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Chronic Inflammatory
Demyelinating Polyneuropathy
Updates in diagnosis and management.
By Christopher J. Lamb, MD and P. James B. Dyck, MD

Chronic inflammatory demyelin- radiculoneuropathy), although distal symptoms may be more

ating polyradiculoneuropathy severe because all distal nerve signals must also pass through
(CIDP) is an immune-mediated the proximal segments. Sparing of pain and temperature sensa-
neuropathy syndrome compris- tion (types III/Ad and IV/C fibers) and autonomic fibers is clas-
ing gradually progressive proxi- sic, because these are thinly myelinated or unmyelinated.5
mal and distal weakness, large- Chronic, progressive or relapsing-remitting, proximal and
fiber sensory disturbances, areflexia, and features of acquired distal large-fiber symptoms with small-fiber sparing should
demyelination (eg, slowed conduction velocities, conduction raise CIDP as a diagnostic consideration after an initial clinical
block, temporal dispersion, and onion-bulb formation).1,2 encounter. These features can be gleaned mostly from a clinical
CIDP likely represents a heterogeneous group with similar history that may include difficulty walking or climbing stairs,
clinical features; however, the term remains useful because of imbalance (particularly with eyes closed in the shower or in
similarities in confirmatory diagnostic features, shared inflam- dark environments), and “dead” or prickling numbness of the
matory demyelination pathophysiology, and responsiveness limbs. Pain, especially when described as “burning,” is consid-
to immune therapy. The lack of a single specific biomarker, ered atypical and raises caution against a diagnosis of typical
presence of several mimickers and chameleons, and pressure CIDP. In practice, neuropathic pain is present in a substantial
on healthcare providers to find treatable causes of disability minority of persons with typical CIDP (20%-41% in a single-
increase the difficulty of diagnosing and managing CIDP and center study6) but occurs later in the disease course, usually
its variants. These factors, among others, have led to the par- long after large-fiber impairment. CIDP should not be the first
adox of simultaneous ‘overdiagnosis’3 and ‘underdiagnosis’4 diagnosis to consider if there are only distal, painful, sensory-
of CIDP. Success in managing CIDP may depend on individual predominant symptoms without weakness, areflexia, or large-
patient factors and type of CIDP and often requires a trial of fiber dysfunction. Generally, systemic symptoms (eg, rash,
immunotherapy using objective treatment endpoints. The weight loss, and cardiorespiratory or gastrointestinal symp-
purpose of this review is to enhance and update the reader’s toms) should also dissuade a clinician from CIDP diagnosis.
ability to diagnose and manage CIDP and its variants with There are no uniquely specific neurologic physical examina-
recent and practical evidence-based principles. tion signs or laboratory findings in CIDP, although hyporeflexia
or areflexia should be confirmed. Palpable, hypertrophic nerves
Diagnosis rarely occur, but may also be present in hereditary demyelin-
Typical Presentation, History, and Neurologic Examination ating neuropathies. The examination and laboratory studies
CIDP should be suspected when a person has features of dis- should be used to investigate for systemic signs and symptoms,
rupted myelinated nerve fiber function. In typical CIDP, fibers mostly to exclude mimics. Assessment of monoclonal proteins,
with the most myelin are preferentially affected earliest and especially lambda, may be useful to identify polyneuropathy,
most severely. Dysfunction of these fibers evolves over at least organomegaly, endocrinopathy, M-protein, and skin changes
8 weeks, by definition, either progressively or in a relapsing- (POEMS) syndrome or IgM- monoclonal gammopathy of
remitting fashion. Because motor (type 1a/Ad), Golgi tendon undetermined significance (MGUS)-associated distal acquired
organ (Ib/Ad), and large, myelinated sensory fibers (type II/ Ad) demyelinating symmetric and sensory (DADS) neuropathy.
have the most myelin, people with classic CIDP present with Serum testing for antibodies against nodal or paranodal epit-
symmetric weakness, areflexia, and loss of vibration, proprio- opes should be considered in some cases.
ception, touch, and muscle spindle sensations. Fiber caliber,
and therefore, amount of myelin, is greater in proximal seg- CIDP Variants
ments, resulting in both proximal and distal weakness (poly- CIDP variants share a common pathophysiology of inflam-

