,

EMG Pearls
 EMG Pearls
STEVEN A. GREENBERG, MD
Assistant Professor of Neurology
Harvard Medical School
Department of Neurology
Brigham and Women's Hospital
Boston, Massachusetts

ANTHONY A. AMATO, MD
Associate Professor of Neurology
Harvard Medical School
Chief, Neuromuscular Division
Director, Clinical Neurophysiology Laboratory
Vice-Chairman, Department of Neurology
Brigham and Women's Hospital
Boston, Massachusetts

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EMG PEARLS

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Library of Congress Cataloging-in-Publication Data

Greenberg, Steven A. (Steven Alan), 1962-

EMG pearls I Steven A. Greenberg, Anthony A. Amato.
p.; em ..
Includes index.
ISBN-13: 978-1-56053-613-0 ISBN-IO: 1-56053-613-6
I. Electtomyography-Case studies. I. Title: Electromyography pearls. II. Amato,
Anthony A., 1960-111. Title ..
[DNLM: I. Electromyography-methods-Case Reports. 2. Electromyography-methods-Problems and
Exercises. 3. Neuromuscular Diseases--diagnosis-Case Reports. 4. Neuromuscular
Diseases--diagnosis-Problems and Exercises. WE 18.2 G798e 2004]
RC77.5.G745 2004 ..
616.7'407547--dc22 2004040545

ISBN-I3: 978-1-56053-613-0
ISBN-IO: 1-56053-613-6

Printed in the United States of America.

Last digit is the print number: 9 8 7 6 5· 4 3 2

 CONTENTS

Section I. Focal Neuropathies

Introduction .
Patient Page
I. A 60-year-old man with numbness and tingling in his hands for I year 5
2. A 48-year-old woman with numbness and tingling in her left hand 8
3. A 68-year-old woman with numbness and tingling in her hands 12
4. A 53-year-old woman with right hand weakness and numbness after an
axillary dissection 15
5. A 36-year-old man with 2 years of numbness in his left hand digits
4 and 5 19
6. A 55-year-old man with numbness in hand digits 4 and 5 23
7. A 53-year-old woman with left hand weakness 27
8. A 40-year-old woman referred for possible left carpal tunnel syndrome or ulnar
neuropathy 31
9. A 68-year-old man with metastatic melanoma and progressive left wrist drop
for several months 34
10. A 43-year-old man with scapular winging after a cervical lymph node biopsy 37
II. A 51-year-old man with scapular winging after arthroscopic shoulder surgery 41
12. A 23-year-old woman with progressive weakness, atrophy, and numbness in
one hand 46
13. A 23-year-old man with right arm weakness after a motorcycle accident 51
14. 56-year-old woman 2 days after a left shoulder dislocation and closed
reduction with diffuse left arm weakness 55
15. A 56-year-old man with painful left hand weakness 59
16. A 71-year-old man with left arm weakness for I week 64
17. A 61-year-old woman with breast cancer, right neck and shoulder pain,
and hand weakness and numbness 3 months prior to evaluation 68
18. A 64-year-old man with right foot drop 6 weeks prior. 73
19. A 70-year-old woman with right foot drop after hip replacement 76
20. A 38-year-old man with left foot weakness and numbness after prior
treatment for a posterior thigh sarcoma 79
21. A 73-year-old man with right leg numbness, weakness, and pain 84
22. A 46-year-old woman with right leg pain and intermittent paresthesias 87
23. A 57-year-old man with right leg weakness after repair of abdominal aortic
and internal iliac artery aneurysms 91

Section II. Generalized Neuropathies

Introduction 94
24. A 73-year-old man with an IgG-lC paraprotein and several years of
progressive numbness and weakness in the feet and hands 97
25. A 43-year-old man with diabetes and multiple neurologic symptoms 103
26. A 73-year-old man with diabetes and several months of chest pain 107
27. A 55-year-old woman with 6 months of progressive numbness in her feet 110
28. A 71-year-old woman with several years of progressive numbness in her
hands and feet 112
29. A 38-year-old man with numbness and weakness in the hands and feet and a
skin rash 115
30. A 64-year-old woman with severe left shoulder pain and weakness of
shoulder abduction for 3 days, followed by progressive neurologic deficits 120

v
Patient Page
31. A 38-year-old man with fever and acute generalized weakness and distal
numbness 126
32. A 66-year-old woman with numbness in her hands and feet and progressive
generalized weakness 131
33. A 43-year-old woman with progressive generalized weakness and numbness
for 5 months ' 137
34. A 48-year-old man with acute progressive numbness and paresthesias in his
hands and feet and generalized weakness 140
35. A 49-year-old woman with numbness in her right hand and both feet 144
36. A 35-year-old woman with numbness in her hands and feet for 3 weeks and a
right facial palsy for several days 148
37. A 74-year-old woman with progressive weakness in her hands 153
38. A 68-year-old woman with slowly progressive weakness in her legs without
paresthesias 156
39. A 32-year-old woman with a severe neuropathy since childhood 158
40. A 33-year-old woman with long-standing asymmetric weakness in all four
limbs and a family history of neuropathy 160
41. A 72-year-old woman with 12 years of progressive numbness and paresthesias in
her hands and feet and mild gait ataxia 164
42. A 43-year-old woman with acute generalized weakness developing 1 month
into her intensive care unit stay for sepsis and multi-organ failure 168
43. A 41-year-old woman with an II-year history of progressive numbness in her
limbs 172

Section III. Neuromuscular Junction Disease

Introduction 175
44. A 17-year-old woman with progressive dysarthria and dysphagia for
4 months 178
45. A 71-year-old man with subacute symmetrical proximal weakness 183
46. A 34-year-old woman with generalized weakness since infancy 187

Section IV. Motor Neuron Disease and Motor Neuropathies

Introduction 192
47. A 55-year-old man with progressive left hand weakness for 6 months 194
48. A 3-month-old boy with congenital hypotonia and weakness 197
49. A 66-year-old man with a 5-year history of progressive weakness and wasting
of his distal right leg 199
50. A 45-year-old woman with a 5-year history of cramps and twitching and
2-year history of weakness in her left forearm 206

Section V. Myopathies
Introduction 211
51. A 49-year-old woman with progressive proximal leg weakness for 3 years 212
52. A 6-month-old girl with generalized weakness since birth 215
53. A 29-year-old man with a cardiomyopathy 218
54. A 62-year-old man with slowly progressive weakness 221
55. A 46-year-old woman with a 2-year history of proximal leg and arm weakness 225
56. A 59-year-old woman with proximal weakness and a rash 229
57. A 70-year-old man with difficulty climbing stairs for several years 232
58. A 50-year-old woman with HIV infection and weakness of her arms and legs
for 6 months 235
59. An 85-year-old woman with progressive leg weakness and myalgias 239
60. A 38-year-old man with myasthenia gravis complaining of muscular stiffness
and rippling 242
vi:
Patient Page
61. A 75-year-old woman with difficulty holding her head up 245

Section VI. EMG-Guided Botulinum Toxin Therapy of Focal Dystonias

Introduction 249
62. A 39-year-old woman with 3 to 4 years of progressive difficulty using her right
hand 251
63. A 47-year-old man with progressive difficulty in writing for 5 years 253
64. A 45-year-old woman with several years of difficulty with her right hand
limited to writing 255
65. A 40-year-old physician with progressive difficulty writing for 3 years 258

Index 261

vii
 PREFACE

This casebook of neuromuscular disorders focuses on electrodiagnostic studies. The reader is

expected to have basic competence in the performance of nerve conduction and needle EMG studies.
The case studies should be read carefully and not rushed through. We have aimed to convey how a neu-
romuscular specialist thinks about electrodiagnostic studies rather than focusing on technique.
All case descriptions are of actual patients and the studies performed on them; the case studies
have not been altered or fictionalized to idealize the approach. Some of these studies are, therefore, not
electrodiagnostically "ideal"; patient discomfort or limited studies done to answer specific questions
are always practical considerations for the electromyographer.
The value of diagnostic procedures is usually dependent on normal values, and this is a sur-
prisingly complex issue for nerve conduction studies. Limits of normality vary widely within the
electrodiagnostic literature and reflect not only technical differences, but also differences in selec-
tion of "normal" subjects, design of the study, and statistical procedures. We like to think of electrodi-
agnostic results as ranges of clearly abnormal and normal values separated by a range of uncertainty
and to interpret studies conservatively, erring on the side of normality. For the purposes of this book,
we use the values listed below as the limits of normality:

Sensory Motor
Amplitude Velocity Amplitude DML (msec) or
Nerve ~(V) (m/sec) Nerve (mV) Velocity (m/sec)
Median 12 44 Median 4.8 4.5
Ulnar 10 44 Forearm 48
Sural 5 36 Upper arm 50
Superf. peroneal 3 36 Ulnar 4.5 3.9
F-waves Forearm 48
Across-elbow 48
Nerve Min Latency Persistence Upper arm 50
Median 32 msec 50% Peroneal 1.5 6.5
Ulnar 32 msec 50% Leg 36
Peroneal 56 msec 0% Across fib. 34
Tibial 56 msec 50% Tibial 2.0 7.0
Leg 36

Abbreviations used repeatedly in this book are standard and as follows:

• SNAP = sensory nerve action potential
• CMAP = compound muscle action potential
• CNAP = compound nerve action potential
• Median-APB = stimulating median nerve, recording abductor pollicis brevis
• Median-D2 = stimulating median nerve, recording digit 2
• Ulnar-ADM = stimulating ulnar nerve, recording abductor digiti minimi
• Radial-Dl = stimulating radial nerve, recording digit I
• APB = abductor pollicis brevis
• ADM = abductor digiti minimi
• FDI = first dorsal interosseous
• EIP = extensor indicis proprius
• TA = tibialis anterior
• EDB = extensor digitorum brevis
• AH = abductor halluci
Steven A. Greenberg, MD
Anthony A. Amato, MD

ix
 ACKNOWLEDGMENTS

The authors gratefully acknowledge the contributions of the following electrodiagnostic

technicians, residents, and fellows who have contributed to this book through their work in the
Brigham and Women's Hospital Department of Neurology, Division of Neuromuscular Disease:
Essa Kayd, Macharia Waruingi, Hannah Briemberg, Jayashri Srinivasan, Mary Beth Toran,
Gisela Held, and Darlene Young.

xi
 SECTION I. FOCAL NEUROPATHIES

Focal neuropathies are by definition disorders that affect a very limited portion of the peripheral
nervous system, generally confined to a peripheral nerve at a single anatomical location. Nerve root
disease (radiculopathies), plexus disease (plexopathy), and disorders of individual named nerves or
their branches are all considered neuropathies.
The key principle for the electrodiagnosis of focal neuropathies is that of localization. The two
methods of localization are
1. The "intersection" method
2. Demonstration of a focal nerve abnormality
The first method is the traditional one of localization for clinical neurology, adapted for electro-
diagnosis. It consists of the demonstration of a nerve abnormality within two or more pathways and
then consideration of where these pathways intersect. The point of intersection is in general the loca-
tion of the focal neuropathy. For example, an absent median to second digit sensory nerve action poten-
tial implies a lesion anywhere between the C6 or C7 dorsal root ganglia and the median nerve sensory
fibers in the digit, including the upper or middle trunk, lateral cord, or proximal or distal median nerve.
When combined with needle electromyogram (EMG) abnormalities in deltoid and biceps (implying a
lesion anywhere between the C5 or C6 cord anterior gray matter, nerve roots, or upper trunk), with
appropriate other normal studies, the localization of an upper trunk lesion is made because of the com-
mon intersection ofthese two pathways. To use this method, detailed knowledge of the anatomy of the
peripheral nervous system is crucial.
The second method is demonstration of a focal nerve abnormality-focal slowing, conduction
block, or temporal dispersion-across a specific segment of a nerve. These are all usually aspects of
focal demyelination, although on rare occasions other processes can produce these findings (i.e., early
acute axonal injury). For example, a sufficiently prolonged median-abductor pollicis brevis (APB) dis-
tal motor latency localizes the lesion immediately to the across-wrist or palm segment of the median
nerve. Note that this method can only be used when a technique exists to reliably stimulate supramax-
imally the nerve proximal to the affected segment.
It is also important to note that focal neuropathies do not always follow the rules of anatomy. In
particular, partial focal nerve involvement occurs frequently. An L5 radiculopathy may result in nee-
dle EMG abnormalities in tibialis anterior and peroneus longus but not tibialis posterior or gluteus
medius. An ulnar neuropathy at the elbow may result in focal slowing of the across-elbow motor fibers
to the first dorsal interosseous (FDI) but not to the abductor digiti minimi (ADM). It is important in
such cases to thoroughly examine all relevant parameters. In the first example above, the limited find-
ings have a broad localization, including the anterior horn cells, plexus, and common peroneal nerve.
One would want to perform needle EMG on L5 paraspinal muscles and the short and long heads of
biceps femoris and pay attention to the amplitudes of the superficial peroneal sensory nerve action
potentials (SNAPs) and peroneal-extensor digitorum brevis (EDB) compound muscle action potentials
(CMAPs) on both sides, as well as the peroneal-EDB and peroneal-tibialis anterior (TA) across-
fibular-head motor conduction velocities. The results may still be nonlocalizing.

1
 Focal Neuropathies of the Upper Limb: Anatomy for the
Electrodiagnostic Practitioner

Several anatomic points with regard to the upper limb are of great value in the interpretation of
electrodiagnostic studies. The electromyographer should be fluent with the anatomy of the major por-
tions of the brachial plexus and should be able to construct the plexus from memory (see Figure I). In
addition, the proximal course of the nerve fibers represented in various sensory and motor nerve con-
duction studies should be kept in mind, as shown in Table 1. Finally, knowledge of the innervation of
the musculature of the arms, according to both nerve roots and plexus, is invaluable (see Table 2) .

. /

z
 Trunks Cords I Nerves I
Musculoculaneous
C5~ Lateral

C6/ Axillary

Middle Posterior Median

C7 •

C8~ Radial
Medial

T1/ Ulnar
(2)

Trunks Cords I Nerves I

Musculoculaneous

C5 ~.~_/~
C6/ ....'./.
Lateral

Axillary
C5> Upper
--/
/
2 Lateral
........................
C6 ...../ Axillary
/ ..... / .... >:.
/ '. 3 / "'3 .'
Median
C7. Middle t....::::: ::.::) Posterior C7. Middle /. :::.:) Posterior ::::::.
Median

...::.:><
:~r;;;:" .'. Medial
Radial

:~F"';'.--.~' Medial
Radial

Ulnar .... Ulnar

(3) (4)
Figure 1. Drawing the brachial plexus: (1) The trunks, cords, and nerves can be drawn from the pattern shown.
(2) Label the trunks, then the cords, and then the nerves as shown. (3) The difficult part is connecting the trunks
to the cords; use the 3-2-1 rule. The posterior cord gets three branches (one from each trunk, ). the lateral
cord gets two branches (upper and middle,-----), and the medial cord receives one branch (lower, .••••..). (4) The
cords are then connected to the nerves symmetrically, with two branches leaving each cord. Note that this
common textbook representation of the plexus does not account for the C7, ulnar-innervated supply of several
forearm muscles (flexor carpi ulnaris [FCU] and flexor digitorum profundus [FOP] digits 4 and 5). It is not clear
W how these fibers reach the ulnar nerve-whether it is directly from C7 to lower trunk or through middle trunk or
posterior cord. Also note that the median nerve is the only nerve with supply from two different cords.
 Table 1. Root, Trunk, Cord, and Nerve Innervation Relevant to Sensory and
Motor Nerve Conduction Studies in the Upper Limb

Study Root(s) Trunk Cord Nerve

Sensory

Lat anteb cutaneous C6 Upper Lateral ~usculocutaneous

Radial-webspace C6 Upper Posterior Radial
Radial-DI C6 Upper Posterior Radial
~edian-DI C6 Upper Lateral ~edian
~edian-D2 C6,C7 Upper/middle Lateral ~edian
~edian-D3 C7 ~iddle Lateral ~edian
Ulnar-D5 C8, T1 Lower ~edial Ulnar
Dorsal ulnar cutaneous C8, T1 Lower ~edial Ulnar
~ed anteb cutaneous C8, T1 Lower ~edial ~ed anteb cutaneous

Motor

Radial-EIP C7,C8 ~iddle Posterior Radial

~edian-APB T1 > C8 ' Lower ~edial ~edian
Ulnar-FDI T1 > C8 Lower ~edial Ulnar
Ulnar-:-AD~ C8 >T1 -Lower ~edial Ulnar

Table 2. Root, Trunk, Cord, and Nerve Innervation Relevant to Needle EMG
Studies in the Upper Limb

~uscIe Root(s) Trunk Cord Nerve

Serratus anterior C5,C6,C7 Long thoracic

Supraspinatus C5, C6 Upper Suprascapular
Infraspinatus C5,C6 Upper Suprascapular
Teres minor C5, C6 Upper Posterior Axillary
Deltoid C5, C6 Upper Posterior Axillary
Biceps C5, C6 Upper Lateral ~usculocutaneous
Brachioradialis' C5,C6 Upper Posterior Radial
Triceps C6,C7,C8 All Posterior Radial
Supinator C7,C8 ~iddle/lower Posterior Radial
Pronator teres C6,C7 Upper/middle Lateral ~edian
Ext carpi radialis C6,C7 Upper/middle Posterior Radial
Ext carpi ulnaris C7,C8 ~iddle/lower Posterior Radial
Ext dig (EDC and EIP) C7,C8 ~iddle/lower Posterior Radial
Flex carpi radialis C6,C7 Upper/middle Lateral ~edian
Flex carpi ulnaris C7, C8, T1 ~iddle/lower ~edial Ulnar
Flex pollicis longus C7, C8 ~iddle/lower ~edial ~edian
Flex dig sup D2-5 C7, C8, T1 ~iddle/lower ~edial ~edian
Flex dig profD2-3 C7,C8 ~iddle/lower ~edial ~edian
Flex'dig profD4-5 C7,C8 ~iddle/lower ~edial Ulnar
Abd pollicis brevis C8, TI Lower ~edial ~edian
Opponens pollicis C8, Tl Lower ~edial ~edian
Adductor pollicis C8, T1 Lower ~edial Ulnar
First dorsal interosseous C8, T1 Lower ~edial Ulnar
Abd digiti minimi C8, Tl Lower ~edial Ulnar

4
 PATIENT 1

A 60-year-old man with numbness and tingling in his hands

for 1 year

This 60-year-old, left-handed man complained of nocturnal paresthesias in his left hand awaken-
ing him at night beginning I year ago. The intermittent tingling evolved into constant numbness in the
left hand as well as intermittent paresthesias at night in both hands.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (flV) (cm) (m/s)
L. median-dig II Wrist Dig II 4.85 5.70 1.6 13 26.8
R. median-dig II Wrist Dig II 4.00 5.90 5.5 13 32.5
L. ulnar-dig V Wrist DigV 2.45 3.30 13.1 II 44.9
R. ulnar-dig V Wrist DigV 2.60 3.30 16.3 11 42.3
L. radial-sn box Forearm Sn box 1.85 2.50 24.0 10 54.1

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm)' (rn.ls) "
L. median-APB 1. Wrist APB 9.70 1.8 7 -"

2. Elbow " APB 15.20 1.4 27 49.1

R. median-APB 1. Wrist APB 7.20 7.0 7
2. Elbow APB 12.10 6.5 25.5 52.0
L. ulnar-ADM 1. Wrist ADM 3.05 13.1 7
2. B. elbow ADM 7.20 11.1 25 "60.2 '
3. A. elbow ADM 9.40 10.0 13.5 61.4·
R. ulnar-ADM 1. Wrist ADM 3.10 11.0 7
2. B. elbow ADM 7.60 10.2 24.5 54.4
3. A. elbow ADM 9.35 10.2 12 68.6

EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
L. abd poll br NI 2+ 0 0 NI NI NI Full Mild red'
R. abd poll br NI 0 0 0 NI NI NI Full NI

Question: What is the likely clinical diagnosis?

5
 Answer: Bilateral carpal tunnel syndrome

Discussion: The electrodiagnostic abnormal- It can be very helpful to analyze the physio-
ities are: logical implications of electrodiagnostic abnor-
• Reduced amplitudes, left worse than right, of malities one at a time to develop the skill of
bilateral median-D2 SNAPs correlating electrophysiology with actual physiol-
• Reduced conduction velocities, left slower ogy and clinical findings. We proceed with this
than right, of bilateral median-D2 SNAPs exercise for the above six electrodiagnostic
• Reduced amplitude of the left median-APB abnormalities present in this case.
CMAP • Median-D2 SNAP amplitude reduction.
• Prolongation, left more than right, of bilat- Axonal degeneration of sensory axons in the
eral median-APB distal motor latencies median nerve at the wrist and supplying skin
• Fibrillation potentials in left APB of digit 2. These axons come from dorsal root
• Mildly reduced recruitment of motor units in ganglion cells at the level of C6 and C7 prin-
leftAPB. cipally, pass through the upper and middle
The findings are those of bilateral median neu- trunk and lateral cord, and enter the median
ropathies at the wrists, left more severe than right. nerve in the axilla. Accordingly, a lesion any-
The electrodiagnostic approach to the patient where along this pathway, including the C6
with suspected carpal tunnel syndrome aims to: dorsal root ganglia (but not nerve root-
• Demonstrate electro diagnostic abnormalities why?), plexus, or proximal or distal median
of the median nerve .. nerve could be the cause of this finding.
• Localize abnormalities to the across-wrist • Median-D2 distal sensory conduction veloc-
segment of the median nerve. ity reduction. Focal demyelination of the
• Demonstrate normal electrodiagnostic param- median sensory fibers somewhere between
eters of adjacent nerves, to exclude the pres- the point of stimulation and the site of
ence of a more generalized nerve disorder. recording-that is, across the wrist (although
In general, we focus on sensory studies before conceivably a lesion in the palm could pro-
motor and amplitudes before velocities or laten- duce this finding as well). This provides
cies. Abnormalities in the median-D2 SNAP definitive evidence for a median neuropathy
amplitude can be thought of as representative of at the wrist.
the extent of sensory axon degeneration in the • Left median-APE CMAP amplitude reduc-
median fibers supplying the second digit. tion. Axonal degeneration of motor axons in
Abnormalities in the median-APB CMAP ampli- the median nerve at the wrist and supplying
tude can similarly be thought of as representative APB. Again, as an isolated finding this
of the extent of motor axon degeneration in the would only imply a lesion anywhere along
median motor fibers supplying APB. The caveat the course of the motor axons supplying this
is that distal conduction block, of sensory or muscle and traveling within the median nerve
motor axons, may also produce low amplitudes. at the wrist, including intramedullary lesions
Reductions in median nerve conduction study at the level of C8 or T1 myotomes (i.e.,
amplitudes generally satisfy the first criterion syringomyelia or motor neuron diseases),
above, although one should keep in mind that lower trunk or medial cord lesions (Le., neu-
lesions of the lateral cord or upper trunk proximal rologic thoracic outlet syndrome), or proxi-
to the median nerve may also cause SNAP ampli- mal median neuropathies. In the context of
tude loss, and lesions of the medial cord or lower the other abnormalities in this patient's study,
trunk may cause CMAP amplitude reduction. the reduction in amplitude of the CMAP sug-
Accordingly, a low median-D2 SNAP or gests a more severe involvement that might
median-APB CMAP suggests only a median be affecting or threatening to affect the
neuropathy and never localizes the disorder to patient's strength. The caveat of distal con-
the wrist (i.e., does not satisfy the second duction block as a cause of this reduction,
criterion). without axonal degeneration, must still be
To confidently localize any nerve lesion, it is kept in mind.
usually necessary to demonstrate a focal electro- • Median-APE distal motor latency prolonga-
diagnostic abnormality: focal slowing, conduc- tion. Focal slowing of the median motor
tion block, or temporal dispersion across a fibers across the wrist. This provides defini-
specific nerve segment. For this purpose, either tive evidence of a median neuropathy at the
the median-D2 across-wrist sensory conduction wrist. The left greater than right prolongation
velocity or the median-APB distal motor latency adds evidence of a more severe left median
must be abnormal. neuropathy than right.

6
 • Fibrillation potentials in left APE. Acute den- sory) and the right radial nerve (sensory) to
ervation of this muscle. This provides sup- exclude a more generalized disorder, such as
port that the reduced CMAP amplitude (see an acquired demyelinating neuropathy.
third point above) is not entirely due to distal This study is characteristic of patients with
conduction block, and there is at least some moderate or moderate-severe median neuro-
element of axonal degeneration present. pathies. There are abnormalities of sensory and
• Reduced recruitment of motor units in left motor studies as well as needle EMG. Even
APB. Either chronic denervation or conduc- though the slow median-D2 across-wrist sensory
tion block of motor axons can produce this velocities are sufficient by themselves to establish
finding. Physiologically, this reduced recruit- the diagnosis of median neuropathies at the
ment of motor units is the single electrodiag- wrists, the other studies and parameters are essen-
nostic parameter that most likely correlates tial for a fuller clinical understanding of the
with clinical weakness. severity and extent of involvement.
• Finally, it is important to note the normal
studies of both ulnar nerves (motor and sen-

Clinical Pearls
I. Confident localization of a median neuropathy to the wrist requires the presence
of a focal abnormality: conduction block, focal slowing, or temporal dispersion.
2. Each individual electrodiagnostic abnormality has a physiological interpretation
that you should train yourself to think of when performing and interpreting electrodi-
agnostic studies.

REFERENCES .
1. Practice parameter: electrodiagnostic studies in carpal tunnel syndrome: report of the AAEM, AAN, and AAPMR,
Neurology 2002; 58:1589-1592.
2. lablecki, e-K., Andary, M.T., Floeter, M-K., Miller, R.G., Quartly, c.A., Vennix, MJ., Wilson, J.R., Second AAEM
literature review of the usefulness of nerve conduction studies and electromyography for the evaluation of patients with
carpal tunnel syndrome, Muscle Nerve 2002; 26.
 PATIENT 2

A 48-year-old woman with numbness and tingling in her left hand

This 48-year-old woman complained of nocturnal paresthesias in her left hand for 3 months.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (jlV) (cm) (m/s)

L. median-dig II 1. Wrist Dig II 2.95 3.60 23.7 13 44.1

L. ulnar-dig V 1. Wrist DigV 2.10 2.60 22.2 II 52.4

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)

L. median-APB 1. Wrist APB 4.20 7.4 7

2. Elbow APB 8.15 7.1 22 55.7
L. ulnar-ADM 1. Wrist ADM 2.65 9.7 7
2. B. elbow ADM 6.00 9.1 21 62.7
3. A. elbow ADM 7.40 8.8 8 57.1

L Median - Ulnar (Palmar)

2 •

Median-Palm

Ulnar-Palm

10ms 20Jlv

8
 Questions:
I. Do the above studies confidently diagnose or exclude carpal tunnel syndrome?
2. If not, what additional nerve conduction studies should be considered?
3. What study is illustrated in the figure and what does it show?

9
 Answers:
1. No
2. A more sensitive study should be considered.
3. The median and ulnar palm-to-wrist mixed palmar study shows prolongation of the median response.

Discussion: The routine median and ulnar studies of median sensory or mixed nerve con-
studies are within normal limits; however, the duction through the carpal tunnel to radial or
existence of more sensitive tests for carpal tunnel ulnar studies in the same hand are more sensitive
syndrome means this study is incomplete. An than non-comparison studies, and that short seg-
additional study was performed and is shown in mental studies are more sensitive than wrist-digit
the figure. studies. Accordingly, if median wrist-digit sen-
This study is abnormal for a prolonged sory conduction velocity is normal, one of these
median-ulnar mixed palmar interlatency differ- more sensitive tests should be used:
ence (0.95 msec). The upper limit of normality • Median-ulnar mixed palmar interlatency dif-
for this technique has ranged from 0.2 to 0.5 in ference (the difference between median and
the medical literature. We prefer the conservative ulnar palm-wrist mixed compound nerve
value of <0.5. action potential peak latencies)
For detection of mild carpal tunnel syndrome, • Median-ulnar D4 or median-radial 0 I inter-
one often needs to use a more highly sensitive test latency differences
than the standard wrist-digit recordings of SNAP • Median palm-wrist sensory latency com-
latency. This need has stimulated a great deal of pared to median wrist-digit or median
research. The main questions have been: elbow-wrist sensory latencies
• Are sensory studies more sensitive than In our experience, the first and second tests are
motor? used considerably more frequently than the third.
• Are comparison studies (i.e., between Because a normal wrist-digit study should always
median and ulnar or between median and be followed by a more sensitive test in patients
radial) more sensitive than non-comparison with suspected carpal tunnel syndrome, one could
studies? argue that it would be more efficient to skip the
• Are shorter segmental studies, such as wrist-digit study altogether and to routinely per-
median palm-wrist instead of digit-wrist, form just the mixed palmar study or a comparison
more sensitive? study to digit 4 or digit 1. We still prefer to per-
The American Association of Electro diagnostic form the wrist-digit study because of the addi-
Medicine (AAEM) second literature review tional information obtained regarding the
answered "yes" to all of these questions. The amplitude of the SNAP and its implication
AAEM concluded that sensory studies are more regarding axonal degeneration of sensory axons
sensitive than motor studies, that comparison (see case 1 for further discussion).

Mixed Nerve CNAP Results from Figure

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (~V) (em) (m/s)

L. median- 1. Median- Wrist 2.10 2.70 41.0 8 38.1

ulnar palmar
(palmar) 2. Ulnar-palmar Wrist 1.35 1.75 28.9 8 59.3

Clinical Pearls
1. Sensory studies are more often abnormal in carpal tunnel syndrome than are
motor studies.
2. If sensory median-digit studies are normal, one of several roughly equivalent sen-
sory comparison studies should be done because of their increased sensitivity.

10
REFERENCES
I. Practice parameter: electrodiagnostic studies in carpal tunnel syndrome: report of the AAEM, AAN, and AAPMR,
Neurology 2002; 58: 1589-1592.
2. Chang, M.H., Wei, S.1., Chiang, H.L., Wang, H.M., Hsieh, P.F., Huang, S.Y., Comparison of motor conduction techniques
in the diagnosis of carpal tunnel syndrome. Neurology 2002; 58(11): 1603-1607.
3. Jablecki, C.K., Andary, M.T., Floeter, M.K., Miller, R.G., Quartly, C.A., Vennix, M.1., Wilson, 1.R., Second AAEM
literature review of the usefulness of nerve conduction studies and electromyography for the evaluation of patients with
carpal tunnel syndrome, Muscle Nerve 2002; 26 ..
4. Kothari, M.1., Rutkove, S.B., Caress, 1.B., Hinchey, 1., Logigian, E.L., Preston, D.C., Comparison of digital sensory studies
in patients with carpal tunnel syndrome. Muscle Nerve 1995; 18(11):1272-1276 ..
5. Preston, D.C., Logigian, E.L., Sensitivity of the three median-to-ulnar comparative tests in diagnosis of mild carpal tunnel
syndrome. Muscle Nerve 1994; 17(8):955-956.

11
 PATIENT 3

A 68-year-old woman with numbness and tingling in her hands

Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (~V) (cm) (m/s)

R. median-dig II 1. Wrist Dig II Absent 13

L. median-dig II I. Wrist Dig II Absent 13
R. ulnar-dig V 1. Wrist DigV 1.90 2.50 41.2 II 57.9
L. ulnar-dig V 1. Wrist DigV 1.95 2.45 22.8 II 56.4
R. radial-sn box 1. Forearm Sn box. 1.55 2.05 23.3 10 64.5
L. radial-sn box 1. Forearm Sn box 1.65 2.10 47.5 10 60.6

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)

R. median-APB 1. Wrist APB Absent

L. median-APB 1. Wrist APB 12.20 2.6 7
2. Elbow APB 17.25 2.7 22 43.6
R. ulnar-ADM 1. Wrist ADM 2.85 7.3 7
2. B. elbow ADM 6.20 7.0 21 62.7
3. A. elbow ADM 8.30 6.7 13 61.9
L. ulnar-ADM 1. Wrist ADM 2.60 9.6 7
2. B. elbow ADM 6.50 9.5 20 51.3
3. A. elbow ADM 9.00 9.1 13 52.0
R. ulnar-FDI 1. Wrist FDI 3.70 9.8
2. B. elbow FDI 7.25 9.1 21 59.2
3. A. elbow FDI 9.30 9.3 13 63.4
L. median-vs. ulnar 1. Median Lumb 9.90 0.5 10
2. Ulnar Inteross 3.05 6.4 10
R. median-vs. ulnar 1. Median Lumb 11.65 0.5 10
2. Ulnar Inteross 3.15 6.2 10

12
 R Median - vs Ulnar

2
Median;
20ms2mV

Ulnar 2

Left

Questions:
1. What finding is demonstrated in the figure?
2. How would the electrodiagnostic interpretation change if this particular study was not performed
on the right side?
3. How would the electrodiagnostic interpretation change if this particular study was not performed
on the left side?

13
 Answers:
I. Abnormal bilateral median-lumbrical, ulnar interosseous interlatency differences
2. Unlocalized median neuropathy
3. No change

Discussion: The figure shows the result of median-APB CMAP aIlows for determination of
the comparison of median-lumbrical vs. ulnar- the distal motor latency, which is prolonged and
second interosseous (2UO) motor study. This provides a localization at the wrist, as well. As a
comparison is made by stimulating at identical dis- general rule, carpal tunnel syndrome affects the
tances the median and ulnar nerves at the wrist and SNAP amplitude before the median-APB CMAP
recording over a single surface location under amplitude, and this before the median-second
which are 'both the second lumbrical (median lumbrical CMAP.
innervated) and second palmar interosseous (ulnar This special technique, first described in 1992
innervated). As apparent in the figure and from the by Preston and Logigian,4 generally does not pro-
numerical values in the results table, there is a vide insight beyond more standard electrodiag-
marked delay in both median responses. Without nostic techniques for patients with mild or
this study on the right, the absent median-D2 sen- moderate carpal tunnel syndrome but is invalu-
sory and median-APB motor responses aIlow for a able in some patients with severe carpal tunnel
diagnosis of median neuropathy but not a more syndrome. In many such patients, the absence of
specific localization. A lesion anywhere along the recordable median-digit SNAPs and median-
course of the median nerve from the axilla to the APB CMAPs makes it otherwise impossible to
palm could result in an absent median SNAP and localize the median neuropathy to the wrist. In the
CMAP. In this situation, the 2UO study can be large study of Loscher et af} 36 patients had
very helpful if a median-lumbrical CMAP is pres- absent median-D2 and median-APB responses.
ent, as it provides definitive localization of the Of these, 31 did have recordable median-lumbrical
median neuropathy at the wrist. Had the study not responses, abnormal in all cases. Preston and
been performed on the left side, the interpretation Logigian's technique has proven extremely valu-
would not have changed, as the recordable able in this circumstance.

Clinical Pearl
Severe carpal tunnel syndrome, with absent median-digit sensory and median-APB
motor responses, creates a diagnostic challenge in demonstrating a focal abnormality at
the wrist. In this situation, the median-ulnar second lumbrical interosseous (2UO) test
is especially valuable.

REFERENCES
I. Boonyapisit, K., Katirji, 8., Shapiro, 8.E., Preston, D.C., Lumbrical and interossei recording in severe carpal tunnel
syndrome, Muscle Nerve 2002; 25(1):102-105.
2. Chang, M.H., Wei, S.l, Chiang, H.L., Wang, H.M., Hsieh, P.F., Huang, S.Y., Comparison of motor conduction techniques
in the diagnosis of carpal tunnel syndrome, Neurology 2002; 58( II): 1603-1607.
3. Loscher, W.N., Auer-Grumbach, M., Trinka, E., Ladurner, G., Hartung, H.P., Comparison of second lumbrical and
interosseous latencies with standard measures of median nerve function across the carpal tunnel: a prospective study of
450 hands, J. Neurol, 2000; 247(7):530--534.
4. Preston, D., Logigian, E.L., Lumbrical and interossei recording in carpal tunnel syndrome, Muscle Nerve 1992;
15:1253-1257.
5. Preston, D.C., Ross, M.H., Kothari, MJ., Plotkin, G.M., Venkatesh, S., Logigian, E.L., The median-ulnar latency difference
studies are comparable in mild carpal tunnel syndrome. Muscle Nerve 1994; 17(12):1469-1471.
(See cases 1 and 2 for further references on carpal tunnel syndrome.)

14
 PATIENT 4

A 53-year-old woman with right hand weakness and numbness

after an axillary dissection

This 53-year-old woman underwent lumpectomy and axillary dissection oflymph nodes for breast
cancer and immediately postoperatively noticed numbness of her right hand digits 1 to 3 and weak-
ness. In particular, she had difficulty flexing her fingers, as noted in the figures.
Electrodiagnostic Study: Electrodiagnostic studies were performed 5 months later.

15
 Sensory NCS
BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (IlV) (em) (m/s)

R. median-dig II I. Wrist Dig II 2.95 3.70 2.8 13 44.1

L. median-dig II I. Wrist Dig II 2.30 3.00 108.3 13 56.5
R. ulnar-dig V I. Wrist Dig V 2.10 3.00 52.9 11 52.4
L. ulnar-dig V I. Wrist DigV 1.95 2.75 85.7 II 56.4
R. radial-sn box 1. Forearm Sn box 1.85 2.35 105.8 10 54.1
L. radial-sn box 1. Forearm Sn box 1.75 2.30 79.5 10 57.1
R. latAB cut 1. Elbow Forearm 1.90 2.45 26.1 12 63.2
L. latAB cut I. Elbow Forearm 1.30 1.75 23.5 12 92.3

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (mls)

R. median-APB l. Wrist ADM Absent 7

R. ulnar-ADM l. Wrist ADM 3.85 7.8 7
2. B. elbow ADM 6.05 7.7 13 59.1
3. A. elbow ADM 8.05 7.4 10 50.0

F-Wave

Nerve Fmin (ms) Fmax (ms) Max - Min (ms) %F

R. median Absent
R. ulnar 23.30 27.95 4.65 100

EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. pron teres NI 3+ 0 0 NI NI NI Full Single MU

R. flex poll In NI 2+ 0 0 NI NI NI Full No activity
R. first dors int NI 0 0 0 NI NI NI Full NI
R. ext dig comm NI 0 0 0 Nl NI Nl Full NI
R. abd poll br NI 2+ 0 0 NI NI NI Full No activity

16
 Questions:
I. What is the rationale for performing sensory nerve studies on the asymptomatic hand?
2. Do the nerve conduction studies alone localize the lesion? Could carpal tunnel syndrome produce
these nerve conduction study abnormalities?
3. Could this patient have an anterior interosseous neuropathy?
 Answers:
1. Side-to-side comparison of SNAP amplitudes provides greater sensitivity.
2. No; Yes
3. No. There is clinical sensory involvement.

Discussion: Because the range of normal for superficial is [FDS], second digit). The second
SNAP amplitudes is quite large, significant focal figure makes evident the weakness in the FDP
nerve lesions can produce amplitude reductions and FDS for digit 3 and possibly for digit 4,
that remain within the normal range. Using the though less so. These muscles are all supplied by
additional criterion of greater than 50% side-to- the anterior interosseous nerve (AIN), a branch of
side reduction, subclinical abnormalities can the median nerve. Could this patient have an ante-
sometimes be demonstrated. In evaluation of pos- rior interosseous neuropathy clinically? Not
sible brachial plexus lesions, we often look at likely, as the patient noted numbness of digits I to
multiple sensory nerves bilaterally. In this case, 3; the AIN is purely motor. This would place the
no additional abnormalities outside of the median lesion more proximally, in the proximal median
nerve territory were detected with this approach. nerve. The electrodiagnostic studies confirm this,
The nerve conduction studies are abnormal for showing abnormalities in reduction in the median
reduced amplitude of the median sensory and sensory potential amplitude and needle EMG
absence of the median-APB motor responses, abnormalities in pronator teres as well as the
suggesting a median neuropathy, though without more distal median nerve territory.
localization to the carpal tunnel. The first figure This patient has a proximal median neuropathy
suggests weakness of thumb flexion (flexor pol- as a perioperative complication. The cause is
Iicis longus) and digit 2 flexion at the distal uncertain, and could relate to manipulation of the
interphalangeal (DIP) joint (flexor digitorum brachial plexus during the axillary dissection or
profundus [FDP], second digit) and proximal compression of the median nerve in the upper
interphalangeal (PIP) joint (flexor digitorum arm during the procedure.

Clinical Pearls
1. The evaluation of brachial plexus abnormalities typically requires multiple bilat-
eral sensory studies.
2. Consider proximal median neuropathy when routine studies demonstrate a
median neuropathy that does not localize to the wrist.

REFERENCES
I. Gross, P.T., Jones, H.R., Jr., Proximal median neuropathies: electromyographic and clinical correlation, Muscle Nerve
1992; 15:390-395.
2. Gross, PT., Tolomeo, E.A. Proximal median neuropathies, Neural. Clin. 1999; 17:425--445.
3. Stewart, 1.0., Proximal median neuropathies: electromyographic and clinical correlation, Muscle Nerve 1993; 16:321-322.
4. Veilleux, M., Richardson, P., Proximal median neuropathy secondary to humeral neck fracture, Muscle Nerve 2000;
23:,426-429.

18
 PATIENT 5

A 36-year-old man with 2 years of numbness in his left hand

digits 4 and 5

This 36-year-old right-handed man noted progressive constant numbness and pins-and-needles
paresthesias in his left hand digits 4 and 5 over 2 years. He worked as a laboratory researcher and spent
many hours each day leaning on his fully flexed elbows. Neurological examination demonstrated a sen-
sory disturbance in the left medial and dorsal hand up to the wrist crease and digits 5 and the medial
half of digit 4.
Electrodiagnostic Study:

Sensory NCS
Recording Onset Peak Amplitude Distance . Velocity
Nerve Sites Site (ms) (ms) (IlV) (cm) (m/s)
L. median-dig II I. Wrist Dig II 2.35 2.90 36.6 13 55.3
R. median-dig II I. Wrist Dig II 2.30 2.75 51.9 13 56.5
L. ulnar-dig V I. Wrist DigV 2.50 3.45 19.9 II ·44.0
R. ulnar-dig V I. Wrist DigV 2.15 2.75 44.4 II 51.2
L. ulnar dorsal I. Wrist Dors hand Absent 10 .
R. ulnar dorsal I. Wrist Dors hand 1.80 2.35 12.4 10 55.6

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)
L. median-APB I. Wrist APB 3.15 9.9 7
2. Elbow APB 7.05 9.3 22 . 56.4
L. ulnar-ADM I. Wrist ADM 2.40 7.4 7
2. B. elbow ADM 5.65 6.5 19 58.5
3. A. elbow ADM 8.55 7.0 14.5 50.0·
R. median-APB 1. Wrist APB 3.05 12.0 7
2. Elbow APB 6.80 12.2 ·23 61.3
R. ulnar-ADM I. Wrist ADM 2.15 9.6 7
2. B. elbow ADM 5.45 9.3 20 60.6
3. A. elbow ADM 8.10 8.5 14 52.8

Questions:
I. Would you consider any other nerve conduction studies at this point to clarify the diagnosis?
2. What is the likely diagnosis?
3. What electrodiagnostic studies would be helpful?
4. What abnormality is illustrated in the figure?

19
 L Ulnar - ADM

• 22 2 • 2 2

20ms 5mV

20
 Answers:
I. Across elbow ulnar inching studies
2. Ulnar neuropathy at or near the elbow
3. Ulnar motor "inching" across the elbow
4. Prolongation of latencies between the third and fourth stimulation sites

Discussion: The electrodiagnostic study is example of the value of short-segmental incre-

notable for relative reduction in the amplitude of mental studies in general, which also have value
the left ulnar-D5 SNAP compared to the right in the diagnosis of median neuropathies at the
ulnar-D5 SNAP. Greater than 50% reduction in wrist.
side-to-side comparison of SNAP amplitudes is In this case, the technique allows for precise
generally taken to be abnormal. In addition, the localization of the ulnar neuropathy not just to the
left dorsal ulnar cutaneous nerve response is elbow but to the retroepicondylar segment. This
absent on the left and robust (12 ~ V) on the right. provides excellent justification to discourage a
These features, together with the other normal cubital tunnel release as a surgical approach.
SNAPs, suggest a lesion of the lower trunk, Several technical caveats are noteworthy. One must
medial cord, or ulnar nerve. The routine motor be meticulous in inching study distance measure-
studies, including the ulnar-ADM across-elbow ments, as shorter distance segments are prone to a
motor velocity of 50 m/sec are within normallim- greater percentage of error. Furthermore, rigor-
its, leaving the electromyographer unable to fur- ously defined normal values for inching are not
ther localize the lesion. available; 0.8 msec or greater across a single
The additional technique of "inching" across 2.5-cm elbow segment is generally taken as a con-
the elbow is quite helpful in further localizing the servative cutoff for abnormality. We also note a
lesion. The results of this study are listed next. reduction in the amplitude of the dorsal ulnar cuta-
The ulnar-ADM inching study demonstrates neous sensory study is generally taken as evidence
focal slowing of motor nerves in the elbow to the of a lesion proximal to the wrist; however, 21% of
elbow + 2.5 cm proximally segment; this is the normal individuals have a significant asymmetry,
retroepicondylar segment that extends from the thus limiting the value of this technique.5
midpoint of a line drawn between the medial epi- In general, one should note that the electrodi-
condyle and olecranon extending proximally. The agnosis of ulnar neuropathy at the elbow is often
figure shows a visible delay in onset latency not straightforward. Campbe1l3 noted that
between the third and fourth waveforms com- Wilbourne referred to it as the "electromyogra-
pared to the others, counting from the left. pher's nightmare." The complexity of interpreta-
Although the overall motor conduction velocity tion, particularly in relation to normal values that
across the 14.5-cm elbow segment in the routine overlap substantially with abnormal values and
ulnar study was 50 m/sec (normal), across this that are highly dependent on technical factors,
2.5-cm segment there is a I-msec delay (6.75 to along with the very serious consequences of mis-
7.75 msec), translating to a velocity of 25 m/sec diagnosis leading to a failed operation, frequently
through this single short segment. This is an lead to an ambiguous study.

Nerve Sites Recording Site Latency (ms) Amplitude (mV)

L. ulnar-ADM inching 1. Elbow - 5.0 ADM 5.90 7.2

study 2. Elbow - 2.5 ADM 6.25 7.6
3. Elbow ADM 6.75 7.5
4. Elbow + 2.5 ADM 7.75 7.3
5. Elbow + 5.0 ADM 8.30 7.3

Clinical Pearls
1. The localization of an ulnar neuropathy to the elbow is often difficult.
2. Short segmental studies ("inching") of the ulnar nerve across the elbow are a valu-
able technique when more routine studies are nonlocalizing.
3. The amplitude of the dorsal ulnar cutaneous SNAP may be significantly asym-
metric in up to 21% of normal individuals, limiting its diagnostic utility.

2I
REFERENCES
I. AAEM, Practice parameter for electrodiagnostic studies in ulnar neuropathy at the elbow: summary statement: American
Association of Electrodiagnostic Medicine, American Academy of Neurology, American Academy of Physical Medicine
and Rehabilitation, Muscle Nerve 1999; 22(suppl. 8):SI71-S174.
2. AAEM. The electrodiagnostic evaluation of patients with ulnar neuropathy at the elbow: literature review of the usefulness
of nerve conduction studies and needle electromyography, Muscle Nerve 1999; 22(suppl. 8):SI75-S205.
3. Campbell, WW, Ulnar neuropathy at the elbow, Muscle Nerve 2000; 23:450-452.
4. Campbell, WW, Pridgeon, R.M., Sahni, K.S., Short segment incremental studies in the evaluation of ulnar neuropathy at
the elbow, Muscle Nerve 1992; 15(9): 1050-1054 ..
5. Dutra de Oliveira, A., Barreira, A.A., Marques, W, Limitations on the clinical utility of the ulnar dorsal cutaneous sensory
nerve action potential, C/in. Neurophysiol. 2000; III: 1208-121 O.

22
 PATIENT 6

A 55-year-old man with numbness in hand digits 4 and 5

This 55-year-old man with diabetes and bilateral numbness, left more than right, in digits 4 and
5 of the hands was referred to "r/o cubital tunnel syndrome" by an orthopedic surgeon.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (~V) (cm) (m/s)
L. median-dig II 1. Wrist Dig II 2.30 3.00 22.1 13 56.5
R. median-dig II 1. Wrist Dig II 2.40 3.20 24.4 13 54.2
L. ulnar-dig V 1. Wrist DigV 3.65 4.70 10.3 11 30.1
R. ulnar-dig V 1. Wrist DigV 2.10 2.90 19.3 11 52.4
L. radial-sn box 1. Forearm Sn box 1.50 2.10 21.3 10 66.7

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)
L. median-APB 1. Wrist APB 3.55 5.1 7
2. Elbow APB 8.45 4.8 25 51.0
L. ulnar-ADM 1. Wrist ADM 3.00 6.9 7
2. B. elbow ADM 8.00 6.2 25 50.0
3. A. elbow ADM 12.35 1.4 12.5 28.7
R. ulnar-ADM 1. Wrist ADM 2.75 7.7 7
2. B. elbow ADM 7.60 7.0 25 51.5
3. A. elbow ADM 9.50 6.5 10 52.6
L. ulnar-FDI 1. Wrist FDI 4.35 11.8
2. B. elbow FDI 9.05 10.5 24 51.1
3. A. elbow FDI 13.10 4.1 11.5 28.4
R. ulnar-FDI 1. Wrist FDI 3.95 10.1
2. B. elbow FDI 8.65 8.9 25 53.2
3. A. elbow FDI 10.90 8.6 11.5 51.1

EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
L. first dors int Inc 2+ 0 0 NI NI NI Full Mod red
L. abd poll br NI 0 0 0 NI NI NI Full NI
L. flex carp uln NI 0 0 0 NI NI NI Full NI

23
 2

L Ulnar- ADM

Wrist

30ms 2mV

Figure 1

Questions:
1.. What is the conclusion of the electrodiagnostic studies?
2. Do· they confirm the presence of cubital tunnel syndrome and should the patient undergo ulnar
nerve decompression at the cubital 'tunnel?
3. What finding is shown in the figure I?

24
 Answers:
I. A left ulnar neuropathy at or near the elbow is present.
2. No
3. Partial conduction block and focal slowing is shown.

Discussion: The electrodiagnostic studies are the point of exit from the flexor carpi ulnaris. The
abnormal for borderline reduction in the ampli- cubital tunnel is synonymous with HUA localiza-
tude of the left ulnar-D5 SNAP (nearly one-half tion. Furthermore, even when localizing to the
the contralateral ulnar amplitude and at the lower cubital tunnel, an ulnar neuropathy may be due to
limit of normal of 10 11V) and reveal slowing blunt trauma rather than ongoing compression.
of both the ulnar-ADM and ulnar-FDI across- The use of the term cubital tunnel syndrome may
elbow motor segments and conduction block lead to inappropriate surgical procedures for mis-
(>50% reduction in amplitude) in these same seg- takes made in connection with either of these two
ments as well. These findings indicate a left ulnar points. Further electrodiagnostic studies includ-
neuropathy at or near the elbow. Also note the ing motor inching studies can clarify the localiza-
slowing of the left ulnar-D5 across-wrist sensory tion. The results of inching studies are shown in
segment, which perhaps seems greater than one the table below and in figure 2.
would expect as a consequence of the axonal loss The inching studies demonstrate focal slowing
alone. and conduction block of the retroepicondylar
Although the term cubital tunnel syndrome is (between the elbow and 2.5 cm proximally) and
often used synonymously with ulnar neuropathy medial intermuscular septum (2.5 to 5.0 cm) seg-
at or near the elbow, doing so should be avoided. ments. Although focal nerve conduction abnor-
Ulnar neuropathies at or near the elbow (gener- malities do not always correlate with the precise
ally referred to as ulnar neuropathy at the elbow, site of compression, a cubital tunnel decompres-
or UNE) are grouped into several categories by sion was not recommended for this patient. If
localization, listed from proximal to distal: the conservative treatment fails, ulnar nerve trans-
medial intermuscular septum, the retroepicondy- position could be considered, although it is of
lar groove, the humeroulnar arcade (HUA), and unproven value.

Ulnar Nerve Inching Study

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)

L. ulnar-ADM 1. 5.0 cm below elbow ADM 8.20 6.0 2.5

2. 2.5 cm below elbow ADM 8.65 6.0 2.5 55.6
3. At elbow ADM 9.10 5.9 2.5 55.6
4. 2.5 cm above elbow ADM 10.70 2.2 2.5 15.6
5. 5.0 cm above elbow ADM 11.65 1.5 2.5 26.3

L Ulnar-ADM Across-Elbow Inching

30ms 5mV

Figure 2

25
 Clinical Pearls
1. Ulnar neuropathy at or near the elbow is not synonymous with cubital tunnel syn-
drome; patients with ulnar neuropathies often have focal abnormalities at elbow sites
proximal to the cubital tunnel.
2. Ulnar elbow inching studies, when abnormal, can demonstrate focal slowing, con-
duction block, or both.' ,

REFERENCES
1. AAEM, Practice parameter for electrodiagnostic studies in ulnar neuropathy at the elbow: summary statement: American
Association of Electrodiagnostic Medicine, American Academy of Neurology, American Academy of Physical Medicine
and Rehabilitation, Muscle Nerve 1999; 22(suppl. 8):SI71-SI74.
2. AAEM. The electrodiagnostic evaluation of patients with ulnar neuropathy at the elbow: literature review of the usefulness
of nerve conduction studies and needle electromyography, Muscle Nerve 1999; 22(suppl. 8):S 175-S205.
3. Campbell, WW, Ulnar neuropathy at the elbow, Muscle Nerve 2000; 23:450-452.
4. Campbell, WW, Pridgeon, R.M., Sahni, K.S., Short segment incremental studies in the evaluation of ulnar neuropathy at
the elbow, Muscle Nerve 1992; 15(9): 1050-1054.
5. Dutra de Oliveira, A., Barreira, A.A., Marques, W, Limitations on the clinical utility of the ulnar dorsal cutaneous sensory
nerve action potential, Clin. Neurophysiol. 2000; .111: 1208-121 0,

26
 PATIENT 7

A 53-year-old woman with left hand weakness

This 53-year-old woman developed sudden weakness without sensory symptoms in her left hand.
She was evaluated for electrodiagnostic studies 5 months later. Motor examination showed atrophy and
weakness of left FDI with normal ADM, and no sensory loss was observed ...
Electrodiagnostic Study:

Sensory NCS
Recording Onset Peak Amplitude Distance Velocity"
Nerve Sites Site (ms) (ms) (JlV) (cm) (mls)
L. ulnar dorsal-hand \. Wrist Hand 1.55 \.95 25.0 10 64.5
L. median-dig II \. Wrist Dig II 2.10 2.85 43.9 13 6\.9
L. ulnar-dig V \. Wrist DigV \.95 2.75 35.8 11 56.4
R. ulnar-dig V \. Wrist DigV \.80 2.55 41.1 11 61.1

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (mls)
L. median-APB \. Wrist APB 3.45 I\.9 7
2. Elbow APB 6.80 11.3 21 62.7
L. ulnar-ADM \. Wrist ADM 3.40 7.4 7
2. B. elbow ADM 6.65 7.5 21 64.6
3. A. elbow ADM 8.15 7.3 9 60.0
R. ulnar-ADM \. Wrist ADM 2.65 10.7 7
2. B. elbow ADM 6.10 10.2 21 60.9
3. A. elbow ADM 7.85 10.1 10 57.1
L. ulnar-FDI \. Wrist FDI 3.65 \.8
2. B. elbow FDI 7.10 \.6 21 60.9
3. A. elbow FDI 8.15 \.7 7 66.7
R. ulnar-FDI \. Wrist FDI 3.55 12.9
2. B. elbow FDI 6.80 12.0 21 64.6
3. A. elbow FDI 8.65 11.1 10 54.1
L. median-vs. ulnar \. Median Lumb 2.95 3.1 10
2. Ulnar Inteross 3.35 0.6 10
R. median-vs. ulnar \. Median Lumb 3.05 3.6 10
2. Ulnar Inteross 3.05 5.5 10
 Needle EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

L. abd poll br Nl 0 0 0 Nl NI Nl Full Nl

L. abd dig min Nl 0 0 0 Nl Nl Nl Full Nl
L. ext indicis Nl 0 0 0 Nl Nl NI Full Nl
L. flex dig pr IV NI 0 0 0 Nl Nl NI Full Nl
L. first dors int Incr 3+ 0 0 Nl Full Single MUP

Questions:
I. What are the electrodiagnostic abnormalities and conclusion?
2. What is the clinical localization of this lesion?

28
 Answers:
1. Reduced amplitudes of the right ulnar-FDI, ulnar second palmar interosseous CMAPs, fibrillation
potentials, and reduced motor unit recruitment in FDI suggest a lesion of the ulnar deep motor
branch in the palm.
2. Ulnar neuropathy at the distal wrist or in the palm, affecting the deep motor branch only

Discussion: This patient has an ulnar neu- shaped fluid collection adjacent to the hamate and
ropathy that spares the ulnar sensory and dorsal Guyon's canal (arrow), displacing the ulnar nerve
ulnar cutaneous nerve territories and the ADM. and artery (the neurovascular bundle, arrowhead).
These features suggest a distal lesion. Distal ulnar The appearance was typical of a ganglion cyst.
neuropathies have four distinct patterns of pres- Surgical exploration with removal of the mass
entation (figure 1): and subsequent pathologic examination revealed
• At Guyon s canal: the main trunk of the a ganglionic cyst.
nerve is affected, so that deficits are apparent Ulnar neuropathy at the wrist is uncommon and
in both the superficial terminal branch (ulnar may be due to a wide range of structural lesions,
n. sensory loss, ventral medial palm, D5 and including hemorrhage, anomalous musculature;
medial D4 sensory) and the proximal deep lipoma, or ganglionic cyst. External pressure,
terminal branch (all ulnar-innervated intrin- such as in bicycle riders and construction workers
sic hand muscles). operating heavy drills, is another cause; note that
• Deep terminal branch proximal to hypo- the mechanism of external pressure is chronic
thenar branches: no sensory loss, with weak- compression but not entrapment (i.e., compres-
ness of all ulnar innervated intrinsic hand sive neuropathy "# entrapment neuropathy).
muscles. Electrodiagnostic techniques for specific use in
• Deep terminal branch distal to hypothenar the evaluation of ulnar neuropathies at the wrist
muscles: no sensory loss, normal ADM, include short segmental ("inching") studies
other ulnar intrinsic hand muscles weak. recording from FDI, as well as the use of the
• Superficial terminal branch only: ulnar terri- median-ulnar second lumbrical-interosseous
tory sensory loss only. latency difference. The latter study, discussed in
This patient's deficits correspond electrodiag- case 3, is generally of value in the diagnosis of
nos tic ally to the third category. severe carpal tunnel syndrome but may also be
Further evaluation included MRI of the wrist helpful for ulnar neuropathy at the wrist.
(see figure 2), which demonstrated a dumbell-

Distal Ulnar Nerve

Hypothenar Branch

Figure 1

29
 Figure 2

Clinical Pearls
1. Ulnar neuropathy at the wrist is uncommon; consideration of causes should
always include masses.
2. Ulnar neuropathy at the wrist has a variety of patterns with varying sensory and
motor involvement.

REFERENCES
1. Sui-Mansfield, L.T., Williamson, M., Wheeler, D.T., Johnstone, E, Guyon's canal lipoma causing ulnar neuropathy, Am J
. Roentgenol. 2002; ] 78: 1458.
2. Elias, D.A., Lax, MJ., Anastakis; DJ., Musculoskeletal images: ganglion cyst of Guyon's canal causing ulnar nerve
compression, Can. 1. Surg. 200]; 44:331-332.
3. Kothari, MJ., Ulnar neuropathy at the wrist, Neurol. Clin. ]999; ] 7:463-476.
4. Kothari, MJ., Preston, D.C., Logigian, E.L., Lumbrical-interossei motor studies localize ulnar neuropathy at the wrist.
Muscle Nerve 1996; 19: 170--174.
5. McIntosh, K.A., Preston, D.C., Logigian, E.L., Short-segment incremental studies to localize ulnar nerve entrapment at the
wrist, Neurology 1998; 50:303-306.
6. Olney, R.K., Hanson, M., AAEE case report #15: ulnar neuropathy at or distal to the wrist, Muscle Nerve 1988;
II :828-832.
7. Stewart, J.D., Focal Peripheral Neuropathies, 3rd ed., Lippincott Williams & Wilkins, Philadelphia, PA, 2000, pp. 267-268.

30
 PATIENT 8

A 40-year-old woman referred for possible left carpal tunnel

syndrome or ulnar neuropathy

Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (JlV) (cm) (m/s)
L. median-dig II 1. Wrist Dig II 2.50 3.30 51.4 13 52.0
L. ulnar-dig V 1. Wrist DigV 2.00 2.55 58.5 II 55.0
L. median-ulnar 1. Median-palm Wrist 2.00 2.45 47.1 8 40.0
(palmar) 2. Ulnar-palm Wrist 1.20 1.65 27.3 8 66.7
L. median-ulnar 1. Med wrist Elbow 4.30 5.35 10.1 34 79.1
(elbow) 2. Uln wrist Elbow 4.55 5.55 12.0 34 74.7

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (mls)

L. median-APB I. Wrist APB 3.85 7.3 7

2. Elbow APB 7.10 9.2 21 64.6
L. ulnar-ADM 1. Wrist ADM 2.50 9.8 7
2. B. elbow ADM 5.70 7.1 21 65.6
3. A. elbow ADM 7.40 7.0 9 52.9
L. median-ADM 1. Med wrist ADM No response
2. Med elbow ADM 6.75 1.0
L. ulnar-For 1. Wrist For 3.55 1I.4
2. B. elbow For 6.85 7.3
3. A. elbow For 8.40 7.6
L. median-vs. ulnar I. Median Lumb 3.75 2.2 10
2. Ulnar Inteross 3.25 8.1 10

Questions:
1. What is the conclusion of the electrodiagnostic studies?
2. Do you think the drop in the amplitudes of the ulnar-ADM and ulnar-FDI CMAPs going from
wrist to below-elbow stimulation are due to technical reasons? Look at the waveforms in the first
two figures.
3. Note the increase in amplitude and change in morphology of the median-APB CMAP going from
wrist to elbow stimulation. Does this clarify the situation? What additional nerve conduction study
should be done?

31
 2
L Ulnar- ADM

Wrist

20ms 5mV

B.Elbow

A.Elbow

L Median - APB

Wrist

•• ' J:\

20ms 5mV

Elbow

L Median - ADM

Median Elbow

20ms 2mV
 Answers:
I. There is a Martin-Gruber anastomosis.
2. No.
3. Stimulate the median nerve at the elbow recording at ADM.

