MOTOR DISORDERS

Approach to Diagnosispproach to
Diagnosis
Normal motor function depends on the transmission of
signals from the brain to the brainstem or spinal cord by
upper motor neurons, and from there to skeletal muscle
by lower motor neurons (Figure 9-1). A lesion that
involves this pathway anywhere along its length may
impair motor function. Anatomic structures involved in
the regulation or execution of motor activity include the
pyramidal and extrapyramidal systems, cerebellum, and
lower motor neurons in the cranial nerve nuclei of the
brainstem and anterior horns of the spinal cord.
The pyramidal system (Figure 9-2) consists of upper
motor neuron fibers that descend from the cerebral cortex
through the internal capsule, traverse the medullary pyramid,
and then mostly decussate, to descend in the lateral
corticospinal tract on the opposite side, where they synapse
on interneurons and lower motor neurons in the spinal
cord.All other descending influences on lower motor neurons
belong to the extrapyramidal system and originate
primarily in the basal ganglia and cerebellum. Disorders of
the basal ganglia (see Chapter 11, Movement Disorders)
and cerebellum (see Chapter 8, Disorders of Equilibrium)
are considered separately.
The motor fibers in the cranial and peripheral nerves
arise from the lower motor neurons (Figure 9-3).
Dysfunction at any point in the peripheral nervous system
(anterior horn cell, nerve root, limb plexus, peripheral
nerve, or neuromuscular junction) can impair motor function,
as can disease of the muscles.

History
History
Patients with motor deficits generally complain of weakness,
heaviness, stiffness, clumsiness, impaired muscular
control, or difficulty in executing movements. The term
weakness is sometimes used in a nonspecific way to denote
fatigue or loss of energy, drive, or enthusiasm, and its
meaning must therefore always be clarified. The word is
properly used to mean loss of muscle power, and it is in
this sense that it is employed here.
History of Present Illness
``Mode of Onset
An abrupt onset suggests a vascular disturbance, such as a
stroke, or certain toxic or metabolic disturbances, whereas
subacute onset over days to weeks is commonly associated
with a neoplastic, infective, or inflammatory process
(Table 9-1). Weakness that evolves slowly over several
months or years often has a hereditary, degenerative, endocrinologic,
or neoplastic basis.
``Course
A progressive increase in the motor deficit from its onset
suggests continuing activity of the underlying process.
Episodic progression suggests a vascular or inflammatory
origin; a steadily progressive course is more suggestive of
neoplastic disorder or such degenerative conditions as
motor neuron disease. Rapid fluctuation of symptoms over
short periods (eg, over the course of the day) is characteristic
of myasthenia gravis.

GAMBAR
1. Anatomic basis of the upper motor neuron and lower motor neuron
concepts.

2. Upper motor neuron pathways. Tracts at bottom left are shown outside the
cord for clarity only. (Used with permission from McPhee SJ, Hammer GD:
Pathophysiology of Disease: An Introduction to Clinical Medicine. 6th ed.
New York, NY: McGraw-Hill; 2009.)

``Distribution of Symptoms
The distribution of weakness and the presence of associated
symptoms may indicate the approximate site of the
lesion. For example, weakness in the right arm and leg may
result from a lesion of the contralateral motor cortex or the
corticospinal pathway at any point above the fifth cervical
segment of the spinal cord. Associated right facial weakness
indicates that the lesion must be above the level of the
facial (VII) nerve nucleus in the brainstem, and an accompanying
aphasia (see Chapter 1, Neurologic History & Examination) or visual field defect (see Chapter 7,
Neuro-Opthalmic Disorders) localizes it to the cerebral hemisphere.

