Abstract

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune mediated treatable peripheral neuropathy, the diagnosis of which is straightforward in more than half of cases. Numerous sets of electrophysiological criteria have been published. However, in some cases, electrophysiological data are not sufficient and patients that may benefit from treatment escape accurate diagnosis.

Objective: To describe a step by step diagnostic procedure for neurologists facing a peripheral neuropathy of undetermined cause, to help make an accurate diagnosis of CIDP.

Methods: A group of French experts was established, neurologists and neurophysiologists being recruited on the basis of personal experience with patients suffering from CIDP and also on publications in the field. A full literature review was conducted on the topic of diagnostic criteria and procedures for the diagnosis of CIDP, and meetings were scheduled to reach a consensus on the best diagnostic workup in different clinical situations.

Results: Six meetings were conducted and a consensus was reached, based on the available literature and experience in the management of such patients. Discussions resulted in defining five clinical situations in which a diagnosis of CIDP may be considered, and procedures were detailed in each case, including the location of nerve biopsy and use of non-conventional electrophysiological testing and imaging procedures.

Conclusion: The guidelines in the diagnostic procedure reported here result from a consensus of French experts in the field of peripheral neuropathy and allow a diagnosis of CIDP to be made in the most frequently encountered situations. These recommendations may be of value for physicians as they rely on the rational use of available techniques in typical clinical situations.