Discussion: The waveform from the addi- and then innervating the flexor pollicis brevis
tional study is shown in the third figure. The (FPB) short head through the ulnar nerve at the
median nerve is stimulated at the elbow and a wrist (because the FPB is adjacent to the APB, the
CMAP recorded from the ADM. An initial nega- thenar CMAP is summated by the FPB activation),
tive (upward) deflection indicates that axons inner- and (2) the presence ofaxons innervating ADM in
vating this muscle lie within the median nerve at the ulnar nerve at the wrist but (3) in the median
the elbow. This anatomical variation is the Martin- nerve in the proximal forearm. The changes in the
Gruber anastomosis (MGA). The typical findings proximal evoked median and ulnar CMAPs sug-
of an MGA are those of this case: (I) the median- gest the presence of an MGA. The only way to be
APB CMAP increases with elbow stimulation, sure of one is to stimulate the median nerve at the
(2) the ulnar-ADM CMAP decreases with below- elbow and record over the ADM, as shown in the
elbow stimulation, and (3) a CMAP is present in third figure. In rare circumstances, collision stud-
the ADM or FDI when stimulating the median ies are required. Stimulating ulnar nerve (recording
nerve at the elbow. These findings are due, respec- over thenar muscles) is not helpful, because the
tively, to (1) median nerve axons in the proximal ulnar nerve normally innervates thenar muscles,
forearm crossing over to the ulnar nerve distally including variably APB.

Clinical Pearls
I. Martin-Gruber anastomoses are common anatomical variants that should be con-
sidered when stimulation at the elbow results in a larger median and a smaller ulnar
compound muscle action potential than distal stimulation.
2. Although several types of MGA exist, routine electrodiagnostic studies reliably
detect only median-to-ulnar communications, through stimulation of the median nerve
at the elbow and recording at ADM.

REFERENCES
1. Claussen, G.c., Ahmad, B.K., Sunwoo, I.N., Oh, S., Combined motor and sensory median-ulnar anastomosis: report of an
electrophysiologically proven case, Muscle Nerve 1996; 19:231-233.
2. Crutchfield, C., Gutmann, L., Hereditary aspects of median-ulnar nerve communications, J. Neurol. Neurosurg. Psychiatry
1980; 43:53-55.
3. Oh, S.J., Claussen, G.C., Ahmad, B.K., Double anastomosis of median-ulnar and ulnar-median nerves: report of an
electrophysiologically proven case, Muscle Nerve 1995; 18: 1332-1334.
4. Streib, E.W., Sun, S.F., Martin-Gruber anastomosis: electromyographic studies, Part I, Electromyogr. Clin. Neurophysiol.
1983; 23:261-270.
5. Sun, S.F., Streib, E.W., Martin-Gruber anastomosis: electromyographic studies, Part 2, Electromyogr. Clin. Neurophysiol.
1983; 23:271-285.

33
 PATIENT 9

A 68-year-old-man with metastatic melanoma and progressive left

wrist drop for several months

Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (~V) (cm) (m/s)

L. median-dig II 1. Wrist Dig II 2.60 3.50 17.2 \3 50.0

L. ulnar-dig V 1. Wrist DigV 2.60 3.25 10.8 II 42.3
L. radial-sn box 1. Forearm Sn box 2.00 2.60 9.0 10 50.0
R. radial-sn box 1. Forearm Sn box 2.10 2.75 19.7 10 47.6

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (~V) (cm) (m/s)

L. median-APB 1. Wrist APB 3.85 6.1 7

2. Elbow APB 9.25 6.4 26 48.1
L. radial-EIP I. Forearm EIP 3.25 0.5 7
2. B. spiral gr EIP Not tolerated

F-Wave

Nerve Fmin (ms) Fmax (ms) Max - Min (ms) %F

L. median 33.50 36.95 3.45

Needle EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

L. triceps Incr 3+ 0 0 NI NI NI Full ModRed

L. brachiorad Incr 4+ 0 0 NI NI NI Full SevRed
L. ext dig Incr 3+ 0 0 NI Long NI Full SevRed
comm
L. deltoid NI 0 0 0 NI NI NI Full NI
L. biceps NI 0 0 0 NI NI NI Full NI
L. pron teres NI 0 0 0 NI NI NI Full NI
L. flex carp uln NI 0 0 0 NI NI NI Full NI

34
 Questions:
I. What is the most likely localization suggested by the electrodiagnostic studies?
2. Are the nerve conduction studies alone, without the needle EMG findings, highly suggestive of this
localization, or do they equally support other possible localizations? What single needle EMG mus-
cle study would most distinguish among the remaining possibilities?

3S
 Answers:
I. Proximal radial neuropathy
2. There could be a posterior cord lesion as well. The deltoid also needs to be checked.

Discussion: The nerve conduction studies are This patient has a radial neuropathy. The reduc-
abnormal for a greater than 50% reduction of tion of the SNAP and the neurogenic abnormali-
the left radial-webspace SNAP compared to the ties in the triceps and brachioradialis place the
right. The amplitude of the radial-extensor indicis lesion proximal to the posterior interosseous
proprius (ElP) distal evoked CMAP is also small, nerve alone. Radial neuropathies typically pro-
at 0.5 m V. These findings suggest a radial neu- duce wrist drop. A common cause is acute com-
ropathy or posterior cord plexopathy. Root dis- pression of the nerve, the so-called Saturday-night
ease does not generally reduce SNAP amplitudes, palsy. There is no accepted entrapment cause (the
and the radial SNAP and CMAP have different existence of an entrapment syndrome at the radial
trunk (upper vs. middle/lower) innervations; how- tunnel is dubious and extremely rare at best), and
ever, they both could be reduced from a posterior chronic compression is quite uncommon, but it
cord lesion. Accordingly, the needle EMG muscle does occur in patients using crutches. Confusing
study that would most readily distinguish between physical examination findings in patients with
a radial neuropathy and posterior cord lesion is radial neuropathy may include mild arm flexion
the deltoid muscle, supplied by the axillary nerve weakness (from brachioradialis weakness) and
from the posterior cord. If normal, a radial neu- moderate to severe finger abductor weakness
ropathy is more likely; if abnormal, a posterior because of the flexed position ofthe fingers at the
cord lesion is more likely. This is a general rule: metacarpophalangeal (MCP) joints and the loss
Do not forget to study the deltoid in patients with of mechanical advantage in this position.
suspected radial neuropathy to exclude a more
proximal posterior cord lesion as the cause.

Clinical Pearls
I. Radial neuropathy should always be distinguished from posterior cord lesions by
needle examination of the deltoid.
2. Confusing clinical features of radial neuropathies are apparent ulnar hand weak-
ness (from loss of mechanical advantage) and arm flexion weakness (from brachioradi-
alis weakness).

REFERENCES
I. Carlson, N., Logigian, E.L., Radial neuropathy, Neural. Clin. 1999; 17:499-523.
2. Stewart, J.D., Focal Peripheral Neuropathies, 3rd ed., Lippincott Williams & Wilkins, Philadelphia, PA, 2000.

36.
 PATIENT 10

A 43-year-old man with scapular winging after a cervical lymph

node biopsy

This 43-year-old man with lymphoma developed recurrent lymphadenopathy in his left neck and
underwent biopsy under local anesthesia. After returning home, he noticed difficulty raising his left
arm over his head that persisted, and he was referred for electrodiagnostic studies 7 months later.
General physical examination was notable for a well-healed scar in the posterior triangle of the left
neck. Neurologic examination was notable for the appearance of enlargement of the suprascapular
region when viewed anteriorly, as indicated by the arrowhead in figure IB, and a slight drooping of the
left shoulder (arrow). Arm abduction and flexion at the shoulder resulted in lateral and superior dis-
placement of the scapula, as shown in the top portion of the figure, and mild posterior displacement
off the back, as can be seen in the second figure. A dimpling in the area of the middle .trapezius was
noted with these maneuvers (arrow in figure lA) ..
Electrodiagnostic Study: Sensory and motor nerve conduction studies are normal.

Figure 1

31
 Figure 2

Needle EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

L. deltoid NI 0 0 0 Nl NI NI Full NI
L. serr ant NI 0 0 0 NI NI NI Full NI
L. infra-spinatus . NI 0 0 0 NI NI NI Full NI
L. teres minor . NI 0 0 0 NI NI NI Full Nl
L. trapezius upper Incr 1-2+ 0 0 NI NI Few Full Nt
L. trapezius middle Incr 2-3+ 0 0 No units
L. trapezius lower NI 0 0 0 NI NI Few Full NI
L. strenoclei domastoid NI 0 0 0 NI NI Few Full NI

Questions:
I. What is the localization suggested by the electrodiagnostic studies?
2. What nerve lies in the posterior triangle of the neck?

38
 Answers:
I. Spinal accessory nerve branch lesion
2. The spinal accessory nerve

Discussion: Scapula winging may be due to upper trapezius when the patient is viewed from
weakness of serratus anterior, trapezius, or rhom- the front (see arrowhead in figure IB). In fact,
boid muscles. This patient has an iatrogenic upward displacement of the scapula because of
injury to the accessory nerve, which is a recog- middle and lower trapezius paralysis and the
nized complication of cervical lymph node unopposed action of levator scapulae and rhom-
biopsy in the posterior triangle of the neck. Any boids gives this appearance, which is evident
surgeon performing such biopsies should be from behind. With flexion or abduction, the
aware of this potential complication and must scapula moves laterally and sometimes upward
meticulously avoid this nerve. The lesion is distal typically with only mild posterior displacement of
to the branch to the sternocleidomastoid, which is the scapula (see figures I and 2). Atrophy of the
not involved. Marked differential involvement of trapezius may be evident (middle trapezius atro-
the three portions of the trapezius is present in phy, as indicated by arrow in figure IA).
this case; no motor units are recruited in the Complete absence of recruitable motor units in
middle trapezius, and normal recruitment pat- the middle trapezius at 7 months suggests a very
terns are present in the upper and lower portions. poor prognosis in this particular clinical setting,
Presumably, this is a lesion of the fascicle or but not in all. The absence of demonstrable nerve
branch of the nerve limited to supplying the mid- continuity in the setting of a likely surgical lesion
dle trapezius. of the nerve makes recovery unlikely. In contrast,
Trapezius weakness results in drooping of the had this been a stretch or anesthetic injury to the
shoulder at rest. Figure 3 shows another patient accessory nerve, the absence of recruitable motor
with such drooping from a spinal accessory nerve units at 7 months would warrant at least some
palsy after radical neck dissection for oral cancer. optimism that recovery at 12 to 15 months was
Often there appears to be hypertrophy of the still possible.

Figure 3

Clinical Pearls
I. The spinal accessory nerve lies in the posterior triangle of the neck and is sus-
ceptible to injury with lymph node biopsies.
2. Scapula winging may be due to weakness of serratus anterior, trapezius, or rhom-
boid muscles; the pattern of movement of the scapula with abduction and flexion of the
arm is different among these different causes.

39
REFERENCES
I. Berry, H., MacDonald, E.A., Mrazel, A.C., Accessory nerve palsy: a review of 23 cases, Can. 1Neural. Sci. 1991;
18:337-34l.
2. Gordon, S.L., Graham, w.P., Black, J.T., Miller, S.H., Accessory nerve function after surgical procedures in the posterior
triangle, Arch. Surg. 1977; 112:264--268 ..
3. Friedenberg, S.M., Zimprich, T., Harper, C.M., The natural history of long thoracic and spinal accessory neuropathies,
Muscle Nerve 2002; 25(4):535-539.
4. Saeed, M.A., Gatens, P.E, Singh, S., Winging of.the scapula, Am. Fam. Physician 1981; 42: 139-143.
5. Stewart, J.D., Focal Peripheral Neuropathies, 3rd ed., Lippincott Williams & Wilkins, Philadelphia, PA, 2000.

40
 PATIENT 11

A 51-year-old man with scapular winging after arthroscopic

shoulder surgery

This 51-year-old man developed right shoulder pain while lifting the top on his convertible and
could not use his arm for several days because of pain with movement. Weakness of external rotation
was noted by an orthopedic specialist. Rotator cuff tear was suspected and confirmed with MRI arthro-
gram, which demonstrated a partial thickness tear of the supraspinatus and infraspinatus tendons. The
patient underwent arthroscopic surgery performed with an interscalene brachial plexus block followed
by general anesthesia. Postoperatively, he had difficulty with forward flexion of the arm and raising it
above his head. Two weeks later, a physical therapist noted winging of his right scapula, and he was
referred for electrodiagnostic consultation 6 months postoperatively. Neurologic examination was
notable for a thin man with prominent scapula bilaterally. At rest, the right scapula was displaced supe-
riorly compared to the left, and marked atrophy of infraspinatus was present (see figure I). With arm
abduction and forward flexion, marked winging of the right scapula was characterized by medial
movement of the scapula as well as posterior displacement of the inferior angle of the scapula off the
back (see figure 2).

Figure 1

~l
 Figure 2

42
 Figure 4

Stop and Consider: Scapular winging may be seen with weakness of any of three muscle groups.
Name these groups and the distinct patterns of scapular winging seen with each. Plan the electrodiag-
nostic study.

Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (~V) (cm) (m/s)
R. median-dig II 1. Wrist Dig II 2.55 3.40 46.9 13 51.0
R. ulnar-dig V 1. Wrist DigV 2.35 3.10 28.0 11 46.8
R. radial-sn box I. Forearm Sn box 1.75 2.35 47.8 10 57.1
R. medAB cut 1. Elbow Forearm 2.00 2.50 23.5
R. latAB cut 1. Elbow Forearm 2.25 2.80 22.0 12 53.3

Motor NCS
Recording Latency Amplitude Distance Velocity
Nerve Sites Site (ms) (mV) (cm) (m/s)
R. median-APB 1. Wrist APB 3.95 13.8 7
2. Elbow APB 7.90 13.8 22 55.7
R. ulnar-ADM 1. Wrist ADM 3.05 13.2 7
2. B. elbow ADM 6.60 13.1 21 59.2
3. A. elbow ADM 8.55 12.4 II 56.4

F-Wave
Nerve Fmin(ms) Fmax(ms) Max - Min (ms) %F
R. median 27.70 29.25 1.55 100

4J
 Needle EMG Summary Table
SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. serr ant Incr 3+ 0 O. Nl Nl Nl Full No activity

R. infraspinatus Incr 3+ 0 0 Nl Nl Nl Full No activity
R. deltoid Nl 0 0 0 Nl Nl Nl Full NI
R. supra spinatus Nl 0 0 0 Nl NI Nl Full NI
R. levator scapulae NI 0 0 0 Nl NI Nl Full NI
R. rhomb maj Nl 0 0 0 Nl Nl Nl Full NI
R. trapezius (U) Nl 0 0 0 Nl Nl NI Full Nl
R. trapezius (L) NI 0 0 0 Nl Nl Nl Full Nl
R. lattisimus dorsi Nl 0 0 0 Nl Nl Nl Full NI
R. teres major Nl 0 0 0 Nl Nl Nl Full Nl

Questions: .
1. What muscle does the arrow in figure 3 point to?
2. What is the value of the sensory and motor studies in this case?
3. What is the conclusion of the electrodiagnostic studies?
4. Given this study 6 months postoperatively, what is the prognosis and what management do you rec-
ommend now?
 Answers:
I. Lower trapezius
2. To exclude a more generalized brachial plexopathy
3. Long thoracic neuropathy and branch suprascapular neuropathy
4. Prognosis is uncertain; it is too early to assess. Conservative therapy is recommended.

Discussion: Scapula winging may be due to anterior and infraspinatus, but not supraspinatus,
weakness of serratus anterior, trapezius, or rhom- suggesting lesions of the long thoracic nerve and a
boid muscles (see patient 10 as well). Certainly distal or fascicular lesion of the suprascapular
more generalized shoulder girdle weakness-for nerve. The lack of sensory and motor abnormali-
example, from facioscapulohumeral muscular dys- ties in the arm provides evidence against a more
trophy or myasthenia gravis~an also result in generalized brachial plexopathy, as might be seen
scapula winging. When due to a focal neuropathy, in acute brachial neuritis. Additional EMG studies
the pattern of winging is distinctive among the provide evidence against an accessory neuropathy,
three causes. This patient has a long thoracic neu- dorsal scapular neuropathy, or a lesion of the cer-
ropathy. Lesions ofthe long thoracic nerve result in vical plexus, from which the levator scapulae
serratus anterior weakness. Arm flexion and abduc- receives a predictable motor supply. The long tho-
tion result in medial movement of the scapula, par- racic neuropathy was likely a perioperative com-
ticularly the inferior angle, as well as more marked plication attributable to the interscalene plexus
posterior displacement off the back than that seen anesthetic block or surgical positioning or traction
with trapezius or rhomboid weakness. The arrow in on the shoulder. The distal or fascicular lesion of
the third figure points to the contracting lower the suprascapular nerve is not well understood in
trapezius that is attempting to compensate for ser- this case; the patient had weakness of external
ratus anterior weakness and counteract the poste- rotation prior to surgery. Complete absence of
rior displacement of the scapula. recruitable motor units in serratus anterior at 6
Accessory nerve lesions result in trapezius weak- months suggested a poor prognosis, although it
ness, which was shown in another patient in case 10 was considered too early to give this prognosis to
(see figures). Isolated dorsal scapular neuropathies the patient. Repeat clinical and electrodiagnostic
resulting in rhomboid and possibly levator scapulae assessment at 12 and 18 months was done. At 12
weakness are rare, and the resulting pattern of months, motor unit action potentials were present
winging is not well described in the literature. in the serratus and at 18 months movement of the
In this case, the electrodiagnostic studies scapula had nearly recovered (see figure 4 and
demonstrate fibrillation potentials in the serratus compare to figure 2).

Clinical Pearls
1. In long thoracic nerve palsies, arm flexion and abduction result in medial move-
ment of the scapula, particularly the inferior angle, as well as more marked posterior
displacement off the back than that seen with trapezius or rhomboid weakness.
2. Unless surgical or traumatic laceration of the nerve is suspected, prognosis cannot
be adequately predicted prior to I to 2 years.

REFERENCES
l. Friedenberg, S.M., Zimprich, T., Harper, C.M., The natural history oflong thoracic and spinal accessory neuropathies,
Muscle Nerve 2002; 25(4):535-539.
2. Frank, O.K., Wenk, E., Stern, J.C., Gottlieb, R.D., Moscatello, A.L., A cadaveric study of the motor nerves to the levator
scapulae muscle, Otolaryngol. Head Neck Surg. 1997; 117(6):671-680.
3. Saeed, M.A., Gatens, P.E, Singh, S., Winging of the scapula, Am. Fam. Physician 1981; 42: 139-143.
4. Stewart, J.D., Focal Peripheral Neuropathies, 3rd ed., Lippincott Williams & Wilkins, Philadelphia, PA, 2000.
5. Wiater, J.M., Flatow, E.L., Long thoracic nerve injury, Clin. Orthop. 1999; 368: 17-27.

45
 PATIENT 12
A 23-year-old woman with progressive weakness, atrophy, and
numbness in one hand

This 23-year-old woman noted at age 13 intermittent right arm pain, numbness, and forearm fin-
ger flexor cramps. Progressive weakness and atrophy of the right hand and numbness in the medial
forearm and medial palm developed at age 20. Examination showed moderate weakness without atro-
phy of ADM and severe atrophy and weakness of FDI and APB (see figure 1). Strength was normal
for the pronator teres, flexor pollicis longus, all finger flexors, and flexor carpi ulnaris. A sensory dis-
turbance was present in the right medial hand and forearm (see figure 2).

c
Figure 1

46
 Figure 2

Electrodiagnostic Study:

Sensory NCS
Nerve Amplitude (IlV) Velocity (m/s) .
R. median-dig II 75 52.0
R. ulnar-dig V Absent
R. ulnar dorsal Absent
R. latAB cut 19 61.5
R. medAB cut Absent
L. median-dig II 64 52.0
L. ulnar-dig V 53 48.9
L. ulnar dorsal 19 62.5
L. medAB cut 12 63.2

Motor NCS
Nerve Sites Latency (ms) Amplitude (m V) Velocity (m/s)
R. median-APB 1. Wrist Absent
R. ulnar-FDI 1. Wrist 4.55 4.7
2. B. elbow 8.55 4.8 42.5
3. A. elbow 9.95 4.7 71.4
4. Axilla 12.10 4.3 46.5
R. ulnar-ADM 1. Wrist 3.85 5.3
2. B. elbow 7.35 5.4 48.6
3. A. elbow 8.75 5.3 71.4
4. Axilla 10.70 5.2 51.3
L. ulnar-ADM 1. Wrist 2.55 9.8
2. B. elbow 5.90 9.5 56.7
3. A. elbow 7.45 9.8 64.5

F-Wave
Nerve F min (ms) F max (ms) Max-min (ms) Persistence
R. ulnar-ADM 34.65 37.00 2.35 6/10
L. ulnar-ADM 26.60 27.75 1.15 10/10

41.
 EMG Summary Table
SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. ext dig comm NI 0 0 0 NI NI NI Full NI

R. abd poll br NI 2+ 0 0 Large NI NI Full Sev red
R. ext indicis NI 0 0 0 NI NI NI Full NI
R. abd dig min NI 1+ 0 0 NI NI NI Full Mild red

Questions:
1. Why is this not the combination of a median and ulnar neuropathy? Why is this not a Tl radicu-
lopathy? What is the localization suggested by the electrodiagnostic studies?
2. Are there F-wave abnormalities and what are their significance?
3.."What additional electrodiagnostic studies might be helpful?
4. Would the presence of a right Homer's syndrome be helpful for localization?

48
 Answers:
I. The median SNAP is normal; it is not T1 because the ulnar SNAP is abnormal. Electrodiagriostic
studies suggest a medial cord lesion.
2. A prologed right ulnar F-wave minimum latency suggests focal demyelination.
3. Needle EMG of cervical paraspinals, pronator teres, and FDI
4. A Homer's syndrome would have suggested a T1 localization.

Discussion: This patient has the unusual and no neurological abnormalities are given this
combination of severe motor axonal loss to APB diagnosis. Pain without neurologic symptoms is
with a normal median SNAp, making a typical not usually due to a disease of the peripheral
median neuropathy unlikely. The absence of the nervous system, and the diagnosis of n- TOS is
medial antebrachial cutaneous SNAP is not a fea- reserved for patients with an actual neurologic
ture of an ulnar neuropathy, as this nerve comes deficit. Other processes that may affect the
directly off the medial cord of the brachial plexus. medial cord of the plexus in a young woman
Radiculopathy would also not account for the would include metastatic invasion and a schwan-
reduced amplitudes of SNAPs. The electrodiag- noma. Syringomyelia may produce hand wasting
nostic findings are those of a lesion of the medial and a sensory deficit but does not produce this
cord ofthe brachial plexus. The lower trunk is also electrodiagnostic picture (i.e., reduction in SNAP
a reasonable localization to consider, although C8 amplitudes).
motor axons in the posterior cord, such as those This patient has n- TOS due to bilateral cervical
supplying the extensor digitorum communis ribs, which were seen on plain radiographs of the
(EDe) and extensor indicis proprius (EIP), are a cervical spine (figure 3). MRI of the cervical
part of the lower trunk, and needle EMG of the spine and brachial plexus were interpreted as nor-
EDC and EIP was normal in this case. The mal by experienced chest radiologists. Distinct
right ulnar-ADM minimum F-wave latency is angulations of the two cervical ribs probably
markedly prolonged compared to the other side accounts for the presence of a unilateral disorder.
and, in the setting of only mild reduction in the She underwent surgical decompression of the
CMAP amplitude, provides evidence of focal right plexus (figure 4), and at 6 months had mod-
demyelination of motor axons supplying the erate improvement, having regained the ability to
ADM. The localization of this study is defensible snap her fingers and button on that side. n-TOS is
as is, although further EMG studies, including rare and tends to present in young to middle-aged
lower cervical paraspinal muscles, median nerve women. Greater involvement in thenar than
innervated lateral cord muscles (pronator teres hypothenar muscles is characteristic, as in this
and flexor carpi radialis), and additional ulnar- case. The disorder is often due to a rudimentary
innervated medial cord muscles (FDI, flexor carpi radiolucent band from the cervical rib or elon-
ulnaris), would be of interest. Recall that the lat- gated C7 transverse process to the first thoracic
eral cord does not contribute to the ulnar nerve. rib. In this case, there was no band; the rib itself
In a young person with a longstanding progres- compressed and stretched the lower brachial
sive unilateral medial cord or lower trunk lesion, plexus. It has also been stated that the sensory
true neurologic thoracic outlet syndrome (n- TOS) loss in n-TOS does not split the fourth finger but
is the most likely cause. The diagnosis of TOS is includes both medial and lateral portions; in this
a wastebasket; many patients with strictly pain case, the sensory disturbance did split this digit.
 Figure 3

Figure 4

Clinical Pearls
I. The finding of thenar and FDI weakness with ulnar sensory loss is highly sug-
gestive of true neurologic thoracic outlet syndrome.
2. Plain cervical spine films are more effective than MRI imaging for confirming
this diagnosis.

REFERENCES
I. Le Forestier, N., Mou1onguet, A., Maisonobe, T., Leger, J.M., Bouche, P., True neurogenic thoracic outlet syndrome:
electrophysiological diagnosis in six cases, Muscle Nerve 1998; 21: 1129-1134.
2. Wilbourn, A.J., Thoracic outlet syndromes, Neuro/ Clin. 1999; 17(3):477--497.

50
 PATIENT 13

A 23-year-old man with right arm weakness after a motorcycle

accident

This 23-year-old man suffered a severe right arm injury in a motorcycle crash. The patient, who
was wearing a helmet, apparently lost control of his motorcycle and rolled the bike over on an exit
ramp, leaving the highway at approximately 70 miles per hour. On physical examination, he was awake
and alert. There was some soft tissue swelling of the cervical spine and of the right clavicular fossa.
There was some thoracic spine tenderness and a laceration over the right scapula. There was a cir-
cumferential injury around the right upper extremity extending down to the biceps and triceps and del-
toid muscles. His vascular examination was 2+ throughout. There was no motor function from the
right elbow distally and no sensation in the right hand. Electrodiagnostic studies performed 18 and
22 months after the injury are presented below.
Electrodiagnostic Study:

Sensory NCS
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (/lV) (cm) (mls)

R. median-dig II I. Wrist Dig II 1.90 2.70 20.0 13 68.4

R. median-dig III I. Wrist Dig II 1.50 2.70 24.9 13 86.7
R. ulnar-dig V I. Wrist DigV 1.75 2.65 27.7 II 62.9
R. radial-sn box I. Forearm Sn box 1.45 2.05 6.9 10 69.0
R. medAB cut I. Elbow Forearm 1.60 2.25 10.4 12 75.0
R. lat AB cut I. Elbow Forearm 1.40 2.05 16.3

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)
R. median-APB 1. Wrist APB Absent 7
2. Elbow APB Absent
R. ulnar-ADM 1. Wrist ADM Absent 7
2. B. elbow ADM Absent
3. A. elbow ADM Absent

Needle EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R. deltoid Incr 3+ 0 0 No activity
R. biceps Incr 3+ 0 0 No activity
R. triceps Incr 3+ 0 0 No activity
R. pron teres Incr 3+ 0 0 No activity
R. first dors int Incr 3+ 0 0 No activity

Continued

51
 Needle EMG Summary Tabl~ont'd

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. C5 paraspinal Incr' 3+ o o
R. C6 paraspinal Incr 3+ o o
R. C7 paraspinal Incr 3+ o o
R. C8 paraspinal Incr 3+ o o

EMG Summary Table of Repeat EMG Studies Performed 5 Months Later

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. deltoid Incr 4+ 0 0 No activity

R. biceps Incr 4+ 0 0 No activity
R. triceps Incr 4+ 0 0 No activity
R. pron teres Incr 3+ 0 0 No activity
R. first dors int Incr 3+ 0 0 No activity
R. ext.dig comm Incr 3+ 0 0 No activity

Figure 1

5iZ
 Figure 2

Questions:
1. What important prognostic question should be addressed in traumatic brachial plexopathy studies?
2. What abnormalities are demonstrated in the MRI images in the figures?

53
 Answers:
1. Is there root avulsion?
2. The presence of pseudomeningoceles provides evidence of nerve root avulsion.

Discussion: The severe and diffuse motor In this case, we note intact lateral antebrachial
involvement is evident from the absent CMAPs, cutaneous (C6), median-D3 (C7), ulnar-D5 (C8),
widespread fibrillation potentials, and inability to and medial antebrachial cutaneous (T I) SNAP
recruit motor units. An important question in amplitudes (although ideally they should have
traumatic brachial plexopathy is whether the been compared to contralateral studies), but
nerve roots remain intact or have been avulsed severe motor axonal loss in C6 (deltoid, biceps,
from their attachment to the spinal cord. Because pronator teres), C7 (triceps, pronator teres, EDC,
the dorsal root ganglia contain the cell bodies of paraspinals), C8 (EDC, FDI, paraspinals), and Tl
the primary sensory neurons, nerve root avulsion (FDI) myotomes. This suggests nerve root avul-
does not in general affect SNAP amplitudes. This sion of C6, C7, C8, and Tl.
allows for an important rule: a root distribution MRI imaging, shown in the figures, confirmed
with severe motor axonal loss and normal SNAP nerve root avulsion at least at C7 and C8 and
amplitudes strongly suggests nerve root avulsion. showed the presence of pseudomeningoceles at
Of course, root avulsion and more peripheral these levels.
injury (with reduced SNAP amplitude) may coex-
ist. Brachial plexus studies accordingly focus on
the preservation of SNAPs from different root ter-
ritories, as shown in the following table:

SNAP Root

Radial-D I or radial-webspace C6
Lat antebrachial cutaneous C6
Median-D3 C7
Ulnar-D5 C8
Med antebrachial cutaneous C8/Tl

Clinical Pearl
Root avulsion and more peripheral injury often coexist in severe brachial plexus
trauma. Electrodiagnostic findings of normal SNAP amplitudes in root distributions
with severe motor axonal loss are highly suggestive of root avulsion. Reduction in
SNAP amplitudes indicates more peripheral injury, although root avulsion can be pres-
ent as well.

REFERENCES
I. Aminoff, MJ., Olney, R.K., Parry, G.J., Raskin, N.H., Relative utility of different electrophysiologic techniques in the
. evaluation of brachial plexopathies, Neurology 1988; 38(4):546-550.
2. Di Benedetto, M., Markey, K., Electrodiagnostic localization of traumatic upper trunk brachial plexopathy, Arch. Phys.
Med. Rehabil. 1984; 65(1):15-17.
3.. Ferrante, M.A., Wilbourn, A.J., The utility of various sensory nerve conduction responses in assessing brachial
plexopathies, Muscle Nerve 1995; 18(8):879-889.
4. Stewart, J.D., Focal Peripheral Neuropathies, 3rd ed., Lippincott Williams & Wilkins, Philadelphia, PA, 2000.
5. Wilbourn, AJ., Electrodiagnosis ofplexopathies, Neural. Clin. 1985; 3(3):511-529.

54
 PATIENT 14

A 56-year-old woman 2 days after a left shoulder dislocation and

closed reduction with diffuse left arm weakness

Neurological exam was notable for normal left shoulder shrug and slight arm abduction, and oth~
erwise complete paralysis of all other arm muscles.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) ().tV) (cm) (m/s) ,
L. median-dig II 1. Wrist Dig II 3.05 3.95 17.3 13 42.6,'
L. ulnar-dig V 1. Wrist DigV 2.05 2.60 40.6 II 53.7
L. radial-sn box 1. Forearm Sn box 1.65 2.15 26.4 10 60.6 "

Motor NCS

Recording Latency Amplitude Distance Veloci~

Nerve Sites Site (ms) (mV) (cm) (m/s)
L. median-APB 1. Wrist APB 4.40 8.6 7
2. Elbow APB 8.10 7.6 20 54.1
3. Axilla APB 9.45 6.5 12 ' 88.9
L. ulnar-ADM 1. Wrist ADM 2.60 7.1 7

F-Wave
Nerve Fmin (ms) Fmax (ms) Max - Min (ms) %F
L. median No response
L. ulnar No response

EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
L. deltoid NI 0 0 0 No activity
L. teres minor NI 0 0 0 No activity
L. biceps Nl 0 0 0 No activity
L. triceps NI 0 0 0 No activity
L. pron teres NI 0 0 0 No activity
L. first dors int NI 0 0 0 No activity

Continued

55
 EMG Summary Table-cont'd

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

L. supra spinatus Nl 0 0 0 Nl Nl Nl Full Nl

L. infra spinat Nl 0 0 0 Nl Nl Nl Full Nl
L. lev scapuli Nl 0 0 0 Nl Nl Nl Full Nl

A second follow-up electrodiagnostic study was performed 5 weeks later, and the results follow.

Sensory NCS
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (IlV) (cm) (mls)

L. median-dig II 1. Wrist Dig II Absent

L. ulnar-dig V 1. Wrist DigV 2.20 2.95 5.1 11 50.0
L. radial-sn box 1. Forearm Sn box 1.40 1.95 23.8 10 71.4

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (mls)

L. median-APB 1. Wrist APB 4.65 1.0 7

2. Elbow APB 10.65 0.7 21 35.0
L. ulnar-ADM 1. Wrist ADM 2.95 3.7 7
2. B. elbow ADM 6.55 3.1 18 50.0
3. A. elbow ADM 8.55 3.1 10 50.0

F-Wave

Nerve Fmin(ms) Fmax(ms) Max - Min (ms) %F

L. ulnar Absent
L. median Absent

EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

L. deltoid Nl 3+ 0 0 Nl Nl Nl No activity
L. biceps Nl 4+ 0 0 Nl Nl NI No activity
L. first dors int Nl 4+ 0 0 Nl Nl Nl No activity
L. abd dig min Nl 4+ 0 0 Nl Nl Nl No activit

56
 EMG Summary Table--cont'd

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
L. flex dig pr II Nl 4+ 0 0 Nl Nl Nl Full Single MU
L. flex dif pr III Nl 4+ 0 0 Nl Nt Nl Full Single MU
L. ext dig comm Nl 3+ 0 0 Nl Nl Nl Full Single MU

Questions:
I. What electrodiagnostic studies are most likely to be abnormal immediately after an acute motor
nerve lesion?
2. An experienced clinician's assessment of the patient was conversion disorder or hysterical paraly-
sis. What features of the neurological exam and the electrodiagnostic study refute or support this
opinion?
3. What abnormalities did you expect to see in this follow-up study?
4. After reviewing the follow-up study, how would you account for the combination offindings related
to the radial sensory study and the needle EMG study of the deltoid and biceps? What additional
studies not performed would have been of value in this regard?

5J.
 Answers:
1. Needle EMG interference pattens and possibly F-waves
2. Hysterical paralysis of an arm usually includes shoulder shrug.
3. Correlates of axonal degeneration
4. Stretch lesions are often not discrete; paraspinal needle EMG should have been performed.

Discussion: This case illustrates the value The second electrodiagnostic studies demon-
and limitations of electrodiagnostic studies in the strate the correlates of axonal degeneration not
period of time immediately after a nerve lesion. yet present in the first study. To summarize the
Even complete nerve transection is followed by implications:
normal distal evoked amplitudes, distal latency • Absent median-D2 SNAP suggests an upper
and velocities, and no fibrillation potentials in trunk or lateral cord lesion; the normal radial
the hyperacute period, typically for several days. SNAP favors the lateral cord localization.
F-wave abnormalities are then quite valuable in • Reduced ulnar-D5 SNAP, median-APB
demonstrating the proximal nature of the lesion. CMAP, and ulnar-ADM CMAP suggest a
If the lesion is incomplete, than needle EMG lower trunk or medial cord lesion.
will generally show a reduced (neurogenic) inter- • Moderately reduced ulnar-ADM CMAP but
ference pattern in weak muscles. If the lesion inability to recruit motor units voluntarily
is complete, the complete absence of recruited suggests at least partial focal demyelination
motor units makes it impossible to say whether of motor nerves in addition to the axonal loss
the weakness is suprasegmental (i.e., reduced effort, evidenced by the abundant fibrillation poten-
unconscious hysterical paralysis, or upper motor tials present.
neuron weakness) or truly due to lower motor neu- • Normal radial SNAP with complete inability
ron weakness. to recruit motor units in deltoid is suggestive
In this case, the abnormalities are the absence of C6 nerve root avulsion. An alternative
of F-wave responses (despite normal distal possibility would be combined lesions of the
CMAPs) and the complete lack of motor unit axillary nerve and musculocutaneous nerves.
recruitment in multiple arm muscles. In hypera- The last finding is surprising in light of the clini-
cute nerve lesions, the F-wave studies and needle cal history. Neglected in this study was examination
EMG interference patterns are generally the only of the paraspinal muscles, which can be invaluable
studies likely to be abnormal. Occasionally, in determining nerve root avulsion. This possibility
median and ulnar somatosensory evoked poten- was not considered by the electromyographer dur-
tials may be useful as well. ing the study. Finally, it can be noted that stretch
In addition to the absent F-wave responses injuries of the plexus are not discrete lesions, and it
noted above, two additional features serve to is possible to differentially affect fibers within the
argue strongly against an hysterical cause of this same nerve bundle; this may be the explanation for
patient's weakness. Hysterical paralysis of an arm fmdings that do not precisely localize.
usually includes the upper trapezius; patients Evolution of electrodiagnostic abnormalities after
generally are not aware that the spinal accessory acute axonal injury is generally occurs as follows:
nerve accounting for shoulder shrug has an • Abnormalities in F-wave, H-retlexes, and
anatomic pathway distinct from the other nerves needle EMG interference patterns
supplying arm function. In addition, both the • Reduction in CMAP amplitude without fib-
infraspinatus (suprascapular nerve) and teres rillation potentials due to neuromuscular
minor (axillary nerve) contribute to external rota- transmission failure
tion of the arm at the shoulder. In this case, • Reduction in SNAP amplitude after Wallerian
the former has a full interference pattern, and the degeneration has proceeded distally to the
latter shows no recruitable motor units. It is point of nerve stimulation
doubtful that such a discrepancy can be attributed • Continued reduction in CMAP amplitude
to a conscious or unconscious psychogenic with development of fibrillation potentials as
mechanism. Wallerian degeneration reaches distally

Clinical Pearl
Although fibrillation potentials may take several weeks to develop after an acute axonal
injury, other abnormalities (F-waves, motor unit recruitment patterns) are present
immediately, and electrodiagnostic studies can be helpful even early after an injury.

58
 PATIENT 15

A 56-year-old man with painful left hand weakness

This 56-year-old man developed aching pain in his left upper arm that lasted several days and was
followed by an inability to bend his thumb, index, and middle fingers. Pain resolved, but weakness per-
sisted, and he underwent electrodiagnostic studies at 8 months. There was no numbness in his hand or
arm. Neurological exam was notable for normal sensation and weakness of forearm pronation and
supination; the findings are shown in the figure. - '

C5

C6
> Trunks

Upper
---,
.....
"'/
/ .....
/
2
/
Cords

Lateral ....

·····x
....•....
Nerves
Musculocutaneous

Axillary

/ "3
C7 • ..-_M_i_dd_l_e~<'.
_ :: Posterior '. ..... Median
.... ...----
.....
' . ......-
··>:: ....._R_a_d_ia_l_
Medial ....
".

Ulnar
 Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (flV) (cm) (m/s)

L. median-dig II 1. Wrist Dig II 2.30 3.05 26.0 13 56.5

R. median-dig II 1. Wrist Dig II 2.80 3.45 22.4 13 46.4
L. ulnar-dig V 1. Wrist DigV 2.25 2.85 21.2 II 48.9
L. radial-sn box 1. Forearm Sn box 1.60 2.35 19.8 10 62.5
L. medAB cut I. Elbow Forearm 1.90 2.40 7.4 12 63.2
R. medAB cut I. Elbow Forearm 2.10 2.55 6.0 12 57.1
L. latAB cut I. Elbow Forearm 2.65 3.15 5.9 12 45.3
R.latAB cut I. Elbow Forearm 2.45 2.95 7.5 12 49.0

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)

L. median-APB 1. Wrist APB 3.35 7.0 7

2. Elbow APB 7.80 6.2 22.5 50.6
L. ulnar-ADM 1. Wrist ADM 3.05 8.5 7
2. B. elbow ADM 6.25 8.5 21 65.6
3. A. elbow ADM 7.75 8.3 10 66.7

F-Wave

Nerve Fmin(ms) Fmax (ms) Max - Min (ms) %F

L. median 29.70 32.30 2.60 70

L. ulnar 29.70 31.05 1.35 90

Needle EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

L. deltoid NI 0 0 0 NI NI Few Full NI

L. biceps NI 0 0 0 NI NI NI Full NI
L. triceps NI 0 0 0 NI NI NI Full NI
L. pron teres NI 3+ 0 0 NI NI NI Sub max NI
L. flex carp rad NI 2+ 0 0 NI NI NI Full NI
L. flex poll In NI 2+ 0 0 NI NI NI Full No activity
L. supinator NI 1+ 0 0 NI NI NI Full NI
L. ext dig comrn NI 0 0 0 NI NI NI Full NI
L. brachio-rad NI 0 0 0 NI NI NI Full NI
L. APB NI 0 0 0 NI NI NI Full NI

60
 Questions:
1. How do you best explain the pronator teres and flexor carpi radialis involvement without the APB
involvement in this case? Putting aside the supinator involvement for a moment, are the remaining
findings best explained by a proximal median neuropathy? Give three electrophysiological reasons
why "no" is the answer to this question.
2. The patient was asked to make a fist with both hands (see figure). What are the findings present?

61
 Answers:
1. The median-D2 SNAP is of normal amplitude, the median-APB CMAP is of normal amplitude, and
the needle EMG study of APB is normal.
2. Weakness of left FPL and flexion of digits 2 and 3

Discussion: The figure shows severe weak- explained by a lesion of the median nerve portion
ness of flexion of the left thumb distal phalanx of the lateral cord. Recall the median nerve
and less ability to fully flex the left second and receives a branch from the lateral cord and from
third digits (note that the proximal interpha- the medial cord; PT and FCR are the two lateral
langealjoints of the index and ring fingers are not cord median-innervated muscles, while all other
as fully tucked in on the left as on the right). The median-innervated muscles are medial cord. In
thumb weakness is that of flexor pollicis longus this case, the patient appears to have a lesion
(FPL), and one cannot tell from the picture if the of the anterior interosseous nerve and of the
difficulty with digits 2 and 3 is due to weakness unnamed branch from the lateral cord to the
of flexion at the distal interphalangeal joints median nerve (marked with an X in the figure). A
(flexor digitorum profundus, FDP) or at the prox- more proximal lateral cord lesion is probably not
imal interphalangeal joints (flexor digitorum present given the normal lateral antebrachial cuta-
superficialis, FDS). Clinical exam demonstrated neous SNAP and the normal needle EMG study
weakness of FDP digits 2 and 3 with normal of the biceps. In addition, the patient seems to
strength for FDS 2 and 3. Strength was normal for have isolated supinator involvement, without
FDP digits 4 and 5. other features of a radial neuropathy (normal
The electrodiagnostic study showed symmetric radial SNAP and EMG ofbrachioradialis), poste-
amplitudes of all sensory nerve action potentials. rior cord lesion (normal radial SNAp, deltoid, tri-
The needle EMG study was notable for the com- ceps, brachioradialis, and EDC), or C7 or C8 root
plete absence of recruitable motor units in the lesion (normal triceps and EDC). Such isolated
FPL, as well as fibrillation potentials in the and unusual focal neuropathies are characteristic
pronator teres, flexor carpi radialis, and supinator. of the diagnosis of acute brachial neuritis.
Clinically, the patient appears to have at least an Acute brachial neuritis, also referred to as neu-
anterior interosseous neuropathy, with weakness ralgic amyotrophy or the Parsonnage- Turner syn-
of flexion of the distal tips of digits I to 3, a drome, is a disorder of unknown cause, with
deficit he reported in the initial history. Given the typical features of acute and severe pain in the
involvement of the pronator teres and flexor carpi shoulder, shoulder blade, or arm for several days
radialis as well, one is tempted to suggest a prox- or a week or so, followed by multi focal weakness
imal median neuropathy rather than an anterior of the arm with variable and usually relatively
interosseous neuropathy. There are three argu- minor sensory symptoms and findings. The diver-
ments against this: (I) the median-D2 SNAP has sity of this disease is impressive and includes
normal amplitude, (2) the median-APB CMAP remarkable focality such as involvement of the
has normal amplitude, and (3) the needle EMG nerve branch to a single muscle. Involvement of
study of APB was normal. Recall also that the certain nerves, such as the anterior interosseous or
patient did not have any sensory symptoms or posterior interosseous, occurs so regularly in this
findings. disorder, and not in the much more common com-
The involvement of the pronator teres (PT) and pression syndromes, that their involvement always
flexor carpi radialis (FCR) is probably best raises acute brachial neuritis as a consideration.

Clinical Pearls
I. Acute brachial neuritis may present with more distal appearing lesions.
2. Two median nerve muscles are lateral cord innervated-pronator teres and flexor
carpi radialis.

REFERENCES
I. England, J.D., Sumner, A.J., Neuralgic amyotrophy: an increasingly diverse entity, Muscle Nerve 1987; 1O:6~8.
2. Lahrmann, H., Grisold, w., Authier, F.J., Zitko, U.A., Neuralgic amyotrophy with phrenic nerve involvement, Muscle Nerve
1999; 22:437-442.
3. Ma1amut, R.I., Marques, w., England, J.D., Sumner, A.J., Postsurgical idiopathic brachial neuritis, Muscle Nerve 1994;
17:320-324.

6Z
 4. Parsonage, M.J., Turner, 1.W.A., Neuralgic amyotrophy: the shoulder girdle syndrome, Lancet 1948; i:973-978.
5. Pierre, P.A., Laterre, C.E., Van Den Bergh, P.Y., Neuralgic amyotrophy with involvement of cranial nerves IX, X, XI, and,
XII, Muscle Nerve 1990; 13:704-707.
6. Turner, 1.W.A., Parsonage, M.J., Neuralgic amyotrophy (paralytic brachial neuritis), Lancet 1957; ii:209-2l2.

I
(

63
 PATIENT 16

A 71-year-old man with left arm weakness for 1 week

This 71-year-old man had brief loss of consciousness and fell in the shower I week prior to this
study and awoke with painless weakness of his left arm, with no sensory disturbance.
Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (flV) (em) (rn/s)

L. median-dig II 1. Wrist Dig II 3.10 4.15 19.2 13 41.9

L. ulnar-dig V 1. Wrist DigV 2.45 3.30 13.2 II 44.9
L. radial-sn box 1. Forearm Sn box 1.95 2.50 24.6 10 51.3

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (em) (m/s)

L. median-APB 1. Wrist APB 4.50 7.2 7

2. Elbow APB 8.90 7.2 24 54.5
L. ulnar-ADM 1. Wrist ADM 3.00 6.0 7
2. B. elbow ADM 7.30 6.2 23 53.5
3. A. elbow ADM 10.00 5.9 13 48.1

EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
L. deltoid NI 0 0 0 NI NI Nl Full SevRed
L. biceps NI 0 0 0 NI NI NI Full SevRed
L. triceps NI 0 0 0 NI NI NI Full ModRed
L. pron teres NI 0 0 0 NI NI NI Full ModRed
L. first dors int NI 0 0 0 NI NI NI Full NI
L. ext dig comm NI 0 0 0 NI NI NI Full NI
L. supra spinatus NI 1-2+ 0 0 NI NI NI Full MildRed
L. infra spinat NI 1-2+ 0 0 NI NI NI Full MildRed
L. C4 paraspinal NI 0 0 0
L. C5 paraspinal NI 3--4+ 0 0
L. C6 paraspinal NI 3--4+ 0 0
 Question: How do you explain the presence of fibrillation potentials in some but not all mus-
cles supplied by a common nerve root?

... '

6'
 Answers: Ongoing wallerian degeneration of motor axons creates a "proximal-to-distal" gradient
of fibrillation potentials.

Discussion: The results of nerve conduction of fibrillation potentials is a typical sign of acute
studies are normal. The results of EMG studies axonal injury, as Wallerian degeneration proceeds
are abnormal for: (1) fibrillation potentials with a from proximal to distal muscles. Follow-up nee-
proximal-to-distal gradient in left C5 and C6 cer- dle EMG study in 2 weeks would likely show
vical paraspinal muscles and supraspinatus and abundant fibrillation potentials in the distal mus-
infraspinatus, and (2) reduced recruitment of cles as well.
motor unit action potentials that is severe for the In general, the electrodiagnostic examination
left deltoid and biceps, moderate for the pronator of suspected radiculopathy targets muscles that
teres and triceps, and mild for the supraspinatus both sample and overlap the C5- T 1 roots. For
and infraspinatus. example, the muscles shown in the first table
The presence of marked reduction in interfer- below serve this purpose. This set of muscles
ence patterns in the deltoid, biceps, and pronator samples all these roots, with at least two muscles
teres with the absence of fibrillation potentials for each root, as well as the five major nerves of
could be due to a purely demyelinating lesion or the arm. The specific pattern of needle EMG
to acute axonal injury prior to the development of abnormalities in cervical radiculopathies is gen-
Wallerian degeneration. In this case, the latter is erally distinct for each root, although one should
more likely given the fibrillation potentials pres- be cautious because published myotome tables
ent in more proximal C6 muscles including are quite variable and individual variation clearly
paraspinals. The most proximal muscles, the exists. An excellent discussion of these patterns is
paraspinals, had 3 to 4+ fibrillation potentials; the noted by Levin et ai., I whose findings are sum-
next distal muscles, supraspinatus and infraspina- marized in the second table below. In this case,
tus, had I to 2+ fibrillation potentials; and more the abnormalities are most suggestive of a C6
distal muscles did not show fibrillation potentials. localization and do not fit neatly into the scheme
The presence of this "proximal-to-distal gradient" suggested by Levin et at.

Five Muscles Useful for Needle EMG Screening of the Arm, Sampling All Roots
with at Least Two Muscles per Root and All Five Major Nerves of the Arm

Muscle Root Trunk Cord Nerve

Deltoid C5-C6 Upper Posterior Axillary

Biceps C5-C6 Upper Lateral Musculocut
Pronator teres C6-C7 Upper Lateral Median
Extens dig comm C7-C8 Middle Posterior Radial
First dorsal inteross C8, Tl Lower Medial Ulnar

Pattern of Needle EMG Abnormalities in Sinyle Root Lesions as Determined by

Levin et al.

Root Muscles Involved

C5 Rhomboids, spinati, deltoid, biceps, brachialis, and brachioradialis

C6 Either the above-C5 or below-C7 pattern
C7 Triceps, anconeus, pronator teres, and flexor carpi radialis
C8 All ulnar innervated + finger extensors + flex pollicis longus

Clinical Pearl
Needle EMG screening for radiculopathy should include muscles with overlapping root
segments and cover all trunks, cords, and the five major nerves to the arm. The first
table above suggests one possible combination that achieves this.

66
REFERENCES
1. Levin, K.H., Maggiano, HJ., Wilbourn, AJ., Cervical radiculopathies: comparison of surgical and EMG localization of
single-root lesions, Neurology 1996; 46:1022-1025.
2. Wilbourne, AJ., Aminoff, MJ., AAEM minimonograph 32: the electrodiagnosis examination in patients with
radiculopathies, Muscle Nerve 1998; 21: 1612-1631.

67,
 PATIENT 17
A 61-year-old woman with breast cancer, right neck and shoulder
pain, and hand weakness and numbness 3 months prior to
evaluation

. Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (I.tV) (cm) (m/s)

R. median-dig II 1. Wrist Dig II 2.35 3.00 27.1 13 55.3

L. median-dig II 1. Wrist Dig II 1.95 2.70 35.8 13 66.7
R. ulnar-dig V 1. Wrist DigV 2.05 2.70 27.5 11 53.7
L. ulnar-dig V 1. Wrist DigV 1.70 2.45 25.3 11 64.7
R. ulnar dorsal 1. Wrist Dors hand Absent
L. ulnar dorsal 1. Wrist Dors hand Absent
R. radial-sn box 1. Forearm Sn box 1.50 2.00 34.5 10 66.7
L. radial-sn box 1. Forearm Sn box 1.45 1.90 34.6 10 69.0
R. medAB cut 1. Elbow Forearm 1.65 2.10 10.1 12 72.7
L. medAB cut 1. Elbow Forearm 1.65 2.25 10.0 12 72.7
R. lat AB cut I. Elbow Forearm 1.85 2.25 11.0 12 66.0
L. latAB cut 1. Elbow Forearm 2.00 2.40 14.0 12 60.0

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)

R. median-APB 1. Wrist APB 3.35 10.5 7

2. Elbow APB 6.90 10.3 20.5 57.7
L. median-APB 1. Wrist APB 3.15 8.8 7
2. Elbow APB 6.80 8.4 21.5 58.9
R. ulnar-ADM 1. Wrist ADM 2.70 5.5 7
2. B. elbow ADM 5.65 5.3 19 64.4
3. A. elbow ADM 7.40 5.1 12 68.6
L. ulnar-ADM 1. Wrist ADM 2.20 10.0 7
2. B. elbow ADM 5.15 9.3 19.5 66.1
3. A. elbow ADM 6.70 8.6 10 64.5
R. ulnar-FDI I. Wrist FDI 3.55 4.7
2. B. elbow FDI 6.75 4.6 19 59.4
3. A. elbow FDI 8.25 4.6 10 66.7
L. ulnar-FDI 1. Wrist FDI 3.25 11.7
2. B. elbow FDI 6.25 10.7 20 66.7
3. A. elbow FDI 7.80 9.8 10 64.5

68
 F-Wave

Nerve F min (ms) F max (ms) Max - Min (ms) %F

R. median 25.95 27.35 1.40

R. ulnar 27.05 29.75 2.70

Needle EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R. first Incr 1+ 0 0 Many Large Many Long Many Full Discrete
dors int
R. abd NI 0 0 0 Many Large Many Long Few Full Mod red
poll br
R. abd Incr Rare 0 0 Many Large Many Long NI Full Mild red
digmin
R. flex Nl 0 0 0 Most Large Most Long Nl Full Discrete
poll In
R. ext Incr 2+ 0 0 Many Large Many Long Many Full Mod red
poll In
R. deltoid NI 0 0 0 Nl Nl Nl Full NI
R. triceps NI 0 0 0 Nl Nl NI Full NI
R. pron Nl 0 0 0 Nl NI Nl Full Nl
teres
R.C? NI 0 0 0 Nl NI Nl Full NI
para-
spinal
R. C8 para- Incr 2+ 0 0 Nl Nl Nt Full NI
spinal
R. TI para- Incr 2+ 0 0 NI NI NI Full Nt
spinals
R. thoracic NI 0 0 0 NI Nl Nl Full NI
PSP
upper
L. first Nl 0 0 0 NI NI NI Full Nl
dors int
--
L. C8 para- NI 0 0 0 Nl Nl Nl Full NI
spinal

Questions:
I. Are the nerve conduction studies more suggestive of a radiculopathy or a plexopathy?
2. What is the electrodiagnostic diagnosis?

69.
 Answers:
I. Radiculopathy
2. C8 radiculopathy

Discussion: The nerve conduction studies gesting motor axonal loss in these muscles. As
show symmetric and normal sensory studies. their innervation is in the C8 and T 1 roots, lower
Given the patient's sensory complaints, this trunk, or medial cord and the ulnar nerve, a nor-
immediately favors a root more likely than plexus mal ulnar-D5 SNAP suggests the proximal root
localization. The bilateral absence of the dorsal localization. The needle EMG study abnormali-
ulnar. cutaneous SNAPs is a normal finding in ties in the ulnar-innervated FDI and ADM mus-
some. individuals. The motor studies show rela- cles, as well as in the radial finger extensor EPL
tively reduced amplitudes (approximately or less and median muscle FPL and APB, is the pattern
than.50% those of contralateral studies) of the of a C8 lesion (see discussion and tables for
right ulnar-ADM and ulnar-FDI CMAPs, sug- patient 16).

Clinical Pearl
Sensory nerve action potential amplitudes are normal in cervical radiculopathy despite
sensory symptoms and signs-this is because a lesion proximal to the dorsal root gan-
. glion does not result in degeneration of the peripheral sensory axons.

REFERENCES
I. Levin, K.H., Maggiano, HJ., Wilbourn, AJ., Cervical radiculopathies: comparison of surgical and EMG localization of
single-root lesions, Neurology 1996; 46: I 022-1 025.
2. Wilbourne, AJ., Aminoff, MJ., AAEM minimonograph 32: the electrodiagnosis examination in patients with
. .-radiculopathies, Muscle Nerve 1998; 21: 1612-1631.

70
 Focal Neuropathies of the Lower Limb: Anatomy for the
Electrodiagnostic Practitioner

As with the arm, the electromyographer needs to think about the anatomy of the leg from the per-
spective of nerve conduction studies and needle EMG. The five lumbar roots LI to L5 and first three
sacral roots SI to S3 supply the leg. The lumbosacral plexus (figure 1.2) is formed from a combination
of the lumbar plexus and the sacral plexus. The lumbar plexus is formed from the ventral rami of
L1-L4; the sacral plexus is formed from the ventral rami of S I-S4 together with a communication
between the two, the lumbosacral trunk, which distally gives rise to the lateral portion of the sciatic
nerve and eventually the common peroneal nerve. The electrodiagnostic tools available for examina-
tion of the leg are more restricted than those for the arm. Table 1 shows the nerve conduction studies
available and the portions of the peripheral nervous system from which they are derived. As can be
seen, nerve conduction studies reliably assess axons derived from the L5, SI, and S2 roots, the lum-
bosacral trunk, and the sacral plexus, but they do not reliably assess the lumbar plexus. The saphenous
SNAp' which could possibly be of value in assessing the lumbar plexus, unfortunately has not been
obtainable in some healthy controls in several studies. 1,4,6 The absence of sensory nerve studies of the
lumbar plexus limits the diagnostic power to separate root from plexus disease, particularly as the pres~
ence ofIumbar paraspinal muscle abnormalities (the only other electrodiagnostic technique available)
can be seen in healthy individuals.2 ' .....
It is also noteworthy that, unlike the arm, the dorsal root ganglia for lumbosacral dorsal roots
sometimes lie within the spine canal; thus, intraspinal processes may result in amplitude reductions for
the superficial peroneal SNAP2 and, in theory, the sural SNAP.

Table 1. Root, Plexus, and Nerve Innervation Relevant to Sensory and Motor
Nerve Conduction Studies in the Lower Limb
Study Root(s) Plexus Nerve
Sensory

Sural SI Sacral Tibial

Superficial peroneal L5 Lumbosacral trunk Superficial peroneal
Saphenous L4 Lumbar Femoral
Lateral femoral cut L2,3 Lumbar Lateral femoral cutaneous
Lateral plantar SI Sacral Tibial
Medial plantar ?L5, SI Sacral Tibial

Motor

Peroneal-EDB L5,SI Lumbosacral trunk Deep peroneal

Tibial-AH SI,2 Sacral Tibial
Peroneal- TA L4,5 Lumbosacral trunk Deep peroneal

Table 2. Root and Nerve Innervation Relevant to Needle EMG Studies in the
Lower Limb
Muscle Root(s) Nerve
Iliopsoas L2,L3 Lumbar plexus
Adductor magnus L2,L3,L4 Obturator + Sciatic
Adductor longus L2,L3,L4 Obturator

Continued
 Table 2. Root and Nerve Innervation Relevant to Needle EMG Studies in the
Lower Limb

Muscle Root(s) Nerve

Quadriceps L2,L3,L4 Femoral

Tibialis anterior L4,5 Peroneal
Peroneus longus L5,SI Peroneal
Tibialis posterior L5, SI Tibial
Biceps femoris, short head L5,SI Sciatic, lateral
Biceps femoris, long head L5,SI Sciatic, medial
Extensor hallucis longus L5,SI Peroneal
Flexor hallucis longus L5,SI Tibial
Gastrocnemius SI,S2 Tibial
Gluteus medius L4,L5,SI Superior gluteal
Tensor fascia lata L4,L5,SI Superior gluteal
Gluteus maximus L5, SI,S2 Inferior gluteal

The Lumbosacral Plexus

Iliohypogastric
____________ ::~~,---------------------[] L1
Ilioinguinal. - - - - - - --

L2
- - - - -. Purely cutaneous nerve
-- Motor and sensory nerve Genitofemoral -< - - -
__ Sciatic nerve, lateral trunk
L3
. ---:--. Sciatic nerve, medial trunk

Lateral femoral cutaneo~J::: %: =~ - L4

L5
Femoral
81

82

83

':, .'. .'