``Associated Symptoms
The presence and distribution of any sensory abnormalities
also help in localizing the lesion. Sensory abnormalities
lateralized to the same side as weakness suggest a hemispheric
lesion; a cortical lesion is implied by sensory neglect
or inattention, agraphesthesia (inability to identify by touch
a number written on the skin), astereognosis (inability to
identify by touch an object placed in the hand), abarognosis
(inability to judge the weight of an object placed in the
hand), or impaired two-point discrimination, when peripheral
sensory function is intact. Sensory loss below a particular
segmental level on the trunk suggests a spinal cord
lesion, whereas distal sensory changes in the limbs favor a
peripheral nerve lesion. Diseases of the anterior horn cells,
neuromuscular junctions, or muscles are not accompanied
by altered sensation.
The character of any associated symptoms may suggest
the nature of the lesion. Thus progressive leg weakness
caused by myelopathy is often preceded or accompanied by
pain in the back or legs when the myelopathy is due to a
compressive lesion—but not when it has a metabolic or
hereditary basis.
``Severity of Symptoms
The functional severity of a motor deficit is evaluated by
determining whether there has been any restriction of
daily activities, difficulty in performing previously easy
tasks, or reduction in exercise tolerance.
The nature of the functional disturbance depends on
the muscles involved. Weakness of proximal muscles in the
legs leads to difficulty in climbing or descending stairs or
in getting up from a squatting position, whereas weakness
in the arms leads to difficulty with such tasks as combing
the hair. Distal weakness in the arms may lead to clumsiness,
difficulty with such fine motor tasks as doing up buttons
or tying shoelaces, and eventually the inability to pick
up or grasp objects with the hands, so that even eating
becomes difficult or impossible.
Involvement of the muscles supplied by the cranial
nerves may lead to diplopia (oculomotor [III], trochlear
[IV], or abducens [VI] nerve); difficulty in chewing (trigeminal
[V] nerve) or sucking, blowing, or grimacing
(facial [VII] nerve); or difficulty in swallowing, with nasal
regurgitation and dysarthria (glossopharyngeal [IX], vagus
[X], and hypoglossal [XII] nerves).
Weakness of the respiratory muscles leads to tachypnea,
the use of accessory muscles of respiration, and
anxiety at a stage when arterial blood gases are usually still
normal. A vital capacity of less than 1 L in an adult generally
calls for ventilatory support, especially if weakness is
increasing.

GAMBAR
3. Anatomic components of the motor unit.

Table 9-1. Some Causes of Weakness of Acute or

Subacute Onset.
Supraspinal lesions
Stroke
Other structural lesions
Spinal cord lesions
Infective: human immunodeficiency virus (HIV) infection
Inflammatory: transverse myelitis, multiple sclerosis
Compressive: tumor, disk protrusion, abscess
Vascular: infarction, hematomyelia
Anterior horn cell disorders
Poliovirus, coxsackievirus, West Nile virus infection
Peripheral nerve disorders
Guillain-Barre syndrome
Diphtheria
Paralytic shellfish poisoning
Porphyria
Arsenic poisoning
Organophosphate toxicity
Neuromuscular junction disorders
Myasthenia gravis
Botulism
Aminoglycoside toxicity
Muscle disorders
Necrotizing myopathies
Acute hypo- or hyperkalemia
Periodic paralyses

Past Medical History

The importance of the past history depends on the
patient’s present complaint and the nature of any previous
illnesses. For example, in a patient with lung cancer, limb
weakness may be due to metastasis or to a remote (nonmetastatic)
complication of the cancer. Leg weakness in a
diabetic may reflect peripheral nerve, plexus, or multiple
root involvement, and hand weakness in a myxedematous
patient may be associated with carpal tunnel syndrome.
All drugs taken by the patient should be noted. Drugs
can cause peripheral neuropathy, impair neuromuscular
transmission, or lead to myopathy (Table 9-2).
Developm ental History
When symptoms develop before adult life, it is particularly
important to obtain a full developmental history, including
details of the delivery, the birth weight, the patient’s condition
in the neonatal period, and the dates at which motor
milestones were attained. Congenital or perinatal cerebral
disease accounts for most causes of infantile diplegia
(weakness of all four limbs, with the legs more severely
affected than the arms).
Fam ily History
Hereditary factors may be important, and the patient’s family
background therefore must be explored. Some types of
myopathy, motor neuron disease, and peripheral neuropathy
have a genetic basis, as do some spinocerebellar
degenerations, hereditary spastic paraparesis, and certain
other neurologic disorders. It is sometimes necessary to
examine other family members to determine whether the
patient’s disorder has a hereditary basis.

