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July 25, 2017 E-Pearl of the Week: Anti-myelin-associated Glycoprotein (MAG) neuropathy

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July 25, 2017

Anti-myelin-associated Glycoprotein (MAG) neuropathy

Anti-MAG neuropathy is an acquired demyelinating polyneuropathy associated with IgM monoclonal gammopathy of undetermined significance (MGUS). Most patients present with symmetric, distal sensorimotor deficits, ataxia, areflexia, hand tremor or pseudoathetosis. Electrodiagnostic studies usually show marked prolongation of distal motor latencies.1 Rituximab has been shown to reduce anti-MAG antibody titers and improve neuropathy. Patients with high anti-MAG titers and severe sensory symptoms at baseline are more likely to benefit from Rituximab treatment.2

References:

  1. Talamo G, Mir MA, Pandey MK, Sivik JK, Raheja D. IgM MGUS associated with anti-MAG neuropathy: a single institution experience. Ann Hematol 2015; 94: 1011-1016.
  2. Dalakas M. C., Rakocevic G, Salajegheh M, et al. Placebo-controlled trial of rituximab in IgM anti-myelin-associated glycoprotein antibody demyelinating neuropathy. Ann Neurol 2009; 65: 286-293. 

Submitted by Tim Tien Lai, MD, Department of Neurology - University of California, Irvine Medical Center. 

Dr. Lai reports no disclosures.

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