84
I ' ~'

,.
".~
I
.-
d'

:
Post cutaneous n.
of the thigh

REFERENCES
I. Izzo, K.L., Sridhara, C.R., Rosenholtz, H., Sensory conduction studies of branches of superficial peroneal nerve, Arch.
Phys. Med. Rehabil. 1981; 62:24-27.
2. Levin, K.H., L5 radiculopathy with reduced superficial peroneal sensory responses: intraspinal and extraspinal causes,
Muscle Nerve 1998; 21 :3-7.
3. Nardin, R.A., Raynor, E.M., Rutkove, S.8., Fibrillations in lumbosacral paraspinal muscles of normal subjects, Muscle
Nerve 1998; 21(10):\347-\349.
4. Senden, R., Van Mulders, J., Ghys, R., Rosselle, N., Conduction velocity of the distal segment of the saphenous nerve in
normal adult subjects, Electromyogr. Clin. Neurophysiol. 1981; 21 :3-10.
5. Subramony, S.H., Wilbourn, A.J., Radicular derivation of sensory action potentials in lower extremity, Arch. Phys. Med.
Rehabil. 1981; 62( II ):590-592.
6. Tranier, S., Durey, A., Chevallier, B., Liot, E, Value of somatosensory evoked potentials in saphenous entrapment
neuropathy,.!. Neurol. Neurosurg. Psychiatry 1992; 55(6):461-465.
 PATIENT 18

A 64-year-old man with right foot drop 6 weeks prior

This man could not recall the precise onset of his right foot weakness but believed it had not
changed since first noticed. There was no numbness or pain that he was aware of. His wife noted his
tendency to fall asleep with his right leg crossed over his left. Examination showed grade 2/5 strerigth
in the right tibialis anterior, eversion, and toe extensors, with all other muscles 5/5. There was a sen-
sory disturbance to light touch over the dorsum of his foot and lateral leg to the mid lower leg.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Amplitude Distance Velocity

Nerve Sites Site (ms) (I-lV) (cm) (mls)

L. sural-I at mall 1. Mid calf Ankle 4.2 16.2 14 33.3

R. sural-Iat mall 1. Mid calf Ankle 4.5 19.9 14 31.1
R. sup peroneal 1. Lat leg Ankle 3.8 9.2 14 36.8
L. sup peroneal 1. Lat leg Ankle 3.9 6.3 14 36.0

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (mls)

R. peroneal-EDB 1. Ankle EDB 7.8 0.8 8

2. Fib head EDB 14.7 0.8 24 34.7
3. Pop fossa EDB Absent
R. peroneal-tib ant 1. Fib head Tib ant 4.2 0.8 8
2. Pop fossa Tib ant 9.4 0.3 9 17.3
R. tibial-AH 1. Ankle AH 5.3 2.2 8
2. Pop fossa AH 14.5 1.5 35 38.0

EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R. tib ant NI 2+ 0 0 None Full Sev dec
R. per long NI 2+ 0 0 NI NI NI Full Sev dec
R. tib post NI 0 0 0 NI NI NI Full NI
R. bic fern SH NI 0 0 0 NI NI NI Full NI

Questions:
1. What is the clinical differential diagnosis?
2. What specific nerve conduction and EMG studies should be performed to address this differential
diagnosis?
3. Ifthe nerve conduction velocities had all been normal, what additional nerve conduction and needle
EMG studies might have been helpful?
 Answers:
1. Common peroneal neuropathy vs. LS radiculopathy
2. SNAP amplitudes, across fibular head conduction velocity, and tibialis posterior needle EMG
3. Contralateral nerve conduction studies and ipsilateral more proximal needle EMG studies

Discussion: The clinical localization of a EDB or peroneal- TA motor segments-

unilateral foot drop is typically either.a common As we have emphasized before, a focal
peroneal neuropathy or LS radiculopathy. A sci- abnormality such as these are definitive in
atic neuropathy, although uncommon, may show localization.
differential involvement of the lateral portion, • Peroneal-EDB F-wave minimum latency and
which is the common peroneal nerve. Other local- persistence, compared to the other side-
izations may occur, including central. The clinical Particularly when the distal evoked CMAP is
features that may distinguish an LS radiculopathy normal, the absence of F-waves on one side
from a common peroneal neuropathy at the fibu- with good persistence on the other provides
lar head include the presence of low back and good evidence of a proximal demyelinating
posterior leg pain, weakness of foot inversion and (or too recent) lesion but will not distinguish
hip internal rotation (LS but not common per- peroneal nerve from LS lesions.
oneal nerve), and a pattern of sensory distur- • Needle EMG abnormalities in common per-
bance. In an LS lesion, this may include a portion oneal innervated muscles (tibialis anterior,
of the distal sole and ventral surfaces of the toes, peroneus longus) but not LS non-peroneal
as well as the lateral calf above the mid-leg to the nerve-innervated muscles (tibialis posterior,
knee. In a peroneal neuropathy at the fibular head, gluteus medius, LS paraspinal muscles)--
the sensory loss does not usually go onto the ven- Additionally, the short head of biceps
tral foot or above the mid-lower leg (lateral cuta- femoris is supplied by the common peroneal
neous nerve of the calf territory, a branch of the division of the sciatic nerve, while the long
lateral cord of the sciatic nerve and essentially head is supplied by the tibial division.
common peroneal nerve, but above the popliteal Demonstrating normality of these helps to
fossa). In addition, signs of mechanical irritabil- exclude LS and sciatic neuropathies as the
ity of the peroneal nerve at the fibular head (Le., cause of the foot drop.
tingling paresthesias in its distribution provoked In this particular case, the clinical features sug-
by tapping or rubbing the nerve over the fibular gested a peroneal neuropathy, particularly the full
head) may also be helpful in localization. strength for ankle inversion and the sensory
The electrodiagnostic parameters that may be deficit sparing the territory of the lateral cuta-
of help include: neous nerve of the calf (LS, but off of peroneal
• The amplitude of the superficial peroneal division of sciatic nerve at or just above the
SNAP-SNAPs may be helpful in distin- popliteal fossa). The electrodiagnostic abnormal-
guishing root from more peripheral lesions, ities include reduced amplitude of the per-
although not always;3 however, common oneal-EDB CMAP (uncommon but does occur
peroneal nerve lesions at the fibular head with LS lesions) and a definitive focal abnormal-
due to compression are often predominantly ity-an across-fibular-head velocity of 17 m/s for
demyelinating, which will not affect the the peroneal- TA motor segment-localizing the
distal evoked superficial peroneal SNAP lesion to a peroneal neuropathy at the fibular
amplitude unless there has been secondary head. The needle EMG studies provide more sup-
Wallerian degeneration. port of this. Additional nerve conduction studies
• The amplitude of the peroneal-EDB CMAP- that would have been helpful if focal slowing was
The peroneal-EDB CMAP amplitude is often not present would have included contralateral
not affected by an LS lesion but may be by a peroneal-EDB and peroneal- TA CMAP and
common peroneal neuropathy at the fibular superficial peroneal SNAP amplitudes to com-
head (although, again, pure demyelinating pare to the symptomatic side. Additional needle
lesions will not affect it). EMG studies would have included both heads of
• The presence of focal slowing or conduction biceps femoris, gluteus medius, and paraspinal
block across the fibular head of peroneal- muscles.

14'
 Clinical Pearls
I. The differential diagnosis of a foot drop includes upper motor neuron lesions, L4
or L5 anterior horn or root lesions, lumbosacral trunk, sciatic or peroneal neuropathies,
and disorders of the neuromuscular junction and muscle.
2. When the tibialis anterior has needle EMG abnormalities in patients with foot
drop, other muscles that should be examined include peroneus longus (distinguishes
common peroneal from deep peroneal neuropathies), tibialis posterior (abnormal in L5
but not peroneal neuropathies), and gluteus medius or lumbar paraspinal muscles (both
abnormal in L5 but not sciatic neuropathies).

REFERENCES
I. Bendszus, M., Koltzenburg, M., Visualization of denervated muscle by gadolinium-enhanced MRI, Neurology 200 I;
57: 1709-171l.
2. Katitji, 8., Peroneal neuropathy, Neural. Clin. 1999; 17:567-59l.
3. Levin, K.H., L5 radiculopathy with reduced superficial peroneal sensory responses: intraspinal and extraspinal causes,
Muscle Nerve 1998; 21:3-7.

75
 PATIENT 19

A 70-year-old woman with right foot drop after hip replacement

This woman was referred for right foot drop immediately after right hip replacement several
months prior. Examination was notable for severe weakness of tibialis anterior and ankle eversion with
probable normal ankle inversion and plantarflexion. The sensory disturbance included the dorsal and
lateral aspects of the foot and the lateral leg up to the knee.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (/-lV) (cm) (m/s)

R. sural-Iat mall 1. Mid calf Ankle Absent

R. sup peroneal 1. Lat leg Ankle Absent
L. sural-Iat mall I. Mid calf Ankle 3.10 3.60 4.3 14 45.2
L. sup peroneal 1. Lat leg Ankle 2.10 2.90 10.6 12 57.1

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)

R. peroneal-EDB 1. Ankle EDB Absent 8

L. peroneal-EDB 1. Ankle EDB 3.60 3.1 8
2. Fib head EDB 9.55 2.9 27 45.4
3. Pop fossa EDB 10.55 2.8 6 60.0
R. peroneal-tib ant 1. Fib head Tib ant Absent
L. peroneal-tib ant 1. Fib head Tib ant 2.70 4.6 7
2. Pop fossa Tib ant 3.90 3.6
R. tibial-AH 1. Ankle AH 3.60 6.0 8
2. Pop fossa AH 11.25 3.7 36 47.1
L. tibial-AH 1. Ankle AH 3.35 10.8 8
2. Pop fossa AH 10.70 7.4 33 44.9

EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. tib ant Inc 3+ 0 0 None None None

R. tib post Nl 0 0 0 NI Nl NI Full Nl
R. gastroc med NI 0 0 0 NI NI NI Full NI
R. vast med NI 0 0 0 NI Nl NI Full NI
R. bic fern SH Inc 2+ 0 0 NI Nl Many Full Mild red
R. glut Nl med Nl 0 0 Nl Nl NI Full Nl
R. L5 paraspinals Nl 0 0 0

16
 Questions:
1. What are the main clinical considerations?
2. What is the localization, based on the electrodiagnostic studies?
 Answers:
I. Peroneal neuropathy, sciatic neuropathy, or L4 or L5 rdiculopathy
2. Sciatic neuropathy

Discussion: As discussed in case 18, the main this localization. The needle EMG abnormalities
considerations for a foot drop are a common per- in the short head of the biceps femoris at least tell
oneal neuropathy at the fibular head, sciatic us that it is not a common peroneal neuropathy at
neuropathy, and an L4 or L5 radiculopathy. the fibular head. Additional nerve conduction
Particularly after hip replacement, a sciatic neu- studies that might have been of help would have
ropathy with predominant involvement of the lat- included bilateral tibial F-waves. These are gener-
eral division needs to be considered. In this case, ally easy to obtain, so normal studies on the left
the superficial peroneal SNAp, peroneal-EDB with poor persistence on the right could have pro-
CMAp, and peroneal- TA CMAP were all absent, vided strong evidence of a sciatic localization.
as would be seen in a lesion of the common per- Several key anatomical points relating to the
oneal nerve or its proximal extension. However, sciatic nerve include:
the sural SNAP is also absent, while normal on • It is formed from the sacral plexus + the lum-
the contralateral side, and the patient's postopera- bosacral trunk.
tive clinical sural territory sensory loss goes • The sciatic nerve is separated into lateral and
along with this finding. The sural is predomi- medial trunks; the lateral trunk appears more
nantly a branch of the tibial nerve, although it susceptible to injury.
may receive some axons from the communicating • In the biceps femoris, the long head is inner-
sural division of the common peroneal nerve. An vated by the sciatic nerve, medial trunk;
absent sural SNAP indicates that the lesion, if the short head by the sciatic nerve, lateral
there is just one, localizes to the sciatic nerve. The trunk.
asymmetry of the tibial-AH CMAP amplitudes, • The adductor magnus has a dual nerve sup-
although not at a level of abnormality (i.e., 50% ply: obturator nerve (L2-L4) and sciatic
side-to-side difference), does add some support to nerve (L4-L5).

Clinical Pearl
Clinically, many sciatic neuropathies look like common peroneal neuropathies because
of apparently increased susceptibility to injury of the lateral division of the sciatic
nerve, which gives rise to the common peroneal nerve. Subclinical involvement of por-
tions of the tibial nerve (i.e., reduction of sural SNAP or tibial-AH CMAP amplitude,
tibial-AH F-wave abnormalities, needle EMG abnormalities in the long head of the
biceps femoris) supports sciatic neuropathy over peroneal neuropathy.

REFERENCES
1. Asnis, S.E., Hanley, S., Shelton, P.D., Sciatic neuropathy secondary to migration of trochanteric wire following total hip
arthroplasty, Clin. Orthop. 1985; 196:226-228.
2. Edwards, B.N., Tullos, H.S., Noble, P.c., Contributory factors and etiology of sciatic nerve palsy in total hip arthroplasty,
Clin.Orthop. 1987; 218:136-141.
3. Fischer, S.R., Christ, DJ., Roehr, B.A., Sciatic neuropathy secondary to total hip arthroplasty wear debris, J. Arthroplasty
1999; 14:771-774.
4. Yuen, E.C., So, YT., Sciatic neuropathy, Neural. Clin. 1999; 17:617--{j31.
5. Yuen, E.C., So, Y.T., Olney, R.K., The electrophysiologic features of sciatic neuropathy in 100 patients. Muscle Nerve
1995; 18:414-420.
6. Yuen, E.C., Olney, R.K., So, YT., Sciatic neuropathy: clinical and prognostic features in 73 patients, Neurology 1994;
44: 1669-1674.
7 .. Zechmann, J.P., Reckling, F.w., Association of preoperative hip motion and sciatic nerve palsy following total hip
arthroplasty, Clin Orthop. 1989; 241: 197-199
 PATIENT 20

A 38-year-old man with left foot weakness and numbness after

prior treatment for a posterior thigh sarcoma

This 38-year-old man was treated at the age of31 for a liposarcoma of the left posterior thigh with
surgery and radiation therapy. Seven years later, he developed weakness of ankle plantar flexion and
numbness of the lateral foot and sole and the lateral and medial heel, as well as enlargement of his calf
muscle. Six months after onset of symptoms, neurological exam showed mild to moderate weakness
of gastrocnemius and toe flexion and mild weakness of tibialis anterior and toe extension, with a sen-
sory deficit as described above by the patient's symptoms. Despite gastrocnemius weakness with
resulting inability to stand on the ball of his left foot, there was substantial hypertrophy of the left lat-
eral calf (indicated by arrowhead in figure 1). There were frequent fasciculations in the left calf. The
superior and lateral portion of the calf demonstrated semirhythmic contractions similar to fascicula-
tions but distinct in having a nearly regular, rhythmic quality. Note the scarring in the distal posterior
thigh in figure 1 as well.

Figure 1

Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance . Velocity
Nerve Sites Site (ms) (ms) (l1V) (cm) (m/s)
L. sural-Iat mall 1. Mid calf Ankle 3.45 4.35 8.9 14 40.6
R. sural-I at mall 1. Mid calf Ankle 3.00 3.50 6.1 14 46.7
L. sup peroneal 1. Lat leg Ankle 2.70 3.60 7.9 12 44.4

19
 Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)
L. peroneal-EDB 1. Ankle EDB 5.60 6.5 8
2. Bel FibHd EDB 13.50 5.7 35 44.3
3. AbvFibHd EDB 15.95 5.6 10 40.8
L. tibial-AH 1. Ankle AH 6.15 14.4 8
2. Pop Fossa AH 16.35 9.5 44 43.1
R. tibial-AH 1. Ankle AH 5.25 14.5 8

L TIBIAL RTIBIAL
1.1

1.2

1.3'

104.

1.5

1.6

1.7

1.9

1.10

1.11

1,12'

100ms 500~N

Figure 2

Questions:
'1. The patient had marked weakness of toe flexion. How do you explain this in the face of the motor
nerve conduction studies above? What additional nerve conduction studies might be of value in test-
. ing your hypothesis?
2. The patient had marked numbness in the left sural nerve territory. How do you explain this in the
face of the sensory nerve conduction studies above?
5. What sensory' nerves appear clinically affected in this patient?
4. What are the potentials seen in the right half of the left tibial nerve study in figure 2? Are they
F-waves? (Hint: Look at the bottom two stimulation tracings in the panel)
5. 'How do you interpret the F-wave results together with the normal tibial-AH CMAP amplitude?
6. What is the electrodiagnostic term for spontaneous activity consisting of semi-rhythmic bursts of
grouped motor unit action potentials?

80
 Answers:
1. Focal demyelination and conduction block proximal to stimulation sites; consider F-wave
2. Focal demyelination and conduction block of sensory axons proximal to stimulation sites
3. Sural, lateral plantar, calcaneal nerves
4. Background surface motor unit potentials
5. Focal conduction block proximally
6. Myokymic potentials

Discussion: The sensory territory affected is CMAP amplitudes indicates focal conduction
that of the sural, lateral plantar, and calcaneal block of motor axons proximal to the ankle. The
nerves, all branches of the tibial nerve. An needle EMG study demonstrated semirhythmic
impressive disturbance of sensation in the sural bursts of motor unit action potentials. These are
nerve territory with a preserved SNAP amplitude shown in figure 3, where tracing (a) shows three
may be seen in any of the following: hyperacute bursts of grouped motor unit discharges; the mid-
axonal injury; focal demyelination and conduc- dle group of motor unit discharges in (a) is
tion block of sensory axons proximal to the point enlarged in (b). Examine the figure and then do
of nerve conduction stimulation; or a lesion prox- the following:
imal to the dorsal root ganglion-root, spinal I. From tracing (a), calculate the approximate
cord, or brain. Similarly, the marked toe flexion interburst frequency.
weakness together with robust and symmetric 2. From tracing (b), calculate the approximate
tibial-AH CMAP amplitudes implies the pos- range of interpotential frequencies within a
sibilities of hyperacute axonal injury, focal burst (or grouped discharge).
demyelination and conduction block proximal to Figure 3A shows myokymic potentials. These
the stimulation site, or a central nervous system typically consist of semirhythmic or rhythmic
(CNS) lesion. The clinical history excludes grouped discharges of motor unit potentials, typ-
hyperacute axonal injury from consideration. ically with a significantly longer interburst period
One additional nerve conduction study that than intraburst (i.e., between potentials) period. In
might be of help is the tibial F-wave study. the example above, we calculate the interburst
Tibial-AH F-waves are generally robust and easy frequency using the 325-ms latency between the
to obtain, unlike peroneal-EDB, which may first and second groups. Thus, 1000 ms divided
be bilaterally absent in healthy individuals. by 325 ms gives 3 cycles/s, or 3 Hz. The interpo-
Abnormalities in F-wave responses would likely tential frequencies within a burst vary; the first
distinguish between conduction block and a CNS two potentials in (b) are 12 ms apart, translating
lesion. The results are shown in figure 2. to a frequency of 1000 divided by 12, or 83 Hz.
In the left tibial nerve study, the potentials The second and third potentials in (b) are 33 ms
could be F-waves, but they do not look like the apart, translating to a frequency of 1000 divided
typical multiphasic tibial F-waves, as in the right by 33, or 30 Hz. The range is thus on the order of
tibial study. The last two tracings do not have a 30 to 83 Hz. In general, myokymic discharges
tibial-AH CMAP ("M wave") present; the stimu- have interpotential discharge frequencies of less
lator was not applied to the patient, yet similar than 100 Hz. Note also two other characteristic,
potentials are present. This indicates that the sur- although not universal, features of myokymic,dis-
face electrode is picking up EMG activity, likely charges: (1) reduction in amplitude of the poten-
motor unit discharges from incomplete relaxation tials from start to finish within a given burst, and
or fasciculations. The marked asymmetry of F- (2) variation in the number of potentials within
wave responses, absent on the left and robust each burst. The tabular results of the EMG stud-
on the right, with symmetric normal tibial-AH ies are provided next.

81
 325 msec
R Lateral Gastrocnemius 1mvL
100 ms

Figure 3

Needle EMG Summary Table

SA

Amplitude Duration Recruitment

IA Fib Fasc Other (MUs) (MUs) Activation Pattern

L. gastroc med Inc 0 Occ Myokymic NI NI Full Sev dec

discharges
L. tib ant NI 2+ Occ 0 NI NI Full Mild dec
L. tib post Inc 2+ Occ Myokymic NI NI Full NI
discharges
L. vast med NI 0 0 0 NI NI Full NI
L. glut med NI 0 0 0 NI NI Full NI
L. L5/S I paraspinals NI 0 0 0 NI NI Full NI

These additional studies show involvement of and clinically visible accompaniment of the
peroneal-nerve-innervated tibialis anterior. The myokymic potentials, namely semirhythmic con-
gastrocnemius does not show any fibrillation traction and dimpling of a portion of the gastroc-
potentials despite severely reduced interference nemius muscle. This later clinical finding does not
pattern, another point supporting conduction have a generally accepted name; it is not the irreg-
block of motor axons supplying this muscle. ular undulating movements resulting from frequent
In summary, this patient has a sciatic neuropathy fasciculations and generally referred to as clinical
with myokymic potentials characteristic of radia- myokymia. In addition, this patient's syndrome is
tion-induced focal neuropathy. A continuous marked by clinical and electrophysiological evi-
motor unit activity syndrome is also present (see dence of focal demyelination, likely at the site of
patient 50) with resulting hypertrophy of the calf radiation nerve injury in the posterior thigh.

82
 Clinical Pearls
1. Myokymic potentials are semirhythmic bursts of groups of motor units and are
characteristic of radiation-induced nerve injury.
2. Substantial weakness of a muscle with normal bulk or even hypertrophy, in the
setting of a peripheral nerve disorder, is likely due to focal demyelination of motor
axons.

REFERENCES
1. Daube, 1.R., Myokymia and neuromyotonia, Muscle Nerve 2001; 24:1711-1712.
2. Gutmann, L., Libell, D., Gutmann, L., When is myokymia neuromyotonia? Muscle Nerve 200 I; 24: 151-153.
3. Gutmann, L., AAEM minimonograph #46: neurogenic muscle hypertrophy, Muscle Nerve 1996; 19:811-818.
4. Harper, C.M., Jr., Thomas, 1.E., Cascino, T.L., Litchy, W.J., Distinction between neoplastic and radiation-induced brachial
plexopathy, with emphasis on the role of EMG, Neurology 1989; 39:502-506.
5. Hart, IX., Maddison, P., Newsom-Davis, 1., Vincent, A., Mills, K.R., Phenotypic variants of autoimmune peripheral nerve
hyperexcitability. Brain 2002; 125: 1887-1895.
6. Vincent, A., Understanding neuromyotonia, Muscle Nerve 2000; 23:655--{)57.

83
 PATIENT 21

A 73-year-old man with right leg numbness, weakness, and pain

Four months ago, this 73-year-old man developed mild pain in his right buttock, more moderate
pain in his right groin and knee, and tingling paresthesias over the right anterior thigh. He noted his
right leg was buckling, and his pain acutely worsened several weeks ago and has gradually improved
since. Neurological examination was notable for moderate weakness of right psoas and mild weakness
of right adductors and knee extensors. Other leg muscles were normal. On sensory examination, light
touch was abnormal at the medial and lateral knee. Reflexes were symmetric at 2+ at the knees.

A B

Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (IlV) (cm) (mls)

R. saphenous Mid calf Ankle Absent

L. saphenous Mid calf Ankle Absent
R. sural-lat mall 1. Mid calf Ankle 3.55 4.35 2.8 14 39.4
L. sural-lat mall 1. Mid calf Ankle 3.50 4.35 3.5 14 40.0

84
 Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)
R. median-APB I. Wrist APB 4.30 5.7 7
2. Elbow APB 9.20 5.9 25.5 52.0
R. peroneal-EDB I. Ankle EDB 4.45 3.5 8
2. Fib head EDB 11.55 3.2 27 38.0
3. Pop fossa EDB 13.65 2.7 9.5 45.2

Needle EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R. iliopsoas Incr 2+ 0 0 NI NI NI Full NI
R. vast lat Incr 2+ 0 0 NI NI NI Full NI
R. tib ant NI 0 2+ 0 NI NI NI Full NI
R. gastroc med NI 0 1+ 0 NI NI NI Full NI
R. add magnus Incr 2+ 0 0 NI NI NI Full NI
R. L2 paraspinals Nl 0 0 0
R. L3 paraspinals NI 0 0 0
R. L4 paraspinals Nl 0 0 0

Question: What is the clinical differential diagnosis?

8S
 Answer: L2 or L3 radiculopathy or lumbar plexopathy

Discussion: The electrodiagnostic abnormali- implied localization is to the upper lumbar plexus
ties are limited to the needle EMG study (bilateral or the L2 or L3 nerve roots. The figure shows MRI
absence of saphenous SNAPs is a normal finding at images from the patient; note the large L2-L3 cen-
any age). Fibrillation potentials are present in the tral and right paracentral disc herniation. The clini-
iliopsoas, vastus lateralis, and adductor magnus; the cal diagnosis was an L3 radiculopathy.

Clinical Pearl
Needle EMG abnormalities of paraspinal muscles are not necessarily present In
radiculopathy.

86
 PATIENT 22

A 46-year-old woman with right leg pain and intermittent

paresthesias

This patient developed right low back and leg pain and intermittent tingling in her right foot
2 years previously and underwent surgical laminectomy for treatment. Acute pain resolved and she has
noted more mild chronic pain in her right leg since.
Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (~V) (cm) (m/s)
R. sural-Iat mall I. Mid calf Ankle 2.70 3.60 10.5 14 51.9
R. sup peroneal I. Lat leg Ankle 2.35 3.05 11.5 12 51.1

Motor NCS

Relative
Recording Latency Amplitude Amplitude Distance Velocity
Nerve Sites Site (ms) (mV) (%) (cm) (m/s)
R. peroneal- I. Ankle EDB 3.65 5.1 100 8
EDB 2. Fib head EDB 9.45 4.5 88.3 29 50.0
3. Pop fossa EDB 11.10 4.4 85.6 8.5 51.5
L. tibial-AH I. Ankle EDB 3.10 10.5 100 8
2. Fib head EDB 10.15 9.4 90 36 51.1
R. tibial-AH I. Ankle AH 3.55 10.3 100 8
2. Pop fossa AH 10.10 8.9 86.3 35 53.4

F-Wave
Nerve Fmin (ms) Fmax (ms) Max - Min (ms)
R. tibial 43.90 45.30 1.40
L. tibial 44.65 45.90 1.25

H Reflex
Nerve H Latency (ms)
R. tibial-soleus Absent
L. tibial-soleus 29.25

87
 EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. gastroc med Incr 1+ 0 0 NI NI NI Full NI

R. bic fern SH NI 0 0 0 NI NI NI Full NI
R. tib ant NI 0 0 0 NI NI NI Full NI
R. glut med NI 0 0 0 NI NI NI Full NI
R. peron In NI 0 0 0 NI NI NI Full NI
R. S 1 paraspinals NI 0 0 0

L TIBIAL - Soleus

.~ .
.v .. - ..

Figure 1

88
 Figure 2

Questions:
I. What abnormality is shown in figure I?
2. What abnormality is shown in figure 2?
 Answers:
I. Absent right tibial H-reflex
2. L5-S I central and right-sided disc hermination

Discussion: The electrodiagnostic study is of such and could be seen in a tibial or sciatic
abnormal for the inability to obtain H-reflexes neuropathy. However, the radiological findings
from the tibial nerve recording over the soleus on show an L5-S I disc central and right-sided herni-
the right and for fibrillation potentials in the right ation; it is likely that the absent H-reflex is a con-
medial gastrocnemius. These findings suggest a sequence of an S I root lesion.
right S I or S2 radiculopathy but are not specific

Clinical Pearl
Nerve conduction studies are relatively insensitive to detecting radiculopathy, although
an H-reflex when available can be informative.

96
 PATIENT 23

A 57-year-old man with right leg weakness after repair of

abdominal aortic and internal iliac artery aneurysms

This 57-year-old man underwent surgical repair of an abdominal aortic aneurysm and a right
internal iliac artery aneurysm with placement of an aorto-bi-iliac graft. He awoke postoperatively with
pain, numbness, and weakness of his right leg and absence of pulses and Doppler flow signal in his
foot. He was brought back to the operating room and underwent thrombectomy of an occluded right
aorto-iliac graft. His electrodiagnostic study was performed 2 months later.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (J.lV) (em) (m/s)
R. sural-Iat mall 1. Mid calf Ankle Absent 14
R. sup peroneal 1. Lat leg Ankle Absent 12
R. saphenous 1. Med leg Foot Absent 10
L. sural-I at mall 1. Mid calf Ankle 3.00 3.75 16.0 14 46.7
L. sup peroneal 1. Lat leg Ankle 2.60 3.35 8.8 12 46.2
L. saphenous 1. Med leg Foot 2.40 2.95 4.0 10 41.7

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (em) (m/s)
R. peroneal-EDB I. Ankle EDB Absent 8
R. peroneal-tib ant I. Fib head Tib ant Absent
R. tibial-AH 1. Ankle AH 5.90 0.3 8
2. Pop fossa AH Absent
R. femoral-rect fern I. Groin Rect fern Absent
L. peroneal-EDB 1. Ankle EDB 4.20 7.9 8
2. Fib head EDB 11.40 7.0 32 44.4
3. Pop fossa EDB 14.00 6.6 12 46.2
L. tibial-AH I. Ankle AH 5.80 7.5 8
2. Pop fossa AH 13.15 5.9 39 53.1
L. femoral-rect fern I. Groin Rect fern 4.95 10.0

EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R. tib ant Inc 3+ 0 0 NI Nl NI Full Single MU
R. vast lat Inc 3+ 0 0 No activity
R. vast med Inc 3+ 0 0 No activity
R. psoas Inc 1+ 0 0 Nl NI NI Full NI

Continued

9.1
 EMG Summary Table-cont'd
SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. med gastroc Inc 2;t- 0 0 NI NI NI Full Mod dec

R. tib post Inc 2-3+ 0 0 NI NI NI Full Mod dec
R. glut med NI 0 0 0 NI Nl Nl Full Nl
R. add longus Inc 3--4+ 0 0 No activity
R. L3 paraspinal m NI 0 0 0
R. L4 paraspinal m Nl 0 0 0
R. L5 paraspinal m Nl 0 0 0

Questions:
I. What are the electrodiagnostic abnormalities? Do they favor root, plexus, or more peripheral
localization?
2. What is the electrodiagnostic localization?

9l
 Answers:
1. They favor a plexous localization
2. Lumbosacral plexopathy

Discussion: The electrodiagnostic abnormal- strongly suggests a localization distal to the dor-
ities are (1) inability to record the right sural, sal root ganglia (i.e., a lumbosacral plexopathy
superficial peroneal, and saphenous SNAPs, and rather than a lumbosacral polyradiculopathy).
(2) inability to record the right peroneal-EDB, This patient apparently suffered an ischemic lum-
peroneal- TA, and femoral CMAPs with a bosacral plexopathy related to his vascular dis-
severely reduced right tibial-AH CMAP. The ease and its treatment with a surgical procedure.
results ofEMG studies are abnormal for abundant Perioperative ischemic lumbosacral plexopathies
fibrillation potentials and reduction or absence of do occur uncommonly in vascular procedures of
recruitable motor units in muscles as tabulated the infrarenal aorta, femoral, and iliac arteries.
above. The absence of right sensory nerve action Lumbosacral plexopathy may be the presenting
potentials with normal responses on the left feature of an iliac artery aneurysm.

Clinical Pearls
1. Sensory nerve studies are generally valuable in distinguishing root from plexus dis-
ease; these may need to be performed bilaterally to be confident of their abnormality.
2. Disease of the infrarenal aorta, femoral, and iliac arteries, and its management,
may produce ischemic lumbosacral plexopathy.

REFERENCES
I. Dougherty, M.1., Calligaro, K.D., How to avoid and manage nerve injuries associated with aortic surgery: ischemic
neuropathy, traction injuries, and sexual derangements, Semin. Vase. Surg. 2001; 14(4):275-281.
2. Gloviczki, P, Cross, S.A., Stanson, A.W., Carmichael, S.w., Bower, T.C., Pairolero PC., Hallett, 1.W., Jr., Toomey, B.1.,
Cherry, K.1., Jr., Ischemic injury to the spinal cord or lumbosacral plexus after aorto-iliac reconstruction, Am. J. Surg.
1991; 162(2): 131-136.
3. Lefebvre, v., Leduc, 1.1., Choteau, P.H., Painless ischaemic lumbosacral plexopathy and aortic dissection, J. Neural.
Neurasurg. Psychiatry 1995; 58(5):641.
4. Luzzio, C.C., Waclawik, A.1., Gallagher, C.L., Knechtle, S.1., Iliac artery pseudoaneurysm following renal transplantation
presenting as lumbosacral plexopathy. Transplantation 1999; 67(7): 1077-1078.
 SECTION II. GENERALIZED NEUROPATHIES
I

The diversity of presentation of generalized peripheral neuropathies is impressive. This area of

clinical neuromuscular practice is one of the most subtle and fascinating. As is always the approach in
neurological diagnosis, one focuses on the localization before the differential diagnosis. For general-
ized neuropathies, we also take the approach of characterizing the neuropathy prior to considering spe-
cific causes.
The characterization of peripheral neuropathy by anatomic and physiological involvement is of
great value in determining the underlying cause. Neuropathies can be categorized by a combination of
anatomical distribution plus extent of physiological involvement. Anatomical distribution is described
by terms such as distal, proximal> distal, diffuse (meaning relatively equal proximal and distal
involvement), symmetric, and asymmetric. The physiological involvement is described using terms
such as sensory, sensory> motor, motor> sensory, and autonomic. Together, we have groupings of
specific anatomical and physiological patterns of neuropathy that help to narrow the potential causes
to manageable groups (Tables I to 6).

Table 1. Distal Symmetric Purely Sensory or Sensory> Motor Neuropathies

Drug and toxic neuropathies, including alcohol Toxic

Diabetic distal symmetric polyneuropathy Paraproteinemic neuropathies
Idiopathic Amyloidosis
Vitamin deficiency: B12' E, thiamine Confluent vasculitis

Table 2. Diffuse Motor> Sensory Neuropathies

Guillain-Barre syndrome Diphtheria

Chronic inflammatory demyelinating polyneuropathy (CIDP) Acute arsenic poisoning
Osteosclerotic myeloma Inherited neuropathies
Drugs: Amiodarone, gold, perhexilene Acute intermittent porphyria

Table 3. Diffuse Sensory Neuropathies (Neuronopathies)

Sjogren's syndrome
Anti-Hu paraneoplastic sensory neuronopathy

Table 4. Neuropathies with Prominent Autonomic Involvement

Diabetes
Guillain-Barre syndrome
Acquired and familial amyloidoses
Paraneoplastic autonomic neuropathy
Hereditary sensory and autonomic neuropathies

Table 5. Symmetric and Multifocal Sensory and Motor Neuropathies

Multifocal CIDP
Some inherited neuropathies

94
 Table 6. Asymmetric, Multifocal Sensory and Motor Neuropathies
Vasculitis Hereditary neuropathy with liability to pressure palsies (HNPP)
Multifocal cmp (MADSAM) Lyme disease
Neoplastic HIV-associated CMV multiple mononeuropathy
Diabetic radiculoplexus Sarcoidosis
neuropathy
Nondiabetic radiculoplexus Amyloidosis
neuropathy
Leprosy Acute brachial neuritis

Accordingly, the electrodiagnostic approach to generalized peripheral neuropathies has thefol-

lowing goals:
• Confirm or reject the presence of a peripheral neuropathy.
• Characterize involvement of sensory or motor nerves or both.
• Establish extent of symmetry.
• Establish length dependence or lack thereof.
Establish primary physiology as that of axonal degeneration or demyelination.

These goals are achieved by the following approach:

I. Bilateral sural and superficial sensory nerve studies
a. Abnormalities in the amplitudes of the sural and superficial sensory nerve action potentials
(SNAPs) are the most sensitive indicators in most axonal neuropathies.
b. Bilateral studies are required to establish symmetry or asymmetry of distal sensory involvement
and help to distinguish disorders in Table I from Table 6.
2. Bilateral peroneal-extensor digitorum brevis (EDB) and tibial-AH motor studies
a. Amplitudes of the compound muscle action potentials (CMAPs) establish whether involvement
includes motor axons and degree of symmetry as in I(b) above.
3. Unilateral or possibly bilateral median, ulnar, and radial sensory nerve studies
a. When foot SNAP amplitudes are reduced, determination of hand SNAP amplitudes character-
izes the extent of the neuropathy (i.e., limited to the feet or spread to the hands).
b. When foot SNAP amplitudes are not reduced but hand SNAP amplitudes are, this establishes a
non-length-dependent process that may be seen in demyelinating neuropathies and sensory neu-
ronopathies (Tables 2 and 3).
c. Peripheral neuropathies with superimposed focal neuropathies, such as median at the wrist and
ulnar at the elbow, may be demonstrated and further characterize the neuropathy. For example,
distal symmetric axonal neuropathies with diabetes often have superimposed bilateral median
and ulnar neuropathies.
d. Same reason as l(b).
4. Unilateral or bilateral median-abductor pollicis brevis (APB) and ulnar-abductor digiti minimi
(ADM) motor studies
a. When foot CMAP amplitudes are reduced, determination of hand CMAP amplitudes character-
izes the extent of the neuropathy (i. e., limited to the feet or spread to the hands), analogous to
point 3(a) above.
b. The median and ulnar forearm motor velocities are more reliable indicators of a primary
demyelinating neuropathy than the leg motor velocities and need to be studied, at least unilater-
ally, in all patients with a generalized peripheral neuropathy.
c. Same reason as 3(c) above.
d. Same reason as 3( d) above.
5. F-wave studies
a. F-wave studies are important when demyelinating neuropathies are a relevant consideration, par-
ticularly when acute.
6. Needle EMG studies
a. Needle EMG studies are valuable in establishing the length dependence of the process. Reduced
amplitudes of foot CMAPs should indicate EMG studies in foreleg muscles. Reduced ampli-
tudes of hand CMAPs should indicate EMG studies in hand and forearm muscles and, if the

95
 latter are abnormal, more proximal muscles. For example, finding fibrillation potentials in tib-
ialis anterior, gastrocnemius, and peroneus longus but not hamstrings or gluteus medius local-
izes the involvement to long motor axons and not roots or a focal cord anterior horn lesion.
b. Needle EMG studies reveal symmetry or asymmetry, multifocality vs. confluence.

REFERENCES
I. Amato, A.A., Approach to peripheral neuropathy, in Dumitru, D., Amato, A., Zwarts, M.l, Electrodiagnostic Medicine,
Hanley & Belfus, Philadelphia, PA, 2002.
2. Barohn, R.l, Approach to peripheral neuropathy and neuronopathy, Semin. Neurol. 1998; 18:7-18.
3., Donofrio, P.O., Albers, lW., AAEM minimonograph 34: po\yneuropathy--classification by nerve conduction studies and
electromyography, Muscle Nerve 1990; 13:889-903.
4. Dyck, P.J., Dyck, P.J., Grant, LA., Fea1ey, R.D., Ten steps in characterizing and diagnosing patients with peripheral
neuropathy, Neurology 1996; 47: 10--17 .

.'.

96
 PATIENT 24

A 73-year-old man with an IgG-K paraprotein and several years of

progressive numbness and weakness in the feet and hands

This 73-year-old man was referred for electrodiagnostic evaluation because of several years of
progressive numbness and weakness that started in his feet and later spread to the hands. Neurological
examination demonstrated symmetric paralysis of all toe and ankle movements and moderate weakness
and atrophy of intrinsic hand muscles, along with symmetric distal sensory loss in the hands and feet.
Consider the main possibilities for localization and how you would approach the electrodiagnos-
tic study.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (j.!V) (cm) (m/s)
R. median-dig II I. Wrist Dig II 2.50 3.55 5.3 13 52.0
R. ulnar-dig V I. Wrist DigV 2.55 3.45 3.5 11 43.1
R. radial-sn box 1. Forearm SnBox 2.30 3.05 6.5 10 43.5
R. lat AB cut I. Elbow Forearm Absent 12
R. sural-I at mall I. Mid-calf Ankle Absent 14
L. sural-I at mall I. Mid-calf Ankle Absent 14

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (j.!V) (cm) (m/s)
R. median-APB 1. Wrist APB 4.60 5.5 7
2. Elbow APB 10.00 4.9 25 46.3
3. Axilla APB 12.55 4.8 12 47.1
R. ulnar-ADM 1. Wrist ADM 3.20 3.0 7
2. B. elbow ADM 7.55 2.6 22 50.6
3. A. elbow ADM 10.65 2.5 15 48.4
R. peroneal-EDB I. Ankle EDB Absent 8
L. peroneal-EDB I. Ankle EDB Absent 8
L. peroneal-tib ant I. Fib head Tib ant 3.15 0.2 II
2. Pop fossa Tib ant 5.15 0.2 10 50
R. tibial-AH 1. Ankle AH Absent 8
L. tibial-AH 1. Ankle AH Absent 8

F-Wave
Nerve Fmin (ms) F max (ms) Max - Min (ms) %F
R. median 38.50 42.20 3.70 30
R. ulnar 36.85 38.65 1.80 80
R. peroneal Absent
R. tibial Absent
L. peroneal Absent
L. tibial Absent

9"ol
 EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. vast med Jnc 2+ 0 0 NI NI NI Full Nl

R. bic fern SH Inc 2+ 0 CRD Nl Nl Nl Full Nl
R. tib ant Inc 3+ 0 0 Nl Nl Nl Full Single MU
R. tib post Inc 3+ 0 0 Nl No activity
R. gastroc med Dec 0 0 0 Nl No activity
R. first dors int Inc 3+ 0 0 Large Long Nl Full Sev red
R. abd poll br Inc 3+ 0 0 Large Long Nl Full Sev red
R. biceps Nl 0 0 0 Nl Nl Nl Full Nl
R. pron teres NI 0 0 0 Nl Nl Nl Full Nl
R. ext dig comm Nl 0 0 0 Nl Nl Nl Full Nl

Questions:
I. Is the process symmetric or asymmetric? What additional electrodiagnostic studies would be valu-
..able in this regard?
2..Is the process length dependent?

98
 Answers:
1. Symmetric; left sensory studies and left leg needle EMG studies
2. Yes

Discussion: The salient electrodiagnostic Paraproteinemic neuropathies are associated

abnormalities can be grouped in a meaningful with the monoclonal production of a specific
way as follows: population of immunoglobulins or immunoglobu-
• Absent foot (sural) SNAPs and low- lin fragments. In some disorders, the monoclonal
amplitude hand SNAPs (median, ulnar, and protein is highly and specifically directed at nerve
radial on one side), suggesting length- antigens and disease is entirely limited to a neu-
dependent axonal degeneration of sensory ropathy. In other disorders, the neuropathy is one
axons in the feet and hands feature of a systemic malignancy of antibody-
• Absent foot (bilateral peroneal-EDB and tib- producing plasma cells. Serum immunofixation
ial-AH) CMAPs with moderately reduced (IF) is more sensitive than serum protein elec-
amplitude of the ulnar-ADM CMAP in the trophoresis (SPEP) and is the essential laboratory
hand, suggesting length-dependent axonal test for detection of most of these syndromes.
degeneration of motor axons in the hands and These disorders are summarized in the next table.
feet Monoclonal gammopathy of undetermined sig-
• EMG abnormalities with a distal to proximal nificance (MGUS) is the term applied to the pres-
gradient in the right leg (look at recruitment ence of a serum paraprotein without any other
patterns of foreleg muscles tibialis anterior, identified disease. An IgG or IgA MGUS is asso-
medial gastrocnemius, and tibialis posterior, ciated with neuropathies, although the causal
all similar to each other and in contrast to relationship is unclear. The same is true for
thigh muscles vastus medialis and biceps approximately 50% of IgM MGUS; however, in
femoris); similarly, a distal to proximal gra- the other 50% of MGUS patients, the antibody is
dient in the right arm likely pathogenic and results in the neuropathy
• Prolongation of the right median-APB mini- syndrome associated with anti-myelin-associated
mum F-wave latency in the setting of fibril- glycoprotein (MAG). MAG-associated peripheral
lation potentials in this muscle neuropathy appears to result from an IgM-K
These features establish the presence of a gen- antibody directed against the MAG or related
eralized peripheral neuropathy characterized by components (e.g., sulfatides or sulfoglucuronyl
length-dependent distal degeneration of sensory paragloboside [SGPGD of peripheral nerve. This
and motor axons and symmetry in the legs. distinctive syndrome looks clinically like a distal
Further study of left hand SNAPs and bilateral sensory> motor neuropathy, although a greater
superficial peroneal sensory and needle EMG of degree of ataxia is generally present. Electro-
left leg and arm muscles could provide even fur- diagnostic studies, however, demonstrate a unique
ther support of a symmetric process. In this case, picture of sensory and motor demyelination with
this severe axonal neuropathy, with fibrillation much greater distal than proximal involvement.
potentials present even in thigh muscles, was asso- Multiple myeloma (MM) is a plasma cell
ciated with an IgG-K paraprotein, although the malignancy, usually but not always producing
relationship is unclear. Other potential considera- IgG or IgA and usually but not always a K light
tions for this category of neuropathies are listed in chain. Typical presentation is in middle or late age
the table below. No other definite cause for this with fatigue, anemia, and hypercalcemia, with
particular patient's neuropathy was established bone pain and renal insufficiency being other
after sural nerve biopsy (showing axonal degener- prominent aspects. Neuropathy is present in about
ation), bone marrow biopsy, and skeletal survey. 10% of patients and may be the presenting fea-
ture. It is typically a distal axonal sensory and
motor neuropathy. The most common pathogene-
Distal Symmetric Purely Sensory
sis of neuropathy in patients with multiple
or Sensory> Motor Neuropathies
myeloma results from AL-type amyloidosis, pres-
Drug and toxic neuropathies, Toxic ent in at least 30 to 40% of MM neuropathies.
including alcohol Other causes may be related to metabolic and
Diabetic distal symmetric Paraproteinemic toxic consequences of the disease or its treatment.
polyneuropathy neuropathies Osteosclerotic myeloma is also a plasma cell
Idiopathic Amyloidosis malignancy and may overlap with multiple
Vitamin deficiency: B12' E, Confluent myeloma; it is usually but not always due to an
thiamine vasculitis IgG or IgA and is almost always a ')..,light chain.
Although much less common than multiple

99
myeloma, the association with neuropathy is proteins (termed amyloid fibrils) in organs and tis-
much higher (50%) and accordingly neuropathy sues. In one disorder (AL-amyloidosis), amyloid
is a much more common presenting feature. The fibrils consist of fragments of immunoglobulin
POEMS syndrome (polyneuropathy, organomegaly, light chains. For all the other amyloidoses, the
endocrinopathy, M-protein, and skin changes) amyloid fibrils are not immunoglobulins. Some
may result. The associated neuropathy is said to cases of multiple myeloma may be associated with
be similar to that of chronic inflammatory deposition of amyloid fibrils derived from
demyelinating polyneuropathy (CIDP) with prox- immunoglobulins but are not classified as princi-
imal and distal weakness and generalized hypo- pal amyloidoses; this distinction appears to be
or areflexia. Electrodiagnostic studies, however, solely semantic. The neuropathy of AL-type amy-
are those of axonal sensory and motor loss with loidosis is more distinctive than the generic distal
moderate or marked slowing of nerve conduction axonal neuropathy seen in multiple myeloma.
velocities, but without the definitive findings of Early painful sensory and autonomic involvement
focal conduction block or temporal dispersion with concomitant carpal tunnel syndromes is char-
more commonly diagnostic of CIDP. Cerebro- acteristic. Familial amyloidotic polyneuropathy
spinal fluid (CSF) protein is often quite elevated (FAP), when due to mutations in amyloidogenic
(> 100 mg/dl). A skeletal survey is more sensitive transthyretin (ATTR), may be diagnosed through
than radioisotope bone scans for detection of sequencing of the transthyretin (TTR) gene.
sclerotic lesions. Cryoglobulinemias are a heterogeneous group
Waldenstrom s macroglobulinemia is a plasma of disorders. Type I cryoglobulins are associated
cell disorder limited by definition to IgM produc- with the plasma cell dyscrasias or Iymphoprolif-
tion; it produces fatigue, anemia, epistaxis, and a erative disorders. These are the disorders just dis-
hyperviscosity syndrome. The pathogenesis of an cussed above. Type II cryoglobulins consist of a
associated neuropathy is heterogeneous. For most monoclonal immunoglobulin, typically an IgM-K
patients, a distal axonal sensory and motor neu- with anti-IgG activity (rheumatoid factor), and
ropathy similar to that seen in multiple myeloma polyclonal IgG. The major cause of type II cryo-
is present, sometimes secondary to AL-type amy- globulinemia is chronic hepatitis C infection
loidosis. Other patients with an IgM-K protein which produces a confluent distal mononeuritis
with anti-MAG activity and the characteristic multiplex, resulting in a distal axonal sensory
features of a MAG-associated neuropathy (see and motor neuropathy. Type III cryoglobulins
below) have also been described. are a mixture of only polyclonal immunoglobu-
Amyloidosis is a group of disorders with a com- lins and are more typically present in rheumatoid
mon endpoint: deposition of insoluble fibrillar arthritis and connective tissue diseases.

100'
 Paraproteinemic Neuropathies
Disorder Paraprotein Systemic Features Neuropathy Of Diagnostic Value
MGUS IgM, IgG, or IgA, usually lC None Distal axonal sensory and
motor
Anti-MAG/SGPG IgM-lC None Sensory> motor with ataxia Distinctive NCS findings
Multiple myeloma IgG, IgA, or IgM (usually Bone pain, anemia, Distal axonal sensory and motor Bone marrow bx Skeletal survey
IgG or IgA) renal
Osteosclerotic myeloma IgG or IgA, almost always A. POEMS Distal axonal sensory and motor Skeletal survey
with demyelinating-range
nerve conduction velocities
Walden strom 's IgM Fatigue, Distal axonal sensory and motor
macroglobulinemia hyperviscosity
Amyloidosis AL-light chains A. or lC(3: l) Heart and kidney Autonomic + axonal sensory and AL-fat pad bx + serum/urine
AA-not paraprotein motor neuropathy; + carpal AA-fat pad bx + stain AA
Familial ATTR tunnel syndrome protein
ATTR-fat pad bx + TTR
mutation
Lymphoma Any Systemic, Distal axonal sensory and motor; Chest! Abd CT
hematologic mononeuritis multiplex from
nerve lllvaSlOn
Cryoglobulinemia
Hepatitis C IgM-lC Complement- Confluent mononeuritis multiplex HepCAb, RF
mediated immune
complex deposition
vasculitis
 Clinical Pearls
1. Establishing length dependence and symmetry are important principles in the
electrodiagnostic assessment of peripheral neuropathies.
2. The relationship between paraproteins in MGUS and neuropathy is uncertain.

REFERENCES
I. Falk, R.H., Comenzo, R.L., Skinner, M., The systemic amyloidoses, N. Engl J Med. 1997; 337:898-909.
2. Gorson, K.C., Ropper, A.H., Axonal neuropathy associated with monoclonal gammopathy of undetermined significance, J
Neural. Neurosurg. Psychiatry 1997; 63:163-168.
3. Notermans, N.C., Wokke, lH.J., Lokhorst, H.M., Franssen, H., van der Graaf, Y, Jennekens, F.G.I., Polyneuropathy
associated with monoclonal gammopathy of undetermined significance: a prospective study of the prognostic value of
clinical and laboratory abnormalities, Brain 1994; 117:1385-1393.
4. Notermans, N.C., Wokke, J.H.J., van den Berg, L.H. et aI., Chronic idiopathic axonal polyneuropathy:comparison of
patients with and without monoclonal gammopathy, Brain 1996; 119:421-427.
5. Ropper, A.H., Gorson, K.C., Neuropathies associated with paraproteinemia, N. Engl. J Med. 1998; 338:1601-1607.
6. Simovic, D., Gorson, K.C., Ropper, A.H., Comparison ofIgM-MGUS and IgG-MGUS polyneuropathy, Acta Neurol.
Scand. 1998; 97: 194-200.
7. Van den Berg, L.H., Hays, A.P., Nobile-Orazio, E. et al., Anti-MAG and anti-SGPG antibodies in neuropathy, Muscle
Nerve 1996; 19:63-143 .

. ..

102
 PATIENT 25

A 43-year-old man with diabetes and multiple neurologic

symptoms

This 43-year-old businessman with a history of diabetes for 7 years presented in August 2000 with
a history summarized as follows:
• Summer 1999-right lower abdominal pain and a bulge in the right lower abdomen
• Late 1999-multiple postprandial gastrointestinal complaints (fullness, belching)
• January 200O--constant tingling paresthesias in left thigh and deep aching pain
• March to May 2000-progressive weakness of left leg
• June 2000-HbA1C 12.1%; initiation of insulin therapy
• July 2000-Right thigh weakness and pain; left leg strength starting to improve
• February to August 2000-40-lb weight loss
• Exam in August 2000:

Strength
Legs Psoas Quads Adductor TA Hams Toe Flex Toe Ext Glut Max Glu Med
Right 2 2 2 5- 4- 4 4 5 5
Left 3 4 2 4 4- 4 4 5 5

• Reflexes-Absent bilateral knees and ankles.

• Sensory-Absent vibration in toes bilaterally; light touch abnormal in right saphenous n. territory;
decreased pin prick up to the ankles bilaterally and in the right saphenous n. territory.
• Gait-Walk with a walker only.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (JlV) (cm) (rn/s)
R. median-dig II 1. Wrist Dig II 3.4 4.5 7.4 13 38
R. ulnar-dig V 1. Wrist DigV Absent II
R. radial-sn box 1. Forearm Sn box 2.5 3.3 7.8 10 43.5
R. sural-Iat mall I. Mid Calf Ankle Absent 14
L. sural-Iat mall 1. Mid Calf Ankle Absent 14

Motor NCS
Recording Latency Amplitude Distance Velocity
Nerve Sites Site (ms) (mV) (cm) (rn/s)
R. median-APB 1. Wrist APB 4.5 7.8 7
2. Elbow APB 10.7 6.4 25 40
R. ulnar-ADM 1. Wrist ADM 3.9 7.0 7
2. B. elbow ADM 8.1 4.8 18 43
3. A. elbow ADM 12.5 6.5 18 41
R. peroneal-EDB 1. Ankle EDB Absent
L. peroneal-EDB 1. Ankle EDB 6.1 0.3 8
2. Bel fib hd EDB 18.0 0.2 33.5 28
Continued

103
 Motor NCS--cont'd

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)

3. Abv fib hd EDB 21.6 0.2 11 31

R. peroneal-tib ant 1. Fib head Tib ant 4.2 4.0 8
2. Pop fossa Tib ant 6.4 3.6 8 36
L peroneal-tib ant 1. Fib head Tib ant 2.6 2.5 8
2. Pop fossa Tib ant 6.9 1.8 11 26
R. tibial-AH 1. Ankle AH Absent
L. tibial-AH 1. Ankle AH Absent

EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R.-vast med Inc 1+ 0 0 Nl Nl Nl Full Sev dec
R. add long Inc 1+ 0 0 Nl Nl Nl Full Sev dec
L.vast lat Inc 1+ 0 0 Inc Inc Nl Full Mod dec
L. tib ant Inc 1+ 0 0 Nl Nl Nl Full Mod dec
L. gastroc med - . Inc 1+ 0 0 Inc Inc Nl Full Normal
L. L3 paraspinals Nl 0 0 0
L. IA paraspinals Nl 0 0 0

Questions:
1. Does the electrodiagnostic study support the clinical characterization of this syndrome?
2. How would you characterize this syndrome, assuming it is a neuropathy?

104
 Answers:
1. Yes
2. Asymmetric, multi focal sensory and motor neuropathies

Discussion: This syndrome is clinically c1e weakness. Thoracoabdominal neuropathy may

classified as a neuropathy with multi focal asym- occur bilaterally.
metric sensory and motor involvement. The elec- Diabetic proximal neuropathy (diabetic radicu-
trodiagnostic studies support this view and loplexus neuropathy) is an even more curious
further characterize the physiology as that of entity. Subacute proximal thigh pain, paresthe-
axonal degeneration. These findings put this dis- sias, and weakness (unilateral or bilateral) may
order in the category represented by Table 6 in the progress for several months before stabilizing.
introduction to this section. Additional notewor- Pain is often severe and the major component of
thy clinical characteristics are that it is painful this syndrome, leading to poor appetite and
and has a subacute progression, and the multifo- depression. Involvement can be bilateral from the
cality is present in time as well, with worsening start or can be on one side for months before the
involvement in one leg while the other is in fact other side becomes involved. The initial side may
improving. be improving when the other side is evolving.
Several specific and separate disorders associ- Although predominant involvement in the quadri-
ated with diabetes are present in this patient: ceps suggesting a femoral neuropathy is the rule,
• Proximal diabetic neuropathy (diabetic radicu- involvement of a wider territory is universal,
loplexus neuropathy, diabetic amyotrophy) localizing this disorder to the lumbosacral plexus
• Thoracoabominal neuropathy more commonly. Weakness of adductor muscles
• Diabetic gastroparesis and medial thigh numbness (obturator nerve) and
• Distal symmetric polyneuropathy sometimes portions of the lateral trunk of the sci-
Diabetic neuropathies other than the distal atic nerve (common peroneal nerve, foot drop)
symmetric polyneuropathy (DSP) are of several are common and suggest specific localization to
types and may occur independently of DSP. the lumbar plexus or lumbosacral trunk, with
Autonomic involvement is common in diabetes, involvement of the sacral plexus less common.
and a separate form of neuropathy, termed auto- The cause is unknown, but it is often associated
nomic neuropathy, may be present and exist inde~ with weight loss. It may be a result of a vasculitis
pendently of, or together with, distal symmetric of small or microscopic vessels. The syndrome
neuropathy. Diabetic gastroparesis is a common has been attributed to both poor diabetic control
manifestation of diabetic autonomic neuropathy. as well as institution of good diabetic control (i.e.,
Postural lightheadedness and erectile dysfunction initial use of insulin); as the latter usually occurs
in men are other less common features. because of the former, it may be more reasonable
Focal cranial neuropathies with diabetes are to assume that poor diabetic control is the cause.
well described and relatively common for the The major emphasis oftreatrnent is on weight sta-
third and sixth cranial nerves. These palsies are bilization and pain control. The syndrome is often
typically acute and painful but have excellent protracted but has an excellent prognosis for good
prognosis for full recovery. recovery of motor function, even in wheelchair-
Other multi focal diabetic neuropathies include bound patients, over 6 to 24 months after symp-
thoracoabdominal neuropathy and diabetic proxi- toms stabilize.
mal neuropathy. Diabetic thoracoabdominal For this patient, treatment was directed at
neuropathy is a curious syndrome of acute or sub- emphasizing weight gain, pain control, and tighter
acute pain and paresthesias, typically in the dis- diabetic control. Over the ensuing 9 months, the
tribution of one or several adjacent unilateral pain resolved and there was continued gradual
thoracic dermatomes. As the pain resolves, a improvement in strength. By April 2002, the
bulging of the abdominal musculature may patient had regained all weight and was walking
appear and is likely due to focal abdominal mus- without a walker. His strength was as follows:

Strength

Toe Toe Glut Glu

Legs Psoas Quads Adductor TA Hams Flex Ext Max Med
Right 5 4 5 5- 5 4 4 5 5
Left 5 4 5 5- 5 4 4 5 5

105
 Clinical Pearls
I. Diabetic neuropathies comprise a number of distinct syndromes.
2. Diabetic proximal neuropathy generally has an excellent long-term prognosis for
recovery.

REFERENCES
I. Asbury, AX., Proximal diabetic neuropathy, Ann. Neurol. 1977; 2: 179-180 .
.2. Barohn, R.1., Sahenk, Z., Wannolts, lR., Mendell, lR., The Bruns-Garland syndrome (diabetic amyotrophy): revisited 100
years later, Arch. Neurol. 1991; 48:1130-1135.
3. Dyck, P.1., Windebank, A.1., Diabetic and nondiabetic lumbosacral radiculoplexus neuropathies: new insights into
pathophysiology and treatment, Muscle Nerve 2002; 25:477-491.
4. Dyck, P.l, Norell, lE., Dyck, P.l, Microvasculitis and ischemia in diabetic lumbosacral radiculoplexus neuropathy,
Neurology 1999; 53:2113-2121.

'.

.\ .

" "

106
 PATIENT 26

A 73-year-old man with diabetes and several months of chest pain

This 73-year-old man with diabetes for several years developed right-sided, lower chest and upper
abdominal continuous burning pain and tingling paresthesias 9 months prior to this study. He n.otea
that a light touch, such as his clothes or a bed sheet, over the area provoked pain.
Electrodiagnostic study:

Sensory NCS
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (~V) (cm) (mls)
R. median-dig II 1. Wrist Dig II 3.85 4.75 11.5 13 33.8
L. median-dig II 1. Wrist Dig II 3.10 3.95 16.3 13 41.9
R. ulnar-dig V 1. Wrist DigV 2.60 3.45 9.9 11 42.3
L. ulnar-dig V 1. Wrist DigV 2.65 3.50 13.4 II 41.5
R. radial-sn box 1. Forearm Sn box 1.70 2.45 16.9 10 58.8
R. sural-Iat mall 1. Mid-calf Ankle 3.65 4.40 4.9 14 38.4

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (mls)
R. median-APB 1. Wrist APB 5.20 6.5 7
2. Elbow APB 9.80 5.4 22 47.8
L. median-APB I. Wrist APB 4.80 4.8 7
2. Elbow APB 9.45 4.5 21 45.2
R. ulnar-ADM 1. Wrist ADM 3.65 8.2 7
2. B. elbow ADM 7.30 7.1 19 52.1
3. A. elbow ADM 10.05 5.6 12 43.6
L. ulnar-ADM 1. Wrist ADM 3.55 8.3 7
2. B. elbow ADM 7.45 7.7 19.5 50.0
3. A. elbow ADM 10.50 6.8 12.5 41.0
R. ulnar-FDI 1. Wrist FDI 5.20 9.5
2. B. elbow FDI 9.15 8.5 19 48.1
3. A. elbow FDI 11.65 7.7 12 48.0
R. peroneal-EDB 1. Ankle EDB 5.00 5.4 8
2. Fib head EDB 12.90 4.6 29 36.7
3. Pop fossa EDB 15.25 4.6 9 38.3

107
 EMG Summary Table
SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. thoracic PSP mid NI 2+. 0 o

R.Jhoracic PSP lower NI 2+ 0 o
R. thoracic PSP upper NI o 0 o
L. thoracic PSP mid NI 1+ 0 o

Question: What is the clinical diagnosis?

~. J ' •

108
 Answer: Diabetic thoracoabdominal neuropathy

Discussion: The nerve conduction studies This patient has diabetic thoracoabdominal neu-
show mild abnormalities in the reduction of the ropathy. This syndrome is typicalIy that of suba-
right median distal sensory velocity (33.8 m/s) cute progressive pain and paresthesias in the
and prolongation of the right median-APB distribution of several contiguous thoracic der-
distal motor latency (5.2 ms), both suggestive of matomes on one side or sometimes bilaterally.
a right carpal tunnel syndrome. In addition, there Sometimes only the sensory territories of the ven-
is mild or borderline slowing of the ulnar-ADM tral ramus are involved. The pain may last for
across-elbow motor velocities (43.6 and 41 m/s) weeks to months before it plateaus and then typi-
suggesting subclinical ulnar neuropathies at the calIy resolves spontaneously. Many patients with
elbow. These fmdings are commonly seen in patients proximal diabetic neuropathy (diabetic amyotro-
with diabetes and are often asymptomatic. There phy) have a preceding history of diabetic thora-
is no evidence of a distal symmetric polyneuropa- coabdominal neuropathy. Sometimes there is a
thy in this patient (normal right sural SNAP and unilateral bulge in the abdominal wall from weak-
peroneal-EDB CMAP amplitudes). The study is ness of abdominal musculature. The authors have
most remarkable for the presence of fibrilIation seen several patients thought to have an abdominal
potentials in bilateral mid-thoracic and right mass. One patient underwent ultrasound of this
lower thoracic paraspinal muscles. The ideal apparent mass; gallstones were seen in the gall
study would have included further demonstration bladder, and he underwent cholecystectomy (with-
of the limits of these abnormalities (such as nee- out benefit). It is noteworthy that the anatomic dis-
dle EMG of cervical and lumbosacral paraspinal tribution of this syndrome is typicalIy wide and not
muscles and several arm and leg muscles). due to a disorder of a single thoracic root.

Clinical Pearl
Diabetic thoracoabdominal neuropathy is a poorly understood disorder that is not con-
fined to a single root or spinal nerve territory and may be mistaken for an internal tho-
racic or abdominal disorder.

REFERENCES
I. Brown, M.J., Asbury, A.K., Diabetic neuropathy, Ann. Neural. 1984; 15:2-12.
2. Simmons, Z., Feldman, E.L., Update on diabetic neuropathy, Curr. Opin. Neural. 2002; 15:595-603.
3. Sun, S.F., Streib, E.W., Diabetic thoracoabdominal neuropathy: clinical and e1ectrodiagnostic features, Ann. Neural. 1981;
9:75-79.