Table 9-2. Motor Disorders Associated With Drugs.

Drugs that cause motor (or predominantly motor)
peripheral
neuropathy1
Dapsone
Imipramine
Sulfonamides (some)
Drugs that can impair neuromuscular transmission
ACTH
Aminoglycoside antibiotics
-Blockers
Chloroquine
Colistin
Corticosteroids
Lithium
Magnesium-containing cathartics
Penicillamine
Phenothiazines
Phenytoin
Polymyxin
Procainamide
Quinidine, quinine
Tetracycline
Drugs associated with myopathy
-Blockers
Chloroquine
Clofibrate
Corticosteroids
Drugs causing hypokalemia
Emetine
-Aminocaproic acid
HMG-CoA reductase
inhibitors
Penicillamine
Zidovudine
1 A number of drugs cause mixed sensory and motor neuropathies
(see Table 10-2).

Neurologic Examination
Neurologic Examination
Motor System
A systematic approach to examining the motor system helps
to prevent important abnormalities from being overlooked.
A sequential routine for the examination is important.
``Muscle Appearance
1. Wasting, or muscle atrophy, suggests that weakness is
due to a lesion of the lower motor neurons or the
muscle itself. The distribution of wasting may help to
localize the underlying disorder. Upper motor neuron
disorders are not usually accompanied by muscle wasting,
though atrophy may occasionally occur with
prolonged
disuse.
2. Pseudohypertrophy of muscles occurs in certain forms
of myopathy, but the apparently enlarged muscles are
actually weak and flabby.
3. Fasciculations—visible irregular flickerings over the
surface of the affected muscle caused by spontaneous
contractions of individual motor units—suggest
that weakness is due to a lower motor neuron lesion.
Fasciculations are most common in anterior horn cell
disorders, but may also occur in normal individuals.
4. Flexor or extensor spasms of the limbs are sometimes
seen in upper motor neuron disorders as a result of
impaired supraspinal control of reflex activity.
``Muscle Tone
For clinical purposes, tone can be defined as the resistance
of muscle to passive movement of a joint. Tone depends
on the degree of muscle contraction and on the mechanical
properties of muscle and connective tissue. The degree of
muscle contraction depends, in turn, on the activity of
anterior horn cells, which is governed by spinal and supraspinal
mechanisms.
Tone is assessed by observing the position of the
extremities at rest, by palpating the muscle belly, and particularly
by determining the resistance to passive stretch
and movement. To assess resistance to passive movement,
the patient relaxes while each limb is examined in turn by
passively taking the major joints through their full range
of movement at different speeds and estimating whether
the force required is more or less than normal.
Postural abnormalities may result from the increased
activity of certain muscle groups caused by disturbances
of reflex function, as exemplified by the typical hemiplegic
posture—flexion of the upper limb and extension of
the ipsilateral lower limb—of many patients who have
had a stroke.
Hypertonia—Two types of increased tone can be
distinguished.
a. Spasticity—consists of an increase in tone that affects
different muscle groups to different extents. In the
arms, tone is increased more in the flexor and adductor
muscles than the extensors and abductors; in the
legs, it is greater in the extensor muscles than flexors.
The resistance of an affected muscle is not the same
throughout the range of movement, but tends to be
most marked when passive movement is initiated and
then diminishes as the movement continues (the
clasp-knife phenomenon). The increase in tone is
velocity dependent, so that passive movement at high
but not low velocities meets increased resistance.
Spasticity is caused by an upper motor neuron lesion,
such as a stroke that involves the supplementary
motor cortex or corticospinal tract. It may not become
apparent for several days after onset of an acute lesion.
b. Rigidity—consists of increased resistance to passive
movement that is independent of the direction of the
movement; that is, it affects agonist and antagonist
muscle groups equally (lead-pipe rigidity). The
term cogwheel rigidity is used when there are
superimposed ratchet-like interruptions in the passive
movement, probably related to underlying
tremor. In general, rigidity indicates extrapyramidal
dysfunction and is due to a lesion of the basal ganglia
(eg, Parkinson disease).
2. Hypotonia (flaccidity)—This is characterized by
