Treatment experience in patients with anti-myelin-associated glycoprotein neuropathy

Muscle Nerve. 2001 Jun;24(6):778-86. doi: 10.1002/mus.1069.

Abstract

We report our experience with 24 consecutively treated patients (15 men and 9 women, median age 64 years) with anti-myelin-associated glycoprotein (anti-MAG) neuropathy. The rates of response to plasma exchange (40%), immune globulin (16%), and cyclophosphamide-based therapy (36%) were similar. Five (24%) responded to the first treatment modality, 32% to a second, alternative modality, and 31% to a third. Only 4 of 12 responders had sustained improvement; the others relapsed after a median of 7 months. In those 4 patients, the median immunoglobulin M (IgM) level dropped by 25% compared to an increase of 24% in the nonresponders (P = 0.04). Thus, most patients with anti-MAG neuropathy failed to have sustained improvement after treatment, and none of the therapies emerged as superior. Disability improved transiently after therapy in approximately 50% of cases. A 25% reduction of the IgM level predicted sustained improvement, but was difficult to achieve. There were no clinical or electrodiagnostic features associated with a treatment response, nor did a reduction of the anti-MAG antibody titer correlate with clinical improvement.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Autoantibodies / blood*
  • Autoimmune Diseases / physiopathology
  • Autoimmune Diseases / therapy*
  • Cyclophosphamide / therapeutic use
  • Electrophysiology / methods
  • Female
  • Follow-Up Studies
  • Humans
  • Immunoglobulin M / blood
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunotherapy
  • Male
  • Middle Aged
  • Motor Neurons / physiology
  • Myelin-Associated Glycoprotein / immunology*
  • Neural Conduction / physiology
  • Neurons, Afferent / physiology
  • Plasma Exchange
  • Polyneuropathies / immunology
  • Polyneuropathies / physiopathology*
  • Polyneuropathies / therapy*
  • Recurrence
  • Retrospective Studies

Substances

  • Autoantibodies
  • Immunoglobulin M
  • Immunoglobulins, Intravenous
  • Myelin-Associated Glycoprotein
  • Cyclophosphamide