109
 PATIENT 27
A 55-year-old woman with 6 months of progressive numbness in
her feet

A 55-year-old woman complains that six months ago she began to experience a constant mild
numbness and tingling paresthesias in the toes of her left foot with a gradual progression since that
time. Several months later, she is experiencing similar numbness in the toes of her right foot with grad-
ual progression since. Over the last month, some mild numbness and tingling in her left thumb has also
be«n noted .
. , Physical Examination: Normal strength; light touch, pinprick, and vibration sense in toes, left
more than right; normal ankle reflexes.
Stop and Consider: Using the approach of characterizing the neuropathy, characterize this
patient's neuropathy clinically.
Electrodiagnostic Study: Relevant electrodiagnostic studies follow (all motor studies were
normal).

Sensory

Recording Amplitude
Nerve Sites Site Onset (ms) Peak (ms) (~V)

R. median-dig I 1. Wrist Dig I 2.2 2.7 20.1

L. median-dig I 1. Wrist Dig I 2.1 2.5 9.7
R. sup peroneal 1. Lat leg Ankle Absent
L. sup peroneal I. Lat leg Ankle 3.0 3.3 5.7
R. sural-I at mall I. Mid-calf Ankle 3.2 3.5 3.7
L. sural-lat mall I. Mid-calf Ankle 3.1 3.4 2.6

Questions:
1. Does the electrodiagnostic study support your characterization of this patient's neuropathy?
2. Why is this not a distal symmetric axonal sensory neuropathy?

110
 Answers:
1. Yes
2. Asymmetric involvement of left thumb and asymmetric onset in left foot

Discussion: The clinical features and the elec- This patient's laboratory studies showed an
trodiagnostic study both suggest characterization as IgM-K monoclonal protein with quantitative IgM
a multi focal asymmetric sensory neuropathy. A dis- of 2190 mg/dl (normal < 230 mgldl), a negative
tal asymmetric neuropathy could also be consid- skeletal survey, and a bone marrow biopsy with
ered, although the lateral thumb numbness makes 30% cellularity with small lymphoid cells, Iym-
the term distal less preferable compared to multifo- phoplasmacytic forms, and plasma cells. Flow
eal. The reduction in the left median-D I and right cytometry confirmed a B-cell Iymphoprolifera-
superficial peroneal SNAP amplitudes compared tive disorder with pathological diagnosis of Iyrri~
to the other sides are important clues to avoiding a phoplasmacytic lymphoma. As nerve biopsy was
mistaken label as a symmetric axonal sensory neu- not indicated, it is difficult to determine the
ropathy. A brief differential diagnosis of multi focal pathophysiology in this case, although the main
asymmetric neuropathies should include vasculitis, considerations would be amyloid deposition or
neoplastic, Lyme disease, and sarcoidosis. lymphomatous infiltration.

Clinical Pearl
The symmetry and possible muItifocality of a distal axonal neuropathy should be
closely examined through the study of bilateral sural and superficial peroneal sensory
studies at a minimum. Asymmetries or muItifocality should prompt evaluation for
disorders including vasculitis, neoplastic infiltration of nerves, Lyme disease, and
sarcoidosis.

REFERENCES .. ,
I. Kraus, M.D., Lymphoplasmacytic lymphomaiWaldenstrom macroglobulinemia: one disease or three?, Am, J Clin. Path9l. ,
2001; 116(6):799-801. '
2. Pangalis, G.A., Angelopoulou, M.K., Vassilakopoulos, T.P., Siakantaris, M.P., Kittas, C., B-chronic lymphocytic leukemia,
small lymphocytic lymphoma, and lymphoplasmacytic lymphoma, including Waldenstrom's macroglobulinemia: a clinical,
morphologic, and biologic spectrum of similar disorders, Semin. Hematol. 1999; 36(2): 104-114.

III
 PATIENT 28
A 71-year-old woman with several years of progressive numbness
in her hands and feet

A 71-year-old woman developed numbness in her left hand. Nine months later she developed
numbness in her left foot, followed a month later with right foot numbness. Over the ensuing 2 years,
numbness came to involve the right hand and increased in intensity in the other limbs, and burning
dysesthesias in her feet became prominent. She then developed an anterior uveitis, and workup includ-
ing chest CT demonstrated diffuse reticular nodular disease throughout both lungs and scattered medi-
astinal, pretracheal, and aortopulmonic window lymph nodes.
Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (JlV) (em) (m/s)

L. median-dig II 1. Wrist Dig II Absent 13

L. ulnar-dig V 1. Wrist DigV 3.05 4.55 15.5 11 36.1
L. radial-sn box 1. Forearm Sn box 1.60 2.50 18.5 10 62.5
L. sural-Iat mall 1. Mid-calf Ankle Absent 14
L. sup peroneal 1. Lat leg Ankle Absent 12
R. sural-l at mall 1. Mid-calf Ankle Absent 14
R. sup peroneal 1. Lat leg Ankle Absent 12

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (em) (m/s)

L. median-APB 1. Wrist APB 4.40 9.0 7

2. Elbow APB 8.65 8.7 21 49.4
L. ulnar-ADM 1. Wrist ADM 3.35 7.2 7
2. B. elbow ADM 7.65 7.0 20.5 47.7
3. A. elbow ADM 10.20 6.7 13 51.0
L. peroneal-EDB 1. Ankle EDB Absent 8
2. Fib head EDB Absent
L. tibial-AH 1. Ankle AH Absent 8
2. Pop fossa AH Absent

EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

L. tib ant Inc 1+ 0 0 NI NI NI Full NI

L. gastroc med NI 0 0 0 NI NI NI Full NI
R. tib ant NI 0 0 0 NI NI NI Full NI
R. gastroc med NI 0 0 0 NI NI NI Full NI
L. pronator teres NI 0 0 0 NI NI NI Full NI
L. abd pollicis brevis NI 0 0 0 NI NI NI Full NI

lJ:2
 Questions:
1. What is the possible relationship of the eye and lung disease to the neurological syndrome?
2. How would you proceed with management of this patient?

113
 Answers:
1. Suggestive of sarcoidosis
2. Nerve and muscle biopsy

Discussion: The electrodiagnostic study de- The history of uveitis and pulmonary and medi-
monstrates a polyneuropathy with symmetric dis- astinal lymphadenopathy suggest sarcoidosis, and
tal degeneration of sensory and motor axons in this is a potential cause of a neuropathy of this type.
the feet and possibly multi focal involvement Sarcoid neuropathy requires long-term treatment
given the absence of the left median SNAP with with steroids with often less than satisfactory out-
normal amplitudes for the left ulnar and radial comes. Because of this, we were not comfortable
SNAPs. The left ulnar distal sensory segment embarking on therapy without a tissue diagnosis,
does have reduced velocity, also possibly another so the patient underwent superficial peroneal nerve
indication of multi focal involvement. Although and peroneus brevis muscle biopsy. Muscle biopsy
the median SNAP may be absent because of a is done because of the possibility of detecting gran-
carpal tunnel syndrome, the normal median-APB ulomas in muscle tissue in patients with sarcoido-
distal motor latency argues somewhat against sis, regardless of the presence or absence of a
this, as a carpal tunnel syndrome severe enough myopathy. Nerve biopsy demonstrated granulomas.
to result in an absent median SNAP will usually Peripheral neuropathy from sarcoidosis is rare.
have a prolonged distal motor latency. Accor- Non-caseating granulomas in nerve are the patho-
dingly, this is probably not best classified as a dis- logical hallmark. In a recent series of II patients
tal symmetric neuropathy but rather as an with biopsy-proven sarcoid neuropathy,4 the neu-
asymmetric sensory and motor neuropathy, with ropathy was focal or multifocal in 6 patients. Only
differential diagnosis that of Table 6 in the intro- 2 patients had elevated serum angiotensin convert-
duction of Section II. This electrodiagnostic inter- ing enzyme, reflecting the limited value of this test
pretation would by highly supported by the in this diagnosis. All patients improved somewhat
clinical history of asymmetric onset and evolution with steroid treatment, but 2 developed severe
of her symptoms. complications of steroids, including 1 death.

Clinical Pearl
As noted in case 27, multifocality (in this case, preferential involvement of one nerve
over another in the same anatomic region) should prompt consideration for some less
common causes of peripheral neuropathy.

REFERENCES
I. Gainsborough, N., Hall, S.M., Hughes, R.A., Leibowitz, S., Sarcoid neuropathy,.!. Neural. 1991; 238(3):177-180.
2. Heck, A. w., Phillips, L.H., Sarcoidosis and the nervous system, Neural. Clin. 1989; 7(3):641-654.
3. Nemni, R., Galassi, G., Cohen, M., Hays, A.P., Gould, R., Singh, N., Bressman, S., Gamboa, E.T., Symmetric sarcoid
polyneuropathy: analysis of a sural nerve biopsy, Neurology 1981; 31(10): 1217-1223.
4. Said, G., Lacroix, C., Plante-Bordeneuve, v., Le Page, L., Pico, E, Presles, 0., Senant, 1., Remy, P., Rondepierre, P.,
Mallecourt, 1., Nerve granulomas and vasculitis in sarcoid peripheral neuropathy: a clinicopathological study of II
patients, Brain 2002; 125(pt. 2):264--275.

114
 PATIENT 29

A 38-year-old man with numbness and weakness in the hands and

feet and a skin rash

A 38-year-old man developed progressive numbness and weakness in all four limbs over a 3-year
period. He was an immigrant to Boston from Cape Verde and had not left the United States for
10 years. He was treated by a physician with monthly doses of Chinese herbal medicine, which was
activated by boiling it in water. The patient spilled the boiling water on his right arm and was hospi-
talized with a third-degree burn of his right arm, requiring a skin graft from his thigh. During hospi-
talization, a rash was noticed as well as hand weakness, and both dermatology and neurology
consultants were asked to see him. The figures show his hand deformities and one of his skin lesions.

B
Figure 1

Figure 2

115
 Figure 3

Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (~V) (em) (m/s)
L. median-dig II 1. Wrist Dig II Absent 13
L. ulnar-dig V 1. Wrist DigV Absent 11
L. radial-sn box 1. Forearm Sn box Absent 10
R. sural-Iat mall 1. Mid-calf Ankle Absent 14
L. sup peroneal 1. Lat leg Ankle Absent 12
L. sural-Iat mall 1. Mid-calf Ankle Absent 14

Motor NCS
Recording Latency Amplitude Distance Velocity
Nerve Sites Site (ms) (mV) (em) (m/s)
L. median-APB 1. Wrist APB Absent 7
L. ulnar-ADM 1. Wrist ADM Absent 7
R. peroneal-EDB 1. Ankle EDB Absent 8
L. peroneal-EDB 1. Ankle EDB Absent 8
R. tibial-AH 1. Ankle AH Absent 8
L. tibial-AH 1. Ankle AH 4.45 3.7 8
2. Pop fossa AH 12.90 2.8 40 47.3
R. peroneal-tib ant 1. Fib head Tib ant 4.15 1.3
2. Pop fossa Tib ant 7.00 0.6
L. peroneal-tib ant 1. Fib head Tib ant 7.05 0.3 8
2. Pop fossa Tib ant 9.20 0.1 10 46.5

U6
 Questions:
I. The right hand could not be studied because of bandaging from his burn. What potential value
might there have been to studying it?
2. What is the most likely diagnosis?

111
 Answers:
I. Further establishment of a multifocal, asymmetric process
2. Lepromatous neuropathy

Discussion: The patient's left hand (see surrounded by Th2 cells. Of note, bacilli cannot
figure 1) is notable for marked atrophy of the dor- be demonstrated. The more superficial nerves in
sal and palmar interossei muscles. The electrodi- the vicinity of the skin lesions may also be
agnostic studies show absent sensory nerve action affected. In addition, there is a predilection for
potentials and absent or variously reduced CMAP involvement of specific nerve trunks. Typical
amplitudes, suggesting a peripheral neuropathy. sites include the ulnar nerve at the medial epi-
This peripheral neuropathy is characterized by condyle, the median nerve at the distal forearm,
multifocality and asymmetry. For asymmetry, note the peroneal nerve at the fibular head, the sural
the absent right tibial-AH response with normal nerve, the greater auricular nerve, and the super-
amplitude for the left tibial-AH CMAP and the ficial radial nerve at the wrist. These nerves can
much greater reduction in amplitude of the left become encased within granulomas, causing
peroneal-TA compared to the right peroneal-TA them to become thickened and easily palpable.
CMAPs. For multifocality, note the absence of the The most common neurological manifestation of
left peroneal-EDB and severe reduction ofthe left tuberculoid leprosy is mononeuropathy or
peroneal-TA CMAPs with normal amplitude of mononeuropathy multiplex.
the left tibial-AH response, suggesting a peroneal In lepromatous leprosy, cell-mediated immu-
neuropathy with relatively spared tibial nerve nity is significantly impaired, resulting in an
function. Further studies of the right hand might extensive infiltration of bacilli. Histologically, the
possibly have provided further evidence of asym- lesions in lepromatous leprosy demonstrate large
metry and multifocality. Accordingly, this neu- number of infiltrating bacilli, Th2 lymphocytes,
ropathy is best characterized as an asymmetric and organism-laden, foamy macrophages with
multifocal motor and sensory axonal neuropathy minimal granulomatous infiltration. Clinical
with causes listed in Table 6 in the introduction to manifestations tend to be more severe in the lep-
Section II. Of these causes, the one most associ- romatous subtype, but as in the tuberculoid form
ated with a large erythematous skin lesion with cooler regions of the body are more susceptible.
patchy hypopigmentation, as shown in figure 2, is The organisms multiply virtually unchecked and
leprosy. Skin biopsy indeed showed acid-fast hematogenously disseminate, producing conflu-
bacilli in this patient with lepromatous neuropa- ent and symmetrical areas of rash, anesthesia, and
thy. Figure 3 shows an additional finding charac- anhidrosis. Typically, a slowly progressive sym-
teristic of this disease-nerve enlargement (in this metric sensorimotor polyneuropathy develops
case, the greater auricular nerve). over time. In early phases of the disease, the
Although uncommon in developed countries, superficial cutaneous nerves of the pinnae and
leprosy is estimated to be the most common cause distal extremities are affected. The continued
of a peripheral neuropathy worldwide. Leprosy is multiplication and infiltration of the organism
caused by the acid-fast bacillus Mycobacterium into the epi-, peri-, and endoneurium results in
leprae. The bacteria reproduce maximally at tem- sensory loss of the ears, dorsum of the feet and
peratures of 27 to 30" which accounts for its hands, dorsal medial aspect of the forearms, and
predilection for cooler regions of the body (e.g., anterior-lateral aspects of the lower legs.
pinnae of the ears, bridge of the nose, distal Although superficially similar to the stocking-
extremities). The clinical and pathological spec- glove pattern of involvement of most other types
trum of leprosy is dependent on the host's of distal symmetric axonal polyneuropathies
immune response to M. leprae and reflects the (e.g., idiopathic, toxic, diabetic) that are length
relative balance between Thl (helper) and Th2 dependent, the pattern of involvement in lepro-
(suppressor) T cells. Tuberculoid leprosy and lep- matous leprosy is temperature dependent and
romatous leprosy represent the two extremes of distinct from other forms of neuropathy. Distal
disease manifestation. In tuberculoid leprosy, the weakness ensues as the motor nerves become
cell-mediated immune response is intact, leading involved in the infiltrative process. Large sensory
to focal, circumscribed, inflammatory lesions fiber modalities are relatively spared as are mus-
involving the skin or nerves. The skin lesions cle stretch reflexes. As with the tuberculoid sub-
appear as well-defined, scattered hypopigmented type, nerve trunks may be affected with time,
patches and plaques with central anesthesia and leading to superimposed mononeuropathies. In
raised, erythematous borders. Pathologically, the advanced disease, facial neuropathies can occur.
tuberculoid form is characterized by granuloma Patients with borderline leprosy have the high-
formed by macrophages and Th 1 cells that are est incidence of neurologic complications. These

118
patients may show clinical and histological fea- ofloxacin, sparfloxacin, minocycline, and clar-
tures of both the lepromatous and tuberculoid ithromycin are also effective. Treatment typically
forms of leprosy. Although impaired cellular requires two years of therapy in order to achieve full
immunity of borderline patients results in eradication of the organism. A potential complica-
mycobacterial spread, the immune system is still tion of therapy, particularly in the borderline lep-
active enough to generate an inflammatory rosy, is the reversal reaction, which can occur at any
response. Patients may develop generalized sym- time during treatment of the disease. The reversal
metric sensorimotor polyneuropathy, mononeu- reaction occurs as a result of a shift to the tubercu-
ropathies, and mononeuropathy multiplex, loid end of the spectrum with an increase in cellu-
including multiple mononeuropathies in atypical lar immunity. Upregulation of the cellular response
locations, such as the brachial plexus. is characterized by excessive release of tumor
Rarely, patients with leprosy present with iso- necrosis factor-alpha, gamma-interferon, and inter-
lated peripheral neuropathy without skin lesions. leukin-2 with new granuloma formation. This may
Lepromatous neuropathy should be suspected in result in an exacerbation of the rash and the neu-
individuals without skin lesions who live in ropathy and in the appearance of new lesions. High-
endemic areas. Virtually all cases of pure neuritic dose corticosteroids appear to blunt this adverse
leprosy have the tuberculoid or borderline tuber- reaction and may even be used prophylactically in
culoid subtypes of the disease. high-risk patients at treatment onset.
Electrodiagnostic studies can demonstrate a A second type of reaction to treatment is ery-
mononeuropathy, mononeuropathy multiplex, or thema nodosum leprosum (ENL), which occurs in
generalized sensorimotor polyneuropathy. Nerve patients at the lepromatous end of the disease
conduction studies reflect a primarily axonal or spectrum. ENL is associated with the appearance
mixed axonal and demyelinating process. of multiple erythematous, sometimes painful,
Electromyography can demonstrate active dener- subcutaneous nodules; exacerbation of the neu-
vation and decreased recruitment of large ropathy may also occur. The reaction of ENL is
polyphasic motor unit potentials in the affected due to the slow degradation of antigens (bacterial
nerve distributions. debris) resulting in antigen-antibody complex
Multidrug therapy with dapsone, rifampin, and formation and complement deposition in affected
clofazimine is the mainstay of treatment, although tissue. ENL may be treated with corticosteroids
other agents, including thalidomide, pertloxacin, or, if available, thalidomide.

Clinical Pearls
1. As in the previous two cases, multifocality is an important clue to the cause of a
peripheral neuropathy when present.
2. Look for enlargement of nerves when considering leprosy as a diagnosis

REFERENCES
I. Altman, D., Amato, A.A., Lepromatous neuropathy, J. Clin. Neuromusc. Dis. 1999; 1:68-73.
2. Nations, S.P., Barohn, RJ., Peripheral neuropathy due to leprosy, Curr. Treat. Options Neural. 2002; 4(3): 189-196.

119
 PATIENT 30

A 64-year-old woman with severe left shoulder pain and weakness

of shoulder abduction for 3 days, followed by progressive
neurologic deficits

This 64-year-old retired nurse developed increasing headaches over a 6-week period in August
and September followed by severe left shoulder blade pain for several days and then sudden onset of
complete paralysis of left shoulder abduction. She had electrophysiological studies 3 days after her
shoulder paralysis occurred.
Initial Electrodiagnostic Study:
 EMG Summary Table-cont'd
SA Amplitude Duration
PolyP Recruitment
IA Fib Fasc Other MUs MUs MUs Activation Pattern
L. triceps Nl 0 0 0 NI NI NI Full NI
L. pron teres NI 0 0 0 NI NI NI Full NI
L. first doTSint NI 0 0 0 NI NI NI Full NI
L. C4 para-spinal NI 0 0 0
L. C5 para-spinal NI 0 0 0
L. C6 para-spinal NI 0 0 0

Second Electrodiagnostic Study (8 Days After Initial Study):

Sensory NCS
BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (jlV) (em) (m/s)
R. median-dig II 1. Wrist Dig II 2.50 3.40 27.5 13 52.0
L. median-dig II 1. Wrist Dig II 2.20 3.10 40.2 13 59.1
R. ulnar-dig V 1. Wrist DigV 2.40 3.05 18.2 II 45.8
L. ulnar-dig V 1. Wrist DigV 2.10 2.95 33.7 11 52.4
R. radial-sn box 1. Forearm Sn box 1.90 2.40 35.4 10 52.6
R. medAB cut 1. Elbow Forearm 2.05 2.65 1.8 12 58.5
R. LatAB cut 1. Elbow Forearm 2.10 2.65 12.3 12 57.1
L. Lat AB cut 1. Elbow Forearm 2.10 2.55 28.4 12 57.1
R. sural-I at mall 1. Mid-calf Ankle 3.10 3.75 10.9 14 45.2
L. sural-Iat mall 1. Calf Lat mall 2.85 3.75 11.0 14 49.1
R. sup peroneal 1. Lat leg Ankle 2.95 3.85 4.2 12 40.7
L. sup peroneal 1. Lat leg Ankle 3.40 4.25 4.0 12 35.3

Motor NCS
Recording Latency Amplitude Distance Velocity
Nerve Sites Site (ms) (mV) (em) (rn/s)
R. median-APB 1. Wrist APB 3.15 6.1 7
2. Elbow APB 7.45 5.9 23 53.5
L. median-APB 1. Wrist APB 2.70 8.3 7
2. Elbow APB 6.80 7.1 21.5 52.4
R. ulnar-ADM 1. Wrist ADM 2.60 9.9 7
2. B. elbow ADM 6.05 9.0 20 58.0
3. A. elbow ADM 7.95 8.5 10 52.6
L. ulnar-ADM 1. Wrist ADM 2.55 9.6 7
2. B. elbow ADM 5.65 8.5 18 58.1
3. A. elbow ADM 7.65 8.9 10 50.0
R. peroneal-EDB 1. Ankle EDB 3.80 7.9 8
2. Fib head EDB 11.15 5.4 30 40.8
3. Pop fossa EDB 13.55 4.1 9.5 39.6
L. peroneal-EDB 1. Ankle EDB 4.60 7.3 8
2. Fib head EDB 12.20 6.4 31 40.8
3. Pop fossa EDB 15.00 2.3 7.5 26.8
L. peroneal-tib ant 1. Fib head Tib ant 3.85 6.4 13.5
2. Abv fib hd Tib ant 6.45 4.3 8 30.8
R. tibial-AH 1. Ankle AH 5.20 18.3 8
2. Pop fossa AH 14.60 10.0 43 45.7
L. tibial-AH 1. Ankle AH 4.70 18.1 8
2. Pop fossa AH 14.35 11.5 40 41.5

Ul
 EMG Summary Table
SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

L. tib ant Nl 0 0 0 Nl Nl Few Full Mod red

L. peron In Nl 0 0 0 Nl Nl Nl Full Mod red
L. tib post Nl 0 0 0 Nl Nl Nl Full Nl
L. vast lat Nl 0 0 0 Nl Nl Nl Full Nl
L. bic fern SH Nl 0 0 0 Nl Nl Nl Full Nl
L. thoracic PSP lower Incr 0 0 0
L. deltoid Nl 0 0 0 Nl Nl Few Full Mod red
L. ext dig comm Nl 0 0 0 Nl Nl Nl Full Mild red
L. triceps Nl 0 0 0 Nl Nl Nl Full Nl
R. pron teres Nl 0 0 0 Nl Nl Nl Full Nl
R. flex dig pr I Nl 0 0 0 Nl Nl Nl Full Nl
R. abd poll br Nl 0 0 0 Nl Nl NI Full Mod red
R. first dors int Nl 0 0 0 Nl Nl Nl Full NI

L Peroneal - EDB

Ankle 1
50ms5mV

A .. 1

-------<V .L--- . FibHead 2

50ms5mV

Pop Fossa 3
50ms5mV

122
 Questions:
I. The initial electrodiagnostic study is suggestive of either of two different localizations; what are
they?
2. How might one best distinguish between them?
3. Suggest two alternative explanations for the absence of fibrillation potentials.

123
 Answers:
I. Upper trunk brachial plexopathy or C5 radiculopathy
2. Relevent sensory potentials and paraspinal muscle needle EMG
3. Pure demyelination or insufficient time for wallerian degeneration

Discussion: This patient has normal nerve Table 6 in the introduction to Section II. The repeat
conduction studies. Needle EMG studies show electrodiagnostic studies should emphasize sen-
marked reduction in recruitment of motor units in sory potentials (root versus more distal involve-
the left infraspinatus, deltoid, and biceps. This ment), although preservation of them could still
is a pattern suggestive of either an upper trunk reflect a very acute process distal to the dorsal root
brachial plexopathy or a C5 radiculopathy, as ganglia. In addition, repeating the previous needle
these three muscles share different nerves and EMG studies of the deltoid and biceps and study-
cords but the same trunk and root. In general, the ing the left peroneal and right median nerves and
best way to distinguish between root and more innervated muscles could potentially be valuable.
peripheral localizations is by examination of rele- The important findings in the repeat study are:
vant sensory potentials and paraspinal muscle • Mild relative but not definite reduction in the
needle EMG. The SNAPs potentially affected by right median and ulnar SNAPs (27 and 18
an upper trunk lesion are the lateral antebrachial f.l V, respectively, compared with left side of
cutaneous, the radial-webspace or radial-D I, and 40 and 33 f.lV)
the median-D I (see Table I). Two of these were • Definite reduction in the right lateral ante-
studied and are normal, although the short history brachial cutaneous SNAP amplitude (12.3
could explain this. One reason fibrillation poten- f.lV compared to left of 28.4 mY; also
tials may be absent is the short history of symp- reduced compared to previous study's right
toms and insufficient time for Wallerian side value of 21 f.l V)
degeneration ofaxons to have occurred; fibrilla- • Definite reduction in the right medial ante-
tion potentials can take up to 3 weeks to develop brachial cutaneous SNAP amplitude (1.8 f.lV)
after acute axonal nerve injury. An alternative compared to the left side studied 8 days ear-
explanation for reduction in motor unit recruit- lier (15.5 f.lV)
ment in the absence of fibrillation potentials is a • Apparent conduction block (amplitude 6.4 to
pure demyelinating lesion. The electrodiagnostic 2.3 mV) and focal slowing of the left per-
interpretation was an acute C5 radiculopathy. oneal-EDB across-fibular-head segment (see
One week later, this patient developed a left figure)
mid-thoracic radiculopathy and a left foot drop. • Needle EMG abnormalities limited to
Examination showed weakness in the right proxi- decreased recruitment in the left common
mal median nerve territory including flexor polli- peroneal distribution, right APB but not first
cis longus (FPL) and APB, weakness of left dorsal interosseous (FDI) and left extensor
tibialis anterior and peroneus longus but not tib- digitorum communis (EDe) but not FDI,
ialis posterior with decreased sensation in the left suggesting peripheral lesions of the left com-
lateral leg from the ankle to halfway up the leg, mon peroneal nerve, right distal median, and
and weakness of left finger extensors but not left posterior interosseous nerves
wrist extensors or triceps. There was an area of These findings support the clinical impression
sensory disturbance corresponding to approxi- discussed earlier. The conduction block for the
mately the complete left T9 dermatome, including peroneal nerve could represent true injury to
the most posterior and anterior extent. She under- myelin or might reflect the pseudo-conduction
went repeat electrodiagnostic studies, now II block seen with acute axonal injury (further dis-
days after onset of the shoulder weakness and cussion of this concept can be found in case 31).
I day after the foot drop. Over the ensuing week, The patient's CSF showed 250 WBC (all
she developed hoarseness (suggestive of a recur- mononuclear), protein of 150 mgldl, and normal
rent laryngeal nerve palsy) and mild bilateral glucose. Serum Lyme ELISA and Western blot
asymmetric facial weakness and had a general- were positive. CSF Lyme Western blot was posi-
ized tonic-clonic seizure with a post-ictal right tive and CSF /serum Lyme IgG indices were
hemiparesis. markedly elevated. This is a case of early dissem-
Clinically, her peripheral nerve involvement is inated Lyme disease with a polyradiculopathy as
characterized as a multifocal neuropathy; there are well as more peripheral involvement. Her only
features of a polyradiculopathy, peripheral multiple known potential exposure was a trip 4 to 6 weeks
mononeuropathy, and cranial neuropathies. This is before onset of symptoms to Cape Cod for 2 days,
an asymmetric muitifocal motor and sensory during which she spent almost all of her time
process whose differential diagnosis is that listed in indoors in an ice-skating rink. She was never

124
/ aware of a skin rash. She was treated with intra- neuropathies, and radiculoneuritis, a term that
venous ceftriaxone and has made a remarkably reflects the combination of a polyradiculopathy
full recovery over several months. and a more peripheral mononeuritis multiplex.
Lyme disease is caused by the spirochete Severe intrascapular pain has been noted as a fre-
Borrelia burgdorferi, transmitted by tick bite quent early symptom,2 as in this case. Thoracic
except in very rare congenital cases. It is charac- radiculopathy has also been commented on.8
terized by three stages of infection: early local Asymmetrical dermatomal and myotomal abnor-
(with skin manifestations only), early disseminated malities may be present, also as in this case,
(with skin, peripheral, and central nervous system; although this presentation is said to be common in
heart; and eye manifestations), and late infection. Europe (where it has been referred to as lympho-
The neurological manifestations of early dis- cytic meningoradiculitis or Bannwarth's syn-
seminated infection are those of meningitis, cranial drome) and is unusual in North America.

Clinical Pearls
1. Early disseminated Lyme disease may present with a polyradiculopathy; particu-
larly when a thoracic radiculopathy is present, a high index of suspicion should be
maintained.
2. Focal slowing and apparent conduction block may occur in acute axonal injury.

REFERENCES
I. Avanzi, S., Messa, G., Marbini, A., Pavesi, G., Granella, E, Isolated neuritis of the sciatic nerve in a case of Lyme
disease, Ital. J Neurol. Sci. 1998; 19:81-85.
2. Coyle, P.K., Schutzer, S.E., Neurologic aspects of Lyme disease, Med. Clin. North Am. 2002; 86:261-284.
3. Deltombe, T., Hanson, P., Boutsen, Y., Laloux, P., Clerin, M., Lyme borreliosis neuropathy: a case report, Am. J Phys.
Med. Rehabil. 1996; 75(4):314-316.
4. Gilchrist, J.M., AAEM case report 26: seventh cranial neuropathy, Muscle Nerve 1993; 16(5):447--452.
5. Krishnamurthy, K.B., Liu, GT, Logigian, E.L., Acute Lyme neuropathy presenting with polyradicular pain, abdominal
protrusion, and cranial neuropathy. Muscle Nerve 1993; 16(11):1261-1264.
6. Logigian, E.L., Peripheral nervous system Lyme borreliosis, Semin. Neural. 1997; 17:25-30.
7. Logigian, E.L., Steere, A.C., Clinical and electrophysiological findings in chronic neuropathy of Lyme disease, Neurology
1992; 42:303-31 I. .
8. Mormont, E., Esselinckx, W., De Ronde, T., Hanson, P., Deltombe, T., Laloux, P., Abdominal wall weakness and
lumboabdominal pain revealing neuroborreliosis: a report of three cases, Clin. Rheumatol. 2001; 20:447--450.
9. Pachner, A.R., Steere, A.C., The triad of neurological manifestations of Lyme disease: meningitis, cranial neuritis, and
radiculoneuritis, Neurology 1985; 35:47-53.
10. Scelsa, S.N., Herskovitz, S., Berger, A.R., A predominantly motor polyradiculopathy of Lyme disease, Muscle Nerve
1996; 19:780-783.
I I. Steere,A.C., Lyme disease, N Engl. J Med. 2001; 345:115-125.

12$
 PATIENT 31

A 38-year-old man with fever and acute generalized weakness and

distal numbness

This 38-year-old man developed fever to 102° and myalgias. Over the subsequent 5 days, he
developed numbness of the medial hands and feet bilaterally. He then developed weakness in his legs
and underwent electrodiagnostic testing within 3 days of the onset of weakness. He was admitted to
the hospital, where examination showed fever to 101S and symmetric diffuse (proximal and distal)
weakness (generally grade 4/5) and distal sensory loss in the arms and legs.
Initial Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (IlV) (cm) (m/s)

L. median-dig II 1. Wrist Dig II 2.40 3.10 43.9 13 54.0

L. ulnar-dig V 1. Wrist Dig V 2.10 2.80 22.1 11 52.0
R. sural-I at mall 1. Mid-calf Ankle Absent

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)

L. median-APB 1. Wrist APB 3.2 14.6 7

2. Elbow APB 7.1 5.9 24 62
L. ulnar-ADM 1. Wrist ADM 2.6 11.3 7
2. B. elbow ADM 6.7 2.2 20.5 50
3. A. elbow ADM 8.8 2.5 14 67
R. peroneal-EDB 1. Ankle EDB 3.7 4.6 8
2. Fib head EDB 10.9 2.0 34 47
3. Pop fossa EDB 13.7 0.8 12 43
R. tibial-AH 1. Ankle AH 4.8 11.0 8
2. Pop fossa AH 14.7 3.4 44 44

F-Wave

Nerve Fmin (ms) %F

R. peroneal Absent
R. tibial 53.1 60
L. ulnar 26.2 80
L. median 26.3 80

126
 Left Ulnar nerve Recording site ADQ
Stimulus site 2 ms

Wrist

85.5mR
Below elbow
5mV

65.1mR
Above elbow
5mV

~5m~
2ms

Figure 1

Right Tibial nerve Recording site AHB

Stimulus site ms

Ankle 62.8mR
5mV

Knee 62.6mR
5mV

Figure 2

127
 Repeat Electrodiagnostic Studies 5 Days Later:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (IlV) (em) (m/s)
L. median-dig II I. Wrist Dig II 2.40 3.00 26.1 13 54.0
L. ulnar-dig V I. Wrist Dig V 2.00 2.50 4.1 11 55.0

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (em) (m/s)
L. median-APB I. Wrist APB 3.1 4.8 7
2. Elbow APB 7.3 4.5 24 57
L. ulnar-ADM I. Wrist ADM 3.0 1.2 7
2. B. elbow ADM 7.2 1.0 23.5 56
3. A. elbow ADM 8.9 1.1 10 59
R. peroneal-EDB 1. Ankle EDB 5.6 0.3 8
2. Fib head EDB 12.5 0.3 34 50
R. tibial-AH 1. Ankle AH 5.1 2.0 8
2. Pop fossa AH 14.7 1.0 44 43

Left Ulnar nerve Recording site ADO

Stimulus site
'2 mV/D' "2 ms/D

Wrist

Below elbow

Above elbow

~2~V
2ms

Figure 3

128
 Right Tibial nerve Recording site AHB

Stimulus site
3-ms/D

Ankle

Knee

~2m~
3ms

Figure 4

Questions:
I. In the initial electrodiagnostic study, what are the major electrodiagnostic abnormalities?
2. Look at the waveforms in figures I and 2. What do they demonstrate? What is your clinical and
electrodiagnostic diagnosis?
3. What clinical feature of this case is highly unusual? What additional tests should be considered?
4. What do the repeat electro diagnostic studies show?
5. Is there still evidence of conduction block?

1'29
 Answers:
1. Absent sural SNAP and amplitude drops of CMAPs with proximal stimulation
2. Amplitude drops in CMAPs with proximal stimulation-possible conduction block
3. Fever; consider nerve biopsy
4. The previously apparent "conduction block" was actually "pseudoconduction block"
5. No.

Discussion: The electrodiagnostic abnonnali- stimulation (see figures). The findings are now
ties in the initial study are the absent sural SNAP those of degeneration of sensory and motor
and the drop in amplitudes of all CMAPs with axons. This patient has a vasculitic neuropathy.
proximal compared to distal stimulation. The Laboratory evaluation was notable for elevated
wavefonns in figures I and 2 demonstrate the erythrocyte sedimentation rate of 69, negative
amplitude drops without temporal dispersion and hepatitis C antibodies and hepatitis B surface
suggest conduction block antigen, negative ANA and ANCA, and serum
The patient was diagnosed with Guillain-Barre lyme. Nerve biopsy showed necrotizing vasculitis
syndrome and treated with intravenous immuno- with true fibrinoid necrosis of medium-sized
globulin (IVIg). Guillain-Barre syndrome is epineurial arterioles and mild patchy axonal loss.
associated with infections and the patient had He was treated with prednisone and gradually
fever and myalgias. Guillain-Barre syndrome is improved. Long-term follow-up is not known.
associated with infections, although as a post- As we have emphasized previously, acute
infectious sequela. The presence of continued axonal nerve injury, from any cause, may produce
fever during active progression of the neuro- apparent conduction block and this condition
logical symptoms is highly atypical and raises has been termed pseudo-conduction block. As
the· possibility. of other diagnoses. Repeat elec- Wallerian degeneration proceeds and neuromus-
trodiagnostic studies and nerve biopsy were cular transmission failure occurs, the distal
perfonned, both 5 days after the initial electrodi- evoked CMAP amplitudes drop as well. In this
agnostic study . case, the evolution of changes clearly defines this
. The repeat electrodiagnostic studies show evo- process as not that of conduction block from focal
lution of reduction in distal evoked SNAP and demyelination. The repeat ulnar nerve study
CMAP amplitudes and the loss of the previous shows the distal wrist evoked CMAP becoming
apparent conduction block, with proximal stimu- the same size and shape as the initial study's prox-
lation resulting in similar amplitudes as distal imal evoked CMAPs (see first and third figures).

Clinical Pearls
1. Apparent conduction block is sometimes pseudo-conduction block, demonstrable
through repeat nerve conduction studies several days to weeks afterward. Therefore, in
acute neuropathies, keep in mind that apparent conduction block may be acute axonal
injury and consider a follow-up diagnostic study.
2. Guillain-Barre syndrome is a post-infectious process; the presence of fever
should raise suspicion of an alternative diagnosis.

REFERENCES
I. Briemberg, H.R., Levin, K., Amato, A.A., Multifocal conduction block in peripheral nerve vasculitis, J. Clin. Neuramusc.
Dis. 2002; 3(4):153-158.
2. Dyck, P.J., Benstead, T.J., Conn, D.L. et al., Non-systemic vasculitic neuropathy, Brain 1987; 110: 843-854.
3. Greenberg, S.A., P-ANCA vasculitic neuropathy with 12 year latency from onset to systemic symptoms, BMC Neural.
2002; 2:10-13.
4. Hawke, S.H., Davies, L., Pamphlett, R., Guo, Y.P., Pollard, J.D., McLeod, J.G., Vasculitic neuropathy: a clinical and
pathological study, Brain 1991; 114:2175-2190.
5. Kissel, J.T., Slivka, A.P., Warmolts, J.R., Mendell, J.R., The clinical spectrum of necrotizing angiopathy of the peripheral
nervous system, Ann. Neural. 1985; 18:251-257.
6. Magistris, M.R., Kohler, A., Estade, M., Conduction block in vasculitic neuropathy, Eur. Neural. 1994; 34:283-285.
7. McCluskey, L., Feinberg, D., Cantor, c., Bird, S., "Pseudo-conduction block" in vasculitic neuropathy, Muscle Nerve 1999;
22:1361-1366.
8. Nadeau, S.E., Neurological manifestations of systemic vasculitis, Neural. Clin. 2002; 20:123-150.
9. Sandbrink, F., Klion, A.D., Floeter, M.K., "Pseudo-conduction block" in a patient with vasculitic neuropathy, Electramyagr.
Clin. Neurophysiol. 2001; 41:195-202.

130
 PATIENT 32

A 66-year-old woman with numbness in her hands and feet and

progressive generalized weakness

This 66-year-o]d woman developed mild numbness in her hands and feet 9 months prior and over
the last month has developed progressive severe generalized weakness. She was hospitalized because
she was unable to sit up in bed unsupported and had severe generalized weakness and areflexia, as well
as distal sensory loss.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (~V) (cm) (m/s)
L. median-dig II I. Wrist Dig II Absent 13
L. ulnar-dig V I. Wrist DigV 2.60 3.45 7.] ]I 42.3.
L. radial-sn box I. Forearm Sn box 1.55 2.10 28.1 10 64.5
L. sural-Iat mall ]. Mid-calf Ankle 3.30 4.20 36.3 14 42.4

Motor NCS
Recording Area Latency Amplitude Distance Velocity
Nerve Sites Site mVms (ms) (mV) (cm) (mls)
L. median-APB 1. Wrist APB 14.9 16.95 3.7 7
2. Elbow APB 18.0 22.85 3.7 16 27.1
R. median-APB I. Wrist APB 20.2 17.55 5.5 7
2. Elbow APB 20.8 21.85 5.2 20 46.5
3. Axilla APB 24.5 25.35 5.3 19 54.3
L. ulnar-ADM I. Wrist ADM 19.1 4.25 3.7 7
2. B. elbow ADM 17.4 6.90 3.6 16.5 62.3
3. A. elbow ADM 12.4 9.40 2.4 9 36.0
R. ulnar-ADM I. Wrist ADM 18.9 4.20 3.3 7
2. B. elbow ADM 14.9 8.35 3.0 15 36.1
3. A. elbow ADM 3.5 19.10 1.2 II 10.2
L. peroneal-EDB I. Ankle EDB 17.4 11.35 3.5 8
2. Fib head EDB 18.6 17.80 3.4 25.5 39.5
3. Pop fossa EDB 16.8 19.95 3.0 9.5 44.2
L. tibial-AH I. Ankle AH 7.3 14.30 1.0 8
2. Pop fossa AH 5.2 22.95 1.1 35 ·40.5

F-Wave
Nerve F min (ms) F max (ms) Max - Min (ms) %F
L. peroneal 72.20 75.45 3.25 30
L. ulnar Absent
R. median 47.95 52.65 4.70 20
R. ulnar 28.10 33.20 5.10 20

131
 H Reflex

Nerve H Latency (ms)

R. tibial-soleus Absent
L. tibial-soleus Absent

EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

L. vast lat NI 0 0 0 NI NI NI Full NI

L. tib ant Incr 0 0 0 NI NI NI Full Mod red
L. gastroc med Incr 0 0 0 NI NI NI Full Mild red
L. flex hall In Incr 2+ 0 0 NI NI NI Full Mod red
L. deltoid NI 0 0 0 NI NI NI Full NI
L. biceps NI 0 0 0 NI NI NI Full NI
L. triceps Nl 0 0 0 Nl Nl Nl Full Mod red
L. first dors int Incr 3+ 2+ 0 Nl Nl Nl Full Mod red
L. pron teres Nl 0 0 0 NI Nl Nl Full NI

2 R Ulnar- ADM

Wrist

50ms2 mV

B.Elbow

Questions:
1. The sensory studies show a pattern of abnormalities referred to by the abbreviation AMNS. What
does this stand for and what does it imply?
2. Look at the right and left median distal motor latencies and velocities. Do you think the left median
forearm motor velocity represents primary demyelination?
3. The left tibialis anterior muscle was MRC grade 2 in strength and had been this weak for 1 month.
Do you think the weakness was due to loss of motor axons or conduction block of motor axons sup-
plying this muscle?

132
 Answers:
1. "Abnormal median normal sural," suggestive of a demyelinating neuropathy
2. Yes
3. Conduction block

Discussion: This case illustrates an issue cen- demyelinating neuropathy. There are also occa-
tral to many studies of patients with suspected sional circumstances, particularly acute ischemic
peripheral neuropathy: the characterization of injury of a nerve, when these findings are present
the physiology as predominantly axonal or without demyelination as the cause. Furthermore,
demyelinating. The electrodiagnostic literature some demyelinating neuropathies preferentially
supports the use of several concepts in this regard: produce one of these findings but not others; the
• The abnormal median normal sural pattern of lack of partial conduction block with anti-MAG
SNAPs neuropathies and CMTl a are examples.
• Focal demyelination: focal slowing, partial What constitutes electrodiagnostic criteria for
conduction block, or temporal dispersion focal conduction slowing sufficient to conclude
• Reduced needle EMG interference patterns demyelination is the cause? A large body of
without fibrillation potentials or positive literature and criteria has evolved. There is no a pri-
waves in muscles that are chronically weak ori reason to assume that criteria accurate for diag-
The first is a pattern of sensory abnormalities nosis applies to all demyelinating neuropathies; in
referred to as AMNS, which stands for abnormal fact, criteria for demonstrating demyelination in
median normal sural and is a characteristic fea- acute inflammatory demyelinating polyneu-
ture of chronic inflammatory demyelinating neu- ropathies (AIDP), such as Guillain-Barre syn-
ropathies. Of course, isolated carpal tunnel drome, is likely different than that for chronic
syndrome can also produce this picture, but in the inflammatory demyelinating polyneuropathy
particular setting of patients with a neuropathy, (CIDP). Some of the differences in criteria refer-
reduced hand (not necessarily just median) but enced below reflect different diseases to which they
normal sural SNAP amplitudes are highly spe- are optimized. There is consensus, nevertheless, of
cific for a demyelinating neuropathy. one broad principle. The lower limit of normal of
The second concept reflects the electrophysio- velocities is not sufficiently accurate as a cutoff for
logical correlates of focal demyelination of a demyelinating neuropathies. Clearly, axonal neu-
nerve: focal conduction slowing, partial conduc- ropathies commonly produce mild slowing, and the
tion block, and temporal dispersion. These three characterization of a neuropathy as primarily
electrodiagnostic findings may occur together or demyelinating on the basis of mild slowing is one of
independently in a nerve segment, but all provide the most frequent and detrimental mistakes made
evidence of focal demyelination. Abnormalities in by electrodiagnostic practitioners. As slowing in
nerve segments that are common sites of com- axonal neuropathy correlates with reduction in
pression (i.e., median at the wrist, ulnar at the CMAP amplitudes, the latter is generally used
elbow, and peroneal at the fibular head) need in the interpretation of the abnormal cutoff for the
to be interpreted cautiously and are not sufficient velocity. Sample proposed research criteria for both
in general for the diagnosis of a generalized AlDP and CIDP are shown below.

Suggestive of Demyelination in AIDP (Albers and Kelly3)

At least 3 of the following:
In 2 or more nerves,
Amplitude> 50% lower limit of normal (LLN) -7 conduction velocity < 90% LLN
Amplitude < 50% LLN -7 conduction velocity < 80% LLN
In 2 or more nerves,
Amplitude normal -7 distal latency > 110% upper limit of normal (ULN)
Amplitude < normal -7 distal latency > 120% ULN
In I or more nerves,
Conduction block as defined by amplitude reduction> 30%, or
Temporal dispersion as defined by proximal-distal duration increase of 30% or more
In I or more nerves,
Minimum F response latency> 120% ULN

133
 Suggestive of Demyelination in CIDP (Albers and Kelly3)

At least 3 of the following:

In 2 or more nerves,
Conduction velocity < 75% LLN
In 2 or more nerves,
Distal latency.> 130% ULN
In 1 or more nerves,
Conduction block as defined by amplitude reduction> 30%, or
Temporal dispersion as defined by proximal-distal duration increase of 30% or more
In I or more nerves,
Minimum F response latency> 130% ULN

Suggestive of Demyelination for Both AIDP and CIDP

(Cornblath,8 AAN AIDS Task Force4)

At least 3 of the following:

In 2 or more nerves,
Amplitude> 80% LLN ~ conduction velocity < 80% LLN
Amplitude < 80% LLN ~ conductiori velocity < 70% LLN
In 2 or more nerves,
Amplitude> 80% LLN ~ distal motor latency> 125% ULN
Amplitude < 80% LLN ~ distal motor latency> 150% ULN
In 1 or more nerves, .
Conduction block as defined by amplitude' reduction> 20%, or
Temporal dispersion as defined by proximal-distal duration increase of 15% or more
(Conduction block is considered possible only if temporal dispersion abnormal.)
In 2 or 'more nerves,
Amplitude> 80% LLN ~ minimum F-wave latency> 120% ULN
Amplitude < 80% LLN ~ minimum F-wave latency> 150% ULN

Other criteria, the same for both AIDP and duction velocity of less than 70% the LLN-had
CIDp, ,have been proposed: a sensitivity of 66% and specificity of 100%. This
finding suggests that the criteria listed above are
. Suggestive of Demyelination for too cumbersome and can be improved upon for
. Both AIDP and CIDP (Saperstein clinical use. In particular, a nerve with conduction
et a1.13) velocity of less than 70% the LLN was not seen
in patients with strictly motor axonal degenera-
Similar to AAN criteria above, except 2 out of 4 tion, such as motor neuron disease, or patients
(instead of 3 out of 4) criteria are required, and with distal symmetric axonal neuropathies and is
the definition of conduction block is per AAEM a powerful indicator of a primary demyelinating
consensus criteria: disorder. This roughly translates into the follow-
For example, to provide evidence of primary ing conservative rule: Motor conduction veloci-
demyelination, for a median-APB motor nerve ties of <30 m/s in the forearm or <25 m/s in the
study with CMAP amplitude of 3.6 (=75% of leg reliably indicate the presence of primary
LLN), the forearm motor velocity would have to demyelination.
be less than 70% of 44 mis, which is 30.8 mls. Its Regarding question 2 above and this case, the
distal latency would have to be at least 150% of left median motor forearm segment has a velocity
4.5 ms, which is 6.8 msec. of 27 mls. We think this is sufficiently abnormal to
consider as reflective of primary demyelination.
These criteria are complex, and those for CIDP Question 3 deals with criteria for partial con-
were reviewed by Bromberg.7 Both Albers and duction block of motor nerves. The above criteria
the AAN criteria were found to be approximately use variously 20% or 30% reduction in CMAP
50% sensitive and 100% specific in a population amplitudes as indicative of conduction block.
that included patients with distal symmetric This is likely too liberal and does not factor in
polyneuropathy and motor neuron disease; how- such considerations as distal CMAP amplitude
ever, a single criterion-one nerve with a con- reduction; that is, if distal CMAP is already very

134
small, such as 0.4, then proximal CMAPs will (I) definite conduction block cannot be judged
commonly meet this degree of reduction (in this present if the distal CMAP is less than 20% the
example, 0.3 is a 25% drop). Criteria have been LLN or if temporal dispersion is greater than
devised and published as consensus criteria of the 30%; (2) definite conduction block can never be
American Association of Electrodiagnostic judged present in the radial nerve or the tibial
Medicine (AAEM).11 These criteria are shown in nerve in the leg; and (3) definite conduction block
the table below. They are much more conservative should not be concluded based on Erb's point or
and are noteworthy in the following regards: cervical root stimulation studies.

Amplitude Reduction Criteria for Partial Conduction Block (AAEM Conduction

Block Criteria 11)

Temporal Dispersion < 30% Temporal Dispersion 31-60%

Nerve/Segment Definite Probable Definite Probable· .
Median
Forearm >50% 40-50% >5Q%
Arm >50% 40-50% >50%
Proximal (Erb's point) >40% >50%
Ulnar
Forearm >50% 40-50% >50%
Across Elb >50% 40-50% >50%
Arm >50% 40-50% >50%
Proximal (Erb's point) >40% >50%
Radial
Forearm >50% >60%
Arm >50% >60%
Proximal (Erb's point) >50% >60%
Peroneal
Leg >60% 50-60% >60%
Across Fib >50% 40-50% >50%
Thigh (sciatic notch) >50% >60%."
Tibial
Leg >60% 50-60% >60%
Thigh (sciatic notch) >50% >60%
Note: Criteria for area reduction
are always of magnitude 10% less than amplitude (i.e., amp/area = 50%/40%). Applicable to
CMAP;:: 20% LLN. Erb's point and sciatic notch stimulation are controversial. Nerve root stimulation is not accepted.

With regard to this specific case study, the Question 4 aims to emphasize the fourth con-
right ulnar-ADM across-elbow segment shows a cept relating to the electrodiagnosis of demyeli-
60% reduction in amplitude, from 3.0 to 1.2. nation-namely, that reduced" ("neurogenic")
Inspection of the waveforms in the figure allows interference patterns in weak rriusc\'es without
for a rough calculation of the degree of temporal fibrillation potentials is a likely indicator of
dispersion, calculated as the duration of the nega- demyelination of motor axons. The main excep-
tive phase of the CMAP (time between markers tion is the acute setting, whereby acute axonal
I and 3). For the below-elbow site, this is -100 injury may immediately result in a reduced inter-
msec, and for the above-elbow site, this is -120 ference pattern, while fibrillation potentials may
ms, indicating temporal dispersion of -20% and take up to 3 weeks to appear. Profound chronic
meeting the criteria for definite conduction block denervation with partial and incomplete reinnerc
in the table. Of course, this is a common com- vation may often give this pattern but will almost
pression site and, although reflective of conduc- always have clear abnormalities of motor unit
tion block, should not be taken by itself as morphology (i.e., increased duration and ampli-
convincing evidence of a generalized demyelinat- tude) to distinguish itself from the above findings
ing neuropathy. of pure motor nerve demyelination.

!l3S
 Clinical Pearls
Electrodiagnostic support of a demyelinating neuropathy may come from any of the
following: .
1. The abnormal median normal sural (AMNS) pattern of SNAPs
2. Focal demyelination: focal slowing, partial conduction block, or temporal dispersion
3. Reduced needle EMG interference patterns without fibrillation potentials or pos-
itive waves in muscles that are chronically weak
4. Definitive and highly conservative criteria for focal slowing representative of
demyelination: motor conduction velocities of <30 mls in the arms and <25 mls in the
legs

REFERENCES .
I. Alam, TA., Chaudhry, v., Comblath, D.R., Electrophysiological studies in the Guillain-Barre syndrome: distinguishing
subtypes by published criteria, Muscle Nerve 1998; 21(10): 1275-1279.
2. Albers, J.W, Donofrio, P.O., McGonagle, T., Sequential electrodiagnostic abnormalities in acute inflammatory
demyelinating polyradiculoneuropathy, Muscle Nerve 1985; 8:528-539.
3. Albers, J.W, Kelly, J.1., Jr., Acquired inflammatory demyelinating polyneuropathies: clinical and electrodiagnostic
features, Muscle Nerve 1989; 12(6):435-451.
.4. American Academy of Neurology, Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy
(ClOP): report from an Ad Hoc Subcommittee of the American Academy of Neurology AIOS Task Force, Neurology
1991; 41(5):617-618.
5. Asbury, A.K., Comblath, D.R., Assessment of current diagnostic criteria for Guillain-Barre syndrome, Ann. Neurol.
'1990; 27(suppl.):S21-S24.
6. Barohn, R.1., Kissel, J.T, Warmolts, J.R., Mendell, J.R., Chronic inflammatory demyelinating polyradiculoneuropathy:
.clinical characteristics, course, and recommendations for diagnostic criteria, Arch. Neurol. 1989; 46:878-884.
7. Bromberg, M.B., Comparison of electrodiagnostic criteria for primary demyelination in chronic polyneuropathy, Muscle
Nerve 1991; 14(10):968-976.
8. Comblath, D.R., Electrophysiology in Guillain-Barre syndrome, Ann. Neurol. 1990; 27(suppl.):S 17-S20.
9. Comblath, D.R., Sumner, A.1., Daube, J., Gilliat, R.W, Brown, WE, Parry, G.J., Albers, J.W, Miller, R.G., Petajan, J.,
Conduction block in clinical practice, Muscle Nerve 1991; 14(9):869-871.
10. Meulstee, J., van der Meche, EG., Electrodiagnostic criteria for polyneuropathy and demyelination: application in 135
patients with Guillain-Barre syndrome, Dutch Guillain-Barre Study Group. J. Neurol. Neurosurg. Psychiatry 1995;
59(5):482-6.
II. Olney, R.K., Consensus criteria for the diagnosis of partial conduction block, Muscle Nerve 1999; 22(suppl.
8):S225-S229.
12. Olney, R.K., Aminoff, M.J., Electrodiagnostic features of the Guillain-Barre syndrome: the relative sensitivity of different
techniques, Neurology 1990; 40:471-475.
13. Sander, H.W, Latov, N., Research criteria for defining patients with CIDp, Neurology 2003; 60(8, suppl. 3):S8-S15.
14. Saperstein, D.S., Katz, J.S., Amato, A.A., Barohn, R.1., Clinical spectrum of chronic acquired demyelinating
. polyneuropathies. Muscle Nerve 2001; 24:311-324.
15. Taylor, P.K., CMAP dispersion, amplitude decay, and area decay in a normal population, Muscle Nerve 1993;
16(11):1181-1187.

136
 PATIENT 33

A 43-year-old woman with progressive generalized weakness and

numbness for 5 months

This 43-year-old woman with rheumatoid arthritis since age 37 developed difficulty .walking
upstairs, getting out of a low chair, and raising her arms up when hanging clothes or washing her hair.
Within one month, she then developed constant bilateral perioral, tongue, and lower facial numbness and
tingling paresthesias, as if these areas were "waking up from Novocain." Tingling and numbness in her
hands, left more than right, then developed. Neurological examination was remarkable for diffuse gener-
alized weakness with moderate asymmetries (i.e., tibialis anterior right 4+, left 4-; infraspinatus right 4+,
left 3), sensory disturbances on the lower face and anterior neck and all fingertips, and complete areflexia.
Electrodiagnostic Study:

Sensory NCS
BP
Recording Onset Peak Amplitude Distance Velocity.
Nerve Sites Site (ms) (ms) (M-V) (cm) (m/s)
L. median-dig II I. Wrist Dig II 2.25 3.15 6.7 13 57.8
L. ulnar-dig V I. Wrist Dig V 2.45 3.20 6.0 11 44.9
L. radial-sn box I. Forearm Sn box 1.75 2.45 6.8 10 57.1
R. ulnar-dig V I. Wrist DigV 2.80 4.00 10.4 II 39.3
R. median-dig II I. Wrist Dig II 4.35 5.75 6.0 13 29.9
R. radial-sn box I. Forearm Sn box 2.05 2.85 14.8 10 48.8
L. median-ulnar I. Median-palm Wrist 1.70 2.30 4.9 8 47.1
(palmar) 2. Ulnar-palm Wrist Absent 8
L. sural-Iat mall 1. Mid-calf Ankle 3.85 4.75 9.4 14 36.4
R. sural-Iat mall I. Mid-calf Ankle 3.75 4.70 11.2 14 37.3
L. sup peroneal I. Lat leg Ankle 3.10 4.05 11.1 12 38.7
R. sup peroneal I. Lat leg Ankle 2.80 3.90 14.3 12 42.9.

Motor NCS
Recording Latency Amplitude Distance Velocity
Nerve Sites Site (ms) (mV) (cm) (m/s)
R. median-APB 1. Wrist APB 5.85 9.4 7
2. Elbow APB 10.70 9.6 21 43.3
3. Axilla APB 12.90 9.1 12 54.5
L. median-APB 1. Wrist APB 3.60 6.9 7
2. Elbow APB 7.90 6.6 19.5 45.3
3. Axilla APB 10.80 6.8 12 41.4
R. ulnar-ADM 1. Wrist ADM 3.20 8.1 7
2. B. elbow ADM 6.20 8.3 16.5 55.0
3. A. elbow ADM 8.05 8.5 10 54.1
4. Axilla ADM 9.75 7.9 10 58.8
L. ulnar-ADM 1. Wrist ADM 2.55 8.3 7
2. B. elbow ADM 5.95 7.6 18 52.9
3. A. elbow ADM 7.50 8.0 10 64.5
4. Axilla ADM 9.25 7.8 10 57.1
R. peroneal-EDB 1. Ankle EDB 6.60 4.5 8
2. Fib head EDB 15.35 4.8 33.5 38.3
3. Pop fossa EDB 17.90 4.3 8 31.4
R. tibial-AH 1. Ankle AH 5.65 6.2 8
2. Pop fossa AH 14.65 5.1 39.5 43.9

131
 F-Wave

Nerve Fmin (ms) F max (ms) Max - Min (ms) %F

R. peroneal 58.15 '63.00 4.85 70
R. median 34.20 36.65 2.45 80
R. ulnar 29.80 33.65 3.85 80
L. median 29.35 32.75 3.40 80
L. ulnar 27.20 30.30 3.10 70

Needle EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
L. deltoid Nl 0 0 0 Nl Brief Nl Full Mild red
L. triceps Incr 2+ 0 0 Nl Nl Nl Full Sev red
L. biceps Nl '0 0 0 Small Brief Nl Full Mod red
L ext dig comm Nl 0 0 0 Nl Nl Nl Full Mod red
L. infra spinat Nl 0 0 0 Nl Nl Nl Full Mod red
L. first dors int Inc 2+ 0 0 Nl Nl Nl Full Mod red
L. tib ant Inc 2+ 0 0 Nl Nl Nl Full Mild red
L. vast med Nl 0 0 0 Nl Nl Nl Full Nl

" Questions:
1. What pattern of abnormalities is present in the sensory nerve conduction studies? What does this
suggest?
2. Is there evidence of primary demyelination with this electrodiagnostic study?
3. What clinical diagnoses should be considered?

138
 Answers:
I. The abnormal median normal sural pattern suggests a demyelinating neuropathy.
2. No
3. Chronic inflammatory demyelinating polyneuropathy, vasculitic neuropathy

Discussion: This patient has the AMNS median at 34 ms, by the carpal tunnel syndrome.
(abnormal median normal sural) pattern of abnor- Accordingly, the nerve conduction studies do not
malities on sensory nerve conduction studies (see provide any definitive evidence of primary
case 32). More generally, she has low amplitudes demyelination.
for bilateral median, left ulnar, and left radial The needle EMG study does provide such evi-
SNAPs. The right ulnar and radial SNAPs are prob- dence, however. The presence of moderately
ably reduced as well, especially given the well reduced recruitment patterns in several arm mus-
above normal size of the sural and superficial per- cles (biceps, extensor digitorum communis, infra-
oneal SNAPs. This pattern suggests chronic inflam- spinatus) without fibrillation potentials to suggest
matory demyelinating polyneuropathy (CIDP). acute denervation or motor units with increased
Is there evidence of primary demyelination to duration or amplitude to suggest reinnervation is
support the diagnosis of CIDP? The right median best explained by proximal demyelination of
distal motor latency and distal sensory latency motor nerves.
are prolonged, but this may be due to carpal This patient was diagnosed with CIDP,
tunnel syndrome; the other distal motor and although a vasculitic neuropathy could not be
sensory latencies are normal. The motor segments completely excluded. The CSF protein was nor-
have normal velocities with the exception of mal. She was treated with prednisone (60 mg/day)
the peroneal-EDB across the fibular head, which and at follow-up at 3 weeks she noted "marked~'
is mildly reduced at 31 mis, although the temper- improvement. The right hand numbness for digits
ature in the leg was only 2TC. The peroneal- 2 to 4 had resolved completely, her arm strength
EDB minimum F-wave response is prolonged at had improved, and her left foot drop had resolved.
56 ms, but again the finding is discounted Long-term follow-up of over 1 year has shown
because of the temperature in the leg; the other F- sustained improvement on low doses of pred-
waves are normal or explained, in the case of the nisone (5 to 10 mg/day).