excessive floppiness—a reduced resistance to passive
movement—so that the distal portion of the limb is
easily waved to and fro when the extremity is passively
shaken. In hypotonic limbs it is often possible to hyperextend
the joints, and the muscle belly may look flattened
and feel less firm than usual. Although hypotonia
usually relates to pathologic involvement of the lower
motor neuron supply to the affected muscles, it can
also occur with primary muscle disorders, disruption
of the sensory (afferent) limb of the reflex arc, cerebellar
disease, and certain extrapyramidal disorders such
as Huntington disease, as well as in the acute stage of a
pyramidal lesion.
3. Paratonia—Some patients seem unable to relax and
will move the limb being examined as the physician
moves it, despite instructions to the contrary. In more
advanced cases, there seems to be rigidity when
the examiner moves the limb rapidly but normal tone
when the limb is moved slowly. This phenomenon—
paratonia—is
particularly apt to occur in patients with
frontal lobe or diffuse cerebral disease.
``Muscle Power
To test muscle power, the patient is asked to resist pressure
exerted by the examiner. Based on the history and other
findings, muscles particularly likely to be affected are
selected for initial evaluation, and other muscles are subsequently
examined to determine the distribution of
weakness more fully and to shorten the list of diagnostic
possibilities. For instance, if an upper motor neuron
(pyramidal) lesion is suspected, the extensors and abductors
of the upper extremity and flexors of the lower
extremity are tested in the most detail, because they will
be the most affected. Strength on the two sides is compared
so that minor degrees of weakness can be
recognized.
1. Distinction between upper and lower motor neuron
lesions—The distribution of weakness helps to distinguish
between dysfunction of the upper or lower
motor neurons. Upper motor neuron lesions (eg,
stroke) lead to weakness that characteristically involves
the extensors and abductors more than the flexors and
adductors of the arms—and the flexors more than the
extensors of the legs. Lower motor neuron lesions produce
weakness of the muscles supplied by the affected
neurons; the particular distribution of the weakness
may point to lower motor neuron disturbance involving
the spinal cord, nerve roots, plexus, or peripheral
nerves.
2. Distinction between myopathic and neuropathic
disorders—Weakness may also result from a primary
muscle disorder (myopathy) or from a disorder of lower
motor neurons. In patients with a motor deficit in all
limbs that is not due to an upper motor neuron lesion,
proximal distribution of weakness suggests a myopathic
disorder, whereas predominantly distal involvement
suggests a neuropathic disturbance.
3. Neuromuscular junction disorders—Marked variability
in the severity and distribution of weakness over
short periods of time (eg, over the course of a day) suggests
myasthenia gravis, a disorder of neuromuscular
transmission.
4. Psychogenic disorders—Apparent weakness that is not
organic in nature also shows a characteristic variability;
it is often more severe on formal testing than is consistent
with the patient’s daily activities. Moreover, palpation
of antagonist muscles commonly reveals that they
contract each time the patient is asked to activate the
agonist.
For practical and comparative purposes, power is best
graded in the manner shown in Table 9-3.
The term monoplegia denotes paralysis or severe
weakness of the muscles in one limb, and monoparesis
denotes less severe weakness in one limb, although the two
words are often used interchangeably. Hemiplegia or
hemiparesis is weakness in both limbs (and sometimes the
face) on one side of the body; paraplegia or paraparesis is
weakness of both legs; and quadriplegia or quadriparesis
(also tetraplegia, tetraparesis) is weakness of all four
limbs.

Table 9-3. Grading of Muscle Power According to the

System Suggested by the Medical Research Council.
Grade Muscle Power
5 Normal power
4 Active movement against resistance and gravity
3 Active movement against gravity but not resistance
2 Active movement possible only with gravity eliminated
1 Flicker or trace of contraction
0 No contraction
Used with permission from Aids to the Investigation of Perpheral
Nerve Injuries. London, UK: H.M. Stationary Office; 1943.