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matory demyelination with repeated demyelination and approached with extreme caution. Slowed conduction velocity
remyelination resulting in stacks of Schwann cell processes and prolonged distal latency occur with axonal destruction in
(ie, onion-bulb formation) and a responsiveness to immune any neuropathy because of loss of the fastest-firing large nerve
therapy with typical CIDP. Otherwise, variants defy the typical fibers and secondary segmental demyelination.17 Lastly, the
clinical syndrome of symmetrical proximal and distal weak- CIDP variants may be more challenging to correctly diagnose
ness (polyradiculoneuropathy), areflexia, and sensory loss in because these do not conform to the clinical or EDX patterns
some particular respect. Although variants seem to be more of typical CIDP, relying on smaller number or limited popula-
rare than typical CIDP, 84 of 460 individuals with confirmed tions of involved nerves. Other supportive tests (eg, cerebrospi-
CIDP in a recent series initially had atypical presentations, with nal fluid [CSF] analysis, nerve imaging, somatosensory evoked
substantial proportions later developing typical CIDP.7 potentials [SEPs], response to treatment, or nerve biopsy) can
The number of CIDP variants is daunting and continues increase confidence in diagnosis of CIDP variants.
expanding (Table e1). CIDP variants should enter the neu-
rologist’s mind when 1) signs and symptoms are specifically Misdiagnosis of CIDP
localized to a select population of large, myelinated nerve Avoiding Overdiagnosis
fibers or roots (usually motor or sensory proprioceptive The most likely cause of CIDP overdiagnosis is overinterpre-
fibers); 2) there is relative sparing of thinly myelinated or tation of EDX findings. A recent study at an academic referral
unmyelinated nerves; 3) upper motor neuron features are center estimated that only 0.34% of patients who met EDX
lacking; and 4) other common causes of the clinical phe- criteria for CIDP presented with a distal symmetric neuropa-
notype have been excluded. Although clinical features vary thy phenotype.18 Despite this, a substantial proportion of
considerably, the unifying feature of these variants is inflam- those referred to an academic center for refractory CIDP had a
matory demyelination. Acute CIDP has ongoing active dis- distal symmetric polyneuropathy phenotype and EDX findings
ease that requires ongoing immunotherapy.8 Multifocal CIDP attributable to other common etiologies (eg, nerve compres-
has typical demyelinating features confined to localized body sion) rather than demyelination.3 For others, features of axon
segments, and is often upper limb predominant.9 Sensory loss (eg, conduction slowing) had been overemphasized.3 The
CIDP has demyelinating features on both motor and sensory practical point is to ensure methods of nerve conduction
electrodiagnostic (EDX) testing,10 whereas motor CIDP has studies (NCS) are performed properly at the required limb
no measurable electroclinical sensory involvement.11 Chronic temperature, and rely only on unequivocal evidence of demy-
immune sensory polyradiculopathy (CISP) has normal nerve elination to confirm CIDP. When demyelinating features are
conduction, and CISP-plus has neurophysiologic findings called partial, borderline, possible, or otherwise do not com-
discordant with observed clinical features; in both, sensory pletely fulfill criteria, other diagnoses should be considered.
nerve roots are predominantly affected.12,13 Chronic immune False-positive EDX criteria for CIDP may truly occur when
sensory and motor polyradiculopathy (CISMP) predomi- unequivocal EDX demyelination is present but caused by
nantly involves nerve roots and spares distal nerves.14 another demyelinating disorder. This may occur in condi-
tions in which axon degeneration and demyelination coexist.
Practical Review of EDX Criteria Electroclinical mimics of CIDP that may fulfill EDX criteria for
The European Academy of Neurology (EAN)/Peripheral CIDP include POEMS syndrome, IgM-monoclonal gammopa-
Nerve Society (PNS) 2010 clinical diagnostic and EDX guide- thy-associated neuropathy with or without myelin-associated
lines2 are the most widely applied for CIDP and CIDP variant glycoprotein (MAG) antibodies, hereditary or acquired amyloi-
diagnosis (Table e1). These criteria are sensitive (73%-91%) and dosis (see Neuromuscular Amyloidosis in this issue), multifocal
specific (66%-88%) in comparison to other available criteria,15 motor neuropathy with conduction block (MMN-CB), neuro-
and were most recently revised in June 2021.16 This second revi- lymphomatosis, severe diabetic polyneuropathies, and heredi-
sion emphasizes diagnosis of variants (no longer calling them tary demyelinating neuropathies (eg, Charcot-Marie-Tooth
atypical) and narrows diagnostic categories to CIDP or possible type 1). In our clinics, POEMS syndrome is often the most likely
CIDP (probable CIDP was removed). As with all diagnostic cri- mimic in incorrectly diagnosed CIDP that has not responded
teria, there are limitations that can lead to underdiagnosis (false to immune therapies, because both CIDP and POEMS present
negatives) or overdiagnosis (false positives). To avoid either, with progressive demyelinating neuropathies.19 The presence of
the first and most important priority is to apply these criteria pain, thrombocytosis, or a monoclonal protein should increase
only in the appropriate clinical scenario. Secondly, application suspicion of and initiate a search for an osteosclerotic myeloma.
of these EDX criteria in people with mixed processes in which
the severity of axonal features exceeds demyelinating features Avoiding Underdiagnosis
(ie, low compound motor action potential [CMAP] and sen- Overdiagnosis of CIDP has been emphasized recently
sory nerve action potential [SNAP] amplitudes) should be because it leads to misuse of immune therapies with high costs