Clinical Pearls
I. The spectrum of CIDP is wide and includes multi focal variants.
2. The abnormal median normal sural (AMNS) pattern of nerve conduction abnor-
malities is an important indicator of a demyelinating neuropathy.
 PATIENT 34

A 48-year-old man with acute progressive numbness and

paresthesias in his hands and feet and generalized weakness

This 48-year-ald man with AML underwent bane marraw transplant. He develaped law-grade
fever to. 100SF a manth later. Two. days later he nated paresthesias in his hands and feet; an the fal-
lawing day, he nated weakness; and ane day later he was unable to. walk. The electradiagnastic study
w~lsperfarmed 7 days after anset af the paresthesias.
Electrodiagnostic Study:

Sensory NCS

Recarding Onset Peak Amplitude Distance Velacity

Nerve Sites Site (ms) (ms) (flV) (cm) (m/s)

L. median-dig II 1. Wrist Dig II Absent 13

L. ulnar-dig V 1. Wrist Dig V Absent 11
L. radial-sn bax 1. Farearm SnBax 1.85 2.30 5.4 10 54.1
R. median-dig V 1. Wrist Dig II Absent 11
R. ulnar-dig V 1. Wrist DigV Absent 11
L. sural-lat mall 1. Mid-calf Ankle 2.50 3.30 5.5 14 56.0
R. sural-lat mall 1. Mid-calf Ankle 2.95 3.85 9.6 14 47.5

Motor NCS

Recarding Latency Amplitude Distance Velacity

Nerve Sites Site (ms) (mV) (cm) (m/s)

L. median-APB 1. Wrist APB 6.65 1.6 7

2. Elbaw APB 12.70 1.3 23 38.0
L. ulnar-ADM 1. Wrist ADM 4.85 1.8 7
.. 2. B. elbaw ADM 8.50 1.5 21 57.5
3. A. elbaw ADM 11.45 1.2 15 50.8
L. peraneal-EDB 1. Ankle EDB 7.70 0.3 8
2. Fib head EDB 14.05 0.2 26 40.9
3. Pap fassa EDB 17.00 0.1 10 33.9
R. median-APB 1. Wrist APB 10.00 1.2 7
2. Elbaw APB 14.55 0.9 22 48.4
3. Axilla APB 17.70 0.9 12 38.1

F-Wave

Nerve Fmin (ms)

R. median Absent
L. median Absent
L. ulnar Absent
L. peraneal Absent
R. median Absent

140
 EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
L. deltoid NI 0 0 0 NI NI ,NI Full NI
L. triceps NI 0 0 0 NI NI NI Full Mild red
L. first dors int NI 0 0 0 NI NI NI Full Mod red
L. tib ant NI 0 0 0 NI NI NI Sub max Mod red
L. gastroc med Inc 3+ 0 0 NI NI NI Full Sev red
L. vast med NI 0 0 0 NI NI NI Sub max NI

L Sural
..... - Lat Mall

·2

. , ,

10ms 10~V
, . ,

10ms 20~V

R Median - Dig II R, Sur;3.1- Lat Mall

10ms 20~V 10ms 20~V

Questions:
I, What is the clinical differential diagnosis?
2, Assuming the study suggests a generalized peripheral neuropathy, how would you characterize the
process? (See case 32 for an explanation of characterization of neuropathies,)
3, Is there any evidence for a demyelinating neuropathy from the sensory studies? What finding is
illustrated in the figure? Is there any evidence of a demyelinating neuropathy in the EMG studies?

141
 Answers:
1. Guillain-Barre syndrome, vasculitic neuropathy
2. Diffuse symmetric motor and sensory
3. Yes. The abnormal median normal sural pattern is present.

Discussion: The electrodiagnostic study demyelinating neuropathies (see cases 32 and 33).
shows low amplitudes or absent SNAPs for bilat- The needle EMG recruitment patterns without fib-
eral median and ulnar and one radial sensory rillation potentials suggest demyelination,
nerve, but normal sural SNAP amplitudes. The although they are not definitive. Perhaps the most
motor studies are notable for reduced amplitudes compelling evidence for primary demyelination
of all CMAPs studied, prolongation of bilateral comes from the combination of small hand
right > left median, left ulnar, and left per- CMAPs but no fibrillation potentials in FDI, sug-
oneal-EDB distal motor latencies. All F-waves are gesting distal conduction block. Needle EMG of
unobtainable, although CMAP amplitudes are APB or ADM would have been valuable in this
markedly reduced. Needle EMG studies show regard, as we did not actually record an ulnar-FDI
mostly reduced recruitment patterns without CMAP to compare to the ulnar needle EMG study.
fibrillation potentials, although they are seen in This patient has Guillain-Barre syndrome. The
the gastrocnemius. The neuropathy is best charac- CSF WBC cell count was 1 and the protein was
terized clinically and electrophysiologically as dif- 166 mgldl (normal, <50). He was treated with
fuse, symmetric, motor, and sensory. The IYlg (0.4 gm/kglday for 5 days) and rapidly sta-
electrophysiological study gives support, although bilized despite this initial aggressive course (time
is not definitive, for a demyelinating neuropathy. from onset of paresthesias to inability to walk of
The figure demonstrates the typical abnormal only 2 days). Electrodiagnostic studies 2 weeks
median normal sural (AMNS) pattern of sensory later, terminated prematurely by the patient's res-
abnormalities seen in acute and chronic acquired piratory difficulties, were as follows:

Sensory NCS

Recording Onset Peak Amplitude Distance

Nerve Sites Site (ms) (ms) (JlY) (cm)

L. radial-sn box 1. Forearm Sn box Absent

Motor NCS

Nerve Sites Recording Site Latency (ms) Amplitude (mY) Distance (cm)

L. median-APB 1. Wrist APB Absent 7

L. ulnar-ADM 1. Wrist ADM 12.5 0.9 7

There has been further progression of axonal distal motor latency of 12.5. Clinical follow-up 6
loss and now very clear-cut focal demyelination months later showed marked recovery of motor
of the ulnar-ADM across-wrist segment, with a function with independent ambulation.

Clinical Pearls
1. The electrophysiology of Guillain-Barre syndrome was discussed in case 32 in the
setting ofCIDP and shares similar features; however, there are also important differences.
2. The acute presentation of patients with Guillain-Barre syndrome results more
often in less substantial electrophysiological abnormalities, and a significant number of
patients with GBS have no electrophysiological abnormalities.
3. It is likely that F-wave abnormalities are in general the earliest abnormal and most
sensitive studies in the diagnosis of Guillain-Barre syndrome.
4. Sequential studies demonstrating evolution of initially mild abnormalities can be
helpful in confirming the diagnosis.

142
REFERENCES
I. Alam, T.A., Chaudhry, v., Cornblath, D.R., Electrophysiological studies in the Guillain-Barre syndrome: distinguishing
subtypes by published criteria, Muscle Nerve 1998; 21(10): 1275-1279.
2. Albers, 1.w., Donofrio, P.D., McGonagle, T., Sequential electrodiagnostic abnormalities in acute inflammatory
demyelinating polyradiculoneuropathy, Muscle Nerve] 985; 8:528-539.
3. Albers, 1.w., Kelly, J.1., Jr., Acquired inflammatory demyelinating polyneuropathies: clinical and electrodiagnostic features,
Muscle Nerve 1989; 12(6):435--45l.
4. Asbury, AX., Cornblath, D.R., Assessment of current diagnostic criteria for Guillain-Barre syndrome, Ann. Neural. 1990;
27(suppl.):S21-S24.
5. Cornblath, D.R., Electrophysiology in Guillain-Barre syndrome, Ann. Neurol. 1990; 27(suppl.):S 17-S20.
6. Meulstee, 1., van der Meche, EG., Electrodiagnostic criteria for polyneuropathy and demyelination: application in 135
patients with Guillain-Barre syndrome, Dutch Guillain-Barre Study Group, J Neural. Neurosurg Psychiatry 1995; -
59(5):482--486.
7. Olney, R.K., Aminoff, M.1., Electrodiagnostic features of the Guillain-Barre syndrome: the relative sensitivity of different
techniques, Neurology 1990; 40:471--475.

143
 PATIENT 35

A 49-year-old woman with numbness in her right hand and

both feet

This 49-year-old woman noted episodes 3 years ago of continuous numbness in her right hand last-
ing days to weeks and progressing to continuous numbness in digits 2 to 4 and lateral palm. Two years
ago she developed numbness in her feet limited to the ventral surfaces of digits 2 and 3 bilaterally and in
the right medial leg from the ankle to the knee. Symptoms have remained unchanged for the last year .
. Neurological examination was notable for mild weakness of right APB and otherwise normal
strength. Light touch sensation was impaired in the right median, saphenous, and bilateral distal medial
plantar nerves. Reflexes are listed below:

Side Biceps Triceps Knees Ankles

R 2+ 2+ Absent Absent
L 2+ Absent 2+ Absent

Try to determine the clinical differential diagnosis. Consider the appropriate initial electrodiagnostic
plan.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (~V) (em) (m/s)

R. median-dig II 1. Wrist Dig II 2.4 3.3 2.4 13 54

L. ulnar-dig V 1. Wrist DigV 1.8 2.5 27.1 11 61
R. sural-l at mall 1. Mid-calf Ankle 2.8 3.65 7.4 14 50
L. sural-Iat mall 1. Mid-calf Ankle 2.9 3.60 8.9 14 48

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (em) (m/s)

R. median-APB 1. Wrist APB 3.2 6.6 7

2. Elbow APB 8.9 2.2 19 33
3. Axilla APB 14.9 0.8 12 20
L. median-APB 1. Wrist APB 3.2 9.6 7
2. Elbow APB 7.0 9.2 21 55
3. Axilla APB 8.7 9.0 12 71
R. ulnar-ADM 1. Wrist ADM 2.5 7.5 7
2. B. elbow ADM 5.5 6.5 18 60
3. A. elbow ADM 7.7 6.0 15 68
R. peroneal-EDB I. Ankle EDB 4.1 5.2 8
2. Fib head EDB 9.9 4.9 28 48
3. Pop fossa EDB 12.1 2.4 10 46

144
 F-Wave

Nerve Fmin (ms) Fma" (ms) %F

R. median Absent
L. median 27.4 29.0 100
L. peroneal 42.5 45.0 100

EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. abd poll brev Inc 2+ 2+ 0 NI NI NI Full MildRed

R. pron teres Nl 0 0 0 NI NI Nl Full Normal
R. flex poll long Nl 0 0 0 NI Nl Nl Full Normal
R. flex dig supf NI 0 0 0 NI Nl NI Full Normal
R. first dors inteross NI 0 0 0 Nl Nl NI Full Normal
R. vast med NI 0 0 0 Nl NI NI Full Norma'l

R Median - APB

3 ms

Wrist

. Axilla
Figure 1

145
 R Pe.rsoneaJ - ED!:}
• 2

.Ankle
2

2 ms

FibHead

Pop Fossa
Figure 2

Questions:
1. What do the figures show?
2. How would you interpret the electrodiagnostic study?

146
 Answers:
I. Conduction block
2. It suggests chronic inflammatory demyelinating polyneuropathy (CIDP).

Discussion: The clinical presentation is that These findings taken together are those ofmul-
of a chronic multi focal and asymmetric neuropa- tifocal CIDp, although conceivably the inherited
thy. The differential diagnosis is that discussed in neuropathy X-linked Charcot-Marie- Tooth
the introduction to this section in Table 6. It (CMT-X) could produce this picture as well.
includes vasculitis, multi focal CIDP (also Vasculitic neuropathy would not likely produce
referred to as multi focal acquired demyelinating the finding of presumably long-standing conduc-
sensory and motor neuropathy, or MADSAM), tion block in the median nerve as in this case.
and a variety of other causes. Follow-up electrodiagnostic studies 2 months
The first figure shows conduction block of the later were unchanged, with continued right
right median-APB motor nerve in the forearm, median forearm conduction block.
temporal dispersion in the upper arm, and focal It should be emphasized that CIDP may be
slowing in both locations. As the patient's median strikingly asymmetric and multi focal despite gen-
distribution hand numbness developed 3 years erally accepted criteria for symmetry. Such multi-
prior and she has not noted any change in her focal forms ofCIDP are important to recognize as
symptoms for I year, it is unlikely to be due to such because they share the excellent treatment
acute nerve infarction (pseudo-conduction block; responsiveness to prednisone that symmetric and
see case 31). The second figure shows conduction confluent CIDP typically has and should thus be
block of the right peroneal-EDB motor nerve treated the same way. This stands in marked
across the fibular head. contrast to the syndrome of multi focal motor
The electrodiagnostic study is abnormal for the neuropathy (MMN), which does not respond to
conduction block and focal slowing in the right prednisone and is best not classified or confused
median and peroneal nerves noted above, as well with CIDP and its variants. MMN is discussed
as absent median F-wave responses despite pre- in greater detail through several cases in Section
served distal CMAP amplitude, a corroborative IV on motor neuropathies and motor neuron
finding of demyelination proximal to the wrist. In diseases.
addition, the median-D2 SNAP is reduced.

Clinical Pearl
CIDP may be strikingly asymmetric and multifocal despite generally accepted criteria
for symmetry

REFERENCES
I. Lewis, R.A., Sumner, AJ., Brown, MJ., Asbury, A.K., Multifocal demyelinating neuropathy with persistent conduction
block, Neurology 1982; 32(9):958-964.
2. Saperstein, D.S., Amato, A.A., Wolfe, 0.1., Katz, 1.S., Nations, S.P., Jackson, C.E., Bryan, WW, Bums, O.K., Barohn, RJ.,
Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis Sumner syndrome, Muscle Nerve 1999;
22:560--566.
3. Saperstein, D.S., Katz, 1.S., Amato, A.A., Barohn, RJ., Clinical spectrum of chronic acquired demyelinating neuropathies,
Muscle Nerve 2001; 24:311-324.
4. Van den Berg-Vos, R.M., Van den Berg, L.H., Franssen, H. et al., Multifocal inflammatory demyelinating neuropathy: a
distinct clinical entity?, Neurology 2000; 54:26-32.

141
 PATIENT 36

A 35-year-old woman with numbness in her hands and feet for 3

weeks and a right facial palsy for several days

Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (IlV) (cm) (mls)

R. sup peroneal 1. Lat leg Ankle 2.85 3.60 8.9 12 42.1

R. sural-I at mall I. Mid-calf Ankle 3.30 3.90 11.7 14 42.4
L. sural-Iat mall I. Mid-calf Ankle 3.50 4.05 15.8 14 40.0
R. median-dig II 1. Wrist Dig II 2.25 2.95 36.9 13 57.8
R. ulnar-dig V 1. Wrist DigV 2.15 2.85 28.5 11 51.2

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (mls)

R. median-APB 1. Wrist APB 3.85 7.3 7

2. Elbow APB 8.10 6.8 21 49.4
R. ulnar-ADM 1. Wrist ADM 3.00 9.5 7
2. B. elbow ADM 6.35 8.8 18 53.7
3. A. elbow ADM 9.75 5.9 12.5 36.8
R. ulnar-FDI' 1. Wrist . FDI 5.30 8.7
2. B. elbow FDI 8.45 7.3 19 60.3
3. A. elbow FDI 10.75 5.2 11 47.8
R. ulnar-inching 1. Wrist ADM 3.40 9.6 7
2. Elb - 5 ADM 6.60 9.1 17 53.1
3. Elb - 2.5 ADM 6.95 9.0 2.5 71.4
4. Elb ADM 7.80 7.8 2.5 29.4
5. Elb + 2.5 ADM 9.05 6.2 2.5 20.0
6. Elb + 5 ADM 9.55 6.1 2.5 50.0
L. ulnar-ADM 1. Wrist ADM 3.10 8.3 7
2. B. elbow ADM 6.85 7.2 21 56.0
3. A. elbow ADM 10.55 5.6 11 29.7
R. peroneal-EDB 1. Ankle EDB 10.55 4.9 7
2. Fib head EDB 16.50 4.1 30 50.4
3. Pop fossa EDB 19.70 4.1 9 28.1
R. tibial-AH 1. Ankle AH 7.10 6.6 8
2. Pop fossa AH 16.25 5.2 38 41.5
L. peroneal-EDB 1. Ankle EDB 15.85 2.2 8
2. Fib head EDB 23.80 2.1 30 37.7
3. Pop fossa EDB 26.05 2.1 10 44.4
L. tibial-AH 1. Ankle AH 7.00 8.5 8
2. Pop fossa AH 15.95 5.5 44 49.2

148
 F-Wave

Nerve Fmin (ms) F max (ms) Max - Min (ms) %F

R. median 28.05 31.65 3.60 80

R. ulnar 33.10 35.20 2.10 80
L. peroneal 48.50 50.10 1.60 30
R. peroneal ' A-waves present
R. tibial A-waves present
L. tibial A-waves present

EMG Summary Table

SA Amplitude Duration

PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. first NI 0 0 0 NI Nl NI Full Nl
dors int
R, tib ant Nl 0 0 0 NI NI Nl Full Nt:

1.2

13

1.4'

1,S.

1,6

1.7
1,8

1,9

. 1,10'

. 1.11.
lOOms 500l-N

Right peroneal Right tibial

Question: What do the arrows demonstrate in the figure, and what is the significance of this
finding?

149
 Answer: A-waves suggest a demyelinating neuropathy.

Discussion: The results of nerve conduction and a resulting reentrant electrical impulse at
studies are abnormal for: (1) prolongation of submaximal stimulation. The most common use
bilateral peroneal-EDB distal motor latencies, of the term A-wave, however, is to describe late
(2) focal slowing of bilateral ulnar-ADM across- responses that appear at supramaximal stimula-
elbow motor segments, (3) focal slowing of the tion with constant shape and latency (unlike
right peroneal-EDB across-fibular-head motor F-waves). Although the presence of A-waves in a
segment, (4) prolongation of the right ulnar- single nerve may occur in up to 5% of asympto-
ADM minimum F-wave latency, and (5) the pres- matic individuals, multiple A-waves have diagnos-
ence of A-waves during F-wave studies. The tic relevance. The electrodiagnostic significance of
results of limited EMG studies were normal. multiple, supramaximally stimulated A-waves has
The figure shows the presence of A-waves in the received recent attention,l,4 and demyelinating
right peroneal and tibial nerves, and the table neuropathies, both acquired and inherited, are
indicates that they are present in the left tibial highly associated with them. The presence of
nerve as well. A-waves in multiple nerves, especially if the
The sensory and motor nerve conduction stud- waveforms are complex, supports the electrodiag-
ies alone suggest a demyelinating neuropathy. nosis of a primary demyelinating neuropathy. The
There is focal slowing not just at the common mechanism is unknown, but ephaptic connection
compression sites but also for the peroneal-EDB of neighboring demyelinated axons is one possi-
segments at the ankles, with markedly increased ble mechanism.
distal motor latencies of 10.55 and 15.85 ms. The patient's clinical diagnosis was Guillain-
The nomenclature of late responses being Barre syndrome; cerebrospinal fluid protein was
termed A-waves is not well established. There are normal (36 mgldl), and she was not treated
several distinct phenomena. Rare afterdischarges because she was almost 4 weeks into the illness
that occur at submaximal stimulation of motor with findings limited to numbness and pare-
nerves and disappear with increasing stimulus sthesias in her hands and a right facial palsy.
intensity have been called an axon reflex; these However, she continued to progress over the ensu-
afterdischarges are better viewed as delayed ing weeks and returned with progressive general-
components of the M-response and not as late ized weakness at 10 weeks since onset of hand
responses. The motor axon loop is another numbness. Results of follow-up studies 3 weeks
A-wave attributed to collateral axonal branches later follow:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (flV) (cm) (m/s)

R~ ulnar-dig V 1. Wrist Dig V 2.55 3.65 12.8 11 43.1

L. sural-Iat mall 1. Mid-calf Ankle 3.05 3.75 14.6 14 45.9
R. median-dig II . 1. Wrist Dig II 2.65 3.65 16.3 13 49.1
R. sural-I at mall 1. Mid-calf Ankle 2.75 3.65 17.2 14 50.9

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (rn/s)

R. median-APB 1. Wrist APB 6.85 5.0 7

2. Elbow APB 11.00 4.7 24 57.8
3. Axilla APB 13.80 4.1 17 60.7
L. median-APB 1. Wrist APB 7.70 4.3 7
2. Elbow APB 13.10 4.3 23 42.6
3. Axilla APB 15.45 4.2 17.5 74.5

159
 Motor NCS--cont'd

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)

R. ulnar-ADM I. Wrist ADM 5.05 7.0 7

2. B. elbow ADM 8.45 6.4 18 52.9
3. A. elbow ADM 12.60 5.1 10 24.1
4. Axilla ADM 15.50 4.8 13.5 46.6
L. ulnar-ADM I. Wrist ADM 5.85 5.4 7
2. B. elbow ADM 8.95 4.2 17 54.8
3. A. elbow ADM 13.95 3.7 10 20.0
4. Axilla ADM 15.95 3.5 13 65.0 '
R. peroneal-EDB 1. Ankle EDB 14.45 2.6 8
2. fib head EDB 22.65 1.9 30 36.6
3. Abv fib H EDB 25.10 2.0 8.5 34.7
L. peroneal-EDB I. Ankle EDB 22.85 0.3 8
2. Fib head EDB 29.75 0.2 30.5 44.2
3. Abv fib H EDB 32.20 0.2 9 36.7
R. tibial-AH I. Ankle AH 6.70 1.4 8
2. Pop fossa AH 15.50 1.1 40 45.5
L. tibial-AH 1. Ankle AH 8.90 2.0 8
2. Pop fossa AH 18.95 1.6 38 37.8

F-Wave

Nerve F min (ms) F max (ms)

R. peroneal A-wave at 68.10
R. tibial A-wave at 30.10
L. tibial Absent
L. peroneal A-wave at 35.10
R. median 34.40 39.20
R. ulnar 35.85 39.95
L. median 35.75 38.10
L. ulnar 35.55 45.15

EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
L. first dors int NI 0 0 0 NI NI Nl Full Mild red
L. ext dig comm NI 0 0 0 NI NI Nl Full Mild red.
L. vast med NI 0 0 0 NI NI NI Full Nl
L. tib ant NI 0 0 0 NI NI NI Full Nl
L. tib post NI 0 0 0 NI Nl NI Full NI
L. gastroc med Nl 0 0 0 NI NI NI Full NI
L. flex dig long NI 0 0 0 NI NI NI Full NI
L. ext dig brev Nl 0 0 0 Nl Nl Nl Full NI

151
 In comparison to the prior studies, this study is The patient's CSF protein was now 51 mgldl
remarkable for: (I) reduction in right median-D2 (normal, <44 mgldl). Her continued progression
and ulnar-D5 SNAP amplitudes, (2) prolongation of disease at 10 weeks led to revision of her
of all distal motor latencies (i.e., left ulnar 3.10 ~ diagnosis as CIDp, and treatment with IVIg was
5.85 ms, right median 3.85 ~ 6.85 ms, left per- followed by oral prednisone, with sustained sub-
oneal 15.85 ~ 22.85 ms), (3) increased focal stantial improvement. CIDP may present acutely2
slowing of bilateral ulnar-ADM across-elbow and is difficult if not impossible to distinguish
motor segments (right 48 ~ 24 mis, left 30 ~ 20 from Guillain-Barre syndrome at onset; however,
mls), and (4) further prolongation and reduced continued disease progression past 4 weeks is
persistence of median and ulnar minimum F-wave uncommon for Guillain-Barre, and past 8 weeks
latencies. The results ofEMG studies showed mild is highly unusual.
reductions in motor unit recruitment as tabulated.

Clinical Pearls
I. The presence of A-waves in multiple nerves, especially if the waveforms are com-
plex, supports the electrodiagnosis of a primary demyelinating neuropathy.
2. CIDP may present acutely and is difficult if not impossible to distinguish from
Guillain-Barre syndrome at onset.

REFERENCES
I. Komhuber, M.E., Bischoff, C., Mentrup, H., Conrad, 8., Multiple A waves in Guillain-Barre syndrome, Muscle Nerve
1999; 22:394-399.
2. Mori, K., Hattori', N., Sugiura, M., Koike, H., Misu, K., Ichimura, M., Hirayama, M., Sobue, G., Chronic inflammatory
demyelinating polyneuropathy presenting with features ofGBS, Neurology 2002; 58(6):979-982.
3. Roth, G., Egloff-Baer, S., Motor axon loop: an electroneurographic response, Muscle Nerve 1984; 7:294-297.
4. Rowin, 1., Meriggioli, M.N., The electrodiagnostic significance of supramaximally stimulated A-waves, Muscle Nerve
2000; 23:1117-1120.

152
 PATIENT 37

A 74-year-old woman with progressive weakness in her hands

This 74-year-old woman was referred by an orthopedic surgeon who noted diffuse atrophy and
weakness of her hands. She complained of increasing weakness and atrophy for several years without
paresthesias or reduction in sensation. She reported a mother and sister with high arches in their fee"t
and atrophy of their hands similar to hers.
Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (~V) (cm) (rnIs)
R. median-dig II \. Wrist Dig II 5.75 8.40 3.6 13 22.6
L. median-dig II \. Wrist Dig II 7.55 8.70 2.3 13 17.2
R. ulnar-dig V \. Wrist DigV Absent II
L. ulnar-dig V \. Wrist DigV 1\.05 13.65 1.3 II 10.0
R. radial-sn box \. Forearm Digit I Absent 13
L. radial-sn box \. Forearm Digit I 8.90 10.60 \.8 13 14.6
L. sural-Iat mall \. Mid-calf Ankle 10.40 12.70 1.4 14 13.5

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (~V) (cm) (rnIs)
R. median-APB \. Wrist APB 9.30 \.9 7
2. Elbow APB 21.15 \.2 2\.5 18.1
L. median-APB \. Wrist APB 11.20 0.9 7
2. Elbow APB 20.40 0.6 20 2\.7
3. Axilla APB 25.30 0.7 12 24.5
R. ulnar-ADM \. Wrist ADM 8.15 \.2 7
2. A. elbow ADM 23.70 0.9 26 16.7
L. ulnar-ADM \. Wrist ADM 6.45 4.2 7
2. B. elbow ADM 17.05 4.2 16.5 15.6
3. A. elbow ADM 22.10 4.0 10 19.8
4. Axilla ADM 27.95 3.5 12.5 2 \.4
L. peroneal-EDB 1. Ankle EDB Absent 8
L. peroneal-tib ant 1. Fib head Tib ant 7.30 2.5 7
2. Pop fossa Tib ant 10.95 2.1 9 24.7
L. tibial-AH 1. Ankle AH 17.50 0.1 8
2. Pop fossa Absent

F-Wave
Nerve F min (ms) F max (ms) Max - Min (ms) %F
R. median Absent
R. ulnar Absent
L. median Absent Reproducible A-waves
L. ulnar Absent Reproducible A-waves

15]
 Questions:
1. Would you characterize this neuropathy as primarily axonal or demyelinating?
2. Does the electrophysiology support an acquired or inherited disorder? Why?

154
 Answers:
1. Demyelinating
2. Inherited disorder

Discussion: These studies are notable for dif- patients with intermediate conduction velocities
fusely reduced sensory nerve and compound mus- of 30 to 40 mis, with velocities of 15 to 30 mls
cle action potential amplitudes and marked classified as demyelinating type I and velocities
slowing of conduction velocities. Every nerve seg- of >40 mls as axonal type II.
ment examined has marked slowing, with distal A publication of Lewis and Sumner in 19825
sensory velocities ranging from 10 to 23 mis, has been influential in emphasizing the electrodi-
motor conduction velocities ranging from 16 to 25 agnostic distinction of inherited versus acquired
mis, and distal motor latencies of 6.5 to 9.3 ms in demyelinating neuropathy. This paper empha-
the hands (the 17.5 ms in the tibial-AH study sized the characteristic features of inherited
could reflect the difficulty in measuring an accu- demyelinating neuropathies: uniform conduction
rate take-off of a very small potential). In addition, slowing and the absence of conduction block. The
there are multiple A-waves (present in more than I presence of nonuniform slowing (substantial dif-
nerve; see case 36). These features are those of a ferences in segmental conduction velocities, with
demyelinating neuropathy, and the relatively uni- a mixture of normal and abnormal segments) or
form slowing of nerve conduction velocities is a of conduction block is instead characteristic of an
characteristic feature of inherited neuropathies. acquired demyelinating neuropathy. Two excep-
Dyck and Lambert in 1968 classified domi- tions to this rule have required a revision of this
nantly inherited neuropathies as axonal or view36: X-linked Charcot-Marie- Tooth disease
demyelinating based on a single parameter: the (CMT-X) and hereditary neuropathy with liability
ulnar forearm motor conduction velocity, with 38 to pressure palsies (HNPP) may have nonuniform
mls being the cutoff for demyelinating versus slowing and conduction block. Other exceptions
axonal. Refinements in the 1970s identified may occur as well (see case 38).

Clinical Pearl
Inherited demyelinating neuropathies, with the exception ofCMT-X and HNPP, are par-
tially distinguishable from acquired demyelinating neuropathies by their uniform con-
duction slowing and absence of conduction block; however, exceptions do occur, and
the advent of molecular genetics is increasingly invalidating this basic principle of elec-
trodiagnostic medicine.

REFERENCES
I. Boerkoel, C.F., Takashima, H., Garcia, C.A., Olney, R.K., Johnson, J, Berry, K., Russo, P., Kennedy, S., Teebi, A.S.,
Scavina, M., Williams, L.L., Mancias, P., Butler, I.J., Krajewski, K., Shy, M., Lupski, JR., Charcot-Marie-Tooth disease
and related neuropathies: mutation distribution and genotype-phenotype correlation, Ann. Neurol. 2002; 51: 190-20 I.
2. Dyck, PJ., Lambert, E.H., Lower motor and primary sensory neuron diseases with peroneal muscular atrophy. 1
Neurologic, genetic, and electrophysiolgical findings in hereditary polyneuropathies, Arch. Neurol. 1968; 18:603-618.
3. Kaku, D.A., Parry, GJ., Malamut, R., Lupski, JR., Garcia, C.A., Uniform slowing of conduction velocities in
Charcot-Marie-Tooth polyneuropathy type I. Neurology 1993; 43:2664-2667.
4. Kaku, D.A., Parry, GJ., Malamut, R., Lupski, JR., Garcia, C.A., Nerve conduction studies in Charcot-Marie-Tooth
polyneuropathy associated with a segmental duplication of chromosome 17, Neurology 1993; 43: 1806-1808.
5. Lewis, R.A., Sumner, AJ., The electrodiagnostic distinctions between chronic familial and acquired demyelinative
neuropathies, Neurology 1982; 32:592-596.
6. Lewis, R.A., Sumner, AJ., Shy, M.E., Electrophysiological features of inherited demyelinating neuropathies: a reappraisal
in the era of molecular diagnosis, Muscle Nerve 2000; 23: 1472-1487.
7. Pareyson, D., Charcot-Marie- Tooth disease and related neuropathies: molecular basis for distinction and diagnosis, Muscle
Nerve 1999; 22:1498-1509.

155
 PATIENT 38

A 68-year-old woman with slowly progressive weakness in her legs

without paresthesias

Electrodiagnostic Study:
, . SensoryNCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site Response (ms) (ms) (~V) (cm) (m/s)
R. radial- 1. Forearm SnBox 1.8 2.6 21.5 10 56
sn box
R. sural- 1. Mid-calf Ankle Absent 14
lat mall'

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (rn/s)
L. ulnar-ADM 1. Wrist ADM 3.2 8.4 7
2. B. elbow ADM 6.5 7.6 15 45
3. A. elbow ADM 8.3 7.3 10 56
4. Axilla ADM 9.8 7.9 10 67
L. median-APB 1. Wrist APB 4.7 5.7 7
2. Elbow APB 8.3 4.8 53
R. peroneal-EDB 1. Ankle EDB 8.7 0.3 8
2. Fib head EDB 17.3 0.3 28 32
3. Pop fossa EDB 20.7 0.2 10 29
L. peroneal-EDB 1. Ankle EDB 4.0 0.3 8
2. Fib head EDB 11.6 0.4 26 34
3. Pop fossa EDB 16.1 0.4 10 22
R. peroneal- TA 1. Fib head TA 2.7 3.6 8
2. Pop fossa TA 6.2 2.5 28 32
L. tibial-AH 1. Ankle AH 6.1 0.5 8
2. Pop fossa AH 20.4 0.5 34 24

EMG studies were limited to right tibialis anterior, showing 2+ fibrillation potentials, large ampli-
tude and long-duration motor unit action potentials, and moderately reduced recruitment.

Question: Do the electrodiagnostic studies suggest an inherited or an acquired demyelinating

neuropathy?

156
 Answer: Acquired

Discussion: This study shows axonal degen- mal other segments. In fact, the range of motor
eration of sensory and motor axons in the feet, velocities is 22 to 67 mis, suggesting that this
with an absent sural SNAP and reduced ampli- patient has an acquired demyelinating neuropathy
tudes of bilateral peroneal-EDB and left tib- (see discussion for case 37).
ial-AH CMAPs; however, the motor velocities in Genetic testing in this patient revealed a point
some of the leg segments (left tibial leg, 24 mlsec; mutation in the myelin protein zero (MPZ or PO)
right peroneal leg, 32 m/sec; left peroneal across gene. Accordingly, by genetic classification, this
fibular head, 22 mls) are slower than one would patient has CMTlb (mutations in MPZ), a
feel comfortable with attributing to the axonal demyelinating neuropathy. As noted in the forth-
degeneration alone. The motor velocities in coming discussion for case 40, some patients pre-
nerves eliciting preserved CMAP amplitudes, viously classified electrodiagnostically as axonal
such as the median and ulnar, show a borderline CMT (CMT2) in fact have mutations in the MPZ
reduced ulnar forearm velocity (45 mls) but nor- gene.

Clinical Pearl
Some patients with MPZ mutations have borderline ulnar nerve conduction velocities
not readily classified as axonal or demyelinating neuropathies.

REFERENCES
I. Marrosu, M.G., Vaccargiu, S., Marrosu, G., Vannelli, A., Cianchetti, C., Muntoni, E, Charcot-Marie-Tooth disease type 2
associated with mutation of the myelin protein zero gene, Neurology 1998; 50: 1397-140 I.
2. Mastaglia, EL., Nowak, K.1., Stell, R., Phillips, B.A., Edmondston, 1.E., Dorosz, S.M., Wilton, S.D., Hallmayer, 1.,
Kakulas, B.A., Laing, N.G., Novel mutation in the myelin protein zero gene in a family with intermediate hereditary motor
and sensory neuropathy, J. Neurol. Neurosurg. Psychiatry 1999; 67(2): 174-179.

15.7
 PATIENT 39

A 32-year-old woman with a severe neuropathy since childhood

This 32-year-old woman had significant developmental delay; she learned to walk with help at the
age ·(jf 3 but then required a wheelchair within several years and remained stable into her 20s. At
th~ age of IS, the ulnar motor nerve conduction velocity was II rn/s (CMAP amplitude not known).
Nerve biopsy was abnormal with proliferation of Schwann cells in an onion bulb appearance. Marked
thoracolumbar scoliosis led to surgery with placement of Harrington rods. Over the last 5 to 10 years,
she has noticed increasing weakness in her hands. There was no family history of a neuropathy in either
parent or several siblings.
Neurological examination showed severe weakness of distal and mild weakness of proximal mus-
cles in all limbs. There was marked loss of position sense, and the patient was unsteady sitting in her
wheelchair with eyes closed.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Amplitude Distance Velocity

Nerve Sites Site Response (ms) (mV) (cm) (rn/s)

L. median--<iig II 1. Wrist Dig II No 13

L. ulnar--<iig V 1. Wrist DigV No II
L. radial-sn box 1. Forearm Sn box No to
L. sural 1. Mid-calf Ankle No 14
R. median--<iig V 1. Wrist Dig II No 13
R. ulnar--<iig V 1. Wrist DigV No 11
R.flidial-sn box 1. Forearm Sn box No to

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site Response (ms) (mV) (cm) (rn/s)

L. median-APB 1. Wrist APB No 7

L. ulnar-ADM 1. Wrist ADM No 7
R. median-APB 1. Wrist APB No 7
R. ulnar-ADM 1. Wrist ADM No 7

Question: Assuming this patient has an inherited neuropathy, do you think it is dominantly or
recessively inherited?

158
 Answer: It cannot be determined.

Discussion: The complete absence of sen- point mutation (C264T). This patient has a PMP-
sory and motor responses indicates a severe 22 point mutation with a CMT3 phenotype.
generalized neuropathy but gives little other The severe phenotype of CMT-3, known as
information. The previous history of an ulnar Dejerine-Sottas disease or congenital hypomyeli-
motor nerve conduction velocity of II m/s sug- nation neuropathy, was once classified as a reces-
gests that previously there was evidence of pri- sive demyelinating disorder. It has since become
mary demyelination, as did the nerve biopsy. clearer that most cases are dominant de novo
Could this patient have chronic inflammatory mutations. Patients with a broad range of affected
demyelinating polyneuropathy, which is treat- genes have been reported with this phenotype,
able? The definitive answer came from sequenc- including point mutations of PMP22, MPZ,
ing of her PMP-22 gene, which demonstrated a EGR2, and PRX (periaxin).

Clinical Pearls
I. Severe slowing of nerve conduction velocities is characteristic of CMT-3, which
may result from a variety of mutations in different genes. The disorder is mostly auto-
somal dominant, although a high spontaneous mutation rate results in de novo cases
with no previous family history.
2. The availability of genetic testing for this disorder should replace the use of nerve
biopsy for diagnosis.

REFERENCES
I. Boerkoel, C.F., Takashima, H., Stankiewicz, P., Garcia, C.A., Leber, S.M., Rhee-Morris, L., Lupski, J.R., Periaxin
mutations cause recessive Dejerine-Sottas neuropathy, Am. J. Hum. Genet. 2001; 68:325-333.
2. Fabrizi, G.M., Simonati, A., Taioli, F., Cavallaro, T., Ferrarini, M., Rigatelli, F., Pini, A., Mostacciuolo, M.L., Rizzuto; N.,
PMP22 related congenital hypomyelination neuropathy, J. Neurol. Neurasurg. Psychiatry 200 I; 70: 123-126.
3. Fabrizi, G.M., Cavallaro, T., Morbin, M., Simonati, A., Taioli, F., Rizzuto, N., Novel mutation of the PO extracellular
domain causes a Dejerine-Soltas syndrome, J. Neural. Neurosurg. Psychiatry 1999; 66:386-389. ,
4. Ionasescu, v.v., Ionasescu, R., Searby, c., Neahring, R., Dejerine-Soltas disease with de novo dominant point mutation' of
the PMP22 gene, Neurology 1995; 45:1766-1767.
5. lonasescu, v.v., Searby, C., Greenberg, S.A., Dejerine-Soltas disease with sensorineural hearing loss, nystagmus, and
peripheral facial nerve weakness: de novo dominant point mutation of the PMP22 gene, J. Med. Genet. 1996;
33:1048-1049.
6. Warner, L.E., Shohat, M., Shorer, Z., Lupski, J.R., Multiple de novo MPZ (PO) point mutations in a sporadic
Dejerine-Sottas case, Hum Mutat. 1997; 10:21-24.

159
 PATIENT 40

A 33-year-old woman with long-standing asymmetric weakness in

all four limbs and a family history of neuropathy

This 33-year-old woman has noticed weakness of alllimbs, greater in the right foot and hand than
the left:, since her early 20s with gradual progression since. Her family history is remarkable for a neu-
ropathy affecting males considerably more severely than females and a lack of male-male transmis-
sion. In particular, her mother developed a neuropathy in her 20s and is still ambulatory while her
mother's brother (M.) was severely affected at age 5. M. has a healthy son and an affected daughter
(S.). S. has 3 boys, one of whom is known to be affected. The patient has a severely affected brother
who was diagnosed at age 8, 3 normal sisters, and 4 other normal brothers. The neurological exam for
strength in several muscles is noted below:

Legs APB FDI TA

Right o 3 o
Left: 4 4 4-

Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (~V) (cm) (m/s)
R. median~ig II 1. Wrist Dig II Absent
L. median~ig V 1. Wrist Dig II Absent
R. ulnar~ig V 1. Wrist Dig V Absent
L. ulnar~ig V 1. Wrist DigV Absent
R. radial-sn box 1. Forearm Sn box Absent
L. radial-sn box 1. Forearm Sn box Absent
R. sural-lat mall 1. Mid-calf Ankle Absent
L. sural-lat mall 1. Mid-calf Ankle Absent

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)
R. median-APB 1. Wrist APB 6.0 1.1 7
2. Elbow APB 14.1 1.1 23 28
L. median-APB 1. Wrist APB 5.5 4.2 7
2. Elbow APB 12.0 3.6 21 32
R. ulnar-ADM 1. Wrist ADM 3.4 3.0 7
2. B. elbow ADM 7.3 2.4 19 49
3. A. elbow ADM 9.8 2.3 10 38
L. ulnar-ADM 1. Wrist ADM 4.0 4.4 7
2. B. elbow ADM 8.7 4.0 19 40
3. A. elbow ADM 11.2 3.9 10 40
R. peroneal-EDB 1. Ankle EDB Absent 0.0 8

160
 Motor NCS--cont'd

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)
L. peroneal-EDB 1. Ankle EDB Absent 0.0 8
R. peroneal- TA 1. Fib head TA 4.7 0.2 8
2. Pop fossa TA 6.0 0.2 8S 57
L. peroneal- TA 1. Fib head TA 3.0 2.1 7
2. Pop fossa TA 5.3 0.8 8 35
R. tibial-AH 1. Ankle AH 6.0 0.1 8
2. Pop fossa AH Absent 0.0
L. tibial-AH 1. Ankle AH Absent 0.0

F-Wave

Nerve Fmin (ms)

R. median Absent
L. median 25.9
R. ulnar 30.2
L. ulnar 30.2

Question: What are the clinical diagnostic considerations?

"
 Answer: Inherited neuropathy, X-linked dominant

Discussion: The patient has an inherited neu- asymmetric and multi focal involvement. The
ropathy with greater severity of males and·absence greater severity in males and lack of male-male
of male-male transmission. This pattern of inheri- transmission in this case strongly suggest CMT-
tance suggests an X-linked dominant disorder with X. Genetic testing of this patient showed intact
partial expression in female carriers. The remark- PMP-22 genes with normal sequences and a point
able aspects of the limited motor strength testing mutation in the coding region of Cx32 (832deIT).
exam results are the marked asymmetry and multi- The history of classification of CMT-X reflects
focality. Certainly, the right TA and APB are dra- its unusual inheritance pattern. Premolecular classi-
matically different than the left, establishing fication of CMT looked something like the first
the asymmetry. Furthermore, the right median- figure. The CMT dominant intermediate velocities
innervated APB muscle is also more severely (ulnar motor forearm velocities 000 to 40 m/s) cat-
affected than the right ulnar-innervated FDI, sug- egory fell between the strict criteria for demyelinat-
gesting multifocality as well. Clinically, these fea- ing versus axonal neuropathies of the 1970s.
tures together suggest an asymmetric multi focal Subsequent discoveries, particularly in molecular
X-linked dominantly inherited neuropathy. genetics, have made it clear that most of these CMT
The electrodiagnostic study provides further dominant intermediate families were indeed CMT-
information. There is evidence for primary X, and most of the remainder of these patients have
demyelination in the right median-APB forearm point mutations of myelin protein zero (MPZ;
motor segment, with a velocity of 28 mis, less CMTl-b), as shown in the second figure.
than 70% the lower limit of normal of 44 mls
(=31 mls; see case 32 for discussion of criteria for
primary demyelination). The left median-APB
forearm motor segment velocity of 32 mls meets Pre-Molecular Genetic
such criteria as well (the relative preservation of Classification of CMT
the distal evoked CMAP amplitude would lead to
application of an 80% LLN factor). In addition, Demyelinating Axonal
there is conduction block of the right peroneal- Dominant Dominant
TA across-fibular-head motor segment (ampli- 1
tude drop > 50%). There clearly is distal axonal HNPP 2
loss as well, but the presence of any clear-cut I CMT-Dominar t Intermediate I
demyelination establishes this as a demyelinating
neuropathy. Demyelinating Axonal
The nerve conduction studies also support the Recessive Recessive
notion of a multifocal and asymmetric process.
The range of velocities is 28 to 57 mls. As noted 3 "AR-2"
previously (see case 37), this generally suggests
an acquired demyelinating neuropathy, although
there are exceptions, this being one of them. It is CMT-X
not clear whether to characterize this neuropathy
as having a predilection for compression sites.
Molecular Genetic
There is conduction block at one fibular head, but
Classification of CMT
the other peroneal across fibular head segment
and neither ulnar across-elbow segment demon- Demyelinating Axonal
strates focal demyelination. The median-APB Dominant Dominant
distal motor latencies are not dramatically
increased either.
lalIll_
HNP~ PMP22 2
Given the above views, two inherited neuro-
pathies should be considered: CMT-X (X-linked
1b r MPZ
Ie....
Charcot-Marie-Tooth) and HNPP (hereditary
neuropathy with liability to pressure palsies). Demyelinating Axonal
These disorders are largely due, respectively, to Recessive Recessive
mutations of the connexin 32 (Cx32) gene and 4 MAR-2M
deletions of the peripheral myelin protein 22
(PMP-22) gene. These two inherited neuropathies
CMT-Dominant Intermediate
may show a significant range of motor conduc-
tion velocities and be characterized as having CMT-X

162
 Connexin-32, also called gap junction protein CMTmutations), mostly point mutations. A wide
B 1 (GJB I), is believed to form gap junctions range of severity exists, with null or truncation
within non-compact myelin. At least 264 mutations mutations causing a severe phenotype, similar to
were reported in molecular mutation databases as CMT3 (Dejerine-Sotas disease).
of November 2003 (http://molgen-www.uia.ac.be/

Clinical Pearl
Although uniform slowing of nerve conduction studies and absence of conduction
block have classically been used to distinguish acquired from inherited demyelinating
neuropathies, there are two exceptions: CMT-X and HNNp, both of which may have
nonuniform slowing and conduction block.

REFERENCES .
1. Birouk, N., LeGuern, E., Maisonobe, T., Rouger, H., Gouider, R., Tardieu, S., Gugenheim, M., Routon, M.C., Leger, JM.,
Agid, Y., Brice, A., Bouche, P., X-linked Charcot-Marie-Tooth disease with Connexin 32 mutations: clinical and
electrophysiologic study, Neurology 1999; 52:432-433.
2. Dubourg, 0., Tardieu, S., Birouk, N., Gouider, R., Leger, JM., Maisonobe, T., Brice, A., Bouche, 1', LeGuern, E.,
Clinical, electrophysiological and molecular genetic characteristics of93 patients with X-linked Charcot-Marie-Tooth
disease, Brain 2001; 124:1958-1967.
3. Gutierrez, A., England, JD., Sumner, AJ., Ferer, S., Warner, L.E., Lupski, JR., Garcia, c.A., Unusual
electrophysiological findings in X-linked dominant Charcot-Marie- Tooth disease, Muscle Nerve 2000; 23: 182-188
4. Hahn, A.E, Ainsworth, 1'1., Naus, c.c., Mao, 1., Bolton, C.E, Clinical and pathological observations in men lacking the
gap junction protein connexin 32, Muscle Nerve 2000; 999(9):S39-S48.
5. Hahn, A.E, Ainsworth, 1'1., Bolton, C.E, Bilbao, JM., Vallat, JM., Pathological findings in the X-linked form of
Charcot-Marie- Tooth disease: a morphometric and ultrastructural analysis, Acta Neuropathol. (Berlin) 200 I;
101:129-139.
6. Lewis, R.A., The challenge ofCMTX and Connexin 32 mutations, Muscle Nerve 2000; 23: 147-149.
7. Nicholson, G., Nash, 1., Intermediate nerve conduction velocities define X-linked Charcot-Marie-Tooth neuropathy
families, Neurology 1993; 43:2558-2564.
8. Pareyson, D., Charcot-Marie- Tooth disease and related neuropathies: molecular basis for distinction and diagnosis,
Muscle Nerve 1999; 22:1498-1509.
9. Tabaraud, E, Lagrange, E., Sindou, P., Vandenberghe, A., Levy, N., Vallat, JM., Demyelinating X-linked
Charcot-Marie-Tooth disease: unusual electrophysiological findings, Muscle Nerve 1999; 22:1442-1447.
10. Vital, A., Ferrer, X., Lagueny, A., Vandenberghe, A., Latour, 1', Goizet, c., Canron, M.H., Louiset, P., Petry, K.G., Vital,'
c., Histopathological features of X-linked Charcot-Marie- Tooth disease in 8 patients from 6 families with different
Connexin 32 mutations, 1. Peripher. Nerv Syst. 200 I; 6:79-84.

1'63
 PATIENT 41

A 72-year-old woman with 12 years of progressive numbness and

paresthesias in her hands and feet and mild gait ataxia

At the age of 60, this woman developed numbness and tingling paresthesias in her feet and her
fingertips which have gradually progressed since. The tingling paresthesias in the legs go up to just
below the knees and in the hands remain confined to the fingertips. Her balance is mildly impaired.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (~V) (cm) (mls)
R. median-dig II 1. Wrist Dig II 5.15 6.75 3.0 13 25.2
L. median-dig II 1. Wrist Dig II 4.80 7.15 3.7 13 27.1
R. ulnar-dig V 1. Wrist Dig V Absent 11
L. ulnar-dig V 1. Wrist Dig V 5.00 6.90 3.3 11 22.0
R. radial-sn box 1. Forearm Sn box 2.80 3.60 6.2 10 35.7
R. sural-I at mall 1. Mid-calf Ankle Absent 14
L. sural-Iat mall 1. Mid-calf Ankle Absent 14

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (mls)
R. median-APB I. Wrist APB 8.95 5.2 7
2. Elbow APB 14.25 5.3 20 37.7
L. median-APB 1. Wrist APB 9.30 4.5 7
2. Elbow APB 15.15 3.3 22 37.6
R. ulnar-ADM 1. Wrist ADM 6.30 6.6 7
2. B. elbow ADM 10.00 5.3 17.5 47.3
3. A. elbow ADM 14.40 5.1 10 22.7
L. ulnar-ADM 1. Wrist ADM 6.40 7.3 7
2. B. elbow ADM 10.10 7.2 17.5 47.3
3. A. elbow ADM 14.15 6.8 9.5 23.5
R. Peroneal-EDB 1. Ankle EDB Absent 8
R. Peroneal-tib ant 1. Fib head Tib ant 4.95 3.3 8
2. Pop fossa Tib ant 10.15 2.2 7.5 14.4
R. tibial-AH 1. Ankle AH Absent 8

F-Wave

Nerve F min (ms) F max (ms) Max - Min (ms) %F

R. median 40.70 43.70 3.00 80
R. ulnar 39.80 43.25 3.45 80
L. median 41.60 46.25 4.65 80
L. ulnar 39.25 45.00 5.75 80

164
 Questions:
1. The electrodiagnostic studies demonstrate a generalized peripheral neuropathy. Should it be char-
acterized as primarily axonal or demyelinating?
2. Does the study suggest an inherited or an acquired neuropathy?

," ~
' ..

16S
 Answers:
1. Demyelinating
2. Acquired

Discussion: The electrodiagnostic features cies are at least 70% increased above the upper
are those of an acquired demyelinating neuropa- limit of normal, with normal forearm velocities.
thy. There is nonuniform slowing of motor nerve The median distal motor latencies are at least
conduction velocities (range, 14.4 to 47.3 m/s). 100% increased, with forearm motor velocities
The distal latencies and some of the motor seg- that are not more than 10 to 20% reduced. The dis-
ment velocities are definitively in the demyelinat- tal sensory velocities are similarly substantially
ing range, while other segments have normal reduced, beyond what would be expected from
velocities (ulnar-ADM forearm). As a rule, inher- axonal loss alone. Note the absence of conduction
ited demyelinating neuropathies have uniform block, characteristic of this particular neuropathy.
slowing (with some exceptions, notably CMT-X The clinical features of distal sensory involve-
and HNPP), while nonuniform slowing indicates ment with gait ataxia along with a demyelinating
an acquired demyelinating neuropathy. The study neuropathy with prolonged distal latencies and
shows a predilection for greater slowing at com- absence of conduction block are characteristic of
mon compression sites (median at the wrist, ulnar the myelin-associated glycoprotein (MAG) and
at the elbow, and peroneal at the fibular head), but sulfatide (SGPG) antibody-mediated neu-
other sites are also significantly involved, such as ropathies. This patient has an IgM-K paraprotein
the ulnar across the wrists for both motor and sen- on immunofixation and serum anti-MAG titer of
sory fibers. 1:51 ,200 (normal, <1 :800) and anti-SGPG titer of
One can also argue from this study that, except 1:819,200 (normal, <1:800). These neuropathies
for the compression sites, the slowing is preferen- are generally mild clinically and very impres-
tially distal. All distal motor latencies are signifi- sively abnormal electrodiagnostically; they gen-
cantly prolonged, and to a greater extent than the erally do not require treatment (which is less than
velocity reductions in the other non~compression satisfactory when attempted) and have a favorable
site segments. For example, the ulnar distal laten- prognosis, although there are exceptions.

Clinical Pearls
1. Anti-MAG and anti-sulfatide neuropathy syndromes typically appear to clinically
have sensory loss typical of a distal axonal neuropathy but more gait ataxia than would
be expected from such.
2. Electrodiagnostically, the findings are relatively unique, with distal demyelination
predominant.
3. Unlike in other acquired demyelinating neuropathies, conduction block is not seen
in MAG and sulfatide neuropathies.

REFERENCES
1. Braun, P.E., Frail, D.E., Latov, N., Myelin-associated glycoprotein is the antigen for a monoclonal IgM in polyneuropathy,
J. Neurochem. 1982; 39(5):1261-1265.
2. Capasso, M., Torrieri, E, Di Muzio, A., De Angelis, M.V, Lugaresi, A., Uncini, A., Can electrophysiology differentiate
polyneuropathy with anti-MAG/SGPG antibodies from chronic inflammatory demyelinating polyneuropathy?, Clin.
Neurophysiol. 2002; 113(3):346-353.
3. Dalakas, M.C., Quarles, R.H., Farrer, R.G., Dambrosia, 1., Soueidan, S., Stein, D.P., Cupler, E., Sekul, E.A., Otero, c., A
controlled study of intravenous immunoglobulin in demyelinating neuropathy with IgM gammopathy, Ann. Neural. 1996;
40(5):792-795.
4. Eurelings, M., Moons, K.G., Notermans, N.C., Sasker, L.D., De Jager, A.E., Wintzen, A.R., Wokke, 1.H., Van den Berg,
L.H., Neuropathy and IgM M-proteins: prognostic value of antibodies to MAG, SGPG, and sulfatide, Neurology 200 I;
56(2):228-233.
5. Gorson, K.C., Ropper, A.H., Weinberg, D.H., Weinstein, R., Treatment experience in patients with anti-myelin-associated
glycoprotein neuropathy, Muscle Nerve 2001; 24(6):778-786.
6. Kaku, DcA., England, 1.0., Sumner, A.1., Distal accentuation of conduction slowing in polyneuropathy associated with
antibodies to myelin-associated glycoprotein and sulphated glucuronyl paragloboside, Brain 1994; 117:941-947.
7. Katz, 1.S., Saperstein, D.S., Gronseth, G., Amato, A.A., Barohn, R.1., Distal acquired demyelinating symmetric neuropathy,
Neurology 2000; 54(3):615-620.
8. Kelly, 1.1., Jr., The electrodiagnostic findings in polyneuropathies associated with IgM monoclonal gammopathies, Muscle
Nerve 1990; 13(12):1113-1117.

166
 9. Nobile-Orazio, E., Meucci, N., Baldini, L., Di Troia, A., Scarlato, G., Long-term prognosis of neuropathy associated with
anti-MAG IgM M-proteins and its relationship to immune therapies, Brain 2000; I 23(pt. 4):710-717.
10. Trojaborg, w., Hays, A.P., van den Berg, L., Younger, D.S., Latov, N., Motor conduction parameters in neuropathies
associated with anti-MAG antibodies and other types of demyelinating and axonal neuropathies, Muscle Nerve 1995;
18(7):730-735.

167
 PATIENT 42

A 43-year-old woman with acute generalized weakness developing

1 month into her intensive care unit stay for sepsis and
multi-organ failure

This 43-year-old woman developed acute ischemic bowel complicated by multi-organ failure and
prolonged intensive care unit hospitalization. One month into her stay, she was noted to be nearly quad-
riplegic. Volitional movements were limited to incomplete lateral and vertical eye movements, partial
eye closure, and slight movement of finger flexion.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (IlV) (cm) (rnIs)

R: median-dig II 1. Wrist Dig II 2.45 3.30 3.8 13 53.1

R. ulnar-dig V \. Wrist DigV \.65 2.40 5.3 11 66.7
R. radial-sn box \. Forearm Sn box \.85 2.25 10.8 10 54.1
R. sural-Iat mall \. Mid-calf Ankle 2.90 3.80 2.4 14 48.3

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (rnIs)

R. peroneal-EDB 1. Ankle EDB Absent 8

R. peroneal-tib ant 1. Fib head Tib ant Absent
R. tibial-AH 1. Ankle AH 4.00 0.8 8
2. Pop fossa AH Absent 40 0
R. median APB \. Wrist APB Absent
R. ulnar-ADM \. Wrist ADM 3.00 0.3 7
2. B. elbow ADM 6.20 0.2 16.5 5\.6
3. A. elbow ADM 7.75 0.3 14 90.3

Needle EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. biceps Nl 0 0 0 No activity
R. triceps Incr 1+ 0 0 No activity
R. deltoid Incr 1+ 0 CRD No activity
R. first dors int Incr 2+ 0 0 No activity
R. ext dig comm Incr 3+ 0 0 No activity
R. tib ant Incr 2+ 0 0 No activity
R. vast med Incr 3+ 0 0 No activity
R. abd poll br Incr 3+ 0 0 No activity

168
 R Mediari-APB . . R Ulriar-FDi
2
. 2
3
Intramuscular'
1
20ms 5mV Intramuscular 1
.10ms20mV

Intramuscular 5 Intramuscular 2
, 20m~ 5mV ..
'10ms20mV

Wrist (Surface)
20ms 5mV Wrist (Surface) 3
10ms 20m V

Wr'ist (Su'rface) 4
20ms 5mV

Questions:
I. What are the two most common neuromuscular causes of generalized weakness that develops after
admission to an intensive care unit?
2. Needle EMG of the right ADM was not performed. Assuming it showed the same findings as that
of the right first dorsal interosseous muscle, how would you interpret this together with the right
ulnar-ADM motor nerve conduction study?
3. The distinction between a severe myopathy resulting in inexcitable muscle and a severe neuropathy
resulting in inexcitable motor nerve can be made using additional studies, the results of which are
demonstrated in the figure and the accompanying tabular results. What studies are these? What do
the results imply?

169
 Answers:
I. Critical illness myopathy and critical illness neuropathy
2. Indicative of distal conduction block or ongoing Wallerian degeneration
3. Surface and intramuscular needle stimulation, with needle recording in both, suggests inexcitable
nerve but excitable muscles, which indicates a neuropathy is the cause of weakness.

Discussion: The nerve conduction studies of the nerve is due to either conduction block
are remarkable for moderate reduction in the proximal to the stimulation site or acute axonal
SNAP amplitudes and severely reduced ampli- injury that has not yet resulted in complete distal
tudes of the CMAP amplitudes. These findings neuromuscular transmission failure or axonal
provide evidence for a peripheral neuropathy, degeneration.
\\:,ith motor involvement being much greater than The important differential diagnostic consider-
sensory nerve involvement. The process is likely ation here is between critical illness myopathy
not length dependent, given that the sural SNAP (CIM) and critical illness polyneuropathy (CIP).
is still recordable despite moderate reduction in CIM, also known as acute quadriplegic myopathy,
the median and ulnar SNAP amplitudes and the generally occurs in the setting of treatment with
EMG shows no recruitment of motor units in both high-dose steroids and neuromuscular blocking
proximal and distal arm and leg muscles. These agents, or sepsis with multi-organ failure. Some
features suggest a possible demyelinating neu- but not all patients have elevated serum CK, and
ropathy, although no specific evidence of such is electromyography usually shows fibrillation
available in the electrodiagnostic study. potentials but abundant motor units recruited
A more significant problem is the nature of the early and with full interference patterns despite
patient's profound paralysis. Although the sensory profound weakness of the muscle. However, the
nerve abnormalities do imply the existence of a process may be severe enough that no motor units
peripheral neuropathy, one wonders whether there are recruited at all. CIP is a poorly understood
could be a mild axonal sensory neuropathy together entity that, in our opinion, has not been ade-
with a separate cause for the profound motor abnor- quately defined. Certainly many ICU patients
malities. In particular, a myopathy or neuromuscu- develop mild distal axonal predominantly or
lar junction disorder could account for generalized exclusively sensory neuropathies, with a variety
paralysis with absent or severely reduced CMAP of causes stemming directly from multi-organ
amplitudes and fibrillation potentials. Definitive failure or infection or the treatment of these dis-
localization of weakness by electrodiagnostic stud- orders. Severe acute generalized weakness of
ies requires observation of the pattern of motor unit neurogenic origin has another specific diagnosis,
recruitment, and in this case there is no recruitment namely the Guillain-Barre syndrome and its clin-
to observe. This makes it impossible to be definitive ical variants. The relationship of CIP to
as to the localization of the weakness. For example, Guillain-Barre syndrome variants is uncertain.
lack of effort could result in no recruited units (but The figure shows the results of both surface
would not result in distal CMAP amplitude reduc- stimulation of nerve and needle stimulation of
tion or diffuse fibrillation potentials). If a single muscle, both with needle recording in muscle. The
muscle would have shown a severely reduced pat- left panel is that of the median nerve and APB, and
tern of recruitment, localization of the weakness to the right shows the ulnar nerve and FDI. The top
the motor neuron could have been made. two tracings in each panel show a large repro-
Hypothetically, by assuming that the right ducible needle-recorded compound muscle action
ADM had been studied by needle EMG and potential with direct needle stimulation of the
showed fibrillation potentials and no recruitment muscle, while the bottom tracings show no electri-
of motor units, together with the small but obtain- cal muscle response to surface stimulation of the
able ulnar-ADM CMAP, one would be able to say nerve. Thus, the motor nerve is inexcitable, but the
there was electrophysiological evidence of a muscle membrane is excitable and muscle stimu-
demyelinating neuropathy or acute Wallerian lation can result in a large potential (29 mV in the
degeneration of the motor axons supplying ADM case of the FDI). This study provides definite evi-
proximal to the wrist. This is because the inability dence that this patient has a neuropathy, not a
to voluntarily recruit units in the setting of being myopathy, with nerve membrane inexcitablity
able to electrically excite the distal motor portion accounting for her weakness.

170
 Needle Stimulation and Recording of Muscle

Recording Latency Amplitude

Nerve Sites Site (ms) (mV)
R. median-APB 1. Intramuscular Intramuscular APB 3.45 4.8
2. Intramuscular Intramuscular APB 3.25 3.8
3. Wrist (surface) Intramuscular APB Absent
4. Wrist (surface) Intramuscular APB Absent
R. ulnar-FDI I. Intramuscular Intramuscular FDI 1.30 29.3
2. Intramuscular Intramuscular FDI 1.35 25.5
3. Wrist (surface) Intramuscular FDI Absent

Clinical Pearls
1. Definitive localization of weakness by electrodiagnostic studies requires observa-
tion of the pattern of motor unit recruitment.
2. Direct intramuscular stimulation may sometimes distinguish a neuropathy from
myopathy in patients with critical care neuromuscular weakness.

