Criteria
European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) criteria[1]
Multiple clinical, electrophysiologic, and laboratory criteria have been suggested, with varying degrees of sensitivity and specificity.[1][23][24][25][41][83][129] While most sets of criteria have specificities approaching 100%, sensitivities are around 60% to 70%.[24][26][23] Sensitivity may be higher if two sets of criteria with different parameters are combined.[27] Motor conduction block in a noncompressible site is highly sensitive.[26] Testing more nerves and proximal sites may improve sensitivity.[28][29] Patients who fulfill diagnostic criteria, especially those with a higher degree of demyelination, have a predicted greater treatment response rate than patients who do not fulfill these criteria.
Revised diagnostic criteria were published by the EAN/PNS in 2021, replacing those published in 2010.[1][31]
Clinical diagnostic criteria
a. Typical CIDP
All of the following criteria are met:
Progressive or relapsing, symmetric, proximal and distal muscle weakness of upper and lower limbs, and sensory involvement of at least two limbs
Developing over at least 8 weeks
Absent or reduced tendon reflexes in all limbs.
The term "acute-onset CIDP" (A-CIDP) represents typical CIDP that rapidly progresses within 4 weeks and mimics GBS.
b. CIDP variants
One of the following, but otherwise as for typical CIDP (tendon reflexes may be normal in unaffected limbs):
Distal CIDP: distal sensory loss and muscle weakness predominantly in lower limbs. (Distal neuropathy with IgM paraprotein and anti-MAG antibodies [anti-MAG neuropathy] is considered outside the scope of CIDP as most patients have specific electrodiagnostic and pathologic findings, and intravenous immune globulin and corticosteroids are not effective.)
Multifocal CIDP: sensory loss and muscle weakness in a multifocal pattern, usually asymmetric, upper limb predominant, in more than one limb.
Focal CIDP: sensory loss and muscle weakness in only one limb.
Motor CIDP: motor symptoms and signs without sensory involvement.
Sensory CIDP: sensory symptoms and signs without motor involvement.
c. Disorders not classified as CIDP
Chronic immune sensory polyradiculopathy (CISP): not included in the CIDP variant classification because there is not enough evidence to determine if CISP is demyelinating or related to sensory CIDP.
Autoimmune nodopathies: not regarded as CIDP variants because they have distinct clinical features, no overt inflammation or macrophage-mediated demyelination, and respond poorly to treatments for CIDP (especially intravenous immune globulin).
Motor nerve conduction criteria
(1) Strongly supportive of demyelination:
At least one of the following:
Motor distal latency prolongation ≥50% above upper limit of normal values (ULN) in two nerves (excluding median neuropathy at the wrist from carpal tunnel syndrome), or
Reduction of motor conduction velocity ≥30% below lower limit of normal values (LLN) in two nerves, or
Prolongation of F-wave latency ≥20% above ULN in two nerves (≥50% if amplitude of distal negative peak compound muscle action potential [CMAP] <80% of LLN), or
Absence of F-waves in two nerves (if these nerves have distal negative peak CMAP amplitudes ≥20% of LLN) + ≥1 other demyelinating parameter in ≥1 other nerve, or
Motor conduction block: ≥30% reduction of the proximal relative to distal negative peak CMAP amplitude, excluding the tibial nerve, and distal negative peak CMAP amplitude ≥20% of LLN in two nerves; or in one nerve + ≥ 1 other demyelinating parameter except absence of F-waves in ≥1 other nerve, or
Abnormal temporal dispersion: >30% duration increase between the proximal and distal negative peak CMAP (at least 100% in the tibial nerve) in ≥2 nerves, or
Distal CMAP duration (interval between onset of the first negative peak and return to baseline of the last negative peak) prolongation in ≥1 nerve + ≥1 other demyelinating parameter in ≥1 other nerve
(Low frequency filter [LFF] 2 Hz) median >8.4 ms, ulnar >9.6 ms, peroneal >8.8 ms, tibial >9.2 ms
(LFF 5 Hz) median >8.0 ms, ulnar >8.6 ms, peroneal >8.5 ms, tibial >8.3 ms
(LFF 10 Hz) median >7.8 ms, ulnar >8.5 ms, peroneal >8.3 ms, tibial >8.2 ms
(LFF 20 Hz) median >7.4 ms, ulnar >7.8 ms, peroneal >8.1 ms, tibial >8.0 ms.
