Acquired Immune Axonal Neuropathies

Vera Bril, BSc, MD, FRCPC; Hans D. Katzberg, MD, MSC, FRCPC Peripheral Nervous System Disorders p. 1261-1273 October 2014, Vol.20, No.5 doi: 10.1212/01.CON.0000455882.83803.72
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Purpose of Review: This article discusses the clinical features, pathophysiology, and management of primary and secondary acquired immune axonal neuropathies.

Recent Findings: Although there are many collagen vascular disorders associated with vasculitic neuropathy, a quarter of cases have been described to be due to nonsystemic vasculitis of the peripheral nervous system. Enhanced surveillance and aggressive treatment of conditions such as cryoglobulin-related vasculitic neuropathy with cyclophosphamide, rituximab, and alfa interferons has led to improved morbidity and mortality, however, many cases of immune axonal acquired neuropathy are still associated with poor outcomes. Acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN) are well-characterized variants of Guillain-Barré syndrome.

Summary: Characterizing the clinical and electrophysiologic phenotype can help diagnose conditions such as nonsystemic vasculitic neuropathy, AMAN, AMSAN, and immune small fiber neuropathy, while careful evaluation of systemic features is key to identifying secondary immune axonal neuropathies such as vasculitic neuropathy related to collagen vascular disease. Additional research is needed to determine the exact immune pathogenesis and optimized treatment regimens for all acquired immune axonal neuropathies.

Address correspondence to Dr Vera Bril, Toronto General Hospital, 200 Elizabeth St, 5EC-309, Toronto, ON M5G 2C4, Canada, [email protected].

Relationship Disclosure: Dr Bril serves as a consultant for and her institution receives grants from CSL Behring and Grifols, Canada, Ltd. Dr Katzberg has received travel support from Genzyme Canada and serves as a speaker, and his institution receives grants from CSL Behring; Grifols Canada, Ltd; and the University of Toronto.

Unlabeled Use of Products/Investigational Use Disclosure: Drs Bril and Katzberg report no disclosures.

© 2014 American Academy of Neurology