Neurological rarities

Chronic immune sensory

polyradiculopathy (CISP)
Stephanie Ong, Adam Cassidy

► Additional supplemental ABSTRACT touch extending to the mid shins along with
material is published online
Chronic immune sensory polyradiculopathy reduced joint position sense at the meta-
only. To view, please visit the
journal online (http://​dx.​doi.​ (CISP) is a rare variant of chronic inflammatory tarsophalangeal joints. He could not walk
org/​10.​1136/​practneurol-​ demyelinating polyradiculoneuropathy (CIDP). tandem and Romberg’s sign was positive.
2021-​003070). We describe a man with isolated sensory ataxia Routine blood tests were normal, including
Neurology, Sunderland Royal
whose initial investigations included normal nerve serum vitamin B12, folate, serum electro-
Hospital, Sunderland, UK conduction studies and normal non-­enhanced MR phoresis, glycated haemoglobin, thyroid
imaging of whole spine, but whose subsequent function tests and erythrocyte sedimenta-
Correspondence to
Dr Stephanie Ong, Neurology,
investigations showed delayed somatosensory tion rate. Cerebrospinal fluid (CSF) was
Sunderland Royal Hospital, evoked potential (SSEP) responses of the lower normal except for slightly elevated protein
Sunderland SR4 7TP, Tyne and limbs, elevated cerebrospinal fluid (CSF) protein at 0.58 g/L. Peripheral sensory and motor
Wear, UK; ​stephanie.​ong1@​ and lumbosacral nerve roots enhancement on MR nerve conduction studies (see online supple-
nhs.​net
imaging. We diagnosed CISP and he improved mental tables 1 and 2) and non-­enhanced
Accepted 7 September 2021 following intravenous immunoglobulin. Proposed MR imaging of whole spine were normal.
Published Online First diagnostic criteria for CISP are sensory symptoms Upper limb somatosensory evoked potential
5 November 2021
with a polyneuropathic distribution without (SSEP) responses were normal bilaterally
weakness, and normal motor and sensory nerve though lower limb SSEP responses were
conduction and electromyography (EMG) studies, moderately delayed (see online supple-
plus at least two of: abnormal SSEPs not due to mental table 31 and figure 1). An MR scan of
central nervous system (CNS) involvement, MRI lumbar spine with gadolinium enhancement
showing gadolinium enhancement or hypertrophy showed mild thickening and enhancement
of the nerve roots, cauda equina or plexuses, of the lumbosacral nerve roots (figure 2).
and elevated CSF protein with normal cell count. We diagnosed chronic immune sensory
Intravenous immunoglobulin is an effective polyradiculopathy (CISP) and treated
treatment. him with intravenous immunoglobulin.
Following the first course his bilateral lower
limb numbness almost resolved and his
balance improved significantly. Following the
CASE REPORT second course all symptoms had completely
A 68-­year-­old man gave a 2-­year history of resolved except for a small residual area of
reduced sensation of the lower limbs and reduced sensation of the left lateral foot. He
unsteadiness. He had developed abrupt remains well with 7-­weekly cycles of intra-
onset of reduced sensation in the left foot venous immunoglobulin treatment.
followed a few weeks later by abrupt onset
of reduced sensation of the right foot. His
symptoms had progressed in a stepwise DISCUSSION
manner over the following 2 years, even- CISP was first proposed as a distinct diag-
tually giving reduced sensation extending nostic entity by Sinnreich et al2 in a case
to the knee on the left and to the ankle on series of 15 patients who had presented
the right. During this time, he had devel- with sensory disturbance of the limbs and
oped unsteadiness on walking and regular gait ataxia. All patients had preserved motor
falls. He had a history of benign prostatic function with large fibre sensory abnormal-
hyperplasia and eczema but no history of ities of altered joint position sense or vibra-
© Author(s) (or their
employer(s)) 2022. No
excess alcohol intake. tion sense along with generalised areflexia or
commercial re-­use. See rights On examination, tone and power were hyporeflexia. Peripheral sensory and motor
and permissions. Published normal in all four limbs. Reflexes were nerve conduction studies were normal in
by BMJ.
preserved in the upper limbs and absent all patients, though SSEPs were abnormal
To cite: Ong S, Cassidy A. in the lower limbs. There was bilaterally in all twelve patients that were tested,
Pract Neurol 2022;22:57–59. reduced sensation to pinprick and light meaning that any pathology of the sensory

Ong S, Cassidy A. Pract Neurol 2022;22:57–59. doi:10.1136/practneurol-2021-003070 57

