Treatment experience in patients with anti-myelin-associated glycoprotein neuropathy.
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.
Gorson
K C
KC
Division of Neurology, St. Elizabeth's Medical Center, Tufts University School of Medicine, 736 Cambridge Street, Boston, Massachusetts 02135, USA. kgorson@aol.com
Ropper
A H
AH
Weinberg
D H
DH
Weinstein
R
R
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000503:physiopathology / Q000628:therapy*; D003520:Cyclophosphamide / Q000627:therapeutic use; D004594:Electrophysiology / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007167:Immunotherapy; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D018992:Myelin-Associated Glycoprotein / Q000276:immunology*; D009431:Neural Conduction / Q000502:physiology; D009475:Neurons, Afferent / Q000502:physiology; D010951:Plasma Exchange; D011115:Polyneuropathies / Q000276:immunology / Q000503:physiopathology* / Q000628:therapy*; D012008:Recurrence; D012189:Retrospective Studies
D001323:Autoantibodies; D007075:Immunoglobulin M; D016756:Immunoglobulins, Intravenous; D018992:Myelin-Associated Glycoprotein; D003520:Cyclophosphamide