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and risks. Underdiagnosis also occurs, and more than two- but also occur in inherited neuropathy, radiculoplexus neurop-
thirds of consecutive patients referred to a tertiary specialist athies, POEMS syndrome, sarcoidosis, and other conditions.
clinic for a nonCIDP diagnosis met clinical and EDX criteria A new wave of discovery has begun after the initial recogni-
for CIDP.4 The most common diagnostic chameleon in this tion and continued investigation of IgG4 antibodies directed
group was Guillain-Barré syndrome (GBS), an understandable toward epitopes in neurofascin 155 (NF-155), contactin-1,
finding considering the similarities between GBS and CIDP, and contactin-associated protein-1 (CASPR1), which all local-
and potential need for follow-up with a neuromuscular disease ize adjacent to or at the nodes of Ranvier. Testing for these
specialist after hospital discharge. Underdiagnoses have also antibodies in the appropriate clinical setting (Table e1) has
been attributed to 1) atypical CIDP variants (eg, multifocal become increasingly useful to inform prognosis and guide
CIDP, diabetic polyneuropathy or radiculoplexus neuropathy) therapeutic choices. Involvement of these antibodies may be
in which demyelinating features were missed or called axonal; recognized by the presence of prominent sensory features
2) satisfaction-of-search errors in which hereditary neuropathy with ataxia, upper limb onset or predominance, very high CSF
and CIDP co-occurred; and 3) other circumstances in which protein levels, and the presence of tremor in the case of anti-
proximal weakness was overlooked and laboratory findings (eg, NF-155. Early evidence suggests conventional therapies may be
Lyme antibodies) were overemphasized.4 These findings high- less effective in these conditions, whereas rituximab or other
light the need to closely link the clinical scenario with the EDX immunoglobulin-depleting therapy may be more effective.21,22
findings, which is helped by obtaining the history and exami-
nation at the same time as EMG and NCS. Reliable communi- Management
cation of neurophysiologic test results is also essential. Other Proven treatments and standards of care for typical CIDP
atypical CIDP variants such as CISP, CISP-plus, and CISMP do and most variants are intravenous immune globulin (IVIG),
not have EDX findings of demyelination on routine testing and corticosteroids, and plasmapheresis. These have remained the
may be missed. Clinical suspicion for these diagnoses and spe- best available options for over 20 years. Novel immune thera-
cial evaluations of SEPs, CSF analysis, MRI of proximal nerves, pies and modifications to existing first-line agents are being
or nerve pathology are needed to identify these variants.12-14 If examined, however. There is also an established role for phys-
uncertainty exists about CIDP variants or nonreponse to treat- iotherapy and occupational therapy in treatment of CIDP.
ment, neuromuscular specialist referral may be required.
Therapy Targets
Future Directions for Diagnosis Goals of immune therapy should be established before start-
As previously mentioned, the coexistence of severe axon loss ing medication. We counsel patients that stabilization is the first
and demyelinating findings may cause a floor effect in which goal, and that improvement may follow stabilization. Setting
most motor and sensory responses are absent. When this floor realistic expectations is especially important when longstand-
effect is reached, a demyelinating neuropathy cannot be iden- ing, untreated disease results in axon loss that may take months
tified and diagnosis is challenging. An R1 latency of the blink to years to recover or may never recover at all. We also inform
response longer than 13 ms has been shown to be a reliable patients that although several treatment regimens have been
sign of demyelination even in the setting of low or unobtain- shown effective for people with CIDP as a group, each individual
able ulnar CMAP amplitudes due to secondary axon loss. R1 will require an individualized dose and duration of treatment
latency improvement after treatment correlated to clinical to induce remission. An individualized approach may initially
improvement measured by neuropathy impairment score seem taxing on finances and time, but likely saves funds and
(NIS).20 This finding is not specific to CIDP but may assist with improves outcomes compared to uniform application of stan-
evaluation for the presence and response to treatment of a dard dosing protocols.23 Several reliable outcome measures in
demyelinating process with severe secondary axonal loss. CIDP have been demonstrated and used in research studies.24,25
The role of CSF protein levels in CIDP diagnosis has come In routine clinical practice, treating providers should find meth-
into question since the 2010 criteria were published, after ods that can be easily and systematically used in their office
overreliance on elevated CSF protein was identified as a major during a standard follow-up visit, such as a combination of
contributor to CIDP overdiagnosis.3 A systematic review of patient-questionnaire inventories (eg, Dyck score, inflammatory
over 20 high-quality studies published between 1960 and Rasch-built overall disability Scale [I-RODS], overall neuropathy
2017 in which CSF protein was evaluated in people with and limitations scale [ONLS]), manual motor testing or dynamom-
without neurologic illness showed an inelastic upper limit of etry, combined measures (neuropathy impairment score), or
45 mg/dL should be replaced with an age-adjusted upper limit periodic repeat EDX testing. The minimum clinically important
of 60 mg/ dL at age 50 or more. Physicians also need to under- difference (MCID) for each measure may be set as a target for
stand that elevated CSF protein levels indicate involvement of therapy, and if improvement is not achieved or measures show
proximal nerve segments or roots that are not specific to CIDP worsening, this can serve as a directive for changing therapy.