REFERENCES
I. Lacomis, D., Petrella, J.T., Causes of neuromuscular weakness in the intensive care unit: a study of ninety-two patients,. .
Muscle Nerve 1998; 21 :61 0-617 ...
2. Rich, M.M., Bird, 5.1., Raps, E.C., McCluskey, L.F., Teener, J.w., Direct muscle stimulation in acute quadriplegic· .
myopathy, Muscle Nerve 1997; 20:665--{)73.

l-71
 PATIENT 43

A 41-year-old woman with an ll-year history of progressive

numbness in her limbs

At the age of 30, this woman became aware of decreased sensation in her right hand. One or two
years later similar numbness in the right foot developed, both with gradual progression in intensity
since then. At the age of 35, she became aware of left foot and intermittent left hand numbness.
Symptoms have gradually increased though remained confined to the right leg up to the middle shin,
the left foot up to the ankle, the right hand (particularly D2 and DI), and only intermittently in the left
hand. She notes reduction in the ability to feel heat in her hands. She has had several painless burns in
her right hand. She notes difficulty walking in a dark room.
_ Neurological exam was notable for normal strength and the findings shown in the figure. The
patient was asked to close her eyes and keep her arms steady in the same position and was reminded
of these instructions several times during performance .
. On sensory examination, there was severe loss of joint position sense in the right foot to the ankle
and right hand to the wrist, with moderate left toe and mild left ankle and finger loss. Vibration sense
waS nearly absent in the right foot, moderately diminished in the left foot and right hand, and mildly
diminished in the left hand. Light touch was nearly absent in her hand in the tip of right D2. Pin sen-
sation was reduced moderately bilaterally and distally. Reflexes were absent at the biceps, triceps,
knees,' and ankles, and the plantar responses were flexor. Gait and station were wide based, and she had
difficulty standing with her feet together. Romberg's sign was present.

Eyes Open 15 sec after eyes closed 1 minute after eyes closed

Electrodiagnostic Study:

Sensory NCS
BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site Response (ms) (ms) (l1V) (cm) (m/s)

L. ulnar-dig V I. Wrist DigV No Absent II

L. radial-sn box I. Forearm Sn box No Absent 10
L. med AB cut I. Elbow Forearm No Absent 12
L. lat AB cut I. Elbow Forearm No Absent 12
L. sural-Iat mall I. Mid-calf Ankle No Absent 14
L. median-dig II I. Wrist Dig II No Absent 13
R. median-dig II I. Wrist Dig II No Absent 13
R. ulnar..:.oig V I. Wrist DigV No Absent II
R. radial-sn box I. Forearm Sn box No Absent 10
R. latAB cut 1. Elbow Forearm No Absent 12
R. sural-Iat mall I. Mid-calf Ankle No Absent 14

172
 Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (/lV) (cm) (m/s)
R. median-APB I. Wrist APB 4.10 8.8 7
2. Elbow APB 9.45 6.7 25 46.7
R. ulnar-ADM I. Wrist ADM 2.90 8.3 7
2. B. elbow ADM 7.30 8.4 26 59.1
3. A. elbow ADM 9.55 7.1 13 57.8
R. peroneal-EDB I. Ankle EDB 4.20 4.6 8
2. Fib head EDB 11.00 4.0 33 48.5
3. Pop fossa EDB 12.90 3.9 9 47.4
R. tibial-AH I. Ankle AH 4.85 15.8 8
2. Pop fossa AH 14.30 5.5 40 42.3

EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R. biceps NI 0 0 0 NI NI NI Full NI
R. triceps NI 0 0 0 NI NI NI Full NI
R. vast med NI 0 0 0 NI NI NI Full NI
R. tib ant NI 0 0 0 NI NI NI Full NI

Blink Reflex
Nerve Sites Muscle RI (ms) R2 (ms)
Supraorbital I. Right L.oculi 30.00
2. Right R.oculi 12.25 30.00
Supraorbital I. Left L.oculi 12.05 37.40
2. Left R.oculi 37.30

Questions:
I. What finding is demonstrated in the figure?
2. What is the electrodiagnostic interpretation?
3. Considering the clinical and electrodiagnostic features together, is this process symmetric or asym-
metric? Is it length dependent or non-length dependent?
4. What is the differential diagnosis for this patient?
5. What is the value of a blink reflex in this setting?

173
 Answers:
I. Asymmetric proprioceptive impairment
2. Sensory neuronopathy
3. Asymmetric, non-length dependent
4. Sjogren's syndrome, paraneoplastic
5. Abnormalities in blink reflexes are said to favor a non-paraneoplastic cause.

Discussion: The finding demonstrated in the A chronic progressive dorsal root ganglionopa-
figure is that of a proprioceptive disturbance in thy has no other well-established cause than
the right greater than left arm. A symmetric pos- Sjogren's syndrome. A subacute more rapidly
ture is easily maintained by the patient with her progressive similar clinical syndrome may be
eyes open (left panel), but closing the eyes results seen in the setting of an anti-Hu or anti-CV2 anti-
in a droop in her right fingers and wrist (right body-associated paraneoplastic syndrome. There
panel) that she is unaware of. This progresses to a are other scattered reports of asymmetric progres-
downward drift in her entire right arm and in the sive sensory neuronopathies with HTLV-I infec-
left hand after a minute. This finding is a conse- tion and with cis-platinum toxicity (complicating
quence of substantial, asymmetric proprioceptive treatment of an underlying cancer). Nevertheless,
loss. the syndrome present in this patient should result
. The electrodiagnostic study demonstrates com- in a very high index of suspicion of Sjogren's syn-
plete absence of all sensory nerve action poten- drome. Recognition of her distinct neuropathy led
tials with normal motor and needle EMG studies. to serological testing showing negative ANA,
The loss of sensory axons is non-length depend- anti-Ro, anti-La, and anti-Hu and anti-CV2 anti-
ent and is electrodiagnostically, though not clini- body studies. Minor sicca syndromes of dry
cally, symmetric. This apparent electrodiagnostic mouth and eyes were present for 2 years prior,
symmetry is due to sufficient bilateral, though and minor salivary gland biopsy was normal. The
asymmetric, severity to surpass the limits of potential value of a blink reflex is in demonstrat-
detection by nerve conduction studies. The inter- ing trigeminal sensory neuropathy evident
pretation of the electrodiagnostic study is that of through an absent afferent response, not present
a sensory neuronopathy, also known as a dorsal in this case. Abnormalities in the blink reflex
root ganglionopathy. favor non-paraneoplastic causes.

Clinical Pearls
I. Sensory neuronopathies are distinct clinical syndromes with a very limited dif-
ferential diagnosis. Sensory loss is not length dependent and is typically asymmetric.
2. Electrodiagnostic findings are limited to reduction in amplitude or absence of
sensory nerve action potentials that may be markedly asymmetric.

REFERENCES
I. Auger, R.G., Windebank, AJ., Lucchinetti, C.E, Chalk, C.H., Role of the blink reflex in the evaluation of sensory
. neuronopathy, Neurology 1999; 53:407-408.
2. Caroyer, J.M., Manto, M.U, Steinfeld, S.D., Severe sensory neuronopathy responsive to infliximab in primary Sjogren's
syndrome, Neurology 2002; 59(7):1113-1114.
3. Graus, E, Pou, A., Kanterewicz, E., Anderson, N.E., Sensory neuronopathy and Sjogren's syndrome: clinical and
immunologic study of two patients, Neurology 1988; 38:1637-1639.
4. Kaplan, J.G., Schaumburg, H.H., Predominantly unilateral sensory neuronopathy in Sjogren's syndrome, Neurology 1991;
41:948-949.
5:· Kaplan, J.G., Rosenberg, R., Reinitz, E., Buchbinder, S., Schaumburg, H.H., Invited review: peripheral neuropathy in
Sjogren's syndrome, Muscle Nerve 1990; 13:570-579.
6. Laloux, P., Brucher, J.M., Guerit, J.M., Sindic, CJ., Laterre, E.C., Subacute sensory neuronopathy associated with
Sjogren's sicca syndrome, J. Neural. 1988; 235:352-354.
7. Lauria, G., Pareyson, D., Grisoli, M., Sghirlanzoni, A., Clinical and magnetic resonance imaging findings in chronic
. sensory ganglionopathies, Ann. Neurol. 2000; 47: 104-109.
8. Malinow, K., Yannakakis, G.D., Glusman, S.M., Edlow, D.W, Griffin, J., Pestronk, A., Powell, D.L., Ramsey-Goldman, R.,
Eidelman, RH., Medsger, T.A., Jr. et aI., Subacute sensory neuronopathy secondary to dorsal root ganglionitis in primary
Sjogren's syndrome, Ann. Neural. 1986; 20:535-537.
9. Valls-Sole, J., Graus, E, Font, J., Pou, A., Tolosa, E.S., Normal proprioceptive trigeminal afferents in patients with
Sjogren's syndrome and sensory neuronopathy, Ann. Neural. 1990; 28(6):786-790.

174
 SECTION III. NEUROMUSCULAR JUNCTION DISEASE

The electrodiagnostic assessment of disorders of the neuromuscular junction (NMJ) is frequently

challenging. The techniques used are often not performed frequently enough to ensure the ideal degree
of practical and technical experience. The principle disorder of the NMJ is myasthenia gravis (MG).
Others, including the Lambert-Eaton myasthenic syndrome (LEMS), the congenital myasthenic syn-
dromes (CMSs), botulism, and effects of various toxins, are either extremely rare or quite uncommon.
It is important to recognize that neuromuscular junctions may be abnormal in a number of disorders
that do not primarily involve the NMJ. In acute axonal nerve lesions, neuromuscular transmission fails
before Wallerian degeneration. In chronic disorders of lower motor neurons, such as amyotrophic lat-
eral sclerosis, the immature neuromuscular junctions present during reinnervation may demonstrate
blocking and abnormal degrees of decrement on repetitive stimulation. Accordingly, both repetitive
nerve stimulation and single-fiber EMG may be abnormal in many neuromuscular disorders.
The various elements ofthe electrodiagnostic study in patients with suspected NMJ disorders are:
• Routine sensory and motor nerve conduction studies, and routine needle EMG
• Repetitive nerve stimulation (RNS)
• Low frequency (2 to 5 Hz)
• High frequency (25 to 50 Hz)
• Post-exercise facilitation
• Post-exercise exhaustion
• Special procedures
• Single-fiber electromyography (SFEMG)
• Voluntary
• Axonal-stimulated SFEMG
The purpose of routine studies in the NMJ evaluation is to exclude other diagnoses, such as a neu-
ropathy or myopathy, and to exclude other technically confounding disorders, such as an ulnar neu-
ropathy that produces abnormal decrement in a repetitive nerve stimulation ulnar-ADM study. Very
occasionally, routine studies will provide direct evidence of a NMJ disorder, such as the occurrence of
repetitive CMAPs after a single motor nerve stimulus in patients with organophosphate toxicity or
some of the congenital myasthenic syndromes (AChE deficiency and slow channel syndrome).
It is worthwhile to briefly review the history of repetitive nerve stimulation. Repetitive nerve stim-
ulation was described in 1941 by Harvey and Masland,9 who reported findings in the ulnar-ADM
CMAP in various neurological disorders and 3 patients with MG. In 1952 Botelho et al.4 reported on
21 patients with MG and noted that the greatest decrement occurred at around the 5th potential. In
1968, Siomic et al. 18studied 23 patients with MG and reported that the degree of decrement increased
several minutes after exercise. In 1971, Odzemir and Youngl2 studied 30 patients with MG and 30 nor-
mals, recording CMAPs from ADM, FCR, and deltoid at baseline and post exercise every 30 seconds
for 5 minutes. Using a cutoff of 10% decrement as abnormal, their approach had a sensitivity of 87%.
In 1974, Borenstein and Desmedt3 noted the effects of temperature on RNS in 30 patients with MG:
warming increases and cooling decreases decrement. In 1982 Oh et al. 11recommended avoiding anti~
cholinesterase medication for 12 hours prior to RNS and emphasized distinct RNS sensitivities iri
ocular (17%) and generalized MG (85%).
The range of sensitivities reported for RNS in MG has been 10% to 50% in ocular myasthenia,
and 75% in generalized untreated myasthenia when one distal and one proximal muscle are examined.
The specificity has not been rigorously studied. It is quite clear that focal neuropathy or anterior horn,
cell disease may result in abnormal decrement. Accordingly, normal RNS does not argue appreciably
against MG as a diagnosis (RNS is relatively insensitive). Abnormal RNS strongly suggests an NMJ
disorder, though motor neuron disease should also be considered (RNS is relatively specific).
Single fiber EMG (SFEMG) was first described by Ekstedt in 1964.8 The concept of "jitter" was
reported by Stalberg in 197119 and Sanders et al.15 reported findings in 127 patients with myasthenia
gravis in 1979. Stimulated single fiber EMG, as opposed to the voluntary SFEMG previously reported,
175
was first described by Trontelj et al.20 in 1986. In that year, Sanders and Howardl6 reported that study-
ing 1 muscle demonstrated an abnormality in 85% of patients with MG and 2 muscles demonstrated
an abnormality in 99% of patients with MG. The reported range of sensitivity of SFEMG in MG is
82% to 99% among 3 different studies meeting evidenced based medicine criteria as reviewed by an
AAEM subcommittee in 2001.
This technique may be over-rated as a clinical diagnostic tool. The technical demands ofthe study
both for patients and examiners are substantial. The test is highly sensitive but likely highly non-spe-
cific (its specificity has never been adequately studied and reported in the literature). Many disorders
of motor nerves or muscle result in increased jitter, including neuropathy, some myopathies, and ante-
rior horn cell diseases. Accordingly, an abnormal SFEMG study has limited diagnostic value. The
value of this test is that a normal SFEMG study makes generalized MG highly unlikely. These straight-
forward conclusions suggest the following approach. If the patient clearly has a neuromuscular disor-
der clinically, SFEMG is likely of no added value. If the goal instead is to exclude a neuromuscular
disorder as the cause of symptoms in a patient with a normal neurological examination, the test is quite
likely to do that.
The various technical elements of SFEMG are briefly summarized below:
• SFEMG Technique
• 50 discharges for each pair
• 20 pairs
• 3-4 different skin insertions
• Technical Considerations
• Amplitude of AP > 200 mV
• Rise time < 300 msec
• IPI may be influenced by preceding interdischarge interval
• Effect of variable firing rates on IPI taken into account with MSD
• Temperature: Stalberg et al. 1971: "Lowering of temperature caused an increase of the jitter and
increasing the temperature resulted in a decrease of the jitter"
• SFEMG Criteria For Abnormality
• A study is abnormal if either:
• The mean jitter is increased; OR
• More than 10% of pairs have increased jitter

REFERENCES
1. AAEM. Literature review of the usefulness ofrepetitive nerve stimulation and single fiber EMG in the electrodiagnostic
evaluation of patients with suspected myasthenia gravis or Lambert-Eaton myasthenic syndrome. Muscle Nerve. 200 I;
24:1239-1247.
2. AAEM. Practice parameter for repetitive nerve stimulation and single fiber EMG evaluation of adults with suspected
myasthenia gravis or Lambert-Eaton myasthenic syndrome: summary statement. Muscle Nerve. 200 I; 24: 1236-1238.
3. Borenstein S, Desmedt JE. Temperature and weather correlates of myasthenic fatigue. Lancet 1974; 63-66.
4. Botelho SY, Deaterly CF, Austin S, Comroe JH Jr. Evaluation of the electromyogram of patients with myasthenia gravis.
Arch Neurol Psychiatry 1952; 67:441-450.
5. Bril V, Werb MR, Greene DA, Sima AA. Single-fiber electromyography in diabetic peripheral polyneuropathy. Muscle
Nerve. 1996 Jan; 19(1):2-9.
6. Chaudhry V, Crawford TO. Stimulation single-fiber EMG in infant botulism. Muscle Nerve. 1999 Dec; 22(12): 1698-703.
7. Chaudhry V, Watson OF, Bird SJ, Comblath DR. Stimulated single-fiber electromyography in Lambert-Eaton myasthenic
syndrome. Muscle Nerve. 1991 Dec; 14(12):1227-30.
8. Ekstedt 1. Human single muscle fiber action potential. Acta Physiol Scand 1964; 61(suppl):226:1-96.
9. Harvey AM, Masland RL. A method for the study of neuromuscular transmission in human subjects. Bull Johns Hopkins
Hosp 1941; 68:81-93.
10. Lange OJ, Rubin M, Greene PE, Kang VJ, Moskowitz CB, Brin MF, Lovelace RE, Fahn S. Distant effects of locally
injected botulinum toxin: a double-blind study of single fiber EMG changes. Muscle Nerve. 1991 Jul; 14(7):672-5.
II. Oh SJ, Eslami N, Nishihara T, Sarala PI(, Kuba T, Elmore RS, Sunwoo IN, Ro YI. Electrophysiological and clinical
correlation in myasthenia gravis. Ann Neurol 1982; 12:348-354.
12. Ozdemir C, Young RR. Electrical testing in myasthenia gravis. Ann NY Acad Sci 1971; 183: 287-302.
13. Padua L, Aprile I, Monaco ML, Fenicia L, Anniballi F, Pauri F, Tonali P. Neurophysiological assessment in the diagnosis
of botulism: usefulness of single-fiber EMG. Muscle Nerve. 1999 Oct; 22(10):1388-92.
14. Padua L, Stalberg E, LoMonaco M, Evoli A, Batocchi A, Tonali P. SFEMG in ocular myasthenia gravis diagnosis. Clin
Neurophysiol. 2000 Jul; 111(7): 1203-7.
15. Sanders DB, Howard JF Jr, Johns TR. Single-fiber electromyography in myasthenia gravis. Neurology. 1979 Jan; 29(1):
68-76
16. Sanders DB, Howard JF Jr. AAEE minimonograph #25: Single-fiber electromyography in myasthenia gravis. Muscle
Nerve. 1986 Nov-Dec; 9(9):809-19.

176
17. Single fiber EMG reference values: a collaborative effort. Ad Hoc Committee of the AAEM Special Interest Group on
Single Fiber EMG. Muscle Nerve. 1992 Feb; 15(2):151-61.
18. Siomic A, Rosenfalck A, Buchthal F. Electrical and mechanical responses of normal and myasthenic muscle. Brain Res
1968; 10: 1-78.
19. Stlliberg E, Ekstedt J, Broman A. The electromyographic jitter in normal human muscles. Electroencephalogr Clin
Neurophysiol 1971; 31 :429-438.
20. Trontelj JY, Mihelin M, Fernandez J, Stlliberg E. Axonal stimulation for end-plate jitter studies. J Neurol Neurosurg
Psychiatry 1986; 49:677-685.
21. Trontelj JY. Stimulation SFEMG in myasthenia gravis. Muscle Nerve. 1990 May; 13(5):458-9.
22. Wiechers D. Single fiber EMG evaluation in denervation and reinnervation. Muscle Nerve. 1990 Sep; 13(9):829-32.
23. Weinberg DH, Rizzo JF 3rd, Hayes MT, Kneeland MD, Kelly JJ Jr. Ocular myasthenia gravis: predictive value of single-
fiber electromyography. Muscle Nerve. 1999 Sep; 22(9): 1222-7.

171
 PATIENT 44

A 17-year-old woman with progressive dysarthria and dysphagia

for 4 months

This 17-year-old woman noted progressive nasal and slurred speech and difficulty with both
chewing and swallowing initially only present later in the day. Examination showed bilateral varying
ptosis and facial and palatal weakness.
Electrodiagnostic Study:

Muscle Train Rate (Pps) Amplitude (mV) 4-1 (%)

L. abd dig min Baseline 3 14.1 -7.4

Post I min exercise 3 14.0 -9.1
After I min 3 13.4 -1.6
After 2 min 3 14.8 -10.2
After 3 min 3 13.9 -4.4
After 4 min 3 13.7 -5
L. trapezius (u) Baseline 3 5.2 -2.1
Post I min exercise 3 6.0 -8.5
After I min 3 5.8 0.3
After 2 min 3 5.5 -10.6
After 3 min 3 5.5 -14.5
After 4 min 3 5.3 -15.1
After 5 min 3 5.3 -20.9
L. nasalis Baseline 3 2.5 -28.1
Post 1 min exercise 3 2.5 -25.2
After I min 3 1.3 -18.1
After 2 min 3 1.2 -21.7
After 3 min 3 1.4 -29.6
After 4 min 3 0.7 -25.1

t7S
 L Abd Dig Min

2 msec 5 mV

Baseline

After2 Min

After4 Min

Figure 1

L Nasalis

2 msec 0.5 mV

-----------:A~ftft;e;;r11rM~in

••....
~---------AA:ftft;;e~r22;:;Mu,:in

~i!oo.._---------AAftfue;r"33i1M~~in

Figure 2
 L Trapezius (U) L Trapezius (U) (continued)
.....
2 msec 2mV 2 msec 2 mV

After 2 Min

After 3 Min

Figure 3

Questions:
1. What are the main clinical diagnoses to consider in this patient?
2. The table lists results of repetitive nerve stimulation studies. What procedure is being used and what
abnormalities are being sought?
3,' What is the rationale for performing studies of trapezius or nasalis if the ADM study appears
normal?
4: What is the most likely explanation for the waveforms for nasalis in the second figure?
5. For the nasalis study in figure 2, was it strictly necessary to perform the exercise and post-exercise
recordings for this muscle?
6. For the trapezius study shown in figure 3, again, was it necessary to perform exercise and post-exer-
cise recordings?

180
 Answers:
1. Myasthenia gravis; congenital myasthenic syndrome; oculopharyngeal muscular dystrophy; brain-
stem mass
2. Repetitive nerve stimulation after exercise to look for post-exercise exhaustion
3. Proximal muscles may be more likely to be abnormal.
4. Electrode movement occurred during exercise.
5. No
6. Yes

Discussion: The chief clinical consideration ing the sensitivity of repetitive nerve stimulation
given the history is myasthenia gravis. When this for the diagnosis of MG have been devised. The
diagnosis is considered in children and adolescents, two commonly used ones are (1) performing
congenital myasthenic syndromes should always be more muscle studies (e.g., distal and proximal),
considered as well. The repetitive nerve stimulation and (2) performing exercise to look for post-exer-
studies above were performed as follows: cise exhaustion. Studies suggest that the addition
• Baseline stimulation at 3 Hz for five of a single proximal muscle study to that of a dis-
impulses. This study aims at detecting abnor- tal muscle already performed results in a sensitiv-
malities in the baseline decrement to low fre- ity of 76% in patients with untreated generalized
quency stimulation. MG and 48% in patients with purely ocular MG.
• The patient performed brief exercise for 10 Commonly both approaches are used.
seconds and immediately afterwards, stimu- The phenomenon of post-exercise exhaustion
lation at 3 Hz for five impulses was per- is poorly understood but quite helpful diagnosti-
formed. This study aims at detecting cally. It may be a consequence of depletion of
abnormalities in post-exercise facilitation. acetylcholine reserves. After 1 minute of volunc
Such abnormalities are diagnostic of presy- tary exercise, a train of 5 impulses is delivered
naptic neuromuscular transmission disorders. each minute for 5 minutes. A typical abnormality
• The patient then performed sustained exer- consists of the appearance of a decrementing
cise for 1 minute; subsequent trains of five response (fourth or fifth impulse compared to
impulses were recorded immediately after- first impulse within a single given train) between
wards and then every minute for 5 minutes. 2 and 4 minutes and recovery of the decrement at
This study aims at detecting abnormalities in the fifth minute. However, the appearance of a
post-exercise exhaustion. >10% decrement at any of the time points is taken
An abnormal decrement to low-frequency to be abnormal. This study is very useful in that a
stimulation (2 or 3 Hz) is usually defined as a significant number of patients with MG will have
greater than 10% decrement in the baseline-to- abnormalities limited to post-exercise exhaustion.
peak or peak-to-peak amplitude of the fourth or In the current study, we see from the table that
fifth potential compared to the first. Meticulous the maximal ADM decrement was 10.2% at minute
attention to technical factors is required to 2-a borderline value-so the results of this nerve
achieve this low of a cutoff for abnormality; slight were initially taken as normal, and the electromyo-
movement of the recording electrodes, muscle grapher proceeded with a more proximal study.
position, or stimulating electrodes can easily pro- However, review of the waveforms in the figure
duce larger apparent decrements in normal mus- shows a more impressive drop in the decrement
cles. The measured decrement can also vary when measured to the fifth potential, particularly at
significantly depending on which measure of minute 4. Here, the EMG software-computed drop
amplitude and which waveform (i.e., fourth or from the first to fourth potential was only 5%. For
fifth) is used. Unfortunately, there is no published the fifth potential, estimating from the figure, there
consensus as to which of these measures should is about half of a vertical box drop, which translates
be used or a body of literature that supports one to 2.5 mY from a 13.7-mY first potential, or an
approach over another. Accordingly, and for other 18% decrement, which is clearly abnormal. This
reasons as well, we recommend caution in inter- emphasizes one of the cardinal rules of interpreting
preting borderline values around 10%. repetitive nerve stimulation results: Always review
The abnormal baseline decrement to low- the waveforms. The waveforms must be repro-
frequency stimulation is the hallmark of neuro- ducible and free from technical problems, and com-
muscular transmission defects and occurs in both puter software cannot always be relied upon for
pre- and postsynaptic disorders; however, the test proper computation of decrement.
is relatively insensitive, and many patients with Inspection of the nasalis waveforms in the sec-
myasthenia gravis will have normal baseline ond figure are notable for the change in morphol-
decrement studies. Several approaches to increas- ogy of the CMAP waveform that is present in

181·
all of the post-exercise tracings compared to the As the nasalis baseline study demonstrated a
baseline. This likely reflects movement of one of 28% decrement in amplitude, it was not necessary
the recording electrodes during forced exercise of to perform exercise to demonstrate post-exercise
the muscle. If the waveform morphology changes exhaustion as well. For the trapezius, however, the
within a train of 5, the train needs to be discarded situation is different, and it was necessary to per-
and cannot be interpreted. In this case, the change form exercise to demonstrate an abnormality in
between trains and the subsequent consistency of this muscle, as can be seen in the third figure. The
the waveform morphologies afterward indicate decrements at baseline, immediately after exercise,
that the movement of the electrode does not affect and 1 minute post-exercise are not impressive, but
the demonstration of decrement within a train of at 2, 3, 4, and 5 minutes are quite impressive.
i~pulses, and the study remains interpretable.

Clinical Pearls
1. Look for post-exercise exhaustion in the evaluation of patients with suspected
myasthenia gravis.
2. Always review the waveforms yourself; automated software will calculate decre-
",'
ments but will not detect technical problems with the recordings.

REFERENCES
I. AAEM, Literature review of the usefulness of repetitive nerve stimulation and single fiber EMG in the electrodiagnostic
,evaluation of patients with suspected myasthenia gravis or Lambert-Eaton myasthenic syndrome, Muscle Nerve 200 I;
24:1239-1247.
2. AAEM, Practice parameter for repetitive nerve stimulation and single fiber EMG evaluation of adults with suspected
, myasthenia gravis or Lambert-Eaton myasthenic syndrome: summary statement, Muscle Nerve 200 I; 24: 1236-1238.
3. Gilchrist, J.M., Massey, J.M., Sanders, D.H., Single fiber EMG and repetitive stimulation of the same muscle in
myasthenia gravis, Muscle Nerve 1994; 17(2):171-175.
4. Keesey, J.e., AAEE Minimonograph 33: electrodiagnostic approach to defects of neuromuscular transmission, Muscle
Nerve 1989; 12(8):613-626.
5. Killian, J.M., Wilfong, A.A., Burnett, L., Appel, S.H., Boland, D., Decremental motor responses to repetitive nerve
__ stimulation in ALS, Muscle Nerve 1994; 17(7):747-754.
6. Morita, H., Shindo, M., Yanagawa, S., Yanagisawa, N., Neuromuscular response in man to repetitive nerve stimulation,
Muscle Nerve 1993; 16(6):648-654.
7. Rutkove, S.8., Shefner, J.M., Wang, AX., Ronthal, M., Raynor, E.M., High-temperature repetitive nerve stimulation in
myasthenia gravis, Muscle Nerve 1998; 21(11):1414-1418.
8. Sanders, D.8., Andrews, P.I., Howard, J.E, Massey, J.M., Seronegative myasthenia gravis, Neurology 1997; 48:S40-S45.
9 .. Schumm, E, Stohr, M., Accessory nerve stimulation in the assessment of myasthenia gravis, Muscle Nerve 1984;
.7(2):147-151.
10. Sonoo, M., Uesugi, H., Mochizuki, A., Hatanaka, Y, Shimizu, T., Single fiber EMG and repetitive nerve stimulation of
the same extensor digitorum communis muscle in myasthenia gravis, Clin. Neurophysiol. 2001; 112(2):300-303.
11. Tim, R.W., Sanders, D.8., Repetitive nerve stimulation studies in the Lambert-Eaton myasthenic syndrome, Muscle Nerve
, 1994; 17(9):995-:-1001.

182
 PATIENT 45

A 71-year-old man with subacute symmetrical proximal weakness

This 71-year-old man developed symptoms of symmetric proximal arm and leg weakness I· year
prior, which remitted spontaneously over 3 months and then again recurred 2 to 3 months ago. Brief
neurological examination demonstrated mild symmetric weakness of neck flexion, deltoids, and biceps
and mild-moderate weakness of first dorsal interosseous (FDI) bilaterally. The left biceps reflex was
initially absent, but after 10 seconds of exercise was a normal 2+. Right biceps reflex was trace to 1+.
The patient was referred for a question of motor neuron disease.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (f.LV) (em) (m/s)
L. median-dig II I. Wrist Dig II 3.10 4.00 30.3 13 41.9
L. ulnar-dig V 1. Wrist DigV 2.45 3.35 19.7 11 44.9·
L. radial-snbox 1. Forearm Sn box 1.70 2.40 38.0 10 58.8
L. sural-I at mall 1. Mid-calf Ankle 3.05 3.85 7.0 14 45.9··

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (em) (m/s)
L. median-APB 1. Wrist APB 4.45 10.3 7
2. Elbow APB 8.80 9.6 21 48.3
3. Axilla APB 12.55 9.1 18 48.0
L. ulnar-ADM 1. Wrist ADM 3.60 3.3 7
2. B. elbow ADM 8.90 2.3 24 45.3
3. A. elbow ADM 12.00 2.0 13 41.9
4. Axilla ADM 13.85 1.6 10 54.1
L. ulnar-FDI 1. Wrist FDI 6.15 2.3
2. B. elbow FDI 9.70 1.6 20 56.3
3. A. elbow FDI 12.10 1.4 13 54.2
4. Axilla FDI 13.80 1.2 10 58.8
R. ulnar-ADM 1. Wrist ADM 3.90 6.1 7
2. B. elbow ADM 8.50 5.3 22 47.8
L. peroneal-EDB 1. Ankle EDB 5.25 1.4 8
2. Fib head EDB 13.75 0.9 32 37.6
3. Pop fossa EDB 15.70 0.9 10 51.3
L. tibial-AH 1. Ankle AH 3.85 4.1 8
2. Pop fossa AH 14.85 4.0 36 32.7

Although the reduced CMAP amplitudes are compatible with motor neuron disease, the history
of symmetric proximal weakness that remits and then relapses again is not particularly suggestive of a
motor neuron disorder. Reduced amplitudes of CMAPs should raise the possibility of Lambert-Eaton
myasthenic syndrome and prompt the electromyographer to have the patient exercise the appropriate
muscle for 10 seconds and then repeat the CMAP measurement. High-frequency repetitive stimulation
at 50 Hz for I second can be performed as an alternative in patients who cannot cooperate. Low-fre-
quency stimulation at 2 or 3 Hz should also be performed. Observe the following figures and tables.

183
 Pre- and Post-Exercise CMAPs

Relative
Recording Latency Amplitude Amplitude Distance
Nerve Sites Site (ms) (mY) (%) (em)

L. ulnar-ADM At nist ADM 4.40 3.1 100 7

Immediately post ADM 4.25 6.8 221
10 s exercise
L. median-APB At rest APB 4.05 9.3 100 7
Immediately post APB 4.10 12.3 132
10 s exercise
R. median-APB At rest APB 4.00 6.1 100 7
Immediately post APB 3.90 10.3 168
10 s exercise
R. ulnar-ADM At rest ADM 3.50 2.5 100 7
Immediately post ADM 3.50 4.6 182
10 s exercise

Repetitive Nerve Stimulation

Rate Amplitude 4--1 5-1 Fac Amplitude

Muscle Train Time (Pps) (mY) (%) (%) (%)
L. abd dig min Baseline 0:01:21 3 2.6 -39.9 -30.2 103

L Ulnar-ADM

Post-exercise

Wrist

Pre-exercise 20ms2mV

Figure 1

184
 L Ulnar-ADM

Baseline
3 hz

2 ms 0.5 mV

Post 10 S Exercise

Figure 2

Questions:
1. What are the relevant abnormalities?
2. The bilateral ulnar-ADM and left peroneal-EDB CMAPs are reduced in amplitude. Are these find-
ings compatible with motor neuron disease? What other electrodiagnostic procedures should be
done when multiple CMAPs have reduced amplitude?
3. What finding is shown in figure I?
4. What is the generally accepted upper limit of normal (ULN, as percent of baseline) for physiolog-
ical post-exercise increment of a CMAP amplitude?
5. What finding is shown in figure 2?
6. What additional non-electrophysiological diagnostic evaluation is indicated for this patient?

185
 Answers:
1. Low amplitude bilateral ulnar-ADM CMAPs; post-exercise facilitations, low frequency decrement
2. Yes; post-exercise nerve stimulation
3. Abnormal post-exercise CMAP facilitation
4. 100% increment
5. Abnormal decrement to low frequency stimulation
6. Measurement of serum P/Q voltage-gated calcium channels

Discussion: The first figure shows abnormal small-cell lung cancer, and the other third have an
post-exercise facilitation of the left ulnar-ADM otherwise uncharacterized autoimmune disorder.
CMAP. A 100% increment is generally accepted as The clinical features are muscular weakness
the upper limit of normal; this CMAP shows a (proximal legs greater than arms; diplopia
121% increment. As seen in the table, several other uncommon), hyporeflexia, and cholinergic auto-
nerves have large increments, particularly the right nomic dysfunction (dry mouth, sluggish pupillary
ulnar (82%). The 100% cutoff is likely a very con- reflexes, urinary retention, and erectile dysfunc-
servative estimate; in clinical practice, post-exer- tion in males). Rapid progression over weeks or
cise increments of even 25% or more are quite months favors the paraneoplastic form as a cause.
uncommon, except in myasthenia gravis. The sec- Antibodies directed against the P/Q type volt-
ond figure shows a decrement at low-frequency age-gated calcium channels (VGCCs) of the
stimulation, maximal at the fourth potential, that motor nerve terminals are present in over 90% of
then repairs partially by the tenth potential. patients with LEMS, resulting in destruction and
These findings are diagnostic of a presynaptic loss of these presynaptic calcium channels.
disorder of neuromuscular transmission, and in Arriving action potentials consequently do not
this clinical setting are diagnostic of Lambert- generate sufficient calcium influx for release of
Eaton myasthenic syndrome. Additional studies acetylcholine. Electrodiagnostic studies typically
of value include measurement of serum voltage- demonstrate low-amplitude CMAP amplitudes
gated P/Q calcium channel antibodies and chest that show a greater than 100% increment with
CT scan for evidence of small-cell lung cancer. In high-frequency repetitive stimulation (50 Hz). At
this patient, the calcium channel antibody titer low-frequency stimulation, a decrement may
was highly abnormal at 51 U (normal, <19 U). CT occur in LEMS as in MG. Treatment with 3,4-
scan did not show chest abnormalities but did diaminopyridine, requiring an investigational
show liver lesions, and biopsy revealed small-cell new drug approval from the Food and Drug
cancer. The patient's weakness progressed, and he Administration in the United States, is the treat-
became nonambulatory. The patient was treated ment of choice. Immunosuppressive or immuno-
with chemotherapy and 3,4-diaminopyridine, and modulatory treatments similar to those for MG are
his strength improved to independent ambulation. effective, although less so than with MG.
The Lambert-Eaton myasthenic syndrome is the Treatment of underlying malignancy when present
second most common disorder of neuromuscular is also important and may improve strength.
transmission but is nevertheless rare. Its prevalence As a rule, both pre-synaptic and post-synaptic
is unknown but has been estimated to be as low as neuromuscular junction disorders typically have
a total of 400 cases in the entire United States. abnormal decrements with low-frequency stimu-
Approximately two-thirds of patients have a para- lation; presynaptic disorders have abnormal
neoplastic disorder, primarily associated with increments with high-frequency stimulation.

Clinical Pearls
1. Consider performing 10 seconds of exercise followed by a repeat CMAP record-
ing in patients with low CMAP amplitudes.
2. In Lambert-Eaton myasthenic syndrome, the increment does not necessarily
exceed 100% in all motor nerves.
3. Low-frequency stimulation may produce an abnormal decrement in pre-synaptic
disorders also; abnormalities are not specific to myasthenia gravis.

REFERENCES .
1. Tim, R.W., Massey, 1M., Sanders, 0.8., Lambert-Eaton myasthenic syndrome: e]ectrodiagnostic findings and response to
treatment, Neurology 2000; 54(] 1):2] 76-2178.
2. AAEM Quality Assurance Committee, Literature review of the usefulness ofrepetitive nerve stimulation and single fiber
EMG in the electrodiagnostic evaluation of patients with suspected myasthenia gravis or Lambert-Eaton myasthenic
syndrome, Muscle Nerve 200]; 24: 1239-]247.

1"86
 PATIENT 46

A 34-year-old woman with generalized weakness since infancy

This 34-year-old woman was a floppy baby and has had longstanding generalized weakness lead-
ing to use of a wheelchair for most activities. She carried a diagnosis of spinal muscular atrophy and
was referred for electrodiagnostic studies. Exam was notable for bilateral fatigable ptosis, and bilateral
proximal greater than distal weakness of the upper and lower extremities.
Electrodiagnostic Study: Routine sensory and motor studies were normal.

Repetitive Nerve Stimulation

Rate Amplitude
Muscle Train Time (pps) (mY) 4-1 (%)
R. abd dig min Baseline 0:00:00 3 7.6 -10.2
Post 1 min exercise 0:02:22 3 8.0 -2.3
After I min 0:03:15 3 8.4 -6.3.
After 2 min 0:04:14 3 8.5 -6.8
After 3 min 0:05:19 3 8.1 -7.9
After 4 min 0:06: 14 3 8.5 -9.9 '
After 5 min 0:07:18 3 8.5 -11.8
R. trapezius (u) Baseline 0:00:00 3 4.8 -33
Post 10 s exercise 0:00:33 3 5.3 -8.6
Post I min exercise 0:01:42 3 5.4 -11.1
After 1 min 0:02:49 3 5.4 -27.6
After 2 min 0:03:56 3 5.2 -38.4
After 3 min 0:05:07 3 4.9 -37.8
After 4 min 0:06:13 3 5.3 -41.3
After 5 min 0:07:14 3 5.4 -41.7

Needle EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
lA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R. biceps NI 0 0 0 Small Brief Few Full Early
R. deltoid NI 0 0 0 Small Brief Few Full Early

SFEMG Stimulation
Muscle N Jitter (Ils) Block MIPI (Ils) MCD (Ils) MSD (Ils) FR (Pps)
R. ext dig 1.1 15 46.26 2999 46.26 46.26 3
comm
2.1 99 45.89 2925 45.89 45.89 3
5.1 99 53.07 3171 53.07 53.07 3
6.1 40 56.59 2896 56.59 56.59 3
7.1 98 58.62 2464 58.62 58.62 3
7.2 100 118.44 5 5629 118.44 118.44 3
MIPI = meaninterpotentialinterval,MCD = meanconsecutivedifference,MSD = meansorteddifference,FR = firingrate
Continued

187
 SFEMG Stimulation--cont'd

Muscle N Jitter (~l.s) Block MIPI01S) MCD (~s) MSD (~s) FR (Pps)
8.1 100 139.56 60 11003 139.56 139.56 3
10.1 95 45.25 3809 45.25 45.25 3
ILl 100 68.17 0 3728 68.17 68.17 3
11.2 100 74.12 0 6563 74.12 74.12 3
11.3 100 117.78 20 10748 117.78 117.78 3
11.4 100 133.37 73 14705 133.37 133.37 3
Mean 79.76
% Blocked 46

R upper trapezius

Figure 1

10ms 200llV

Figure 2

188
 11
Block 333ms

12
Block 333ms

13
Block 333ms

91
Block 333ms

92
1 83 IJs 333ms

95
Block 333ms

Figure 3
t
Questions:
1. What do the needle EMG studies suggest?
2. What is the overall interpretation of the electrodiagnostic study?
3. Can you identify what is being demonstrated in each of the figures?
4. What do you think is the most likely diagnosis for this patient?

189
 Answers:
1. A myopathy or neuromuscular junction disorder
2. A neuromuscular junction disorder
3. Abnormal decrement; abnormal jitter; intermittent blocking
4. A congenital myasthenic syndrome

.Discussion: The electrodiagnostic study shows response to low-frequency stimulation, and nega-
an abnormal decrement in the trapezius muscle tive tests for acetylcholine receptor (AChR) anti-
at low-frequency stimulation: 33% at baseline bodies. In some CMSs, however, the onset is
and 42% 5 minutes after exercise. There was no delayed, and patients may present with an appar-
abnormal increment after 10 seconds of exercise, ent myopathy in adulthood.
and the baseline CMAP amplitudes were normal. • CMS with defect in resynthesis/packing: This
There were also no after-discharges seen on sin- disorder is also known as CMS with episodic
gle motor stimuli during routine motor studies. apnea and as familial infantile myasthenia.
The first figure shows intermittent blocking of a The presentation is in infancy or early child-
single fiber potential (arrows) in a series of 10 hood with a range of myasthenic symptoms,
consecutive recordings. including apnea provoked by fever or excite-
The most likely diagnosis for this patient is a ment. The molecular basis is choline acetyl-
congenital myasthenic syndrome. The history of transferase mutations. This enzyme catalyzes
weakness since infancy, fatigable ptosis on exam- the reversible synthesis of ACh from acetyl
ination, and demonstration of a postsynaptic dis- CoA and choline, resulting in stimulus-
order of neuromuscular transmission are all dependent depletion of ACh.
suggestive of such. Laboratory studies showed • Paucity of synaptic vesicles: The clinical fea-
repeatedly absent acetylcholine receptor antibod- tures are similar to myasthenia gravis, the
ies over 2 decades of testing. molecular basis is unknown, and diagnosis
, . The congenital myasthenic syndromes (CMSs) requires specialized techniques (quantitative
are a diverse group of molecular genetic defects electron microscopy of the endplate).
affecting the neuromuscular junction. The classi- • Lambert-Eaton-like CMS: Only two patients
fication and molecular basis for these are still have been described with this syndrome. The
evolving. A review by Engel et al.4 covers this defect lies in a subunit of the presynaptic
topic in depth. The table below highlights the voltage-gated P/Q-type calcium channel or in
main disorders. The diagnosis of a CMS should a component of the synaptic vesicle release
be suspected from a history of fluctuating weak- complex. Patients have marked facilitation
ness involving ocular, bulbar, and limb muscles of CMAPs with high-frequency repetitive
since infancy or early childhood, a history of sim- stimulation.
ilarly affected relatives, a decremental EMG

Congenital Myasthenic Syndromes

Defects Inheritance Molecular Genetics

Presynaptic defects
Defect in ACh resynthesis/packaging AR ChAT
Paucity of synaptic vesicles (1 patient to date) ? Unknown
Lambert-Eaton syndrome like AR Unknown
Reduced quantal release (3 patients to date) ? Unknown
Synaptic defect
Endplate AChE deficiency AR ColQ gene
Postsynaptic defects
Primary kinetic abnormality AR
Slow channel syndromes AD n, 13, or £ AChR subunit
Fast channel syndromes AR n, 0, or £ AChR subunit
Primary AChR deficiency AR AChR £-subunit null mutation
Rapsyn deficiency AR Rapsyn
Myasthenic syndrome with plectin deficiency AR
No identified defect

AR = autosomal recessive; AD = autosomal dominant

190
 • Endplate acetylcholinesterase deficiency: space, and subsynaptic cellular degeneration .
Patients born with a congenital deficiency of This disorder is the only CMS that is autoso-
acetylcholinesterase present soon after birth mal dominant. Repetitive CMAPs evoked
or in early childhood with fluctuating ptosis, from a single supramaximal nerve impulse is
extraocular muscle weakness, generalized a common finding, as in AChE deficiency ...
delay in motor development, weakness exac- • Fast channel syndromes: This disorder is
erbated by exertion, poor cry and suck, and rare. The clinical features are generally
respiratory muscle weakness. The disorder is severe weakness at birth with poor suck and
due to mutations in the gene encoding for weak cry. The AChR channel opens and
CoIQ, the collagen tail of AChE anchoring it closes too quickly, resulting in the ineffec-
to the basement membrane. A single electri- tiveness of acetylcholine to effect muscle
cal stimulus produces repetitive compound depolarization. Mutations in AChR subunits,
muscle action potentials during routine nerve as in slow channel syndromes, appear to be
conduction studies. the cause. A mutation of the fetal O-subunit
• Slow channel syndromes: These disorders may result in arthrogryposis multiplex con-
result from mutations in various subunits of genita due to absence of fetal movement in
the AChR. Individuals with the slow channel utero.
syndrome may present at essentially any time • Primary AChR deficiency: Several patients
(i.e., infancy, childhood, or adulthood). Most have been described with a presentation dur-
patients show selective and severe involve- ing the neonatal period characterized by
ment of cervical, scapular, wrist, and finger feeding difficulties, nasal regurgitation, pto-
extensor muscles. In effect, these patients sis, impaired eye movements, and reduced
have a myopathy due to prolonged opening overall tone. These disorders mainly result
episodes of the AChR channel in the pres- from AChR E-subunit null or low expression
ence of ACh (the channel is slow to close). mutations resulting in severe loss of function
Even choline in its normal serum concentra- of the subunit. In these patients, the few
tion opens the channel and renders it leaky in AChR present include the y-subunit (a fetal
the resting state. The result is cationic over- subunit typically found replacing the E-sub-
loading of the junctional sarcoplasm result- unit during AChR development) instead of
ing in degeneration of the junctional folds the E-subunit. It may be that null mutations of
with loss of AChR, widening of the synaptic the other AChR subunits are lethal.

Clinical Pearl
The diagnosis of a CMS should be suspected from a history of fluctuating weakness
involving ocular, bulbar, and limb muscles since infancy or early childhood, a history
of similarly affected relatives, a decremental EMG response to low-frequency stimula-
tion, and negative tests for acetylcholine receptor (AChR) antibodies.

REFERENCES
1. Brownlow, S., Webster, R., Croxen, R., Brydson, M., Neville, 8., Lin, J.P., Vincent, A., Newsom-Davis, J., Beeson, D.,
Acetylcholine receptor delta subunit mutations underlie a fast-channel myasthenic syndrome and arthrogryposis multiplex
congenita, J. Clin. Invest. 200 I; 108(1): 125-130.
2. Croxen, R., Young, C, Slater, C., Haslam, S., Brydson, M., Vincent, A., Beeson, D., End-plate gamma- and epsilon-subunit
mRNA levels in AChR deficiency syndrome due to epsilon-subunit null mutations, Brain 200]; I 24(pt. 7): 1362-1372.
3. Engel, A.G., 73rd ENMC International Workshop: congenital myasthenic syndromes, 22-23 October, 1999, Naarden, The
Netherlands, Neuromuscul. Disord. 2001; 11(3):315-321.
4. Engel, A.G., Ohno, K., Sine, S.M., Congenital myasthenic syndromes: progress over the past decade, Muscle Nerve 2003;
27:4-25.
5. Ohno, K., Tsujino, A., Brengman, J.M., Harper, C.M., Bajzer, Z., Udd, 8., Beyring, R., Robb, S., Kirkham, F.J., Engel,
A.G., Choline acetyltransferase mutations cause myasthenic syndrome associated with episodic apnea in humans, Proc.
Nat!. Acad. Sci. USA 2001; 98(4):2017-2022.
6. Zhou, M., Engel, A.G., Auerbach, A., Serum choline activates mutant acetylcholine receptors that cause slow channel
congenital myasthenic syndromes, Proc. Natl.Acad. Sci. USA 1999; 96:10466-10471.

191
SECTION IV. MOTOR NEURON DISEASE AND
MOTOR NEUROPATHIES

The electrodiagnosis of motor neuron diseases and of motor neuropathies are intricately associ-
ated. As the principal motor neuron disease, amyotrophic lateral sclerosis (ALS) is universally fatal,
while motor neuropathies are effectively treatable, the physician must thoroughly explore the possibil-
ity that a motor neuropathy is present in patients with suspected motor neuron diseases.
In a patient with a suspected motor neuron disease, the broad goals of the study include:
• Demonstration of a "generalized" disorder of lower motor neurons. "Generalized" can be vari-
ously defined, and the electromyographer should state the definition in the study's report. Because
many clinical trials have adopted the Revised World Federation of Neurology's EI Escorial crite-
ria for patient enrollment,7 this is one commonly used definition. These criteria require needle
EMG abnormalities in two of four regions: bulbar, cervical, thoracic (paraspinals at or below T6,
or abdominal muscles), and lumbar. For the bulbar or thoracic region, one abnormal muscle is
required, while for the cervical or lumbosacral, two abnormal muscles innervated by different
roots and peripheral nerves are required. Another commonly used definition is the presence of
abnormalities in at least two distinct nerve distributions (including root, plexus, or peripheral
nerve) in each of three limbs. Specific abnormalities indicating lower motor neuron (LMN) dis-
ease include signs of acute denervation (fibrillation potentials and positive sharp waves), reinner-
vation (increased amplitude or duration of motor unit action potentials or the presence of satellite
potentials), and reduced interference patterns. Some advocate that the presence of varying motor
unit morphology should also be considered an abnormality. Fasciculation potentials should be
sought, although they do not provide evidence of definite abnormalities by themselves.
• Demonstration of a lack of focal motor demyelination to exclude a treatable motor neuropathy.
An extensive search for a segment of focal motor demyelination should include at least bilat-
eral median-APB and ulnar-ADM motor studies with multiple stimulation sites, including axil-
lary stimulation, and, if possible, bilateral peroneal-EDB and tibial-AH studies as well. A careful
F-wave study should also be performed on all motor nerves studied.
• Demonstration of normal sensory nerve studies, particularly in anatomic territories overlapping
with those of severe motor involvement. The demonstration of normal sensory nerve action poten-
tial (SNAP) amplitudes helps exclude focal neuropathies (such as a median and ulnar neuropathy)
and X-linked bulbospinal neuronopathy, a lower motor neuron disorder that frequently has reduced
SNAP amplitudes in the absence of sensory symptoms or signs. A very strong case for a motor
neuron disorder is often made by the demonstration of normal SNAP amplitudes in distributions
with severe motor axonal loss. For example, an absent median-APB compound muscle action
potential (CMAP) along with normal median-D2 and ulnar-D5 SNAPs would be difficult to
attribute to a focal peripheral lesion, such as a median neuropathy or medial cord plexus lesion.
• Note the presence of suprasegmental recruitment patterns, possibly indicative of upper motor neu-
ron weakness. ALS is a disorder of upper and lower motor neurons. Upper motor neuron weakness
is evidenced on needle EMG studies as a suprasegmental interference pattern: a small number of
motor units firing at low frequency in a weak muscle, despite the patient's stated full effort not lim-
ited by pain. One should not be definitive about concluding the presence of a disorder of upper motor
neurons by needle EMG interference patterns, but such a pattern is highly suggestive of such.
• Note the presence and distribution of fasciculation potentials. Fasciculation potentials by them-
selves are not generally considered abnormal. In the setting of a probably generalized disorder of
lower motor neurons, however, they often indicate a diseased motor unit and may be a prelude to
denervation. As such, they indicate muscles that might be valuable in demonstrating future abnor-
malities should a subsequent electrodiagnostic study be required.
A number of specialized electrodiagnostic techniques, including motor unit number estimation
(MUNE) and the use of transcranial magnetic stimulation for calculation of central motor conduction
time, are currently primarily of research value and are not routine in clinical practice.
192
REFERENCES
I. Brooks, B.R., EI Escorial World Federation of Neurology: criteria for the diagnosis of amyotrophic lateral sclerosis,
J. Neurol. Sci. 1994; I 24(suppl.):96-107.
2. Cornblath, D.R., Kuncl, R.W., Mellits, E.D., Quaskey, S.A., Nerve conduction studies in amyotrophic lateral sclerosis,
Muscle Nerve 1992; 15:1111-1115.
3. Daube, 1.R., Electrodiagnostic studies in amyotrophic lateral sclerosis and other motor neuron disorders. Muscle Nerve
2000; 23:1488-1502.
4. Layzer, R.B., The origin offasciculations and cramps. Muscle Nerve 1994; 17:1243-1249.
5. Olney, R.K., American Association of Electrodiagnostic Medicine. Consensus criteria for the diagnosis of partial
conduction block, Muscle Nerve 1999; 22:(suppl. 8):225-229.
6. Olney, R.K., Aminoff, MJ., So, YT., Clinical and electrodiagnostic features of X-linked recessive bulbospinal
neuronopathy, Neurology 1991; 41:823-828.
7. Ross, M.A., Miller, R.G., Berchert, L., Parry, G., Barohn, RJ., Armon, c., Bryan, W.W., Petajan, 1., Stromatt, S.,
Goodpasture, 1., McGuire, D., Toward earlier diagnosis of amyotrophic lateral sclerosis: revised criteria, Neurology 1998;
50:768-772.
8. Yuen, E.C., Olney, R.K., Longitudinal study of fiber density and motor unit number estimate in patients with amyotrophic·
lateral sclerosis, Neurology 1997; 49:573-578.

193
 PATIENT 47
A 55-year-old man with progressive left hand weakness for
6 months

Over the previous 6 months, this man has noted difficulty writing, buttoning a shirt, and turning
a key. He has noted frequent left hand finger extensor cramps and infrequent similar cramps in the right
hand: Neurological exam showed atrophy and frequent fasciculations diffusely in left arm muscles,
particularly in ventral forearm flexors, thenar, and hypothenar muscles, but also deltoid, biceps, and
triceps. Reflexes were brisker in the left arm than the right; for example, the left biceps was MRC grade
3 strength with a 3+ reflex.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (J.lV) (cm) (mls)

L. median-dig II I. Wrist Dig II 2.80 3.55 13.2 13 46.4

R. median-dig II I. Wrist Dig II 2.65 3.35 21.6 13 49.1
L. ulnar-dig V l. Wrist DigV 2.25 3.05 13.8 11 48.9
R. ulnar-dig V I. Wrist DigV 2.65 3.35 6.4 II 41.5
L. radial-sn box l. Forearm Sn box 1.60 2.15 18.8 10 62.5
L. med AB cut I. Elbow Forearm 2.10 2.50 3.8 12 57.1
R. medAB cut I. Elbow Forearm 1.80 2.30 6.4 12 66.7
L. latAB cut I. Elbow Forearm 2.00 2.50 8.9 12 60.0

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)

L. median-APB I. Wrist APB 5.30 0.6 7

2. Elbow APB 10.25 0.4 24 48.5
R. median-APB 1. Wrist APB 3.80 6.5 7
2. Elbow APB 7.95 6.3 24.5 59.0
3. Axilla APB 11.45 6.5 20 57.1
L. ulnar-ADM 1. Wrist ADM 4.65 0.8 7
2. B. elbow ADM 9.80 0.7 23 44.7
3. A. elbow ADM 12.90 0.6 14 45.2
R. ulnar-ADM }. Wrist ADM 3.35 9.3 7
2. B. elbow ADM 7.25 8.2 23.5 60.3
3. A. elbow ADM 9.20 7.6 11 56.4
4. Axilla ADM 11.65 7.3 15 61.2
L. ulnar-FDI 1. Wrist FDI 5.55 l.l
2. B. elbow FDI 9.75 1.0 23 54.8
3. A. elbow FDI 13.55 0.9 14 36.8

194
 F-Wave

Nerve F min (ms) F max (ms) Max - Min (ms) %F

L. ulnar Absent
R. median 29.60 31.65 2.05 80
R. ulnar 28.30 30.95 2.65 80

Needle EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
Left arm
L. deltoid Incr 2+ 2+ 0 NI NI NI Full NI
L. biceps Incr 2+ 4+ 0 Nl NI NI Full NI
L. triceps Incr 3+ 3+ 0 NI NI Few Full Mod red
L. ext Indicis NI 0 0 0 NI NI NI Full Nt
L. ext dig comm Incr 3+ 2+ 0 NI NI NI Central Sev red
L. first dors int Incr 3+ Rare 0 NI NI NI Central Sev red
Left leg
L. tib ant NI 0 Rare 0 NI NI NI Full NI
L. gastroc med NI 0 Rare 0 NI NI NI Full NI
L. vast med NI 0 0 0 NI NI NI Full NI
L. glut med NI 0 0 0 NI NI NI Full NI
L. bic fern SH NI 0 0 0 NI NI NI Full NI
Thoracic
paraspinals
L. thoracic PSP lower NI 0 0 0 -
L. thoracic PSP mid NI 1+ 1+ 0
L. thoracic PSP upper NI 1+ 1+ 0
Right arm
R. deltoid NI 0 Rare 0 NI Nl NI Full NI
R. biceps Nl 0 0 0 Nl NI Nl Full NI
R. triceps NI 0 0 0 NI NI NI Full NI
R. pron teres NI 0 Rare 0 NI NI NI Full NI
R. abd poll br NI 0 0 0 NI NI NI Full NI
R. first dors int Incr 1+ 0 0 NI Nl NI Full .NI
Right leg
R. tib ant NI 0 0 0 NI NI NI Full NI
R. vast med NI 0 0 0 NI NI NI Full NI

Questions:
1. Does this study provide evidence of a generalized disorder of motor neurons?
2. Is there e1ectrophysiological evidence of a potentially treatable motor neuropathy?
3. Is the prolonged left median-APB distal motor latency likely due to carpal tunnel syndrome?
4. Are the sensory amplitudes normal?
5. What muscles might be valuable to study in a follow-up EMG examination should one be needed
for diagnosis?

195
 Answers:
I. Yes
2. No
3. Possibly
4. All except the right ulnar-D5
5. Right deltoid, pronator teres, left tibialis anterior, gastrocnemius

Discussion: We analyze this case from the normal (ULN), a median DML of greater than
perspective put forth in the introduction to this 6.8 or ulnar DML of greater than 6.0 would be
section: required. In answer to question 3, it is possible
.• Is there electrophysiological evidence of a gen- that the mild prolongation in the left
eralized disorder of motor neurons? We look for median-APB DML is due to carpal tunnel
reduction in CMAP amplitudes and for the pres- syndrome, and we note a mild but not clearly
ence of fibrillation potentials or reduced recruit- abnormal reduction in the left median-D2
ment of motor units. The left median-APB, SNAP amplitude compared to the right (13 11V
ulnar-ADM, and ulnar-FDI CMAPs are all vs. 21 11V). However, the across-wrist sensory
reduced, and fibrillation potentials are present in velocity is normal (46 m/s). Ideally, a more sen-
· at least two myotomes in the left arm, C5/6 (del- sitive test (see case 2) such as the median-ulnar
toid) and C8ffl (FDI). Fibrillation potentials palm-wrist interlatency difference would best
· are also present in thoracic paraspinal muscles answer this question.
and in right FDI. Accordingly, the World • Is the neuronal degeneration selective (i.e.,
Federation of Neurology revised EI Escorial cri- are the SNAP amplitudes normal)? Seven of
. teria for "generalized" are met, but not the crite- eight SNAP amplitudes are normal. The right
ria of at least two distinct nerve distributions in ulnar-D5 SNAP amplitude is reduced in iso-
each of three different limbs. lation, a likely incidental finding or sugges-
• Is there electrophysiological evidence of con- tive of a separate nonlocalizing right ulnar
· duction block of motor axons? We have looked neuropathy. As evidence of selectivity of the
at multiple motor segments including bilateral neuronal degeneration, we note the reduced
median and ulnar nerves and including axillary amplitudes of the left median-APB and
stimulation and F-wave responses. There is ulnar-ADM CMAPs, but preserved left
mild prolongation of left median-APB and median-D2 and ulnar-D5 SNAP amplitudes.
ulnar-ADM distal motor latencies, and all • Which muscles normal in the current study
· other motor segments, including F-waves, are should particularly be studied if afollow-up nee-
normal (no conduction block or focal slowing). dle EMG exam isperfOrmed in thefUture? These
Can this be accounted for by the degree of are the muscles with fasciculations present: the
axonal loss? The answer is probably, but there right deltoid and pronator teres and the left tib-
are no definite data that establish this. If we ialis anterior and gastrocnemius. The appear-
apply the conservative research criteria for ance of fibrillation potentials in these muscles in
focal demyelination commonly used in the a follow-up exam would allow the study to meet
diagnosis of acute inflammatory demyelinating the "generalized" criteria of two distinct nerve
.polyneuropathies (AIDP) or chronic inflamma- distributions in each of three limbs.
tory demyelinating polyneuropathy (CIDP) This patient has ALS. Six months after the initial
(see case 32), requiring a distal motor latency study, his left arm weakness had progressed, and
(DML) of greater than 150% the upper limit of right hand and left leg weakness had developed.

Clinical Pearls
The following questions should be addressed in the electrophysiological evaluation
of possible motor neuron disorders:
I. Is there electrophysiological evidence of a generalized disorder of motor neurons?
2. Is there electrophysiological evidence of conduction block of motor axons?
3. Is the neuronal degeneration selective (i.e., are the SNAP amplitudes norma!)?
4. Which muscles normal in the current study should particularly be studied if a fol-
low-up needle EMG exam is performed in the future?

REFERENCES
See the references listed in the introduction to this section.
196
 PATIENT 48

A 3-month-old boy with congenital hypotonia and weakness

A 3-month-old boy was referred for evaluation of congenital hypotonia and weakness since birth.
The mother noted reduced fetal movements during this pregnancy compared to when she was pregnant
with her other healthy 2-year-old son. On exam, the child was lying in a frog-leg posture. There was
little spontaneous movement of the extremities. He was floppy and muscle bulk was diminished. There
was decreased suck and quivering movements of the tongue. Muscle stretch reflexes were absent.
Electrodiagnostic Study:

Sensory NCS

Recording BP Amplitude Velocity

Nerve Sites Site (~V) (m/s)
L. median-dig II I. Wrist Dig II 16 35
L. sural-Iat mall I. Mid-calf Ankle 15 38

Motor NCS

Recording Latency Amplitude Velocity

Nerve Sites Site (ms) (mV) (m/s)
L. median-APB I. Wrist APB 3.0 0.1 -
2. Elbow APB Absent
L. peroneal-EDB I. Ankle EDB 3.3 0.1
2. Fib head EDB Absent

Needle EMG Summary

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Pattern
L. vast lat Incr 3+ 2+ 0 Many small, Many short, NI Marked red
few large few long
L. tib ant Incr 3+ 2+ 0 Many small, Many short, NI Marked red
few large few long

Questions:
I. What is the differential diagnosis of a floppy infant?
2. How would you proceed in evaluating this child?

