Diagnostic Snares in Chronic Inflammatory Demyelinating Polyneuropathy

Jeffrey A. Allen, MD


January 06, 2020

Making a diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) requires neurologists to identify an array of clinical, electrodiagnostic, and other supportive evidence, and weigh it against inclusion and exclusion criteria.

Guidelines from the European Federation of Neurological Societies and the Peripheral Nerve Society (EFNS/PNS) mandate that patients both satisfy clinical criteria and present demyelination of peripheral nerves on EMG.

The EFNS/PNS guidelines, published in 2010, are globally recognized for CIDP sensitivity and specificity. However, a recent survey of US neurologists showed that only 39% refer to the EFNS/PNS guidelines for diagnosing CIDP. Misdiagnosis is a persistent problem in CIDP for many reasons, among which is variability of clinical practice.

As Dr Jeffrey Allen reports in this ReCAP, the EFNS/PNS joint task force is working to update the guidelines in 2020. Improvements will include more specific definitions of CIDP phenotypes, especially atypical variants, to clarify the clinical and electrodiagnostic features of CIDP for diagnostic benefit.


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