Dr. Dimachkie, Director of the Neuromuscular Disease Division and Executive Vice Chairman for Research Programs, Department of Neurology, The University of Kansas Medical Center, received honorariums from Alnylam, Audentes, Baxalta, Catalyst, CSL Behring, Mallinckrodt, Momemta, Novartis, NuFactor, Sanofi, Shire, RMS Medical, and Terumo for speaking engagements or consulting work, and grants from Alexion, Alnylam, Amicus, Biomarin, BMS, Catalyst, CSL Behring, FDA/OPD, Genentech, Genzyme, GlaxoSmithKline, Grifols, MDA Pharmaceuticals, NIH, Novartis, Octapharma, Orphazyme, Sanofi,TMA, UCB BioPharma, and Viromed.)
Dr. Weimer of Columbia University has received consulting fees from Roche.)
The author reviews advances in the diagnosis and management of patients with anti-MAG neuropathy. Deposits of IgM antibodies can be detected in the small nerve fibers from glabrous skin biopsy. The author also comments on the markedly elevated risk of malignant plasma cell dyscrasias. Patients with anti-MAG neuropathy, therefore, require serial hematologic work-up.
Historical note and terminology
Antimyelin associated glycoprotein antibodies were first described in 1980 in a patient with neuropathy and IgM monoclonal gammopathy (Latov et al 1980; Braun et al 1982). More than 200 patients with this syndrome subsequently have been described. Several studies have delineated the clinical, laboratory, electrodiagnostic, immunologic, pathologic, and therapeutic aspects of this condition (Latov et al 1988; Nobile-Orazio et al 1989; Ellie et al 1996; Chassande et al 1998; Nobile-Orazio et al 2000; Eurelings et al 2001; Gorson et al 2001).
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