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TABLE. EAN/PNS ELECTRODIAGNOSTIC GUIDELINE ON CIDP–2ND REVISION 2021 CHECKLISTa

Criterion Definition Nerves Total
Median Ulnar Fibular Tibial
A Distal latency Prolonged ≥50% (1.5x) of UL Exclude CTS
Conduction Exclude UNE
B Reduced ≥30% (0.7x) of lower limit
velocity andMGA
Normal CMAP amplitude Prolonged ≥30% (1.3x) of UL
F-wave
C Reduced (≥20%) CMAP
latency Prolonged ≥50% (1.5x) of UL
amplitude
F-Wave
D Absence with CMAP amplitude ≥20% of lower limit
absence
Partial motor
Proximal CMAP amplitude ≥50% Reduced compared to
E conduction b
distal CMAP amplitude
block
Abnormal
CMAP duration increases >30% between proximal and
F temporal
distal sites
dispersion
Distal CMAP Prolonged interval between initial onset and baseline >6.5 ms >6.6 ms >7.5 ms >8.7 ms
G
duration return of last negative peak
Abbreviations: CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; CMAP, compound muscle action potential; CTS, carpal
tunnel syndrome; EAN, European Academy of Neurology; PNS, Peripheral Nerve Society; UNE, ulnar neuropathy at elbow; MGA, Martin-
Gruber (median-ulnar) anastomosis; UL, upper limit.
aSecond revision provided via personal communication from Dr. Peter Van Den Bergh. These criteria must only be applied for patients