(2) Weakly supportive of demyelination
As in (1) but in only one nerve.
Sensory nerve conduction criteria
(1) CIDP
Sensory conduction abnormalities (prolonged distal latency, or reduced sensory nerve action potential [SNAP] amplitude, or slowed conduction velocity outside of normal limits) in two nerves.
(2) Possible CIDP
As in (1).
Sensory CIDP with normal motor nerve conduction studies needs to fulfill (a) or (b):
sensory nerve conduction velocity <80% of LLN (for SNAP amplitude >80% of LLN) or <70% of LLN (for SNAP amplitude <80% of LLN) in at least two nerves (median, ulnar, radial, sural nerve), or
sural sparing pattern (abnormal median or radial SNAP amplitude with normal sural nerve SNAP amplitude) (excluding carpal tunnel syndrome).
Supportive criteria
Response to treatment, imaging, cerebrospinal fluid (CSF) analysis, or nerve biopsy may be supportive in patients who fulfill clinical criteria for CIDP, but whose electrodiagnostic criteria only allow for possible CIDP.[1]
Diagnostic categories
The 2021 guideline classifies CIDP into two categories (CIDP and possible CIDP).[1] The category of probable CIDP (as in the 2010 guideline) was removed because the diagnostic accuracy of criteria for probable and definite CIDP did not significantly differ.[1]
Typical CIDP
Typical CIDP
Clinical criteria + motor conduction criteria in two nerves + sensory conduction abnormalities in two nerves; or
Possible typical CIDP + at least two supportive criteria
Possible typical CIDP
Clinical criteria + motor conduction criteria in one nerve + sensory conduction abnormalities in two nerves; or
Clinical criteria + motor conduction abnormalities not fulfilling CIDP motor conduction criteria in one nerve + sensory conduction abnormalities in two nerves + objective response to treatment + one other supportive criterion
Distal CIDP
Distal CIDP
Clinical criteria + motor conduction criteria in two upper limb nerves + sensory conduction abnormalities in two nerves; or
Possible distal CIDP + at least two supportive criteria
Possible distal CIDP
Clinical criteria + motor conduction criteria in one upper limb nerve + sensory conduction abnormalities in one nerve; or
Clinical criteria + motor conduction criteria in two lower limb nerves only + sensory conduction abnormalities in two nerves (possible distal CIDP only, cannot be upgraded by supportive criteria)
Multifocal or focal CIDP
Multifocal or focal CIDP
Clinical criteria + motor conduction criteria in two nerves + sensory conduction abnormalities in two nerves; or
Possible multifocal or focal CIDP + at least two supportive criteria
Possible multifocal or focal CIDP
Clinical criteria + motor conduction criteria in one nerve + sensory conduction abnormalities in two nerves
Focal CIDP fulfilling clinical criteria + motor conduction criteria in one nerve + sensory conduction abnormalities in one nerve (possible focal CIDP only, cannot be upgraded by supportive criteria)
Motor CIDP
Motor CIDP
Clinical criteria + motor conduction criteria in two nerves + normal sensory conduction in four nerves; or
Possible motor CIDP + at least two supportive criteria
Possible motor CIDP
Clinical criteria + motor conduction criteria in one nerve + normal sensory conduction in four nerves
Motor-predominant CIDP
As in motor CIDP but with sensory conduction abnormalities in two nerves
Sensory CIDP
Possible sensory CIDP
Clinical criteria + sensory conduction criteria (possible sensory CIDP only, cannot be upgraded by supportive criteria). Motor conduction must be normal in at least four nerves.
Sensory-predominant CIDP
Possible sensory-predominant CIDP
Clinical criteria + sensory conduction abnormalities in two nerves + motor conduction abnormalities in two nerves or motor conduction criteria fulfillment in one nerve
Sensory-predominant CIDP
Clinical criteria + sensory conduction abnormalities in two nerves + motor conduction criteria fulfillment in two nerves.
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