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NEUROLOGY 1992;42:2175
© 1992 American Academy of Neurology

Long-term neuromuscular dysfunction produced by passive transfer of amyotrophic lateral sclerosis immunoglobulins

O. D. Uchitel, MD, F. Scornik, MSc, D. A. Protti, MSc, C. G. Fumberg, MD, V. Alvarez, MSc and S. H. Appel, MD

Instituto de Biologia Celular (Drs. Uchitel and Fumberg, F. Scornik, D.A. Protti, and V. Alvarez), Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina, and the Department of Neurology (Dr. Appel), Baylor College of Medicine, Houston, TX.

We investigated the role of the immune system in the pathogenesis of amyotrophic lateral sclerosis (ALS) by studying the long-term consequences of ALS immunoglobulin (Ig) application on the levator auris muscle of the mouse. We applied Ig from seven ALS patients, four disease controls, and a pool of normal Ig (6 mg of Ig in 2 weeks) by subcutaneous injection; removed the muscles 4 to 12 weeks after the beginning of treatment; and recorded both spontaneous and evoked release of transmitter. None of the control Ig induced changes in transmitter, whereas five of seven ALS Ig induced a significant increase in the rate of spontaneous release, and all ALS Ig produced significant changes in the quantal content of evoked release. In muscles treated with one of the ALS Igs, synaptic activity was completely absent. Cholinesterase and silver staining demonstrated intact neuromuscular junctions in the control Ig-treated muscles and also in many areas of ALS Ig-treated muscles. Axonal degeneration and denervation were present in most muscles treated with ALS Ig. There was complete denervation when no synaptic activity could be recorded. Thus, ALS Ig appears to lead to long-lasting effects at the neuromuscular junction, and such effects may be an early stage in the immune-mediated pathogenesis of ALS.

Address correspondence and reprint requests to Dr. Osvaldo D. Uchitel, Instituto de Biologia Celular, Facultad de Medicina, Universidad de Buenos Aires, Paraguay 2155 (1121) Buenos Aires, Argentina.

Supported by the Muscular Dystrophy Association, Tuscon, AZ; Consejo Nacional de Investigaciones Cientificas y Tecnicas de la Republica Argentina (CONICET-PID 3041400/88) and the Universidad de Buenos Aires (subsidio ME-067).

Received December 13, 1991. Accepted for publication in final form April 3, 1992.






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