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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an immune mediated neuropathy with an incidence of ~2 / 100,000. CIDP classically presents with subacute or chronic, proximal and distal weakness, sensory loss and areflexia. Variants include multifocal acquired demyelinating sensory and motor neuropathy  (MADSAM), multifocal motor neuropathy (MMN), pure sensory and distal predominant (distal acquired demyelinating neuropathy - DADS).

Reading Material

Allen & Lewis, CIDP Diagnostic Pitfalls and Perception of Treatment Benefit. Neurology. 2015. PMID: 26180143

  • Take home points: CIDP misdiagnosis is common. Sources of error include emphasis on subjective reported of treatment benefit, mild cytoalbuminologic dissociation and relaxed electrophysiologic interpretation.  

Dyck, et al. A Plasma Exchange Versus Immune Globulin Infusion Trial in Chronic Inflammatory Demyelinating Polyradiculoneuropathy. Ann Neurol. 1994 PMID: 7998769

  • Take home points: Small trial of 20 patients who received PLEX (or IVIg) for 6 weeks, then washout period, then other treatment. Improvements in muscle scores and summated CMAPs and SNAPs were seen in both groups.

Hughes, et al. Intravenous Immune Globulin (10% Caprylate-Chromatography Purified) for the Treatment of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (ICE Study): A Randomised Placebo-Controlled Trial. 2008. Lancet. PMID: 18178525

  • Take home points: Using INCAT score - 54% of patients treated with IGIV-C and 21% patients on placebo had an improvement maintained through to week 24. Improvements also recorded in grip strength. Similar results in crossover and extension period (longer time to relapse). First trial showing short and long term benefit in IVIG for CIDP

European Federation of Neurological Societies/Peripheral Nerve Society Guideline on Management of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Report of a Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society--First Revision. 2010 J Peripher Nerv Syst. PMID: 20433600

  • Take home points: There are many CIDP electrodiagnostic criteria. The EFNS/PNS criteria offer sensitivity of ~81% and specificity of 96% for definite/probable CIDP.

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