Article Abstract

Diagnostic insights into chronic-inflammatory demyelinating polyneuropathies

Authors: Johannes J. Roggenbuck, Joseph Boucraut, Emilien Delmont, Karsten Conrad, Dirk Roggenbuck

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare immune-mediated neuropathy with demyelination of nerve fibers as leading morphological feature. The course of disease can be chronic progressive or remitting relapsing. Whereas for acute immune-mediated neuropathies several serological markers have been identified and used successfully in clinical routine, the serological diagnosis of chronic variants such as CIDP has not yet been evolved satisfactory. The typical CIDP and its various atypical variants are characterized by a certain diversity of clinical phenotype and response to treatment. Thus, diagnostic markers could aid in the differential diagnosis of CIDP variants and stratification of patients for a better treatment response. Most patients respond well to a causal therapy including steroids, intravenous immunoglobulins and plasmapheresis. Apart from electrophysiological and morphological markers, several autoantibodies have been reported as candidate markers for CIDP, including antibodies against glycolipids or paranodal/nodal molecules. The present review provides a summary of the progress in autoantibody testing in CIDP and its possible implication on the stratification of the CIDP variants and treatment response.