Chronic inflammatory demyelinating polyradiculoneuropathy—Diagnostic pitfalls and treatment approach. Issue 2 (11th September 2020)

• Record Type: Journal Article
• Authors: Stino, Amro M.; Naddaf, Elie; Dyck, Peter J.; Dyck, P. James B.
• Is Part Of: Muscle & nerve. Volume 63:Issue 2(2021)
• Page Start: 157
• Page End: 169
• Publication Date: 2020-09-11
• Subjects: CIDP, diagnosis, IVIg, plasma exchange, steroids, treatment; Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.27046
• Languages: English
• ISSNs: 0148-639X

Abstract

Abstract: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by progressive weakness and sensory loss, often affecting patientsʼ ability to walk and perform activities of daily living independently. With the lack of a diagnostic biomarker, the diagnosis relies on clinical suspicion, clinical findings, and the demonstration of demyelinating changes on electrodiagnostic (EDx) testing and nerve pathology. As a result, patients can often be misdiagnosed with CIDP and unnecessarily treated with immunotherapy. Interpreting the EDx testing and cerebrospinal fluid findings in light of the clinical phenotype, recognizing atypical forms of CIDP, and screening for CIDP mimickers are the mainstays of the approach to patients suspected of having CIDP, and are detailed in this review. We also review the currently available treatment options, including intravenous immunoglobulin (IVIg), corticosteroids (CCS), and plasma exchange (PE), and discuss how to approach treatment‐refractory cases. Finally, we emphasize the need to adopt objective outcome measures to monitor treatment response.