197
 Answers:
I. Congenital myopathy, congenital myasthenic syndrome, spinal muscular atrophy, others
2. Genetic testing for SMN gene mutations

Discussion: The nerve conduction studies larger units may be appreciated. The MUAPs
were remarkable for low-amplitude CMAPs with numbers are decreased and fire at rapid rates (i.e.,
normal distal latencies. The sensory studies were demonstrate reduced recruitment).
normal. Needle EMG demonstrated fibrillation Muscle biopsy reveals severe groups of
potentials, positive sharp waves, and fasciculation atrophic, rounded, type I and 2 fibers intermixed
potentials at rest. Motor units had a mixed mor- with scattered large rounded fibers (usually type I
phology, and recruitment was markedly reduced. fibers). With DNA testing now available, there is
The overall clinical picture and electrodiagnostic little indication for performing muscle biopsies
findings were suggestive of a lower motor neuron on these patients.
syndrome (e.g., spinal muscular atrophy). Spinal muscular atrophy types 1 to 3 are due to
, Spinal muscular atrophy (SMA) type I or mutations in the spinal motor neuron gene (SMN)
Werdnig-Hoffinann disease manifests within the located on chromosome 5q13. Two almost identical
first 6 months oflife. About 30% of patients man- SMN genes-telomeric SMN (SMNt) and cen-
ifest in utero with decreased fetal movements. tromeric SMN (SMNc}-differ by five nucleotides.
The incidence is approximately 1 in 25,000 live Normal individuals contain two copies of SMNt
births, with males and females being equally and several copies of SMNc' SMA is caused by
affected. Infants manifest with severe generalized mutations in both SMNt alleles. Approximately
weakness and hypotonia. Most infants are never 98% of the causes are associated with deletions,
able to sit without support when placed. Infants usually involving exons 7 and 8, while the other 2%
have weak sucking motions, and difficulty swal- are the result of conversion of the SMNt genes to
lowing and poor clearing of secretions are quite the SMNc sequence. The age of onset and severity
common. Fasciculations are evident in the tongue. of SMA may be modified by the number of intact
A fine tremor of the fingers may be seen second- copies of SMNc and other neighboring genes.
ary to hand intrinsic fasciculations. The SMN protein is present in the cytoplasm of all
Sensory nerve conduction studies are normal in cells and in nuclear structures called gems that asso-
SMA. Motor nerve conduction studies may be ciate with nuclear coiled bodies. These gems and
normal during the early course of the disease coiled bodies are believed to serve as storage sites for
process; however, as the disease progresses and spliceosomes that excise introns from newly synthe-
there is considerable loss of anterior horn cells, a sized small nuclear RNA (snRNA) to produce mes-
resultant drop in CMAP amplitudes is observed. senger RNA (mRNA). However, prior to this
Mild slowing of conduction velocities propor- splicing of the snRNA into mRNA, SMN protein
tional to the loss of large myelinated axons (usu- binds to and shuttles snRNA out of the nucleus and
ally not more than 25% below the lower limit of into the cytoplasm, where they undergo methylation
normal) may be observed. The needle electromyo- to form mature small nuclear ribonucelic protein
graphic examination is abnormal. Fibrillation (snRNP), or "snurps." SMN protein then shuttles the
potentials, positive sharp waves, and fasciculation mature snRNP back into the nucleus, where splicing
potentials are commonly appreciated. Motor unit to mRNA occurs. Thus, the fundamental defect in
action potentials (MUAPs) may be decreased in SMA appears to involve abnormal trafficking and
amplitude and short in duration due to the diffuse splicing of RNA species. How this leads to destruc-
atrophy and lack of reinnervation, although a few tion of motor neurons is unclear.

Clinical Pearl
In the evaluation of generalized weakness in an infant, focus on sensory studies (for
possible neuropathy) and needle EMG to distinguish lower motor neuron from muscle
or neuromuscular junction disease.

REFERENCES
I, Dumitru, D., Amato, A.A., Disorders of motor neurons, in Dumitru, D., Amato, A.A., Swartz, M.J., Eds., Electrodiagnostic
Medicine, 2nd ed., Hanley & Belfus, Philadelphia, PA, 2002, pp. 581-65l.
2. Iannaccone, S.T., Russman, B.S" Brown, R.H. et al., Prospective analysis of strength in spinal muscular atrophy, J Child
Neurol. 2000; 15:97-10l.
3. Morris, G.E., Nuclear proteins and cell death in inherited neuromuscular disease, Neuromusc. Dis. 2000; 10:217-227.

198
 PATIENT 49

A 66-year-old man with a 5-year history of progressive weakness

and wasting of his distal right leg

At the age of 61, this man noted the onset of right ankle weakness that gradually increased in it
slow, stepwise fashion with prolonged periods of relatively little change. His right foot became essen-
tially flail, with very poor plantar flexion and almost no dorsiflexion. There was no pain, numbness,
tingling or any sensory disturbance. Neurological examination at age 66 was remarkable for extensive
atrophy of the right leg below the knee, affecting all muscle groups. Eversion and dorsiflexion of foot
and toes were trace at best, and plantarflexion and inversion were 2/5. The hamstrings and the buttocks
were normal in bulk and strength, and there was no sensory deficit. The knee reflex was normal, the
ankle reflex absent, and the plantar response silent. There was no spasticity. There were no symptoms
in the other three limbs.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (~V) (cm) (m/s)
R. median-dig II I. Wrist Dig II Absent 13
L. median-dig II 1. Wrist Dig II 3.75 4.65 17.2 13 34.7
R. ulnar-dig V I. Wrist DigV 2.65 3.55 23.4 II 41.5
L. ulnar-dig V 1. Wrist DigV 2.80 3.70 26.0 11 39.3
R. sural-Iat mall I. Mid-calf Ankle 2.60 3.40 11.6 14 53.8
L. sural-lat mall 1. Mid-calf Ankle 3.55 4.40 13.3 14 39.4
R. sup peroneal 1. Lat leg Ankle 3.60 4.30 6.8 14 38.9
L. sup peroneal 1. Lat leg Ankle 2.55 3.35 5.7 12 47.1 ..

Motor NCS
Relative
Recording Latency Amplitude Amplitude Distance Velocity'
Nerve Sites Site (ms) (mV) (%) (cm) (rnIs)
R. median-APB I. Wrist APB 10.8 5.7 100 7.
2. Elbow APB 18.4 0.8 14 25.5 26.8.
3. Axilla APB 20.8 0.8 14 12 50
L. median-APB I. Wrist APB 5.35 8.5 100 7
2. Elbow APB 9.05 8.8 103 23 62.2
R. ulnar-ADM I. Wrist ADM 3.95 8.8 100 7
2. B. elbow ADM 7.65 7.1 80.9 21 56.8
3. A. elbow ADM 9.25 7.0 80 10 62.5
4. Axilla ADM 11.15 6.3 71.3 10 52.6
L. ulnar-ADM I. Wrist ADM 3.50 10.5 100 7
2. B. elbow ADM 7.55 6.0 57.2 20.5 50.6
3. A. elbow ADM 9.65 6.1 57.8 1I.5 54.8
L. median-ADM 1. Elbow ADM No Martin-Gruber
R. peroneal-EDB 1. Ankle EDB 7.25 0.4 100 8
2. Fib head EDB 15.65 0.4 97.9 32 38.1
3. Pop fossa EDB 18.30 0.4 96.2 8 30.2
L. peronea1-EDB I. Ankle EDB 7.45 2.7 100 8
2. Fib head EDB 15.90 2.5 90.3 33 39.1

Continued

199
 Motor NCS-cont'd
Relative
Recording Latency Amplitude Amplitude Distance Velocity
Nerve Sites Site (ms) (mV) (%) (cm) (m/s)

3. Pop fossa EDB 18.70 2.6 94.8 10 35.7

R. peroneal-tib ant 1. Fib head tib ant .3.10 1.0 100 10
2. Pop fossa tib ant 6.95 0.9 87.7 8.5 22.1
R. tibial-AH 1. Ankle AH Absent 8
L. tibial-AH I. Ankle AH 6.55 6.0 100 8
2. Pop fossa AH 17.40 4.1 68 40 36.9

F-Wave

Nerve Fmin (ms) F max (ms) Persistence

L. tibial 70.05 74.35 8/10

L. peroneal 66.15 71.35 6/10
R. median 42.35 48.45 9/10
R. ulnar 31.65 34.15 9/10
L. median 32.65 34.95 7/10
L. ulnar 31.90 33.30 7/10

H-Retlex

Nerve H Latency (ms)

L. tibial-soleus Absent
R. tibial-soleus 50.35

EMG Summary Table

SA

Amplitude . Duration PolyP Recruitment

lA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. vasflat NI 0 0 0 NI NI NI Full NI
R. tib ant Incr 2+ 0 0 NI NI NI Full SevRed
R. gastrocmed Incr 3+ 0 CRD NI NI NI Full SevRed
R. peron In Incr 3+ 0 0 NI NI NI Full SevRed
R. bicfem SH NI 0 0 0 NI NI NI Full NI
R. glut med NI 0 0 0 NI NI NI Full NI
R. L5 para-spinals Incr 1+ 0 0 NI NI NI Full NI
R. S 1 para-spinal NI 0 0 0 NI NI NI Full Nl
L. L5para-spinals NI 0 0 0 NI NI NI Full NI

200
 R Median - APB

2 •

Wristl
30ms5mV,

1 2 3 4

--~--~---------~--+- I~I .. Elbow 2

30ms5m0'

• 1 • 2 3 '
vI'- --------r--- I ~
30ms5mV

Figure 1

. L Median R Median
1.1

1.2

1.3

1.4

1.5
1.6

1.1 1.8

1.l;l
. 1.10
1.11
1.12
. 1.13
~ 10~ms ~OO~V.

F Wave: L Median F Wave: R Median

Figure 2

ZOt
 Questions: This patient was initially diagnosed with ALS. How would you classify this syn-
drome? What would be the potential value and rationale for performing extensive motor conduction
studies in all four limbs?

~02
 Answers: Motor neuropathy with conduction block; detection requires extensive motor studies

Discussion: The patient's clinical syndrome, globulin (IVIG) monthly; after 8 months, strength
prior to electrophysiological characterization, is in ankle dorsiflexion and toe extension and flexion
that of a progressive unilateral distal lower motor had improved. Electrodiagnostic studies (see
neuron degeneration. There is marked motor below and figure 3) showed resolution of the
axonal loss in the distal right leg without sensory median nerve conduction block and forearm slow-
or upper motor neuron findings. This syndrome ing and improvement in peroneal and tibial distal
always raises the possibility of ALS but is also evoked CMAP amplitudes.
indicative of multi focal motor neuropathy Multifocal motor neuropathy has a spectrum of
(MMN) and rarely structural disorders. presentations, including a purely demyelinating
The nerve conduction study abnormalities are: form, an asymmetric lower motor neuron degen-
• Absent right median-D2 SNAP and pro- eration (as in this case), and a motor unit hyper-
longed left median-D2 distal sensory latency activity disorder (see case 50). The diagnosis of
• Reduced amplitude of the right peroneal- the asymmetric lower motor neuron degeneration
EDB, peroneal- TA, and tibial-AH CMAPs form ofMMN is one of the most significant con-
• Asymmetric prolongation of bilateral median- tributions the electromyographer can make to the
APB distal motor latencies clinical care of a patient. Accordingly, the elec-
• Conduction block (86% amplitude drop) and trodiagnostic evaluation of patients with sus-
focal slowing of the right median forearm pected ALS and a purely lower motor neuron
motor segment (figure I) syndrome should include extensive search for
• Probable conduction block (43% amplitude demyelination, including motor conduction stud-
drop) of the left ulnar forearm segment (note ies of as many nerve segments as can be accom-
that the amplitude drop was not due to the plished and F-wave studies. Bilateral median
presence of a Martin-Gruber anastomosis) and ulnar studies, including axillary stimulation,
• Focal slowing of the right peroneal-EDB and and bilateral peroneal and tibial studies including
right peroneal- TA across- fibular-head motor F-waves should almost always be performed in
segments this evaluation. When appropriate, radial motor
• Prolongation of the right median, left studies might also be considered. Erb's point
peroneal, and left tibial minimum F-wave and root stimulation to demonstrate conduction
latencies block have the disadvantage that a significant
• Prolongation of the right and absent left tib- number of patients cannot be supramaximally
ial-soleus H-reflex stimulated at these sites, making it impossible
These findings provide evidence of multi focal to confidently demonstrate conduction block.
demyelination of motor nerves and support a diag- American Association ofElectrodiagnostic Medicine
nosis of multi focal motor neuropathy. In addition, (AAEM) consensus criteria do not recognize
there is evidence of sensory involvement for the Erb's point stimulation for the demonstration of
median nerves at the wrist, which might reflect the definite partial conduction block, although they
coexistence of bilateral carpal tunnel syndromes. do allow it for the demonstration of "probable"
In this case, motor axonal degeneration dominates partial conduction block. The criteria do not allow
the clinical and electrodiagnostic picture, but for root stimulation at all. Subtle evidence of
unequivocal evidence of demyelination is crucial demyelination is sometimes limited only to
for diagnosis and management. This patient was F-wave minimum latency prolongation, which
subsequently treated with intravenous immuno- should be interpreted attentively but cautiously.

Electrodiagnostic Studies 8 Months After Monthly IVIG Treatment:

Motor NCS

Relative
Recording Latency Amplitude Amplitude Distance Velocity
Nerve Sites Site (ms) (mV) (%) (em) (m/s)
R. median- 1. Wrist APB 11.40 6.0 100 7
APB 2. Elbow APB 14.60 4.8 79.5 22 68.7
3. Axilla APB 16.85 5.4 89.2 10 44.4

Continued

203
 Motor NCS-cont'd
Relative
Recording Latency Amplitude Amplitude Distance Velocity
Nerve Sites Site (ms) (mV) (%) (cm) (m/s)

R. peroneal- 1. Ankle EDB 9.35 1.6 100 8

EDB 2. Fib head EDB 20.25 1.3 82.6 32 29.4
3. Pop fossa EDB 25.10 1.3 85.4 10 20.6
R. tibial-AH 1. Ankle AH 10.70 0.1 100 8
2. Pop fossa AH 27.15 0.1 79.5 - 38 23.1

F-Wave

Nerve Fmin(ms) Fmax (ms) Max - Min (ms) %F

R. median 44.70 46.95 2.25 7/10

R. peroneal Absent
R. tibial Absent

After 8 months of IVIG treatment - R Median ~APB

R Median - APB
2

5
Wrist 1

50ms 5mV

5 Elbow 2
50ms 5mV

,2

Axilla 3
50ms5mV

Figure 3

Clinical Pearls
1. The electrodiagnostic evaluation of suspected motor neuron disorders should
include a thorough search for conduction block in motor segments, even in asympto-
matic distributions. If possible, bilateral median-APB, ulnar-ADM, peroneal-EDB,
and tibial-AH motor studies, including F-waves and axillary segments, should be per-
formed.
2. Multifocal motor neuropathy (MMN) is a potentially treatable disorder that can be
mistaken for ALS.

!04
REFERENCES
I. Federico, P., Zochodne, D.W, Hahn, A.E, Brown, WE, Feasby, T.E., Multifocal motor neuropathy improved by IVlg:
randomized, double-blind, placebo-controlled study, Neurology 2000; 55(9): 1256-1262.
2. Katz, J.S., Barohn, R.I., Kojan, S., Wolfe, 0.1., Nations, S.P., Saperstein, D.S., Amato, A.A., Axonal multi focal motor
neuropathy without conduction block or other. features of demyelination, Neurology 2002; 58(4):615-620.
3. Katz, J.S., Wolfe, 0.1., Bryan, WW, Jackson, C.E., Amato, A.A., Barohn, R.I., Electrophysiological findings in multifocal
motor neuropathy, Neurology 1997; 48:700-707.
4. Leger, J.M. et aI., Intravenous immunoglobulin therapy in multifocal motor neuropathy: a double-blind, placebo-
controlled study, Brain 2001; 124:145-153.
5. Lewis, R.A., Sumner, A.J., Brown, M.J., Asbury, AX., Multifocal demyelinating neuropathy with persistent conduction
block, Neurology 1982; 32:958-964.
6. Olney, R.K., Consensus criteria for the diagnosis of partial conduction block, Muscle Nerve 1999; 22(suppl. 8):
S225-S229.
7. Pakiam, A., Parry, OJ., Multifocal motor neuropathy without overt conduction block, Muscle Nerve 1998; 21 :243~245: .
8. Pestronk, A. Chaudhry, V, Feldman, E.L., Oriffin, J.W, Cornblath, D.R., Denys, E.H., Olasbery, M., Kuncl, R.W, Olney,
RX., Yee, WC. Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and
high titers of anti glycolipid antibodies, Ann. Neurol. 1990; 27:316-326.
9. Taylor, B.V, Wright, R.A., Harper, C.M., Dyck, PJ., Natural history of 46 patients with multifocal motor neuropathy with
conduction block. Muscle Nerve 23: 900-908, 2000.
10. Van Doom, PA., Van der Meche, EO., IVlg treatment improves multifocal motor neuropathy: easy to start but difficult to
stop, Neurology 2000; 55: 1246-1247.

205
 PATIENT 50
A 45-year-old woman with a 5-year history of cramps and
twitching and 2-year history of weakness in her left forearm

This 45-year-old woman complained of frequent cramping of her left hand for the previous 5 years,
often precipitated by wrist extension or changes in temperature. In addition, she was aware of nearly con-
tinuous left forearm extensor muscle twitching for several years. Over the last year, she noticed weakness
6ffinger extension, particularly for the thumb and digit 3. Examination was most remarkable for contin-
uous twitching, best described as clinical myokymia, in left forearm finger extensor muscles and MRC
grade 2 strength in the thumb and third digit extensors, with grade 4+ strength in the other finger exten-
sors. There has been no numbness or paresthesias or neurological symptoms in her other limbs.
Electrodiagnostic Study:

Sensory NCS

Recording Onset Peak Amplitude Distance Velocity

Nerve Sites Site (ms) (ms) (~V) (em) (m/s)

L. radial-sn box I. Forearm Snbox 1.70 2.25 32 10 58.8

R. radial-sn box I. Forearm Sn box 1.50 2.00 48 10 66.7
L. median I. Wrist D2 2.20 3.00 64 13 59.0
L. ulnar I. Wrist D5 2.00 2.6 58 11 65.0

Motor NCS
Recording Area Latency Amplitude Distance Velocity
Nerve Sites Site (mVms) (ms) (mV) (cm) (m/s)
R. radial-EIP I. Forearm EIP 48.1 1.80 10.1
2. B. spiral gr EIP 39.6 3.20 8.4 9 64.3
3. A. spiral gr EIP 42.4 4.65 8.6 10 69.0
L. radial-EIP 1. Forearm EIP 64.1 2.00 10.3
2. B. spiral gr EIP 57.3 2.50 9.3 3 60.0
3. A. spiral gr EIP 15.7 7.80 2.5 18 34.0
L. median-APB I. Wrist APB 3.0 16.9 7
2. Elbow APB 6.9 16.5 21.5 55.1
3. Axilla APB 10.0 15.8 19.5 62.9
R. median-APB I. Wrist APB 3.6 14.6 7
2. Elbow APB 7.7 14.3 23 56
L. ulnar-ADM I. Wrist ADM 2.9 10.1 7
2. B. elbow ADM 6.1 10.7 21 65.6
3. A. elbow ADM 8.4 10.7 12 52.6
R. ulnar-ADM I. Wrist ADM 3.0 10.5 7
2. B. elbow ADM 5.9 9.7 19.5 67
3. A. elbow ADM 7.9 9.1 10 50
4. Axilla ADM 9.5 8.8 10 62.5

F-Wave

Nerve F min (ms) Persistence

R. median 28.9 10/10

R. ulnar 27.9 10/10
L. median 27.5 10/10
L. ulnar 27.9 10/10

206
 EMG Summary
SA

High-
Frequency Amplitude Duration PolyP Recruitment
IA Fib Fasc Discharges (MUs) (MUs) (MUs) Activation Pattern

L. biceps Nl 0 0 0 Nl Nl Nl Full Nl
L. triceps Nl 0 0 0 NI Nl NI Full Nl
L. ECR Nl 0 3+ Continuous Nl Nl NI Full Mild red
L. brachiorad Nl 0 0 0 Nl NI Nl Full Nl
L. EDC NI 0 3+ 0 Nl NI Nl Full Mod red
L.FDI NI 0 0 0 Nl NI Nl Full Ni

·2
L Radial - EIP
Fore~rm

S'. Spiral Gr

A. Spiral Gr

30ms 5mV

Figure 1

207
 Figure 2

Questions:
I: What findings are shown in the figures?
2. What is the physiological implication of the needle EMG findings for the left extensor digitorum
communis (ED C)?
3. What is the significance of the high-frequency discharges in the left extensor carpi radialis (ECR)?

208
 Answers:
1. Conduction block of left radial-EIP motor nerves; enlargement and increased T2 signal
2. Decreased recruitment without fibrillation potentials suggests focal demyelination
3. Continuous motor unit activity

Discussion: Figure I shows conduction block and reduced, but did not eliminate, cramps and
of the left radial-EIP across the spiral groove fasciculations.
motor segment. As can be seen from the numeri- The spectrum of MMN and its relationship
cal tabular results, there is a 73% reduction in the to several other disorders is outlined in figure 3.
CMAP area. In addition, there is focal slowing Typical chronic inflammatory demyelinating neu-
(34 mls) of this nerve segment. The needle EMG ropathy (CIDP) has symmetric sensory and motor
studies of left EDC show no fibrillation poten- involvement not confined to individual named
tials, normal motor unit morphology, and moder- nerves. There is generally elevated cerebrospinal
ately reduced recruitment of motor units. This fluid (CSF) protein and an excellent treatment
combination implies demyelination of motor response to prednisone. Multifocal CIDP, also
axons (although chronic denervation with partial called multifocal inflammatory demyelinating
reinnervation, despite normal motor unit ampli- neuropathy and multifocal acquired demyelinat-
tude and duration, is a conceivable possible inter- ing sensory and motor neuropathy (MADSAM),
pretation). Furthermore, the left ECR continuous, is characterized by multi focal (symmetric or
high-frequency discharges indicate a motor unit asymmetric) sensory and motor with individual
hyperactivity disorder and can be called continu- named nerve involvement, variable CSF protein,
ous motor unit activity (CMUA). and excellent but variable response to prednisone.
An MRI of the forearm (figure 2) demon- Multifocal motor neuropathy is characterized by
strated enlargement and increased T2 signal of the multi focal (symmetric or asymmetric) purely
left radial nerve in the across fibular head seg- motor with individual named nerve involvement,
ment, corresponding to the electrodiagnostic normal CSF protein, and poor response to pred~
abnormality. The clinical presentation of a motor nisone but excellent response to IVIg. As indi-
unit hyperactivity disorder, with cramps, fascicu- cated in the figure, MMN has several distinct
lations, and continuous motor unit activity, presentations, in decreasing order of incidence:
argued strongly for a diagnosis of multi focal (1) pure, or classic, MMN with prolonged and
motor neuropathy (MNN), and the patient was marked multi focal purely demyelination without
treated with intravenous immunoglobulin (IVIg). axonal loss, often dramatically improved by
The patient described her response to the first IVIG; (2) an asymmetric lower motor neuron
infusion as "miraculous" in that it allowed for the degeneration with subtle evidence of demyelina-
first night of sleep without forearm extensor tion (see case 49); and (3) a rarer motor unit
cramps in over a year. Continued periodic treat- hyperactivity syndrome (this case).
ment resulted in marked improvement in strength

209
 The Spectrum of MMN

Predominantly axonal degeneration with

subtle motor demyelination

"Pure" or "classic" MMN

MMN with motor unit

hyperactivity

MMN with minor sensory

Figure 3

.Clinical Pearl
Multifocal motor neuropathy has a spectrum of presentations, including that of a motor
unit hyperactivity disorder, dominated by cramps and fasciculations rather than
weakness.

REFERENCES
1. Chaudhry, v., Multifocal motor neuropathy, Semin. Neurol. 1998; 18( I ):73-81.
2. Federico, P., Zochodne, D.W, Hahn, A.E, Brown, WE, Feasby, TE., Multifocal motor neuropathy improved by IVlg:
.. randomized, double-blind, placebo-controlled study, Neurology 2000; 55: 1256--1262.
3. Felice, K.J., Goldstein, J.M., Monofocal motor neuropathy: Improvement with intravenous immunoglobulin, Muscle Nerve
2002; 25:674-678.
4. Layzer, R.B., The origin of fasciculations and cramps. Muscle Nerve 1994; 17:1243-1249.
5. Lewis, R.A., Sumner, AJ., Brown, MJ., Asbury, A.K., Multifocal demyelinating neuropathy with persistent conduction
block, Neurology 1982; 32:958-964.
6. Meriggioli, M.N., Sanders, D.B., Conduction block and continuous motor unit activity in chronic acquired demyelinating
polyneuropathy, Muscle Nerve 1999; 22:532-537.
7. O'Leary, C.P., Mann, A.C., Lough, J., Willison, H.J., Muscle hypertrophy in multi focal motor neuropathy is associated
with continuous motor unit activity, Muscle Nerve 1997; 20:479-485.
8. Thomas, P.K., Claus, D., Jaspert, A., Workman, J.M., King, R.H., Lamer, AJ., Anderson, M., Emerson, J.A., Ferguson,
LT, Focal upper limb demyelinating neuropathy, Brain 1996; 119:765-774.
9. Van Doom, P.A., van der Meche, EG., IVlg treatment improves multi focal motor neuropathy: easy to start but difficult to
stop, Neurology 2000; 55: 1246--1247.
10. Van Es, H.W, Van den Berg, L.H., Franssen, H., Witkamp, TO., Ramos, L.M., Notermans, N.C., Feldberg, M.A., Wokke,
J.H., Magnetic resonance imaging ofihe brachial plexus in patients with multi focal motor neuropathy, Neurology 1997;
48:1218-1224.

210
 SECTION V. MYOPATHIES

Electrodiagnostic studies are valuable in patients with weakness for distinguishing among nerve,
muscle, or other causes. For patients with suspected myopathies, the nerve conduction studies are often
normal, and the needle EMG studies are the most informative part of the electrodiagnostic study. There
are some exceptions worth noting. Myopathies may result in reduced compound muscle action poten-
tial amplitudes in affected muscles. Because routine motor nerve compound muscle action potentials
(CMAPs) are recorded from distal muscles (e.g., abductor pollicis brevis, abductor digiti minimi,
extensor digitorum brevis, abductor hallucis), reductions in amplitudes occur only in myopathies with
involvement of hand or foot muscles (such as myotonic dystrophy or critical illness myopathy) ..
The needle EMG study in myopathies generally follows the following principles:
• Studies are often limited to one side of the body. Many patients with suspected myopathies ulti-
mately undergo muscle biopsy in the course of their initial diagnostic evaluation, often shortly
after referral for an EMG study. It is imperative to avoid the possibility of the biopsy sample
including muscle traumatized by a needle EMG study because of the resulting inflammation. The
electrodiagnostic practitioner should clearly state what side was studied.
• Both proximal and distal muscles in an arm and a leg should generally be studied. Most
myopathies have a proximal predominance, and it is helpful to demonstrate a proximal-to-distal
gradient of abnormalities. Muscles that are typically biopsied, such as the biceps and vastus later-
alis, should be studied so that, if abnormal, the electromyographer can suggest biopsy of the cor-
responding muscle on the contralateral study.
• Fibrillation potentials and positive waves may be seen in myopathies. These electrical potentials
should never be referred to as "denervation potentials" as one is misled into viewing their pres-
ence as indicative of nerve disease. When present in myopathies, fibrillation potentials and posi-
tive sharp waves have diagnostic implications, and such myopathies are often reported as
myopathies associated with "membrane irritability" or "membrane instability."
• The electrodiagnostic hallmarks of myopathies are small-amplitude, short-duration motor unit
action potentials (MUAPs), and early recruitment of MUAPs with full interference patterns in
weak muscles. A number of small-amplitude and short-duration motor units are often present in
normal muscles, however, and this criterion is only a reliable indicator when abundant sampling
of motor units has been performed in a given muscle. Early recruitment is a pattern of motor unit
firing in which abundant units are recruited and generate a relatively weak force. Thus, slight
movement is associated with many units, rather than the normal pattern of one or a few MUAPs
at weak effort ..
• Often, the only electrodiagnostic abnormality in patients with myopathies is a full interference pat-
tern in a weak muscle. When there is substantial weakness of a muscle (MRC grade 3 or less) and
the examiner is able to overcome the muscle while recording a full interference pattern, a myopa-
thy (or disorder of neuromuscular transmission) is highly likely. Weakness from neurogenic or
suprasegmental causes have very different interference patterns in this situation.
• Neuromuscular junction disease may demonstrate myopathic needle EMG abnormalities and
should not be neglected as considerations.

REFERENCES
I. Daube, J.R., AAEM mini monograph II: needle examination in clinical electromyography, Muscle Nerve 1991;
14:685-700.
2. Petajan, J.H., AAEM mini monograph 3: motor unit recruitment, Muscle Nerve 1991; 14:489-502.

211
 PATIENT 51

A 49-year-old woman with progressive proximal leg weakness for

3 years

A 49-year-old woman developed difficulty getting out of a chair and climbing stairs 3 years ago.
She now typically climbs stairs on her hands and knees. She also has difficulty lifting her arms over
her head to shampoo her hair and has noticed some protrusion of her shoulder blades. Family history
is remarkable for her moth~r, who had difficulty walking in her late 50s. She had prominent scapulae
and some speech and swallowing disturbances; as well, and made a slapping sound with her feet when
walking. The patient has four daughters, ages 25, 18, 14, and 13, and two sons, ages 23 and 20, who
are alive and well.
Neurological exam was remarkable for bilateral scapular winging and overriding scapulae.
Manual muscle testing revealed the following MRC scores: orbicularis oculi, 5; neck flexion, 5-; neck
extension, 5; shoulder flexion, 4; and infraspinatus, 4. Shoulder abduction was 4; elbow extension, 5;
elbow extension, 4+; wrist extension, 4+ on the left and 5 on the right; wrist flexion, 5; and hand intrin-
sics, 5. In the lower extremities, hip flexion, abduction, and extension were 4; knee extension and knee
flexion were 5; ankle dorsiflexion was 4+; and plantar flexion was 5. On gait testing, she had marked
hyperlordotic posture. Her gait was wide based and waddling. Significant paraspinal atrophy, in addi-
tion to the hyperlordosis and scapular winging, was evident.
Electrodiagnostic Study:

Sensory NCS
BP
Recording Onset Peak Amplitude Distance Velocity Temperature
Nerve Sites Site (ms) (ms) (f.lV) (cm) (m/s) CC)
L. median- 1. Wrist Dig II 3.00 3.90 25.8 13 43.3 34.9
dig II
L. sup 1. Lat leg Ankle 2.55 3.15 17.9 12 47.1
peroneal

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (em) (mls)

L. median-APB 1. Wrist APB 3.65 12.0 7

2. Elbow APB 7.45 11.4 22 57.9
L. peroneal-EDB 1. Ankle EDB 4.10 6.2 8
2. Fib head EDB 10.05 5.5 29 48.7
3. Pop fossa EDB 11.70 5.4 9 54.5

Needle EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R. deltoid Nl 0 0 0 Few Short Few Full Early
R. biceps Nl 0 0 0 Many Short Many Full Early

nil
 Needle EMG Summary Table---cont'd
SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R. FDI NI 0 0 0 NI NI NI Full NI
R. glut med NI 0 0 0 Small Short Few Full Early
R. vast lat NI 0 0 0 Small Short Few Full Early
R. tib ant NI 0 0 0 Small Short Few Full Early
R. mid-thoracic Incr 1+ 0 0
paraspinals

Question: What is your differential diagnosis and how would you proceed?

213
 Answer: Limb girdle muscular dystrophy, fascioscapulohumeral muscular dystrophy

Discussion: There was electrophysiological The patient had a mildly increased serum CPK
evidence of a myopathic process affecting proximal of 1010 lUlL (normal, <200 lUlL). Leftbiceps
muscles more than distal and in which there was lit- muscle biopsy demonstrated variability in muscle
tle evidence of muscle membrane instability. fiber size, scattered necrotic and regenerating
Clinically, the patient has a limb-girdle pattern of fibers, and increased endomysial connective tis-
weakness associated with marked winging of the sue suggestive of a dystrophy. In addition, many
scapulae, hyperlordotic posture, and paraspinal mus- fibers had rimmed vacuoles, a finding typical of
cle atrophy. There was also a family history, which is autosomal dominant LGMD type 1A, which has
most likely autosomal dominant in nature, but we an age of onset ranging from late teens to the late
cannot exclude a mitochondrial inheritance pattern, 60s (mean, 24.8 years). Affected individuals have
as the disorder was passed down only from her proximal greater than distal weakness, with the
mother and there were no other affected family legs more affected than the arms. Distal weakness
members. The most common disorder or form of is evident in a few patients. Rare patients develop
disorders that would cause this pattern of weakness dysarthria secondary to palatal muscle involve-
would be a limb-girdle muscular dystrophy ment and mild facial weakness. Serum creatine
(LGMD). There are at least five genetically distinct kinase (CK) levels are normal or elevated up to 9
forms of autosomal dominant LGMD; three times normal. Muscle biopsies are notable for the
causative mutated genes have been identified (see frequent occurrence of rimmed vacuoles. LGMD
table below). These include mutations directed IA I is caused by mutations in the gene that
against myotilin, lamin AlC, and caveolin-3. In addi- encodes for myotilin located on chromosome
tion, facioscapulohumeral dystrophy can rarely pres- 5q22.3-31.3. Myotilin is a sarcomeric protein
ent with a limb-girdle pattern of weakness. Some that co localizes with a-actinin on the Z-disk.
congenital myopathies can have a limb-girdle pattern Mutations in myotilin do not appear to affect its
of weakness, but most do not present this late in life. binding with a-actinin but may perturb the
One that can is myofibrillar or desmin myopathy. normal structure of the Z-disk.

Limb-Girdle Muscular Dystrophies

Disease Inheritance Gene Locus Gene Product

LGMDlA AD 5q22-q34 Myotilin

LGMDlB AD Iqll-q21 LaminAlC
LGMDIC AD 3p25 Caveolin-3
LGMDID AD 6q22
LGMDlE AD 7
LGMD2A AR 15ql5 Calpain
LGMD2B AR 2pl3 Dysferlin
LGMD2C AR 13q12 y-Sarcoglycan
LGMD2D AR 17q12-21 a-Sarcoglycan
LGMD2E AR 4ql2 ~-Sarcoglycan
LGMD2F AR 5q33-q34 o-Sarcoglycan
LGMD2G AR 17qll-q12 Telethionin
LGMD2H AR 9q3-q34

AD = autosomal dominant; AR = autosomal recessive

Clinical Pearl
Limb-girdle muscular dystrophies are a heteorgenous group of genetically determined dis-
eases; type I diseases are autosomal dominant, and type II diseases are autosomal recessive.

REFERENCES
I. Cohn, R.D., Campbell, K.P., Molecular basis of muscular dystrophies, Muscle Nerve 2000; 23:1456-1471.
2. Gilchrist, J.M., Pericak-Vance, M., Silverman, L., Clinical and genetic investigation in autosomal dominant limb-girdle
muscular dystrophy, Neurology 1988; 38:5-9.
3. Hauser, M.A., Horrigan, S.K., Salmikangas, P. et al., Myotilin is mutated in limb-girdle dystrophy lA, Hum. Mol. Genet.
2000; 9:2141-2147.

214
 PATIENT 52

A 6-month-old girl with generalized weakness since birth

A 6-month-old infant girl was referred for generalized weakness that was noted at birth and for
recent respiratory failure requiring a mechanical ventilator. Her exam revealed generalized hypotonia
and weakness, no ptosis, and fuIl extraocular movements. Muscle stretch reflexes were absent.' .
Electrodiagnostic Study: -

Sensory NCS

BP
Recording Amplitude Velocity
Nerve Sites Site (I1V) (rnIs)
L. median-dig II l. Wrist Dig II 15 22
L. sural-l at maIl 1. Mid-calf Ankle 8 24

Motor NCS

Recording Latency Amplitude VelocitY

Nerve Sites Site (ms) (mV) (rnIs)
L. median-APB l. Wrist APB 3.2 7
2. Elbow APB 7 22
L. peroneal-EDB 1. Ankle EDB 3.3 5
2. Fib head EDB 5 23

Needle EMG Summary Table

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Pattern
L. deltoid Incr 2+ 0 0 Many smaIl Many short Many Early
L. biceps Incr 2+ 0 0 Many smaIl Many short Many Early
L. vast lat Incr 2+ 0 0 Many small Many short Many Early
L. tib ant Incr 2+ 0 0 Many smaIl Many short Many Early

215
 Questions:
I. What is your differential diagnosis and how would you proceed?
2. What other tests would be helpful?
3. What other tests might you consider?

216
 Answers:
I. Mitochondrial disorders, merosin deficiency
2. Serum CK, muscle biopsy
3. Immunostaining of the muscle biopsy for merosin

Discussion: The motor and sensory nerve of patients with CMD have absent or decreased
conduction studies were remarkable for normal merosin. Infants with classical CMD are character-
amplitudes but mildly slow conduction velocities istically hypotonic and weak at birth. Weakness is
for age. The EMG demonstrated abnormalities generalized with a predilection for proximal shoul-
suggestive of a myopathy with muscle membrane der and hip girdle muscles. Sensation appears nor-
instability. Myopathies associated with peripheral mal. Deep tendon reflexes are diminished or
nerve abnormalities in children include mito- absent. Arthrogryposis mayor may not be present.
chondrial disorders and the congenital muscular Patients with partial merosin deficiency have been
dystrophy associated with merosin deficiency identified. These patients have a milder course and
(CMD I). The serum creatine kinase level was can present in childhood with a Duchenne muscu-
elevated over 2000 lUlL. An MRI scan of the lar dystrophy (DMD) phenotype or in early adult-
brain revealed hypomyelination. A muscle biopsy hood similar to Becker muscular dystrophy (BMD)
subsequently demonstrated features of a dystro- or limb-girdle muscular dystrophy (LGMD).
phy. Immunostaining for merosin demonstrated Serum CK levels are elevated, usually over
absence on the sarcolemma. 2000 lUlL, in merosin-negative infants. In con-
The congenital muscular dystrophies (CMDs) trast, most infants with merosin-positive CMD
are a heterogeneous group of autosomal recessive have normal or mildly elevated serum CKs, typi-
inherited disorders characterized by perinatal cally less than 1000 lUlL. Interestingly, magnetic
onset of hypotonia with proximal weakness; joint resonance images (MRIs) demonstrate diffuse
contractures affecting the elbows, hips, knees, white matter abnormalities suggestive of dys-
and ankles (arthrogryposis); dystrophic appearing myelination in most infants with merosin-
muscle biopsies; and exclusion of other recogniz- negative CMD. MRI in merosin-positive patients
able causes of myopathy of the newborn. The is typically normal. Patients with partial merosin
CMDs are classified according to clinical, oph- deficiency mayor may not have cerebral hypo-
thalmological, radiological, and pathological fea- myelination on MRI.
tures. The major categories of CMDs are (I) the The classical-type CMD with cerebral
classic or Occidental/Western type; (2) the hypomyelination is associated with mutations in
Fukuyama type, characterized by defects in neu- the a.-2 subchain of merosin. Merosin binds to a.-
ronal migration (i.e., polymicrogyria) with severe dystroglycan of the dystrophin glycoprotein com-
mental retardation, seizures, and a progressively plex and a.7~ID integrin. Merosinopathies may
deteriorating course; (3) the Walker-Warburg or result in a disruption and loss of integrity of the
cerebral-ocular-dysplasia syndrome; and (4) muscle membrane. Merosin is also present in
Santivouri type, or muscle-eye-brain disease. the basal lamina of myelinated nerves. Abnormal
The classical type of CMD is divided into the expression of merosin may interfere with myelino-
merosin-negative and merosin-positive subgroups. genesis and may account for the hypomyelination
Merosin, or a.-2 laminin, is connected to the dys- evident in the central and peripheral nervous
trophin-glycoprotein complex. At least 40 to 50% system.

Clinical Pearl
The combination of a demyelinating neuropathy and a myopathy in a child suggests a
mitochondrial disorder or congenital muscular dystrophy with merosin deficiency.

REFERENCES
I. Amato, A.A., Dumitru, D., Hereditary myopathies, in: Dumitru, D., Amato, A.A., Swartz, MJ., Eds., Electrodiagnostic
Medicine, 2nd ed., Hanley & Belfus, Philadelphia, PA, 2002, pp. 1265-1370.
2. Pegoraro, E., Marks, H., Garcia, C.A., Crawford, T., Mancias, P., Connolly, A.M., Fanin, M., Martinello, E, Trevisan, C.P.,
Angelini, c., Stella, A., Scavina, M., Munk, R.L., Servidei, S., Bonnermann, C.C, Bertorini, T., Acsadi, G., Thompson,
E.C., Gagnon, D., Goganson, G., Carver, v., Zimmerman, R.A., Hoffman, E.P., Laminin a2 muscular dystrophy:
genotype/phenotype studies of 22 patients, Neurology 1998; 50: 101-1 ] O.

211
 PATIENT 53

A 29-year-old man with a cardiomyopathy

A 29-year-old white man complained of progressive dyspnea related to a dilated cardiomyopathy

since the age of 22. About a year ago, he noted difficulty swallowing. Of note, over the past 7 or
8 years, he had noticed progressive hearing loss. Recent pulmonary function tests showed a forced vital
capacity (FVC) of 80%. He had decreased mean inspiratory and expiratory pressures of 52 and 40%,
respectively, of predicted. Family history was remarkable as follows. He has a 32-year-old sister who
is healthy. Of note, his mother, who is in her mid-50s, has been deaf for over 10 years and had a
cochlear implant 2 years ago. She also has had a history of diabetes for a long period of time. The
patient's father is healthy. The patient has no known medical allergies. General physical examination
was remarkable for his short stature: 5 foot, 3 inches in height.
On neurological examination, there was evidence of pigmentary retinopathy in the macula of both
eyes and mild bilateral ptosis. He had marked limitations of all upward and lateral eye movements with
normal down-gaze. Orbicularis oculi and oris strength was 5/5. Jaw, palate, and tongue strength like-
wise was 5/5. Motor exam revealed thin muscle bulk proximally and distally in the arms and legs. On
manual muscle testing, he had the following MRC scores: neck flexion, 4+; neck extension, 5; shoul-
der abduction, 5; elbow flexion, 5-; and elbow extension, 5. Wrist extension and flexion and hand
intrinsics were grade 5. Hip flexion, abduction, and extension were grade 4; knee extension and flex-
ion, 5; and ankle dorsiflexion and plantar flexion, 5. Complex motor skills revealed normal coordina-
tion. Deep tendon reflexes were 1 at the biceps and at the ankles, but 0 at the triceps, brachioradialis,
and knees. Plantar responses were flexor.
Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (/-lV) (cm) (mls)

L. median-dig II 1. Wrist Dig II 2.50 3.30 111.1 13 52.0

L. radial-sn box 1. Forearm Sn box 1.90 2.45 42.2 10 52.6
L. sural-lat mall 1. Mid-calf Ankle 3.00 3.70 28.0 14 46.7

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (mls)
L. median-APB 1. Wrist APB 3.25 12.7 7
2. Elbow APB 6.95 12.6 19 51.4
L. ulnar-ADM 1. Wrist ADM 2.85 13.8 7
2. B. elbow ADM 5.75 13.5 16.5 56.9
3. A. elbow ADM 7.50 13.4 10 57.1
L. peroneal-EDB 1. Ankle EDB 4.00 6.1 8
2. Fib head EDB 9.35 5.8 26 48.6
3. Pop fossa EDB 11.20 5.7 9 48.6
L. tibial-AH 1. Ankle AH 4.10 20.0 8
2. Pop fossa AH 11.45 16.9 37 50.3

218
 Needle EMG Summary Table
SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
L. deltoid NI 0 0 0 NI NI NI Full NI
L. biceps NI 0 0 0 Nl Nl Nl Full Nl
L. vast lat Nl 0 0 0 Nl NI NI Full Nl
L. tib ant Nl 0 0 0 Nl Nl Nl Full Nl

Questions:
1. How would you interpret these studies?
2. Would normal EMGINCS exclude an underlying neuromuscular disorder in the patient?

219
 Answers:
1. Normal
2. No

Discussion: The patient has normal EMG/ Kearns~Sayre syndrome (KSS). These patients
NCS. Based on his clinical features (ptosis, could represent partial expressions of KSS.
ophthalmoparesis, deafness, short stature, mild Importantly, these deletions are felt to be sporadic
extremity weakness, and cardiomyopathy), a in occurrence and not inheritable. Point mutations
mitochondrial myopathy was suspected. This have been demonstrated within various mitochon-
would also explain his mother's deafness. The drial tRNA (Leu, lIe, Asn, Trp) genes in several
patient had a mildly elevated serum CK and lac- kinships with maternal inheritance of PEO. In
tic acid. He underwent a biopsy ofthe right biceps addition, multiple mtDNA deletions have been
brachii muscle, which demonstrated numerous described in a few kinships with autosomal dom-
ragged red fibers and abnormal mitochondria on inant inheritance.
electron microscopy. Importantly, EMG is often The molecular defects are suspected to lie in
normal in patients with a mitochondrial myopa- nuclear genes involved in regulating the mito-
thy. Some syndromes are associated with periph- chondrial genome. Autosomal dominant PEO
eral neuropathies, which are typical axonal in appears to be genetically heterogeneous, because
nature. His electrophysiological studies were the disorder has been localized to mutations in
completely normal. the genes encoding for adenine nucleotide translo-
This patient falls into the category of pro- cator I (ANTI) on chromosome 4q34-<}35, the
gressive external ophthalmoplegia (PEO), which twinkle gene on chromosome lOq23.3-q24.3, and
is a heterogeneous group of mitochondrial disor- polymerase-yon 15q22-q26. ANTI is responsi-
ders. There are autosomal dominant and mater- ble for transporting adenosine triphosphate across
nally inherited forms of PEO. Some sporadic the inner mitochondrial membrane, while twinkle
patients with PEO have single large mtDNA and polymerase-yare involved in mitochondrial
deletions indistinguishable from those seen in DNA replication.

Clinical Pearl
Many patients with myopathies have normal electrodiagnostic studies. Although it may
be diagnostically useful to perform them in suspected myopathy, normal EMG studies
provide little evidence against a myopathy.

REFERENCES
I. Hirano, M., DiMauro, S., ANTI, Twinkle, POLG, and TP: new genes open our eyes to ophthalmoplegia, Neurology 200 I;
57:2163-2165.
2. Kawai, H., Akaike, M., Yokoi, K., Nishida, Y., Kunishige, M., Mine, H., Saito, S., Mitochondrial encephalomyopathy with
autosomal dominant inheritance: a clinical and genetic entity of mitochondrial diseases, Muscle Nerve 1995; 18:753-760.
3. Li, F.Y., Tariq, M., Croxen, R., Morten, K., Squier, W., Newsom-Davis, 1., Beeson, D., Larsson, C., Mapping of autosomal
dominant progressive external ophthalmoplegia to a 7-cM critical region on IOq24, Neurology 1999; 53:1265-1271.

220
 PATIENT 54

A 62-year-old man with slowly progressive weakness

A 62-year-old right-handed man noted insidious onset of weakness in his neck flexors, hands, and
hips about 3 years ago. He also has a feeling occasionally of food getting stuck in his throat. He denies
dysarthria, dyspnea, ptosis, diplopia, or sensory loss. There is no significant past medical history o'r
family history of neuromuscular disease. Neurological examination was notable for 4-/5 strength of
neck flexors and 5/5 strength of neck extensors. In the upper extremities strength was 5-/5 in the del~
toids, 4+/5 in the biceps, 4/5 in the triceps, 4+ in wrist extensors, 4 on left wrist flexion, 4- in right
wrist flexion, 4+/5 in the finger extensors, 4 on left finger flexors, and 4- on right finger flexors. I'D
the lower extremities, strength was 4/5 in the hip flexors, abductors, and extensors; 3- in knee exten~
sion (quadriceps), 4 in ankle dorsi flexors, and 5 in plantar flexors. On gait testing there was noticeable
hyperextension of the knees. Serum creatine kinase level was 200 IU/L (upper limit of normal).
Electrodiagnostic Study:

Sensory NCS
BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (~V) (em) (m/s) .
L. median-dig II 1. Wrist -Dig II 2.30 3.15 80.1 13 56.5
L. ulnar-dig V 1. Wrist DigV 2.25 3.20 76.5 11 48.9
L. sural-l at mall 1. Mid-calf Ankle 3.15 4.05 12.2 14 44.4

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (em) (m/s)
L. median-APB 1. Wrist APB 3.65 5.4 7
2. Elbow APB 7.55 4.5 21.5 55.1
L. ulnar-ADM 1. Wrist ADM 3.15 10.5 7
2. B. elbow ADM 5.80 9.2 16 60.4
3. A. elbow ADM 7.40 9.4 12.5 78.1
L. peroneal-EDB 1. Ankle EDB 5.30 4.5 8
2. Fib head EDB 11.90 3.7 28 42.4
3. Pop fossa EDB 13.75 4.2 9 48.6

22f
 Needle EMG Summary Table
SA

Amplitude Duration PolyP Recruitment

lA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

L. deltoid Incr 1+ 0 0 NI NI NI Full NI

L. biceps Incr 2+ 0 0 Small Brief Few Full Early
L. triceps Incr 2+ 0 0 Small Brief Many Full Early
L. FCU Incr 3+ 0 CRD Small Brief and long Many Full Early
L: first dors int Incr 1+ 0 0 NI Nt NI Full Nt
L. glut med Incr 2+ 0 0 Small Brief Few Full Early
Lvast lat Incr 3+ 0 0 Small Brief and long Many Full Early
L. iliopsoas Incr 2+ 0 0 Small Briefand long Many Full Early
L. tib ant Incr 2+ 0 0 Small Brief and long Many Full Early
L. thoracic PSP upper Incr 1+. 0 0

Questions:
J. What is your differential diagnosis?
2. How would you interpret this study?
3., What is the most common myopathy in this age group?
4. How would you proceed with the diagnostic evaluation?
5. ~at does the muscle biopsy show?

222
 Answers:
1. Inflammatory myopathy, myasthenia gravis, sarcoid myopathy
2. Myopathy with muscle membrane irritability
3. Inclusion body myositis
4. Muscle biopsy
5. Vacuolated muscle fibers

Discussion: The early recruitment of motor flexors. We have invariably found that the manual
units and the presence of short-duration and muscle scores of the finger and wrist flexors are
small-amplitude motor unit action potentials is lower than those of the shoulder abductors, and the
characteristic of a myopathy, and the fibrillation muscle scores ofthe knee extensors are lower thaN
potentials provide evidence of associated muscle those of the hip flexors in patients with IBM. The
membrane irritability. The most common myopa- opposite relationship between muscles scores is
thy with age of onset over the age of 50 is inclu- present in DM and PM. In addition, muscle
sion body myositis (IBM). A muscle biopsy involvement in IBM is often asymmetric, in con-
would be indicated to pursue the diagnosis, and trast to the symmetrical involvement in DM and
this was done (see figure). PM. The presence of slowly progressive, asym-
On the basis of the exam findings, a diagnosis metric quadriceps and wrist/finger flexor weak-
of IBM was suspected. Muscle biopsy confirmed ness in a patient over 50 years of age' strongly
the diagnosis, showing vacuolated muscle fibers suggests the diagnosis of IBM.
(see figure) and invasion of non-necrotic muscle Serum CK is normal or only mildly elevated
fibers (not shown). IBM is characterized clinically (less than tenfold above normal). EMG studies
by the insidious onset of slowly progressive prox- demonstrate increased spontaneous and inser-
imal and distal weakness that generally develops tional activity, small polyphasic MUPs, and
after the age of 50 years. The slow evolution of the early recruitment. In addition, large polyphasic
disease process probably accounts in part for the MUPs can also be demonstrated in one third of
delay in diagnosis, averaging approximately 6 patients; however, large polyphasic MUPs can
years from the onset of symptoms. Men are much also be seen in DM, PM, and other muscle disor-
more commonly affected than women, in contrast ders (e.g., muscular dystrophies) and probably
to the female predominance seen in dermato- reflect chronicity of the disease process rather
myositis (DM) and polymyositis (PM). The clini- than a neurogenic etiology. Nevertheless, nerve
cal hallmark of IBM is early weakness and conduction studies reveal evidence of a mild
atrophy of the quadriceps, volar forearm muscles axonal sensory neuropathy in up to 30% of
(i.e., wrist and finger flexors), and ankle dorsi- patients.

223
 The characteristic light microscopic findings fibers also contain cytoplasmic clusters of 6- to
are endomysial inflammation, small groups of 10-nm amyloid-like fibrils. Because of sampling
atrophic fibers, eosinophilic cytoplasmic inclu- error, repeat muscle biopsies may be required to
sions, and muscle fibers with one or more identify the rimmed vacuoles and abnormal
rimmed vacuoles lined with granular material. tubulofilament or amyloid accumulation in order
Amyloid deposition is evident on Congo red to histologically confirm the diagnosis of IBM.
staining using polarized light or fluorescence This sampling error probably accounts for IBM
techniques. Increased numbers of ragged red being misdiagnosed as PM.
fibers and COX-negative fibers are also evident The pathogenesis of IBM is unknown. Whether
in IBM compared to DM and PM patients and IBM is a primary inflammatory myopathy like
age-matched controls.' Electron microscopy DM and PM or a myopathy in which the inflam-
demonstrates 15- to 21-nm cytoplasmic and matory response plays a secondary role is the
intranuclear tubulofilaments, although a mini- subject of intense research. IBM is slowly pro-
mum of three vacuolated fibers may have to be gressive and unfortunately does not respond well
scrutinized to confirm their presence. Vacuolated to immunosuppressive medications.

Clinical Pearls
1. The most common myopathy with onset after age 50 is inclusion body myositis.
2. EMG studies typically show muscle membrane irritability with fibrillation poten-
tials and positive sharp waves in the inflammatory myopathies.

REFERENCES
r. 'Barohn, R.J., Amato, A.A., Kissel, IT., Sahenk, Z., Mendell, IR., Inclusion body myositis: response to immunosuppressive
therapy, Neurology 1995; 45: 1302-1304 ..
2. Amato, A.A., Gronseth, G.S., Jackson, C.E., Wolfe, G.I., Katz, IS., Bryan, w.w., Barohn, R.J., Inclusion body myositis:
clinical and pathological boundaries, Ann. Neurol. 1996; 40:581-586.
3. Greenberg, S.A., Sanoudou, D., Haslett, IN., Kohane, I.S., Kunkel, L.M., Beggs, A.H., Amato, A.A., Molecular profiles of
inflammatory myopathies, Neurology 2002; 59: I 170- I 182.

224
 PATIENT 55

A 46-year-old woman with a 2-year history of proximal leg and

arm weakness

This 46-year-old woman noted that approximately 2 years ago she began having difficulty walk-
ing down steps and getting up out of a chair. Additionally, she began having difficulty swallowing
approximately 15 months ago. She has difficulty swallowing solid foods and feels that they get caug~t
at the back of her throat. She has had no numbness or tingling. She has not had any rashes on the face,
neck, or shawl area. Neurological examination was notable for normal muscle bulk and tone ..No action
or percussion.myotonia, paramyotonia, or fasciculations were noted. Muscle strength was as follows:
neck flexion, 4-; extension, 5; deltoids, 4; biceps, 4; triceps, 4; wrist extension, 5-; wrist flexion, 5;
and finger extension and flexion, 5 bilaterally. No winging of the scapula was seen. In the lower
extremities, hip flexion, extension and abduction were 4-/5; knee extension, 5-; knee flexion, 4+; and
ankle dorsiflexion and plantar flexion, 5.
Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (~V) (cm) (m/s)

R. median-dig II 1. Wrist Dig II 2.35 3.05 32.7 13 55.3

R. ulnar-dig V 1. Wrist DigV 1.95 2.65 29.5 11 56.4 .
R. sural-Iat mall 1. Mid-calf Ankle 2.90 3.90 16.4 14 48.3

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)

R. peroneal-EDB 1. Ankle EDB 4.45 5.4 8

2. Fib head EDB 10.75 4.9 33.5 53.2
3. Pop fossa EDB 12.70 4.7 10 51.3
R. tibial-AH I. Ankle AH 4.00 10.8 8
2. Pop fossa AH 13.30 8.5 43 46.2
R. median-APB 1. Wrist APB 3.65 5.4 7
2. Elbow APB 7.45 5.4 22.5 59.2
R. ulnar-ADM 1. Wrist ADM 2.95 11.1 7
2. B. elbow ADM 6.55 9.6 21.5 59.7
3. A. elbow ADM 8.85 9.0 12 52.2

Needle EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. deltoid Incr NI 0 0 NI NI NI Full NI

R. biceps NI NI 0 0 NI NI NI Full NI
R.FDI NI NI 0 0 NI NI NI Full NI

Continued

225
 Needle EMG Summary Table--cont'd

SA

Amplitude Duration PolyP Recruitment

IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern

R. low thoracic . ;Incr 2+ . 0 0

paraspinals
R. glu medius Incr 2+ 0 0 Small Short Many Full Early
R. vastus lateral is Incr 2+ 0 0 Small Short Many Full Early
R.FHL Nl Nl 0 0 Nl Nl Nl Full Nl

Questions:
L How would you interpret the EMGINCS findings?
2.. Does this help narrow the differential diagnosis?
3.· What further tests and work-up would be helpful?
4. What does the muscle biopsy demonstrate?

226
 Answers:
I. Myopathy with membrane irritability in proximal muscles
2. Narrows to myopathy
3. Serum CK, muscle biopsy
4. Invasion of non-necrotic fibers by inflammatory cells

Discussion: There is electrophysiological tional tests are required, including a muscle

evidence of a myopathy with muscle membrane biopsy. Laboratory work-up revealed creatine
irritability in proximal muscle groups. This is phosphokinase (CPK) ranging from 684 to 713
a nonspecific finding and may be seen in lUlL. A muscle biopsy of the left quadriceps was
inflammatory myopathies, muscular dystrophies, performed (see figure).
toxic myopathies, certain congenital myopathies The biopsy demonstrated endomysial inflam-
(e.g., myofibrillar myopathy), and some meta- mation with invasion of non-necrotic muscle fibers
bolic myopathies (e.g., acid maltase deficiency). consistent with the diagnosis of polymyositis.
The differential diagnosis in a middle-aged adult Polymyositis is a major form of idiopathic inflam-
with an insidious onset of progressive, symmetri- matory myopathy, along with dermatomyositis and
cal, proximal greater than distal weakness would inclusion body myositis (IBM). Although it is ofte'n
include a form of motor neuron disease (e.g., thought to be the most common form of myositis,
adult-onset spinal muscular atrophy, Kennedy's it is in fact the least common. Many cases of IBM
disease), a neuromuscular junction disorder (e.g., and limb-girdle muscular dystrophy with inflam-
myasthenia gravis, Lambert-Eaton syndrome), mation have been incorrectly diagnosed as
polyneuropathy (e.g., chronic inflammatory polymyositis. The pattern of muscle weakness with
polyradiculoneuropathy), and various myopathies symmetric proximal greater than distal weakness
(myositis, limb-girdle muscular dystrophy, and can help distinguish polymyositis from IBM. The
endocrine, metabolic, toxic, and some congenital clinical examination and special stains on muscle
myopathies). The electrodiagnostic studies in this biopsy are useful in differentiating polymyositis
case were very helpful in pointing toward a myo- from limb-girdle muscular dystrophy, acid maltase
pathic etiology. Further, the muscle membrane deficiency, and congenital myopathies that may
irritability excludes many of the endocrine have similar clinical presentations and electrodiag-
myopathies (i.e., hypothyroid, Cushing's syn- nostic findings. Polymyositis is usually responsive
drome) and congenital myopathies that have little to corticosteroids. Intravenous immunoglobulin
in the way of abnormal insertional and sponta- (IVlg), methotrexate, azathioprine, or mycopheno-
neous activity. However, the EMG cannot narrow late mofetil may be used in patients refractory to
the differential diagnosis any further, and addi- steroids or as steroid sparing agents.

22'7
 Clinical Pearls
1. Polymyositis is the least common of the inflammatory myopathies.
2. Treatment-resistant polymyositis is usually a mistaken diagnosis-most such
patients have inclusion body myositis (IBM) or a muscular dystrophy.

REFERENCES
I. Amato, A.A., Barohn, R.I., Idiopathic inflammatory myopathies, Neurol. Clin. 1997; 15:615-{i48.
2 .. Briemberg, H.R., Amato, A.A., Dermatomyositis and polymyositis, Curr. Treat. Options Neurol. 2003; 5:349-356.
3. Dalakas, M.C., Progress in inflammatory myopathies: good but not good enough, J. Neurol. Neurosurg. Psychiatry 2001;
.. 70:569-573.
4. Dalakas, M.C., Hohlfeld, R., Polymyosits and dermatomyositis, Lancet 2003; 362: 971-982.
5. Van Der Meulen, M.F., Bronner, I.M., Hoogendijk, 1.E., Burger, H., Van Venrooij, W.I., Voskuyl, A.E., Dinant, H.1.,
Linssen, W.H., Wokke, 1.H., De Visser, M., Polymyositis: an overdiagnosed entity, Neurology 2003; 61 :316--321.