who meet clinical criteria for typical CIDP: symmetric proximal and distal weakness in 4 limbs and sensory disturbance in 2 or more limbs.
Temperature must be maintained at 33° C at the palm and 30° C at the lateral malleolus. Tally the box for each confirmed criterion in the
corresponding nerve, then sum them in the total column. Typical CIDP is diagnosed if there are ≥2 points in rows A, B, C, E, or F or ≥1 point
in row D or G plus 1 point in any other row. Possible CIDP is diagnosed if clinical criteria for CIDP are met, there are 2 supportive criteria
from cerebrospinal fluid analysis, nerve imaging with ultrasound or MRI, response to treatment, or a positive nerve biopsy, and ≥1 point in
row E and ≥2 points in rows A and B or reduced sensory nerve action potential (SNAP). b Tibial conduction block should not routinely be
relied upon for diagnosis because of the presence of 50% depression in normal individuals.
We recommend seeing patients after a prespecified treatment trial of IVIG vs. plasmapheresis.27 Consequently, when begin-
period—often 3 months. During this follow-up visit, we evalu- ning IVIG treatment of newly diagnosed persons with CIDP
ate with the prespecified measure first and then take the clinical or when treating refractory CIDP, we may prescribe doses of
history to remain as objective as possible. 0.4 g/kg once or, in severe cases, twice weekly, for short dura-
tions (eg, 4-8 weeks), and then weekly thereafter. This is done
Standard Immune Therapies alone or sometimes in combination with intravenous methyl-
IVIG. The use of IVIG as a first-line therapy for CIDP is prednisolone (IVMP) 1,000 mg. Reassessment for improve-
supported by high-quality data from multiple randomized ment should still be done at approximately 3-month intervals
controlled trials. It is likely as effective as plasmapheresis, because earlier reassessment may miss subtle improvements.
but better tolerated by many.26,27 A clinical trial established If there is improvement with these intensive dosing regimens,
that IVIG dosing of 1 g/kg over 1 to 2 days every 3 weeks for we maintain or scale down the IVIG dose slowly. If no treat-
up to 24 weeks is more effective than placebo for improv- ment response is noted, alternative diagnoses are considered.24
ing disability and grip strength and increasing time between Use of combined IVIG and IVMP for treatment induction is
relapses.28 This trial provides a reasonable regimen to use currently being investigated in a large randomized controlled
when starting immune therapy. trial,29 and was well-tolerated in a recent pilot study.30
Some persons with CIDP may not respond to regimens of Adjustment of IVIG can be difficult and sometimes patients
3- to 4-week dosing intervals or may have improvement fol- cannot be easily weaned from a weekly to biweekly schedule
lowed by wearing off of benefits during these intervals. Often because of worsening symptoms and neurologic deficits. In
these individuals improve with lower, more frequent doses. such cases, a 1.5-week protocol can be achieved with an alter-
This more frequent dosing approach was used in a controlled nating dosing interval to maintain infusions consistently on the

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same day of the week (eg, scheduling infusions every 10th day of rituximab initiation, and maximum improvement occurred
then every 11th day, on an alternating basis of Monday the first at 2 to 18 months.22 Only 3 of the 11 individuals were tested
week and Thursdays the following week or something similar). for NF-155 antibodies, which were found in 1 of those 3 peo-
Subcutaneous immune globulin (SCIG) is a relatively new ple. In a retrospective study of 200 people who had chronic
means of administering immunoglobulin therapy that was immune neuropathy, 48 had typical or atypical CIDP, or para-
effective and tolerable in a large randomized controlled trial proteinemic demyelinating neuropathy with or without nodal/
with a long-term extension beyond 24 weeks.31 There are paranodal or MAG antibodies. These individual’s conditions
2 published doses (0.4 g/kg and 0.2 g/kg), which were appar- had been refractory to conventional immune therapies, but
ently equally effective in the initial trial. In the extension study, 85.4% of them who received rituximab, cyclophosphamide, or
however, approximately half of those who had dose reduction bortezomib as monotherapy or in combination responded to
from 0.4 g/kg to 0.2 g/kg experienced relapse. treatment. The INCAT-overall disability sum scores (ODSS)
We determine immunoglobulin therapy duration by clini- after these therapies were higher than in people with similar
cal response, attempting to slowly reduce it as individuals conditions who received different agents used as second-,
reach a plateau or have sequential improvements over a 6- to third-, or fourth-line therapies.34 These studies, and others, sug-
8-month period. Tapering is done by decreasing frequency of gest the need for new clinical trials to evaluate these immune
dosing by 1 week at a time (eg, every 1 week to every 2 weeks) therapies, and in routine clinical practice, provide some basis
over 3-month periods to allow for a new steady state to be for trial in those with confirmed CIDP refractory to treatment.
reached. If objective worsening occurs as therapy is tapered,
we return to the most recent previously successful dosing. Key Points
Adverse events with IVIG are uncommon, but include • CIDP is an inflammatory demelinating neuropathy; as
thrombotic events, renal injury, headache/chemical meningitis, such, damage is predominantly to large myelinated fibers.
and allergic reactions. Dosing should be adjusted in persons As a result, CIDP usually presents with muscle weakness and
with pre-existing renal disease, and premedication may be con- sensory ataxia, and rarely with pain or autonomic findings.
sidered to avoid some unwanted effects. • CIDP overdiagnosis is common and often related to over-
Corticosteroids. Corticosteroids remain useful immunother- interpretating EDX findings as demyelination or overrelying
apy in CIDP because of their low cost, widespread availability, on mildly elevated CSF protein and other non-specific find-
and convenience of use and dosing. Corticosteroids are effec- ings seen in mimicking disorders.
tive, although the data supporting efficacy is not as strong as • Even when demyelination is confirmed with EDX, non-
for immunoglobulin therapy,26 and long-term adverse events response to treatment warrants a search for alternative
occur. High-dose monthly oral dexamethasone produced diagnoses with POEMS syndrome at the top of the list.
fewer adverse events than daily use in a comparison to daily • Co-existing axon loss and demyelination may occur in long-
prednisolone and was no less effective.26 Periodic intravenous standing CIDP and makes EDX testing more challenging, but
methylprednisolone with dosing adjusted to clinical response is the R1 latency may be useful to establish the presence of
equally effective and produced fewer adverse effects than daily demyelinating disease in this scenario.
oral prednisone in a small, single-center retrospective study.32 • An objective target for immune therapy should be estab-
Plasmapheresis. Plasmapheresis is well-established and effec- lished before initiating treatment for CIDP to guide therapy.
tive immunotherapy in CIDP with the relative advantage of Dosing should be individualized based on such targets.
working quickly. Plasmapheresis may be more useful in those • The mainstays of therapy are immunoglobulins, cortico-
with very severe disability.26,27,33 We have had personal experi- steroids, or plasmapheresis, but novel emerging immune
ence of plasmapheresis being very effective in treatment of therapies may be used in refractory cases as further high-
ataxia and tremor in some cases of recently described variants quality evidence is obtained. n
caused by antiNF-155, antiCASPR-1, and anticontactin-1—the
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Christopher J. Lamb, MD
Department of Neurology, Mayo Clinic
Rochester, MN
P. James B. Dyck, MD
Professor, Department of Neurology, Mayo Clinic
Rochester, MN
Disclosures
PJBD has disclosures at www.practicalneurology.com