Neurological rarities

studies and abnormal SSEPs consistent with CISP. Subse-

quently, there have been four individual cases reported4–7
and a large series of CIDP patients included only two with
CISP.8 In this study, the authors applied diagnostic criteria
to 460 patients with CIDP to determine the frequency
and evolution of CIDP variants. They proposed clinical
diagnostic criteria for each atypical CIDP form. The
proposed criteria for CISP were sensory symptoms with
a polyneuropathic distribution without weakness and
normal peripheral neurophysiological studies, plus at least
two of: abnormal SSEPs not due to central nervous system
(CNS) involvement, MRI showing gadolinium enhance-
ment or hypertrophy of the nerve roots, cauda equina
or plexuses, and elevated CSF protein with a normal
cell count. Although the patient described here had only
a mildly elevated CSF protein, he fulfilled the proposed
diagnostic criteria due to his clinical presentation, normal
nerve conduction studies and abnormal SSEP and MRI.
Figure 1 SSEP waveforms of the lower limbs showing normal
morphology. SSEP, somatosensory evoked potential.
Proposed diagnostic criteria for chronic immune
sensory polyradiculopathy
► Sensory symptoms with a polyneuropathic distribution
nervous system must have been proximal to the dorsal without weakness.
root ganglia. Thirteen of the 14 patients who underwent
CSF analysis had an elevated CSF protein. On MRI of And
the lumbar spine five patients had thickened lumbar ► Normal motor and sensory nerve conduction and EMG
nerve roots. Three had lumbar sensory rootlet biopsies studies.
that showed evidence of demyelination and endoneurial Plus at least two of
macrophage infiltration. All patients treated with immu- ► Abnormal somatosensory evoked potential not due to
nomodulatory therapy experienced a rapid improvement CNS involvement.
of their symptoms. These findings suggested a variant of ► MR imaging showing gadolinium enhancement or
chronic inflammatory demyelinating polyradiculoneu- hypertrophy of the nerve roots, cauda equina or
ropathy (CIDP) that affected the proximal sensory nerve plexuses.
roots but spared distal nerve segments. ► Elevated CSF protein level with normal cell count.
Only a few cases of CISP have been described since
then. Ayrignac et al3 reported a series of 22 patients with Following the description of CISP a further CIDP
sensory CIDP, 8 of whom had normal nerve conduction variant known as chronic immune sensorimotor

Figure 2 MR scan of the lumbosacral spine showing postcontrast enhancement and mild thickening of the lumbosacral nerve
roots.

58 Ong S, Cassidy A. Pract Neurol 2022;22:57–59. doi:10.1136/practneurol-2021-003070

 Neurological rarities

polyradiculopathy has been postulated.9 This pres- adjunct when symptoms returned within 2 weeks of the
ents with both sensory symptoms and motor weakness first course of intravenous immunoglobulin.5 However,
and is presumed to be caused by demyelination of both for three patients, intravenous methylprednisolone was
the motor and sensory nerve roots. As with CISP, these either ineffective or worsened symptoms. These three
patients have normal peripheral sensory nerve conduc- patients were then treated with intravenous immunoglob-
tion studies but also evidence of denervation on electro- ulin with a good response.4 6 7
myography (EMG) with normal motor nerve conduction The patient described here responded well to intrave-
studies. It is debatable, however, whether the motor nerve nous immunoglobulin monotherapy. We monitored his
conduction studies in these cases were sufficiently exten- symptoms and findings on clinical examination over a
sive to exclude CIDP fully.10 Almost all of these patients 7-­week cycle. However, best practice in managing chronic
had elevated CSF protein as well as lumbar nerve root conditions with high-­ cost therapies is to use objective
thickening on MR imaging and responded to immuno- rating scales such as the Berg Balance Scale to document
modulatory treatment. treatment response and to optimise treatment intervals.
The European Federation of Neurological Societies/
Contributors SO is the first author and AC is the second
Peripheral Nerve Society Guideline on the management author.
of CIDP advises that patients with moderate or severe
Funding The authors have not declared a specific grant for this
disability should be offered treatment with corticosteroids research from any funding agency in the public, commercial or
or intravenous immunoglobulin. As with typical CIDP, not-­for-­profit sectors.
the mainstay of treatment for CISP is immunomodula- Competing interests None declared.
tory therapy. On review of the reported cases of CISP, Patient consent for publication Consent obtained directly from
first line treatment was with intravenous immunoglob- patient(s).
ulin and/or corticosteroids. Of the cases where response Provenance and peer review Not commissioned. Externally
to specific treatments were described, six out of the seven peer reviewed by Yusuf Rajabally, Birmingham, UK.
patients improved following intravenous immunoglob-
ulin.2 3 There was variable success with corticosteroid REFERENCES
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► Patients with unexplained sensory ataxia may require polyradiculopathy (CISP): first juvenile case description.
further investigation with SSEPs, CSF analysis and Neurol Sci 2021;42:333–6.
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Ong S, Cassidy A. Pract Neurol 2022;22:57–59. doi:10.1136/practneurol-2021-003070 59

© 2022 Author(s) (or their employer(s)) 2022. No commercial re-use. See
rights and permissions. Published by BMJ.