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NEUROLOGY 1980;30:1203
© 1980 American Academy of Neurology

Ultrastructural localization of acetylcholine receptor at the motor endplate

Myasthenia gravis and other neuromuscular diseases

Mitsuhiro Tsujihata, M.D., Ryuji Hazama, M.D., Nobuko Ishii, M.D., Yoshihiko Ide, M.D. and Masaharu Takamori, M.D.

First Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki, Japan.

Peroxidase-conjugated alpha-bungarotoxin (P-BGT) was used for the ultrastructural localization of the acetylcholine receptor in motor endplates. Brachial biceps muscle specimens were obtained from six patients with myasthenia gravis (MG) (two ocular and four generalized), five other patients with neuromuscular diseases (limb-girdle dystrophy, polymyositis, and amyotrophic lateral sclerosis) and two controls. In all patients with generalized MG, most of the endplates showed a marked decrease in P-BGT binding. In one of two patients with ocular MG, the amount and distribution of P-BGT binding appeared normal, whereas the other patient showed a slight decrease in P-BGT binding. There was a loose correlation between clinical severity of MG and acetylcholine receptor index or antiacetylcholine receptor antibodies. On the other hand, the amount and distribution of acetylcholine receptor in other neuromuscular diseases was well preserved, even at the endplates denuded of their nerve terminals in amyotrophic lateral sclerosis (ALS) cases.

Address correspondence and reprint requests to Dr. Tsujihata, First Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki, Japan.

Accepted for publication December 14, 1979.

This study was supported by a grant from Muscular Dystrophy Association of America.






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