62 PRACTICAL NEUROLOGY JULY/AUGUST 2021

 NEUROMUSCULAR
DISORDERS

TABLE e1. CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY VARIANTS

Variant Clinical presentation Location of pathology Differential diagnosis
Acute (A-)8 or subacute (S-)35 Rapid <8 weeks (A-CIDP) or Proximal and distal nerves GBS, acute myelopathy
onset chronic inflammatory 4-8 weeks (S-CIDP); proprioceptive and nerve roots as in
demyelinating polyradiculo- loss and ataxia more than in Guillain- typical CIDP
neuropathy (CIDP) Barré syndrome (GBS)8
Multifocal CIDP (Lewis- Asymmetric, relapsing, or progressive,
Sensory and motor nerves Systemic small vessel vasculitides,
Sumner syndrome, more often sensorimotor than sensory-
>nerve root involvement, hereditary liability to pressure nerve
MADSAM)7,9 only, and multiple mononeuropathy
with patchy presentation, palsy (HNPP), cervical or lumbo-
upper/lower limbs equally sacral radiculoplexus neuropathy,
affected perineurioma
Sensory CIDP 10,36 Progressive sensory loss with preserved Postganglionic sensory Myeloneuropathy (dorsal columns),
strength, electrophysiology shows nerves and motor nerves sensory ganglionopathy
subclinical motor involvement that is with more upper than
nonlength dependent, and two-thirds lower limb involvement
progress to typical CIDP within a
mean 5 years7
Motor CIDP2,7,11 Progressive proximal and distal weak- Proximal and distal motor Multifocal motor neuropathy with
ness with preserved sensation and nerve fibers lower>upper conduction blocks (MMN-CB),
sensory sparing on electrophysiology limb involvement motor neuron disease (MND)
Chronic immune sensory Sensory ataxia with normal strength, Preganglionic sensory Myeloneuropathy, sensory
polyradiculopathy (CISP)13 EMG, and nerve conduction studies roots ganglionopathy
(NCS) but abnormal sensory evoked
potentials (SEPs)
Chronic immune sensory Sensory ataxia with minimal distal Predominantly pre- May have electrodiagnostic appear-
polyradiculopathy plus “numbness” or weakness that may ganglionic sensory roots ance of axonal distal symmetric
(CISP-plus)12 appear to have mild, distal axonal with some involvement of polyneuropathy, paraneoplastic or
EMG/NCS findings and abnormal SEPs postganglionic segments metabolic myeloneuropathies
Chronic immune sensory Proximal weakness and sensory loss Root level inflammatory Infiltrative, neoplastic, or structural
and motor polyradiculopathy with hyporeflexia or areflexia and demyelination that spares polyradiculopathy
(CISMP)14 lower>upper limb involvement distal segments
36. Rajabally YA, Wong SL. Chronic inflammatory pure sensory polyradiculoneuropathy: a rare CIDP variant with unusual
electrophysiology. J Clin Neuromusc Dis. 2012;13(3):149-152.

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