'\. '

228
 Answers:
I. Toxic myopathy, primary inflammatory myopathy, HIV associated myopathy
2. Myopathy and distal axonal sensory neuropathy
3. Muscle biopsy
4. Corticosteroids

Discussion: The patient's history and exami- to the antiviral medication AZT is usually associ-
nation suggested a long-standing sensory neu- ated with impaired mitochondria, which on mus-
ropathy that was supported by the nerve cle biopsy is characterized by the presence of
conduction studies demonstrating low-amplitude ragged red fibers. Usually, patients have symmet-
sural sensory nerve action potentials (SNAPs). ric proximal greater than distal weakness and
It is possible that the neuropathy is the HIV- pain. EMG may demonstrate myopathic units, but
associated distal symmetric polyneuropathy there is usually no abnormal insertional or spon~
(HIV-DSP) or a toxic neuropathy related to her use taneous activity. Wasting disease occurs in 25 to
of nucleoside analogs. In addition, the EMG 50% of patients with AIDS and is associated with
demonstrated evidence of a diffuse myopathic severe weight loss and easy fatigability. Serum
process with muscle membrane instability and CK and EMG are usually normal.
small, polyphasic motor units that recruited early. At least 20% of patients with HIV develop
She had a muscle biopsy that demonstrated fea- some form of polyneuropathy during the course of
tures of HIV myositis. She was treated with IVIg their illness. The seven major types of peripheral
without response. Subsequently, she was started on neuropathy associated with HIV infection are (I)
corticosteroids and had significant improvement in distal symmetric polyneuropathy (DSP), (2)
strength and normalization of her serum CK. inflammatory demyelinating polyneuropathy
Patients with HIV infection can manifest with (including both AIDP and CIDP), (3) mononeu-
weakness secondary to central etiologies (e.g., ropathy multiplex (e.g., vasculitis, CMV related),
myelopathy), motor neuropathies, peripheral neu- (4) progressive polyradiculopathy (usually CMV
ropathies, and myopathies. Myopathies associ- related), (5) autonomic neuropathy, (6) sensory
ated with HIV infection are uncommon but ganglionitis, and (7) toxic neuropathy secondary
certainly occur. These include mitochondrial to nucleoside analogs. DSP is the most common
myopathy related to antiretroviral medication form of peripheral neuropathy associated with
(e.g., zidovudine AZT), inflammatory myopathies HIV infection and usually is seen in patients with
(HIV myositis, inclusion body myositis, second- AIDS. The nucleoside analogs zalcitabine
ary infections of muscle), and type 2 muscle fiber (dideoxycytidine, or ddC), didanosine (dideoxyi-
atrophy secondary to HIV-wasting syndrome. nosine, or ddI), stavudine (d4T), lamivudine
Unfortunately, the exact cause of the myopathy is (3TC), and antiretroviral nucleoside reverse tran-
often not clear, as patients may have a com- scriptase inhibitor (NRTI) are used in the treat-
bination of myositis, AZT myopathy, and HIV- ment of HIV infection. One of the major
wasting syndrome, not to mention a peripheral dose-limiting side effects ofthese medications is a
neuropathy related to the infection or medica- predominantly sensory, length-dependent, sym-
tions. Differentiating these conditions requires metric, painful neuropathy. Patients with DSP and
a detailed history, neurological examination, toxic neuropathies related to treatment with nucle-
laboratory studies, neurophysiological testing, oside analogs present with numbness and painful
and sometimes a muscle biopsy. Inflammatory paresthesias of the hands and feet. Nerve conduc-
myopathies are associated with symmetric proxi- tion studies demonstrate features of a length-
mal greater than distal weakness, elevated serum dependent axonal neuropathy. It is often difficult
CKs, and EMG demonstrating myopathic to distinguish whether a patient's neuropathy is
MUAPs and abnormal insertional and sponta- secondary to DSP or is medication induced; dis-
neous activity (e.g., fibrillation potentials and continuation of medication and observation are
positive sharp waves). A toxic myopathy related usually required for such a determination.

Clinical Pearl
HIV or its treatment may produce a myopathy with fibrillation potentials.

237
REFERENCES
I. Barohn, R.1., Gronseth, G.S., LeForce, B.R. et al., Peripheral nervous system involvement in a large cohort of human
immunodeficiency virus infected individuals, Arch. Neural. 1993; 50:167-171.
2. Dalakas, M.C., IlIa, I., Pezeshkpour, G.H., Mitochondrial myopathy caused by long-term zidovudine therapy, New Engl. J
Med. 1990; 322:1098-1105.
3. Simpson, D.M., Citak, K.A., Godfrey, E., Godbold, J., Wolfe, D., Myopathies associated with human immunodeficiency
virus and zidovudine: can their effects be distinguished?, Neurology 1993; 43:971-976.
4. Simpson, D.M., Olney, R.K., Peripheral neuropathies associated with human immunodeficiency virus infection, Neural.
Clin. 1992; 10:685-711.

238
 PATIENT 59

An 85-year-old woman with progressive leg weakness and

myalgias

An 85-year-old woman reported a 2-month history of progressive leg weakness and myalgias with
significant difficulty going up stairs. She has not noted any upper extremity weakness, dysphagia, ocu-
lar symptoms, or significant shortness of breath. She denies any history of cramps or fasciculations.
There are no sensory symptoms. She does suffer from chronic mild low back pain but denies any radic-
ular symptoms. Past medical history is significant for hypertension and hypercholesterolemia. She was
started on atorvastatin I year ago, and additional medications include enalapril, nifedipine, atenolol,
and aspirin. Neurological examination showed mild weakness in her deltoids and biceps bilaterally at
4+/5. Strength in her triceps, wrist extensors, wrist flexors, finger extensors, finger flexors, and
interossei was 515 bilaterally. She had moderate weakness in her hip girdle and hamstrings at 4/5 bilat-
erally. Quadriceps, dorsiflexors, plantar flexors, invertors, and evertors were all 5/5. There was mod-
erate neck flexor weakness at 4-/5. Sensory exam revealed a mild decrease in vibration in her toes
bilaterally. Her gait was narrow based but somewhat shuffling. Her reflexes were 2+ and symmetric at
the biceps, brachial radialis, triceps, knees, and ankles. Plantar responses were flexor.
Electrodiagnostic Study:

Sensory NCS

BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (J.LV) (cm) (m/s)

R. median-dig II 1. Wrist Dig II 2.85 3.90 21.6 13 45.6

R. sural 1. Calf Ankle 3.45 4.55 5.5 14 41

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (mls)
R. median-APB 1. Wrist APB 4.20 5.0 7
R. median-APB 2. Elbow APB 7.70 5.0 17 48.6
R. tibial-AH I. Ankle AH 5.15 3.4 8
R. tibial-AH 2. Pop fossa AH 15.50 2.9 42 40.6

Needle EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R. deltoid Incr 0 0 0 Few small Few short NI Full Early
R. biceps Incr 1+ 0 Myotonia Few small Few short NI Full Early
R. FDI NI 0 0 0 Nt NI NI Full NI
R. iliopsoas Incr 1+ 0 Myotonia Few small Few short Nl Full Early
R. vast lat Incr 0 0 0 Few small Few short NI Full Early
R. tib ant Incr 0 0 0 NI Nl NI Full Nl
R.FHL NI 0 0 0 NI NI NI Full Nl
R. thoracic Incr 2+ 0 0
PSP-mid

239
 Questions:
1. What is your differential diagnosis?
2. What other recommendations and tests would you suggest?

2:40:
 Answers:
1. Myopathy with myotonic discharges
2. Serum CK, muscle biopsy, genetic testing for myotonic dystrophy type 2

Discussion: There was electrophysiologic muscle was chosen because it was clinically weak
evidence of a generalized myopathy with associ- and the contralateral muscle had abnormalities on
ated muscle membrane irritability and myotonic EMG. The muscle biopsy demonstrated scattered
discharges. Myopathies that can present with necrotic and regenerating muscle fibers with
proximal weakness, clinical myotonia, and increased internal nuclei. There was no evidence of
myotonic discharges on EMG include myotonic a primary inflammatory myopathy. The atorvas-
dystrophy (types 1 and 2), myotonia congenita, tatin was subsequently stopped, and the patient was
paramyotonia congenita, and potassium-sensitive closely followed in the clinic. Her serum CKs and
periodic paralysis. Disorders that present with muscle strength gradually returned to normal, and
proximal weakness and myotonic discharges on the myalgias resolved within a couple of months.
EMG but no clinical myotonia include acid mal- Another option would have been to have
tase deficiency, rarely inflammatory myopathies, stopped the atorvastatin and to follow her before
myofibrillar myopathy, certain vacuolar myopa- proceeding with a biopsy; however, the duration
thy, and some toxic myopathies secondary to of the toxic side effects are not well known. We
drugs (e.g., chloroquine, statin agents). have seen patients who continue to complain of
Initial work-up revealed an elevated CK of symptoms for several months after stopping the
between 5300 and 6000 lUlL. In order to exclude medication. We did not want to delay treatment of
a treatable inflammatory myopathy, we opted to a possible inflammatory myopathy in a patient
perform a biopsy of the left biceps muscle. This who is clinically weak, thus the biopsy.

Clinical Pearl
The statin agents may be associated with a myopathy in which myotonic discharges are
frequently seen. In rare cases, rhabdomyolysis may result.

REFERENCES
I. Meriggioli, M.N., Barboi, A., Rowin, 1., Cochran, EJ., HMG-CoA reductase inhibitor myopathy: clinical,
electrophysiologic, and pathologic data in five patients. J. Clin. Neuromusc. Dis. 200 I; 2: 129-134.
2. Thompson, I'D., Clarkson, 1', Karas, R.H., Statin-associated myopathy, JAMA 2003; 289:1681-1690.

241
 PATIENT 60
A 38-year-old man with myasthenia gravis complaining of
muscular stiffness and rippling

A 38-year-old man developed bilateral arm flexion weakness, hypophonia, dysphagia, and nasal
regurgitation of liquids which progressed over 1 year. Serum CK ranged from 130 to 240 mg/dl (nor-
mal, 200 mg/dl). Left deltoid muscle biopsy showed degenerating and regenerating muscle fibers and
epimysial and endomysial inflammatory cell infiltrates. He was diagnosed with an inflammatory
myopathy and treated with prednisone; within 2 weeks, he noted substantial improvement. He tapered
his prednisone over 9 months, and relapsed with proximal arm weakness, diplopia, jaw fatigue, dys-
phagia, and head drop, and he had a marked response to edrophonium testing. Further evaluation
showed a 25% decrement to low-frequency repetitive nerve stimulation of the spinal accessory nerve
and normal needle EMG studies of a single deltoid muscle. The acetylcholine receptor antibody level
was 65.2 (normal, <0.08), and chest CT showed a 3x3x2-cm anterior mediastinal mass. He was treated
with 60 mg/day of prednisone for 6 weeks without benefit, underwent plasmapharesis with improve-
ment, and underwent thymectomy, with pathology revealing a thymoma.
He gradually improved and by the age of 42 was without symptoms on low-dose prednisone for
6 months when he started to note rippling waves of muscle contractions across his chest, back, and
limbs that were precipitated by percussion. Rapid extension of his arms became painful with a sensa-
tion that a muscle was catching. Serum CK increased over an 18-month period of increasing muscular
symptoms from 63 to 788 mg/dl. The acetylcholine receptor antibody level was 5.7. Needle EMG stud-
ies did not show fibrillation potentials or myotonic or neuromyotonic discharges. Electrical silence was
present during episodes of muscle rippling. Carbamazepine was without significant benefit. He con-
tinued to taper his prednisone and went on pyridostigmine alone for 2 years without symptoms of
myasthenia gravis but continued to have persistent symptoms of muscle rippling and stiffness with
rapid limb movement (see figures).

Figure 1

. 242
 Figure 2

Question: What is the name of this rippling muscle phenomenon?

243
/
 Answer: Rippling muscle disease

Discussion: Rippling muscle disease has two contraction that induces stretch of mechanosensi-
forms: genetic and acquired. The genetic form tive calcium channels in the neighboring sarcom-
may have been commented on in 1974 in the der- ere, initiating a new local contraction and a
matology literature as a transient rippling of the repetition of the cycle. Additional clinical fea-
skin in an infant. The syndrome was really identi- tures are myoedema, a localized mounding of
fied in 1975 by Torbergsen, II who described a muscle lasting several to 30 seconds and induced
family with dominant myotonia. At least two dis- by percussion, and percussion contracture, a clin-
tinct genetic loci exist: a lq41-q42 locus, for ical sign identical to percussion myotonia.
which the gene has not been identified, and a Thymoma is most commonly associated with
locus at 3p25 at the caveolin 3 gene. Mutations in myasthenia gravis but may be associated
this gene are also implicated in limb-girdle mus- with polymyositis or the rare syndromes of rip-
cular dystrophy type lc (LGMDlC) and in some pling muscle disease (RMD) or neuromyotonia.
patients with idiopathic elevations of serum CK. Rippling muscle disease in association with
The disorder is a rare, generally benign disor- myasthenia gravis had been reported in seven
der of muscle stiffness, muscular hypertrophy, patients as of 2002, the first in 1996. Two of
and modestly elevated serum CK. It is named for these patients have had thymoma, five have not.
the self-propagating rolling or rippling of muscles Three patients were reported to have electrically
induced by passive stretch or percussion, with active contraction, in stark contrast to all patients
velocity of -0.6 m/s (10 times slower than muscle with genetic RMD and the other four patients
fiber conduction velocity). The muscle contrac- with acquired RMD. Modestly elevated serum
tion is electrically silent and has been hypothe- CK and very high titers of acetylcholine receptor
sized to be a consequence of intracellular local antibodies have been present in all patients.

Clinical Pearl
Rippling muscle disease is typically familial, although an acquired form reported exclu-
sively in association with myasthenia gravis has been reported in a few patients since
1996.

REFERENCES
I. Ansevin, C.F., Agamanolis, D.P., Rippling muscles and myasthenia gravis with rippling muscles, Arch. Neurol. 1996;
53(2): 197-199.
2. Ansevin, C.F., Phenotypic variability in rippling muscle disease, Neurology 2000; 54(1):273-274.
3. Betz, R.C., Schoser, B.G., Kasper, D., Ricker, K., Ramirez, A., Stein, Y., Torbergsen, T, Lee, YA., Nothen, M.M.,
Wienker, TF., Malin, J.P., Propping, P., Reis, A., Mortier, w., Jentsch, TJ., Vorgerd, M., Kubisch, C., Mutations in CAV3
cause mechanical hyperirritability of skeletal muscle in rippling muscle disease, Nat. Genet. 2001; 28(3):218-219.
4. Fine, H.L., Possick, P.A., Myrow, P.F., Transient rippling of the skin (smooth muscle hamartoma?), Arch. Dermatol. 1974;
110:141.
5. Greenberg, S.A., Acquired rippling muscle disease with myasthenia gravis, Muscle Nerve 2004; 29:143-146.
6. Kosmorsky, G.S., Mehta, N., Mitsumoto, H., Prayson, R., Intermittent esotropia associated with rippling muscle disease,
J. Neuroophthalmol. 1995; 15(3):147-151.
7. Koul, R.L., Chand, R.P., Chacko, A., Ali, M., Brown, K.M., Bushnarmuth, S.R., Escolar, D.M., Stephan, D.A., Severe
autosomal recessive rippling muscle disease, Muscle Nerve. 2001; 24(11):1542-1547.
8. Muller-Felber, W., Ansevin, C.E, Ricker, K., Muller-Jenssen, A., Topfer, M., Goebel, H.H., Pongratz, D.E.,
Immunosuppressive treatment of rippling muscles in patients with myasthenia gravis, Neuromuscul. Disord. 1999;
9(8):604-607.
9. So, YT, Zu, L., Barraza, C., Figueroa, K.P., Pulst, S.M., Rippling muscle disease: evidence for phenotypic and genetic
heterogeneity, Muscle Nerve 2001; 24(3):340-344.
10. Stephan, D.A., Hoffman, E.P., Physical mapping of the rippling muscle disease locus, Genomics 1999; 55(3):268-274.
I I. Torbergsen, T, Rippling muscle disease: a review, Muscle Nerve 2002; I I (suppl.):SI03-SI07.
12. Vernino, S., Auger, R.G., Emslie-Smith, A.M., Harper, C.M., Lennon, Y.A., Myasthenia, thymoma, presynaptic
antibodies, and a continuum of neuromuscular hyperexcitability, Neurology 1999; 53(6):1233-1239.
13. Vorgerd, M., Ricker, K., Ziemssen, E, Kress, W., Goebel, H.H., Nix, W.A., Kubisch, c., Schoser, B.G., Mortier, W., A
sporadic case of rippling muscle disease caused by a de novo caveolin-3 mutation, Neurology 2001; 57(12):2273-2277.
14. Walker, G.R., Watkins, T, Ansevin, C.E, Identification of autoantibodies associated with rippling muscles and myasthenia
gravis that recognize skeletal muscle proteins: possible relationship of antigens and stretch-activated ion channels,
Biochem. Biophys. Res. Commun. 1999; 264(2):430-435.

244
 PATIENT 61

A 75-year-old woman with difficulty holding her head up

This 75-year-old woman noted 2 years of increasing difficulty holding her head up straight, some-
times requiring her to use her arm to support it. There was no fluctuation of the weakness over the
course of a day and no weakness in her limbs, swallowing, or other axial muscles. Examination showed
severe weakness of neck extensors with normal neck flexion and limb strength (see figure) ..

245
 Electrodiagnostic Study:

Sensory NCS
BP
Recording Onset Peak Amplitude Distance Velocity
Nerve Sites Site (ms) (ms) (llV) (cm) (m/s)
L. median-dig II I. Wrist Dig II 3.45 4.35 12.8 13 37.7
L. ulnar-dig V I. Wrist DigV 2.50 3.55 17.0 11 44.0
L. radial-sn box I. Forearm Sn box 1.85 2.50 21.8 10 54.1
L. median-ulnar I. Median-palm Wrist 2.20 2.80 20.3 8 36.4
(palmar) 2. Ulnar-palm Wrist 1.60 2.20 13.9 8 50.0

Motor NCS

Recording Latency Amplitude Distance Velocity

Nerve Sites Site (ms) (mV) (cm) (m/s)
L. median-APB I. Wrist APB 4.80 5.7 7
2. Elbow APB 9.35 4.9 21 46.2
L. ulnar-ADM I. Wrist ADM 3.00 5.4 7
2. B. elbow ADM 6.90 5.1 20 51.3
3. A. elbow ADM 8.85 4.1 II 56.4
L. tibial-AH I. Ankle AH 4.65 5.2 8
2. Pop fossa 12.90 3.1 39 47.3

F-Wave
Nerve Fmin (ms) Fmax (ms) Max - Min (ms) %F
L. tibial 59.05 60.90 1.85 80
L. ulnar 31.20 32.05 0.85 80

Needle EMG Summary Table

SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
L. deltoid NI 0 0 0 NI NI NI Full NI
L. biceps NI 0 0 0 NI NI NI Full NI
L. pron teres NI 0 0 0 NI NI NI Full NI
L. ext dig NI 0 0 0 Nl NI NI Full NI
comm
L. vast lat NI 0 0 0 NI NI NI Full NI
L. vast med NI 0 0 0 NI Nl Nl Full NI
L. tib ant Nl 0 0 0 Nl NI Nl Full Nl
L. gastroc NI 0 0 0 NI NI NI Full NI
med
L. L5 para- NI 0 0 0 Nl NI Nl Full Nl
spinals
R. vast lat NI 0 0 0 NI Nl NI Full NI
R. vast med NI 0 0 0 NI Nl NI Full NI
R. tib ant Nl 0 0 0 NI Nl NI Full Nl
R. gastroc Nl 0 0 0 NI Nl NI Full NI
med

246
 Needle EMG Summary Tabl~ont'd
SA
Amplitude Duration PolyP Recruitment
IA Fib Fasc Other (MUs) (MUs) (MUs) Activation Pattern
R. L4 para- Nl 1+ 0 0 Nl Nl Nl Full Nl
spinals
R. L5 para- Nl 1+ 0 0 Nl Nl Nl Full Nl
spinals
R. thoracic Nl 0 0 0 Nl Nl Nl Full Nl
PSP mid
L. thoracic Nl 0 0 0 Nl Nl Nl Full Nl
PSP mid
L. C6 para- Nl 3+ 0 0 Small Brief Nl Full Early
spinal
L. C7 para- Nl 3+ 0 0 Small Brief Nl Full Early
spinal
L. C8 para- Nl 3+ 0 0 Small Brief Nl Full Early·
spinal
L. sternoclei- Nl 0 0 0 Nt Nl Nl Full Nl
domast
R. C6 Para- Nl 3+ 0 0 Small Brief Nl Full Early
spinal
R. C7 Para- Nl 3+ 0 0 Small Brief Nl Full Early
spinal
R. C8 Para- Nl 3+ 0 0 Small Brief Nl Full Early
spinal

Question: What is your limited differential diagnosis?

247
 Answer: Myasthenia gravis, amyotrophic lateral sclerosis, inflammatory myopathies, nemaline
myopathy, and isolated cervical extensor myopathy.

Discussion: A brief differential diagnosis of recruitment in cervical paraspinal muscles. The

neck extensor weakness should include myasthe- mild degree of fibrillation potentials in unilateral
nia gravis, amyotrophic lateral sclerosis (ALS), lumbosacral paraspinal muscles is of uncertain
inflammatory myopathies, nemaline myopathy, significance. Note that sternocleidomastoid, a
and isolated cervical extensor myopathy. An ideal neck flexor, was normal, and note also the lack of
electrodiagnostic study should be directed toward other abnormalities (myopathic or neurogenic) in
establishing a muscle or neurogenic basis for the extensive sampling of other limbs. This patient
weakness and determining the distribution of was diagnosed with an isolated cervical extensor
abnormalities. This patient had normal serum CK myopathy, a condition of uncertain cause and
and negative acetylcholine receptor antibody classification. Atrophy of paraspinal muscles may
studies, arguing against (although not excluding) be visible on CT or MRI in some patients. This
inflammatory myopathies and myasthenia gravis. myopathy may in fact be an axial muscular dys-
Ideally, repetitive stimulation of a proximal mus- trophy and may overlap with other axial weakness
Cle should have been performed for myasthenia of the trunk and low back, impairing standing up
gravis, although the expected yield is low. straight as well.
The abnormalities are that offibrillation poten-
tials, myopathic motor unit morphology, and early

Clinical Pearl
A "dropped head" syndrome is seen in ALS, myasthenia gravis, inflammatory
myopathies, nemaline myopathy, isolated cervical extensor myopathy, and an axial
myopathy with a broader distribution, including a bent spine.

REFERENCES
I. Katz, 1.S., Wolfe, G.I., Bums, O.K. et aI., Isolated neck extensor myopathy: a common cause of dropped head syndrome,
Neurology 1996; 46:917-921.
2. Lomen-Hoerth, c., Simmons, M.L., Dearmond, SJ. et al., Adult-onset nemaline myopathy: another cause of dropped head,
Muscle Nerve 1999; 22:1146-1150.
3. Mahjneh, I., Marconi, G., Paetau, A., Saarinen, A., Salmi, T., Somer, H., Axial myopathy: an unrecognized entity, J. Neurol.
2002; 249:73~734.
4. Oerlemans, w.G., de Visser, M., Dropped head syndrome and bent spine syndrome: two separate clinical entities or
different manifestations of axial myopathy?, J. Neurol. Neurosurg. Psychiatry 1998; 65:258-259.
5. Serratrice, G., Pouget, 1., Pellissier, 1.F., Bent spine syndrome, J. Neurol. Neurosurg. Psychiatry 1996; 60:51-54.
6. Suarez, G.A., Kelly, 1.1., The dropped head syndrome, Neurology 1992; 42:1625-1627.
7. Swash, M., Dropped-head and bent-spine syndromes: axial myopathies?, Lancet 1998; 352:758.
8. Umapathi, T., Chaudhry, v., Comblath, D., Drachman, D., Griffin, 1., Kuncl, R., Head drop and camptocormia, J. Neurol.
Neurosurg. Psychiatry 2002; 73: 1-7.

248
 SECTION VI. EMG-GUIDED BOTULINUM TOXIN THERAPY
OF FOCAL DYSTONIAS

The availability of botulinum toxin therapy (BTX) since the early 1990s has changed the treat-
ment of many patients with focal dystonias. The efficacy of BTX has been established through con-
trolled trials in patients with blepharospasm and cervical dystonia.2 It was approved by the Food and
Drug Administration (FDA) for use in blepharospasm in 1989 and for cervical dystonia in 2QOO. H
appears to be effective for limb dystonias, although no clinical trial has established this. Its mechanis'rri
of action for neuromuscular junction blockade is via uptake into the presynaptic nerve terminal an'd
cleavage by the toxin of one of three SNARE proteins required for vesicle docking, fusion, and exo~
cytosis of acetylcholine. Botulinum toxin A (BoTox) cleaves the synaptosomal-associated protein
SNAP-25, while botulinum toxin B (Myobloc) cleaves the protein synaptobrevin. The mechanism of
relief of dystonia is less clear and presumably relates to changes in basal ganglia and cortical motor
and sensory integration resulting from the induced weakness.
Electromyography (EM G) has two potential roles as an adjunct for botulinum toxin therapy: to
identifj; which muscles should be injected by detection of abnormally firing motor units and to find
muscles already targeted for injection. At least one study has suggested that for cervical dystonia,
EMG studies were superior to clinical prediction alone for identifying which muscles should be tar-
geted.13 In limb dystonias, it has been shown that needle localization is more accurate in finding the
targeted muscle than placement without EMG guidance (Table 1).8 At least to date, this.has not been
shown to translate into a better result for the patient; as the toxin may diffuse to neighboring muscles
after injection, it certainly is possible that EMG guidance does not add benefit. In general, though, the
value of needle EMG for correct localization remains uncertain, and one view, dependent on the loca-
tion and type of dystonia, is shown in Table 2.

Table 1. Accuracy of Needle Placement into Intended Muscles

Intended Muscle Placement Site of Actual Needle Placement (No. of Patients)

FCR (n = 3) FCR (2), FDS II (1) .
FCU (n = 4) FCU (2), FDP II (1), not in muscle (1)
FPB(n=l) APB (1)
FPL (n = 3) FDS II (2), not in muscle (1)
PT (n = 3) PT (1), FDS III (1), FCR (1)
FDS II (n = 5) FDS (1), FCR (3), not in muscle (1)
FDSIV(n=l) FPL (1)
FDP II (n = 4) FDP II (2), FDP III (1), FCR (1)
FDP III (n = 3) FDP III (2), Not in muscle (1)
FDP IV (n = 2) FDP IV (1), FCU (1)
EIP (n = 5) EIP (2), EDC II (2), not in muscle (1)
EDC II (n = 3) EDC II (1), ECR (2)
ECU (n = 1) EDC II (1)

Source: Molloy, EM. et ai., Neurology, 58, 805-807,2002.

249
 Table 2. Need for EMG Guidance in the Treatment of Various Dystonias

Focal dystonia Usually Sometimes Not Usually

Facial dystonias
Blepharospasm X
Oromandibular X
Complex X
Cervical dystonia X
Limb dystonias
Arm X
Leg X
Segmental myoclonus X

. The approach is straightforward. Muscles that appear to be contracting inappropriately and pro-
ducing the abnormal posture are targeted for injection with BTX. These muscles are usually deter-
'rnined by clinical inspection, and then needle EMG is used to localize the muscle, either by the
presence of abundant involuntary motor unit action potentials or more commonly by asking the patient
to selectively contract the muscle. Weakness and apparent clinical benefit generally are observed
within days to a week and last from 2 to 6 months (typically 3 months). Injections may be repeated as
long as benefit is apparent, which may be indefinite. Often, fibrillation potentials are seen with sub-
sequent EMG-guided injections, and atrophy of the muscle becomes clinically apparent.

REFERENCES
I. Byrnes, M.L., Thickbroom, G.w., Wilson, S.A., Sacco, P., Shipman, J.M., Stell, R., Mastaglia, F.L., The corticomotor
representation of upper limb muscles in writer's cramp and changes following botulinum toxin injection, Brain 1998;
121 :977-988.
2. Ceballos-Baumann, A.a., Evidence-based medicine in botulinum toxin therapy for cervical dystonia, J Neurol. 200 I;
248(suppl. I): 14-20.
3. Ceballos-Baumann, A.a., Sheean, G., Passingham, R.E., Marsden, C.D., Brooks, 0.1., Botulinum toxin does not reverse
the cortical dysfunction associated with writer's cramp: a PET study, Brain 1997; 120:571-582.
4. Cole, R., Hallett, M., Cohen, L.G., Double-blind trial of botulinum toxin for treatment of focal hand dystonia, Mov.
Disord. 1995; 10:466-471.
5. Comella, c., Buchman, A.S., Tanner, C.M., Brown-Toms, N.C., Goetz, C.G., Botulinum toxin injection for spasmodic
torticollis: increased magnitude of benefit with electromyographic assistance, Neurology 1992; 42:878-882.
6. Farmer, S.E, Sheean, G.L., Mayston, M.1., Rothwell, J.C., Marsden, C.D., Conway, B.A., Halliday, D.M., Rosenberg, J.R.,
Stephens, J.A., Abnormal motor unit synchronization of antagonist muscles underlies pathological co-contraction in upper
limb dystonia, Brain 1998; 121 :801-814.
7. Guyer, B.M., Some unresolved issues with botulinum toxin, J Neurol. 2001; 248(suppl. I): 1111-1/13.
8. Molloy, EM., Shill, H.A., Kaelin-Lang, A., Karp, B.I., Accuracy of muscle localization without EMG: implications for
treatment oflimb dystonia, Neurology 2002; 58:805-807.
9. Murase, N., Kaji, R., Shimazu, H., Katayama-Hirota, M., Ikeda, A., Kohara, N., Kimura, J., Shibasaki, H., Rothwell, J.C.,
Abnormal premovement gating of somatosensory input in writer's cramp, Brain 2000; 123: 1813-1829.
10. Oga, T., Honda, M., Toma, K., Murase, N., Okada, T., Hanakawa, T., Sawamoto, N., Nagamine, T., Konishi, J., Fukuyama,
H., Kaji, R., Shibasaki, H., Abnormal cortical mechanisms of voluntary muscle relaxation in patients with writer's cramp:
an fMRI study, Brain 2002; 125:895-903.
II. Ross, M., Charness, M.E., Sudarsky, L., Logigian, E.L., Treatment of occupational cramp with botulinum toxin: diffusion
of toxin to adjacent noninjected muscles, Muscle Nerve 1997; 20: 593-598.
12. Speelman, J.D., Brans, J.W.M., Cervical dystonia and botulinum treatment: is electromyographic guidance necessary?,
Mov. Disord. 1995; 10:802.
13. Van Gerpen, J.A., Matsumoto, J.Y., Ahlskog, lE., Maraganore, D.M., McManis, P.O., Utility of an EMG mapping study
in treating cervical dystonia, Muscle Nerve 2000; 23: 1752-1756.
14. Yoshimura, D.M., Aminoff, M.1., Olney, R.K., Botulinum toxin therapy for limb dystonias, Neurology 1992; 42:627--630.

250
 PATIENT 62

A 39-year-old woman with 3 to 4 years of progressive difficulty

using her right hand

The patient has had progressive difficulty using her right hand for writing, but she has no diffi-
culty using it for buttoning, typing, or using utensils.

A B

c D

Question: Some patients with writer's cramp learn to write with the other hand. Do you think
this strategy would work well for this woman over the long-term?

25t
 Answer: No. Left hand dystonia is visible in figure D.

Discussion: Note the normal posture of the be the case. The muscles and doses targeted for
hand holding the pen before writing (A) and the her treatment were as follows:
rapid development of dystonia when starting to • Flexor carpi ulnaris (FCU)-30 U
write. Images (B) and (C) suggest hyperextension • Flexor digitorum superficial is (FDS) digit
at the second metacarpophalangeal (MCP) joint 2-20 U
and excessive flexion at the second proximal • Flexor digitorum superficialis (FDS) digit
interphalangeal (PIP) joint. There is probably 3-20 U
excessive flexion at the third PIP joint, as is evi- Treatment repeated 5 times over a I-year
dent in (C), in comparison to the posture of the period seemed to be producing significant weak-
third digit in (A). The tip of the thumb also slips ness but no benefit in her dystonia. Changes in
off the pen in figure (B). Excessive flexion of the her regimen including injections of FDP fourth
wrist is present in (B) and (D), and abduction of and fifth digits and FCR also produced signifi-
the arm at the shoulder is excessive in (D) . cant weakness but no benefit in her dystonia, so
.. :The predominant aspects of this dystonia are botulinum toxin treatment was abandoned. Note
the flexion at the wrist and fingers as noted the dystonia present in her left hand that is evident
above. The hyperextension of the second MCP in (B) and (D), where she has difficulty holding
joint is likely compensation for the excessive onto the paper properly. Accordingly, she would
flexion at the PIP joint forcing the finger to seem to be at high risk of developing writer's
hyperextend in order to hold onto the pen. The cramp in her left hand should she learn to write
patient reported subjectively that this seemed to with it.

Clinical Pearls
I. Botulinum toxin is not always effective in the treatment of focal dystonias.
2. Look for subtle signs of dystonia in the other hand when observing patients with
writer's cramp.

REFERENCES
1. Sheehy, M.P., Marsden, C.D., Writer's cramp: a focal dystonia, Brain 1982; 105:461-480.
(Also see reference list for the introduction to this section.)

252
 PATIENT 63

A 47-year-old man with progressive difficulty in writing for

5 years

The patient reported that he had difficulty keeping his second and third digits against the pen
because of involuntary extension, and he attempted to compensate by tightening his grip with his
thumb (see figure).

A B
Question: What muscles and dosage would you use based on the views shown in the figure?

253
 Answer: Extensor indicis proprius, 2.5 U; extensor digitorum communis, 5 U; flexor carpi radialis,
15 U

Discussion: This patient responded well to joints; an additional muscle that might be of value
dosing as follows: in this case is the second lumbrical (to third digit),
• Extensor indicis proprius (EIP}-2.5 U which acts to extend the third finger at the PIP
• Extensor digitorum communis (EDC}-5 U joint. This is probably the most evident aspect of
• Flexor carpi radialis (FCR}-15 U his dystonia, and future injections will target this
The radial deviation of the wrist is difficult to muscle as well. This patient had a good response
appreciate in this picture. The EIP and EDC are lasting 3 months to subsequent injections.
responsible for extension principally at the MCP

Clinical Pearl
A wide range of individual dose responsiveness occurs with botulinum toxin treatment;
some patients respond to very small doses, and others require larger doses.

REFERENCES
I. Byrnes, M.L., Thickbroom, G.W., Wilson, S.A., Sacco, P., Shipman,J.M., Stell, R., Mastaglia, FL., The corticomotor
representation of upper limb muscles in writer's cramp and changes following botulinum toxin injection, Brain 1998;
121 :977-988 .
. 2. Ceballos-Baumann, A.O., Evidence-based medicine in botulinum toxin therapy for cervical dystonia, J. Neurol. 200 I;
248(suppl. 1):14-20.
3. Ceballos-Baumann, A.O., Sheean, G., Passingham, R.E., Marsden, C.D., Brooks, DJ., Botulinum toxin does not reverse
the cortical dysfunction associated with writer's cramp: a PET study, Brain 1997; 120:571-582.
4. Cole, R., Hallett, M., Cohen, L.G., Double-blind trial of botulinum toxin for treatment of focal hand dystonia, Mov.
Disord. 1995; 10:466-471.
5. Comella, c., Buchman, A.S., Tanner, C.M., Brown-Toms, N.C., Goetz, C.G., Botulinum toxin injection for spasmodic
torticollis: increased magnitude of benefit with electromyographic assistance, Neurology 1992; 42:878-882.
6. Farmer, S.E, Sheean, G.L., Mayston, MJ., Rothwell, J.C., Marsden, C.D., Conway, B.A., Halliday, D.M., Rosenberg, J.R.,
Stephens, J.A., Abnormal motor unit synchronization of antagonist muscles underlies pathological co-contraction in upper
limb dystonia, Brain 1998; 121 :801-814.
7. Guyer, B.M., Some unresolved issues with botulinum toxin, J. Neurol. 2001; 248(suppl. I): 1/11-1113 .
. 8. Molloy, EM., Shill, H.A., Kaelin-Lang, A., Karp, B.\., Accuracy of muscle localization without EMG: implications for
treatment oflimb dystonia, Neurology 2002; 58:805-807.
9. Murase, N., Kaji, R., Shimazu, H., Katayama-Hirota, M., Ikeda, A., Kohara, N., Kimura, J., Shibasaki, H., Rothwell, J.C.,
Abnormal premovement gating of somatosensory input in writer's cramp, Brain 2000; 123: 1813-1829.
10. Oga, T., Honda, M., Toma, K., Murase, N., Okada, T., Hanakawa, T., Sawamoto, N., Nagamine, T., Konishi, J., Fukuyama,
H., Kaji, R., Shibasaki, H., Abnormal cortical mechanisms of voluntary muscle relaxation in patients with writer's cramp:
an fMRI study, Brain 2002; 125:895-903.
II. Ross, M., Charness, M.E., Sudarsky, L., Logigian, E.L., Treatment of occupational cramp with botulinum toxin: diffusion
of toxin to adjacent non injected muscles, Muscle Nerve 1997; 20: 593-598.
12. Speelman, J.D., Brans,J.WM., Cervical dystonia and botulinum treatment: is electromyographic guidance necessary?,
Mov. Disord. 1995; 10:802.
13. Van Gerpen, J.A., Matsumoto, J.Y., Ahlskog,J.E., Maraganore, D.M., McManis, P.G., Utility of an EMG mapping study
in treating cervical dystonia, Muscle Nerve 2000; 23: 1752-1 756.
14. Yoshimura, D.M., Aminoff, MJ., Olney, R.K., Botulinum toxin therapy for limb dystonias, Neurology 1992; 42:627-630.

254
 PATIENT 64

A 45-year-old woman with several years of difficulty with her

right hand limited to writing

This patient initially held the pen normally (figure lA), but then rapidly developed the dystonia
evident in the subsequent figures ..

c D
Figure 1

255
 4,,, f'S r".
0... 5i::lI •.••••. <'T~ ""y .,x-< .L(~ ~ •

jlN-J. I ~,( hG:.7'(~ t-b ""l( f.,.a..})

~ -L. ~
-Le... ~1>'i!"'4:r ~
, ..-<1'
k.~dL ~"'( +-'( ~3~~A. ~<Ef>r....1 I"
at.. (II(. u... 5~1\""" e (. <.. ( q.CA.{'
bH~~\.~""-v <W( ~ ~ cP,;~ k#"" (
~ cf rtU . Uly ~o(tLJ a~
~oti<~ ~~r~' ~,.,.\.\/~(~
Figure 2

Question: What muscles and what doses would you use for treatment?

156
 Answer: Supinator 20 U; Flexor digitorum superficialis, second digit 10 U; Flexor digitorum
superficialis, third digit IO U; Flexor digitorum superficialis, fourth digit 15 U; Flexor digitorum super-
ficialis, fifth digit 5 U

Discussion: This case shares some similari- • Flexor digitorum superficial is, third digit-
ties to case 62. The patient's hand appears normal 10 U
while holding the pen prior to writing (see part • Flexor digitorum superficial is, fourth
(A) in figure). In (B) there is excessive flexion at digit-IS U
the second PIP joint (FDS digit 2) and the thumb • Flexor digitorum superficialis, fifth digit-
interphalangeal joint (flexor pollicis longus, 5U
FPL). In (C) and (D), note the excessive flexion Alterations to this regimen that might be con-
of the other digits as well; digit 5 is digging into sidered in the future would include injection of
the palm of her hand in (C). It is difficult to FPL and larger doses to the FDS second digit
appreciate from the picture, but there is also (say, 15 to 20 U) than to the fourth digit (say, a
abnormal supination of the forearm and extension decrease to IOU). As apparent, treatment regimen
of the wrist (the latter best seen in (B)). Her treat- selection is often empirically derived from trial
ment regimen was as follows and produced satis- and error after initial estimates based on the pat-
factory results with a frequency of every 6 months: tern of dystonia.
• Supinator-20 U
• Flexor digitorum superficial is, second
digit-IO U

Clinical Pearl
Treatment regimens with botulinum toxin are empirically derived after initial estimates
and trial.

REFERENCES
See reference list for the introduction to this section.

257
 PATIENT 65

A 40-year-old. physician with progressive difficulty writing for

3 years

The patient has had no difficulty with typing or writing on a blackboard.

A B

Question: What muscles would you target for treatment?

258
 Answer: Finger extensors

Discussion: The figure demonstrates the pensate, the patient typically holds the pen as
normal posture of the hand holding the pen before shown in (C), maintaining a modest ability to
writing (A). Within 10 seconds of writing, the write. Injection as follows resulted in moderate
patient develops involuntary extension of the benefit to the patient:
third to fifth digits and can barely hold the pen • Extensor digitorum communis (EDC}-30 U
(B). Soon thereafter, the second digit extends as • Extensor digitorum, fourth finger-20 U
well, and the pen drops out of the hand. To com- • Extensor digitorum, fifth finger-IOU

Clinical Pearl
The focal dystonia can be very dramatic, as in this case. It is worthwhile to follow such
patients using photographs to assess response.

REFERENCES
I. Byrnes, M.L., Thickbroom, G.w., Wilson, S.A., Sacco, P., Shipman, 1M., Stell, R., Mastaglia, F.L., The corticomotor
representation of upper limb muscles in writer's cramp and changes following botulinum toxin injection, Brain 1998;
121 :977-988.
2. Ceballos-Baumann, A.a., Evidence-based medicine in botulinum toxin therapy for cervical dystonia, J Neurol. 200 I;
248(suppl. I): 14-20.
3. Ceballos-Baumann, A.a., Sheean, G., Passingham, R.E., Marsden, C.D., Brooks, OJ., Botulinum toxin does not reverse
the cortical dysfunction associated with writer's cramp: a PET study, Brain 1997; 120:571-582.
4. Cole, R., Hallett, M., Cohen, L.G., Double-blind trial of botulinum toxin for treatment of focal hand dystonia, Mov.
Disord. 1995; 10:466-471.
5. Comella, e., Buchman, A.S., Tanner, e.M., Brown-Toms, N.e., Goetz, e.G., Botulinum toxin injection for spasmodic
torticollis: increased magnitude of benefit with electromyographic assistance, Neurology 1992; 42:878-882.
6. Farmer, S.F., Sheean, G.L., Mayston, M.l, Rothwell, lC., Marsden, e.D., Conway, B.A., Halliday, D.M., Rosenberg, lR.,
Stephens, lA., Abnormal motor unit synchronization of antagonist muscles underlies pathological co-contraction in upper
limb dystonia, Brain 1998; 121 :801-814.
7. Guyer, B.M., Some unresolved issues with botulinum toxin, J Neurol. 2001; 248(suppl. I): 1111-1/13.
8. Molloy, F.M., Shill, H.A., Kaelin-Lang, A., Karp, B.!., Accuracy of muscle localization without EMG: implications for
treatment of limb dystonia, Neurology 2002; 58:805-807.
9. Murase, N., Kaji, R., Shimazu, H., Katayama-Hirota, M., Ikeda, A., Kohara, N., Kimura, 1, Shibasaki, H., Rothwell, lC.,
Abnormal premovement gating of somatosensory input in writer's cramp, Brain 2000; 123: 1813-1829.
10. Oga, T, Honda, M., Toma, K., Murase, N., Okada, T, Hanakawa, T, Sawamoto, N., Nagamine, T, Konishi, 1, Fukuyama,
H., Kaji, R., Shibasaki, H., Abnormal cortical mechanisms of voluntary muscle relaxation in patients with writer's cramp:
an fMRI study, Brain 2002; 125:895-903.
II. Ross, M., Chamess, M.E., Sudarsky, L., Logigian, E.L., Treatment of occupational cramp with botulinum toxin: diffusion
of toxin to adjacent noninjected muscles, Muscle Nerve 1997; 20: 593-598.
12. Speelman, 10., Brans, lW.M., Cervical dystonia and botulinum treatment: is electromyographic guidance necessary?,
Mov. Disord. 1995; 10:802.
13. Van Gerpen, lA., Matsumoto, lY., Ahlskog, lE., Maraganore, D.M., McManis, P.G., Utility of an EMG mapping study
in treating cervical dystonia, Muscle Nerve 2000; 23: 1752-1756.
14. Yoshimura, D.M., Aminoff, MJ., Olney, R.K., Botulinum toxin therapy for limb dystonias, Neurology 1992; 42:627-630.

259
ii· :
 INDEX

A Brachial plexus (Continued)

AAEM. See American Association of proximal median neuropathy, 15-18
Electrodiagnostic Medicine anatomy of, 3f
Abductor digiti minimi, I, 3t Brachioradialis, 3t
Abductor pollicis brevis, 3t
Abnormal median normal sural sensory nerve action C
potentials, 133-136, 137-139 C6 nerve root avulsion, 58
Acetylcholine receptor deficiency, primary, 191 Carpal tunnel syndrome
Acetylcholinesterase deficiency, endplate, 191 bilateral, 5-7
Acute brachial neuritis, 62 evaluations for, 31-33
Acute inflammatory demyelinating polyneuropathy, median-ulnar second lumbrical interosseous test
133,196 for, 13f, 14
Adductor longus, 71 mild, 10
Adductor magnus, 71, 78 sensory nerve action potential affected by, I
Adductor pollicis, 3t Cervical radiculopathy, 70
Adenine nucleotide trans locator I, 220 Charcot-Marie-Tooth (X-linked), 147, 155, 162
AIOP. See Acute inflammatory demyelinating Chronic inflammatory demyelinating
polyneuropathy polyneuropathy
ALS. See Amyotrophic lateral sclerosis description of, 196
American Association of Electrodiagnostic Medicine, diagnosis of, 133, 139, 144-147
10,203 Guillain-Barre, syndrome vs., 142, 152
Amyloidosis, 100, JOlt multi focal, 209
Amyotrophic lateral sclerosis Chronic progressive dorsal root ganglionopathy, 174
description of, 192 ClOP. See Chronic inflammatory demyelinating
diagnosis of, 194-196 polyneuropathy
"dropped head" syndrome associated with, 245-248 CMAPs. See Compound muscle action potentials
multi focal motor neuropathy vs., 199-205 Common peroneal neuropathy, 74
ANT I. See Adenine nucleotide trans locator I Compound muscle action potentials
Anterior interosseous nerve, 62 distal, 134
Anti-myelin-associated glycoprotein, 99 distal evoked, 130
Anti-myelin-associated glycoprotein neuropathy, 166 generalized motor neuron disease evaluations, 196
Anti-sulfatide neuropathy, 166 peroneal-extensor digitorum brevis, I, 74
Atorvastatin, 241 Conduction block, 126-130, 135, 147,209
Autonomic neuropathy, 103 Congenital muscular dystrophy associated with
A-waves, 150---152 merosin deficiency, 215-217
Axon reflex, 150 Congenital myasthenic syndromes, 187-191
Axonal degeneration, 58 Connexin-32, 163
AZT,235 Continuous motor unit activity, 209
Critical illness myopathy, 170
B Critical illness polyneuropathy, 170
Becker muscular dystrophy, 21 7 Cryoglobulinemias, 100, JOlt
Biceps,3t Cubital tunnel syndrome, 25
Biceps femoris, 72, 78
Botulinum toxin therapy, for focal dystonias D
electromyography-guided, 249 Deep peroneal nerve, 71
extensor digitorum communis, 253-254 Dejerine-Sottas disease, 159, 163
extensor indicis proprius, 253-254 Deltoid, 3t
finger extensors, 258-259 Demyelinating neuropathy
flexor carpi radialis, 253-254 diagnosis of, 148-152
flexor digitorum superficial is, 257 inherited, 153-155, 156-157, 158-159
left hand dystonia, 251-252 Demyelinating polyneuropathy
overview of, 249 acute inflammatory, 133, 196
supinator, 257 chronic inflammatory
Brachial neuritis, acute, 62 description of, 196
Brachial plexus diagnosis of, 133, 139, 144-147
abnormalities of Guillain-Barre, syndrome vs., 142, 152
evaluation of, 15-18 multi focal, 209

261
Dermatomyositis Flexor hallucis longus, 72
description of, 221 Flexor pollicis longus, 3t
diagnosis of, 229-231 Floppy infant, 197-198
Diabetic proximal neuropathy, 103-106 Focal cranial neuropathy, 103
Diabetic thoracoabdominal neuropathy, 107-109 Focal dystonias, botulinum toxin therapy for
Diffuse motor neuropathy, 94 electromyography-guided, 249
Diffuse sensory neuropathy, 94 extensor digitorum communis, 253-254
Disc herniation, L5-S I central and right-sided, 90 extensor indicis proprius, 253-254
Distal axonal neuropathy, 110-111 finger extensors, 258-259
Distal evoked compound muscle action potentials, 130 flexor carpi radialis, 253-254
Distal symmetric motor neuropathy, 94 flexor digitorum superficialis, 257
Distal symmetric polyneuropathy, 237 left hand dystonia, 251-252
Distal symmetric sensory neuropathy, 94 overview of, 249
Distal ulnar neuropathies, 29 supinator, 257
Dorsal ulnar cutaneous sensory nerve action Focal motor demyelination, 192
potentials, 70 Focal neuropathies
"Dropped head" syndrome, 245-248 definition of, I
Duchenne muscular dystrophy, 217 localization of, 1
Dystonia lower limb, 71-93
focal. See Focal dystonias upper limb, 1-70
left hand, 251-252 Foot drop
case studies of, 73-75, 76-78
E differential diagnosis, 75
Elbow, ulnar neuropathy at, 19-22, 23-26 F-waves
Electromyography with acute axonal injury, 58
botulinum toxin therapy for focal dystonias guided latency of, 46
by, 249 tibial, 81
needle
generalized neuropathies evaluated using, 95-96 G
myopathy evaluations, 211 Gap junction protein B I, 163
radiculopathy screening using, 66 Gastrocnemius, 72
single fiber GBS. See Guillain-Barre, syndrome
neuromuscular junction disease evaluated using, Generalized neuropathies
175-176 electrodiagnostic approach to, 95-96
technical elements of, 176 types of, 94-95
EMG. See Electromyography Gluteus maximus, 72
Endplate acetylcholinesterase deficiency, 191 Gluteus medius, 72
Erythema nodosum leprosum, 119 Guillain-Barre, syndrome
Extensor carpi radialis, 3t chronic inflammatory demyelinating
Extensor carpi ulnaris, 3t polyneuropathy vs., 142, 152
Extensor digitorum, 3t diagnosis of, 130, 140-143
Extensor digitorum communis, 46, 253-254 Guyon's canal, 29
Extensor hallucis longus, 72
Extensor indicis proprius, 253-254 H
Hepatitis C, 10 It
F Hereditary neuropathy with liability to pressure
Facioscapulohumeral muscular dystrophy, 212-214 palsies, 155, 162
Fasciculation potentials, 192 H-reflex, 90
Fast channel syndromes, 191 Human immunodeficiency virus, 235-238
Fibrillation potentials Humeroulnar arcade, 25
absence of, 120-125 Hysterical paralysis, 58
description of, 58
in myopathies, 211 I
Wallerian degeneration findings, 66 Iliopsoas, 71
Finger extensor muscles, 258-259 Immunofixation, 99
First dorsal interosseous, 1, 3t Inclusion body myositis, 221-224
Flexor carpi radialis Inflammatory myopathies
anatomy of, 3t, 62 description of, 223-224
botulinum toxin therapy of, for dystonia, 253-254 "dropped head" syndrome associated with, 245-248
Flexor carpi ulnaris, 3t inclusion body myositis, 221-224
Flexor digitorum profundus, 62 polymyositis, 225-228
Flexor digitorum superficial is Infraspinatus, 3t
botulinum toxin therapy of, 257 Inherited demyelinating neuropathies, ]53-]55,
description of, 62 156-157, 158-159

262
Inherited neuropathy, 160-163 Median-ulnar second lumbrical interosseous test, 13f,
Intravenous immunoglobulin, 203, 209 14,29
Merosin deficiency, 217
K MG. See Myasthenia gravis
Keams-Sayre syndrome, 220 Mitochondrial myopathy, 235
MMN. See Multifocal motor neuropathy
L Monoclonal gammopathy of undetermined
L5 radiculopathy, 74 significance, 99, JOlt
Lambert-Eaton myasthenic syndrome Motor neuron disease, 192
clinical features of, 186 Motor neuropathies
congenital myasthenic syndrome and, 191 electrodiagnostic goals for, 192
description of, 175 multifocal
evaluations of, 183-186 amyotrophic lateral sclerosis vs., 199-205
voltage-gated calcium channels in, 186 chronic inflammatory demyelinating
Lateral femoral cutaneous nerve, 71 polyneuropathy vs., 209
Lateral plantar nerve, 71 description of, 94, 147
Left arm weakness, 55-58, 64-67 diagnosis of, 203-204, 206-210
Left foot weakness, 79-83 Motor unit action potentials
Left hand in myopathies, 211
dystonia of, 251-252 in spinal muscular atrophy, 198
numbness, 19-22,27-30 Motor unit number estimation, 192
weakness of, 59-63, 194-196 MUAPs. See Motor unit action potentials
Left median-abductor digiti minimi compound Multifocal acquired demyelinating sensory and motor·
muscle action potential amplitude reduction, 6 neuropathy, 209 .
Left ulnar-abductor digiti minimi compound muscle Multifocal asymmetric sensory neuropathy, IJO-111
action potential, 186 Multifocal motor neuropathy
LEMS. See Lambert-Eaton myasthenic syndrome amyotrophic lateral sclerosis vs., 199-205
Lepromatous leprosy, 118 chronic inflammatory demyelinating
Lepromatous neuropathy, 115-119 polyneuropathy vs., 209
Leprosy, 118-119 description of, 94, 147
LFS. See Low-frequency stimulation diagnosis of, 203-204, 206-210
LGMD. See Limb-girdle muscular dystrophy Multifocal sensory neuropathy, 94
Limb-girdle muscular dystrophy, 212-214, 244 Multiple myeloma, 99, JOlt
L2/L3 radiculopathy, 86 Muscular dystrophies
Long thoracic neuropathy, 45 Becker, 217
Lower limb congenital, 215-217
focal neuropathies of, 71-93 Duchenne, 217
nerve innervation of, 71-72 facioscapulohumeral, 212-214
Low-frequency stimulation limb-girdle, 212-214, 244
Lambert-Eaton myasthenic syndrome evaluation, 186 Myasthenia gravis
myasthenia gravis evaluation, 181 "dropped head" syndrome associated with,
L5-S I central and right-sided disc herniation, 90 245-248
Lumbar plexopathy, 86 evaluation for, 178-182
Lumbosacral plexopathy, 93 post-exercise exhaustion associated with, 181-182 .
Lyme disease, 120-125 repetitive nerve stimulation findings, 175, 181
rippling muscle disease associated with, 242-244
M Mycobacterium leprae. 118
MAG. See Myelin-associated glycoprotein Myelin protein zero, 157, 162
Martin-Gruber anastomosis, 31-33 Myelin-associated glycoprotein, 99
Medial plantar nerve, 71 Myelin-associated glycoprotein neuropathy, 166
Median neuropathy, 6 Myokymic potentials, 81-83
Median palm-wrist sensory latency, for carpal tunnel Myopathies
syndrome, 10 electrodiagnostic studies for, 218-220
Median-APB distal motor latency prolongation, for fibrillation potentials in, 211
carpal tunnel syndrome, 6 HIV-associated, 235-238
Median-D2 compound muscle action potentials, in inclusion body myositis, 221-224
Martin-Gruber anastomosis, 33 inflammatory
Median-D2 distal sensory conduction velocity description of, 223-224
reduction, for carpal tunnel syndrome, 6 "dropped head" syndrome associated with,
Median-D2 sensory nerve action potential, 6, 58 245-248
Median-ulnar D4 radial D I interlatency differences, inclusion body myositis, 221-224
for carpal tunnel syndrome, 10 polymyositis, 225-228
Median-ulnar mixed palmar interlatency difference, with membrane irritability, 227
for carpal tunnel syndrome, JO mitochondrial, 235

263
Myopathies (Continued) Neuropathy (Continued)
with myotonic discharges, 239-241 ulnar
with myotonic dystrophy, 232-234 description of, I
needle electromyography studies of, 211 distal, 29
proximal myotonic, 234 at or near the elbow, 19-22, 23-26
Myotilin,214 at wrist, 27-30
Myotonic discharges, myopathy with, 239-241 Numbness
Myotonic dystrophy, myopathy with, 232-234 case study investigations of, 5-7, 8-11, 19-22,
23-26, 46-49, 68-70, 97-102, 110-111,
N 112-114,131-136,140-143,148-152,
Nasalis waveforms, in myasthenia gravis evaluation, 164-167,172-174
181-182 left hand, 19-22, 27-30
Needle electromyography right hand, 144-147
generalized neuropathies evaluated using, 95-96 right leg, 84-86, 91-93
myopathy evaluations, 211
radiculopathy screening using, 66 o
Neuralgic amyotrophy, 62 Opponens pollicis, 3t
Neurologic thoracic outlet syndrome, 46-50 Osteosclerotic myeloma, 99-100, 10 It
Neuromuscular junction diseases
congenital myasthenic syndromes, 187-191 p
description of, 175 Paraproteinemic neuropathies, 99-101
electrodiagnostic studies for, 175 Parsonnage- Turner syndrome, 62
Lambert-Eaton myasthenic syndrome Peri fascicular atrophy, 231
clinical features of, 186 Peripheral myelin protein 22 gene, 159, 162
congenital myasthenic syndrome and, 191 Peripheral neuropathy
description of, 175 description of, 114
evaluations of, 183-186 general ized, 164-166
voltage-gated calcium channels in, 186 Peroneal neuropathy, 74
myasthenia gravis Peroneal-extensor digitorum brevis compound muscle
"dropped head" syndrome associated with, action potentials
245-248 description of, I
evaluation for, 178-182 generalized neuropathies evaluated using, 95
post-exercise exhaustion associated with, L5 radiculopathy and, 74
181-182 Peroneal-tibialis anterior compound muscle action
repetitive nerve stimulation findings, 175, 181 potentials, 74
rippling muscle disease associated with, Peroneus longus, 72
242-244 Plexopathy, lumbosacral, 93
Neuropathy PMP-22 gene, 159, 162
acquired, 164-167 POEMS syndrome, 100
autonomic, 103 Polymyositis
with autonomic involvement, 94 description of, 221
common peroneal, 74 diagnosis of, 225-228
demyelinating, 148-152, 153-155 Polyneuropathy
diabetic proximal, 103-106 acute inflammatory demyelinating, 133, 196
diabetic thoracoabdominal, 107-109 chronic inflammatory demyelinating
distal axonal, 110-111 description of, 196
focal cranial, 103 diagnosis of, 133, 139, 144-147
inherited, 160-163 Guillain-Barre, syndrome vs., 142, 152
lepromatous, 115-119 multifocal, 209
long thoracic, 45 critical illness, 170
median, 6 distal symmetric, 237
multi focal motor, 147 human immunodeficiency virus and, 237
myelin-associated glycoprotein, 166 Posterior interosseous nerve, 62
peripheral, 114 Post-exercise exhaustion, in myasthenia gravis,
peroneal, 74 181-182
proximal, 34-37 . Primary acetylcholine receptor deficiency, 191
proximal median, 15-18 Progressive external ophthalmoplegia, 218-220
proximal radial, 34-37 Pronator teres, 3t, 62
radial Proprioceptive disturbances, 172-174
proximal, 34-37 Proximal median neuropathy, 15-18
wrist drop caused by, 36 Proximal myotonic myopathy, 234
sarcoid, 114 Proximal radial neuropathy, 34-37
sciatic, 78 Pseudo-conduction block, 130
sulfatide antibody-mediated, 166 Pseudomeningoceles, 51-54

264
Q Spinal accessory nerve
Quadriceps, 72 location of, 39
trapezius weakness caused by lesions of, 39, 45 .
R Spinal motor neuron gene, 198
Radial nerve, 208 Spinal muscular atrophy, 198
Radial neuropathy Sulfatide antibody-mediated neuropathy, 166
proximal, 34-37 Superficial peroneal nerve, 71
wrist drop caused by, 36 Supinator
Radiation-induced focal neuropathy, 82 anatomy of, 3t
Radiculoneuritis, 125 botulinum toxin therapy of, 257
Radiculopathy Supraspinatus, 3t
cervical, 70 Sural nerve, 71, 78
L5, 74 Sural sensory nerve action potentials, 130
L2-L3,86 Synaptic vesicles, 190
needle electromyography screening of, 66 Syringomyelia, 46
Repetitive nerve stimulation
history of, 175 T
myasthenia gravis evaluations, 175, 181 Tensor fascia lata, 72
Right arm weakness, 51-54 Teres minor, 3t
Right hand Thoracic outlet syndrome, neurologic, 46-50
numbness, 144-147 Thoracoabdominal neuropathy, 103
weakness of, 15-18 Thymoma, 244
Right leg Tibial F-waves, 81
numbness of, 84-86, 91-93 Tibial H-reflex, 90
pain of, 87-90 Tibial nerve, 71
weakness of, 84-86, 91-93 Tibialis anterior
Rippling muscle disease, 242-244 characteristics of, 72
Root avulsion in foot drop, 75
C6 nerve, 58 Tibialis posterior, 72
case study of, 51-54 Tingling in hands, 5-7, 8-11
Trapezius
S illustration of, 42, 45
Saphenous nerve, 71 weakness of, 39, 45
Sarcoid neuropathy, 114 Triceps,3t
Sarcoidosis, 114 Tuberculoid leprosy, 118
Scapular winging, 37--40, 41--45
Sciatic nerve, 78 U
Sciatic neuropathy Ulnar neuropathy
case study of, 78 description of, 1
with myokymic potentials, 82 distal,29
Sensory nerve action potentials at or near the elbow, 19-22, 23-26
abnormal median normal sural, 133-136, at wrist, 27-30
137-139 Ulnar-abductor digiti minimi studies
carpal tunnel syndrome effects on, I generalized neuropathies evaluated using, 95
in cervical radiculopathy, 70 neuromuscular junction disease evaluated using,
dorsal ulnar cutaneous, 70 175
focal neuropathy diagnosis by, I ulnar neuropathy at elbow evaluated using, 21,
generalized neuropathies evaluated using, 95 23-25
root avulsion findings, 51-54 Ulnar-second lumbrical interosseous test, 14
saphenous, 71 Upper limb nerve innervation, 3t
in spinal muscular atrophy, 198
superficial peroneal, I, 74 V
sural, 130 Voltage-gated calcium channels, 186
Sensory neuronopathies, 172-174
Serratus anterior, 3t W
Serum protein electrophoresis, 99 Waldenstrom's macroglobulinemia, 100, 10 It
Single fiber electromyography Wallerian degeneration, 66
neuromuscular junction disease evaluated using, Weakness
175-176 case studies of, 46--49, 68-70, 97-102, 153-155,
technical elements of, 176 156-157,160-163,199-205,212-214,
Sjogren's syndrome, 174 221-224,225-228,229-231,239-241
Slow channel syndromes, 191 focal demyelination of motor axons as cause of, 83
SMA. See Spinal muscular atrophy generalized, 126-130, 137-139, 168-171, 187-191,
SNAPs. See Sensory nerve action potentials 215-217

265
Weakness (Continued) Wrist drop, 34-36
in infant, 197-198 Writing, botulinum toxin therapy for improvements in,
left arm, 55-58, 64-67 251-259
left foot, 79-83
left hand, 59-63, 194-196 X
neuromuscular causes of, 168-171 X-linked bulbospinal neuronopathy, 192
proximal, 183-186 X-linked Charcot-Marie-Tooth, 147, 155, 162
right arm, 51-54 X-linked dominant inherited neuropathy, 160-163
right hand, 15-18
right leg, 84-86, 91-93 Z
trapezius, 39,45 Z-disk,214
Werdnig-Hoffmann disease, 198 Zinc finger-9, 234
Wrist, ulnar neuropathy at, 27-30

266
 (
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