Acquired Immune Demyelinating Neuropathies

Mazen M. Dimachkie, MD, FAAN, FANA; David S. Saperstein, MD Peripheral Nervous System Disorders p. 1241-1260 October 2014, Vol.20, No.5 doi: 10.1212/01.CON.0000455883.91426.12
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Purpose of Review: Acquired immune demyelinating neuropathies refer to a group of disorders that share overlapping sensory, motor, and autonomic clinical, laboratory, and electrodiagnostic features. It is important to recognize acquired immune demyelinating neuropathies as they are generally responsive to immunosuppressive or immunomodulatory therapies.

Recent Findings: This article reviews recently developed early prognostic tools in Guillain-Barré syndrome and discusses the evolving understanding of chronic demyelinating phenotypes with differing treatment responsiveness.

Summary: While weakness and numbness progress over 2 to 4 weeks in Guillain-Barré syndrome, they continue to evolve beyond 8 weeks in chronic inflammatory demyelinating polyradiculoneuropathy and over 4 to 8 weeks in subacute inflammatory demyelinating polyradiculoneuropathy. Acquired immune demyelinating neuropathies present uncommonly as variants with predominance of ocular, bulbar, sensory, autonomic, or motor manifestations in addition to regional variants, such as paraparetic acquired immune demyelinating neuropathies. Establishing the correct diagnosis is important as these immune disorders differ in response to corticosteroids and other immunosuppressive therapies.

Address correspondence to Dr Mazen M. Dimachkie, University of Kansas Medical Center, 3599 Rainbow Blvd., Mail Stop 2012, Kansas City, KS 66160, [email protected].

Relationship Disclosure: Dr Dimachkie has received grant support from CSL Behring and has served as a consultant, speaker, advisory board member, or steering committee member for Baxter; BioMarin Pharmaceutical, Inc; Catalyst Pharmaceutical Partners, Inc; CSL Behring; Depomed, Inc; Genzyme Corporation; Merck & Co, Inc; NuFACTOR; and Pfizer Inc. Dr Saperstein has served as a consultant, speaker, and advisory board member for Baxter, CLS Behring, Grifols, and NuFACTOR.

Unlabeled Use of Products/Investigational Use Disclosure: Drs Dimachkie and Saperstein discuss the unlabeled use of IV immunoglobulin G, plasma exchange, and gabapentin for the treatment of acquired immune demyelinating neuropathies.

© 2014 American Academy of Neurology