進行性筋ジストロフィーの臨床と筋電図

CLINICAL AND ELECTROMYOGRAPHICAL STUDIES ON PROGRESSIVE MUSCULAR DYSTROPHY

I．緒言

　進行性筋萎縮症は従来末梢神経，筋系疾患群のほかでは日常しばしば遭遇する疾患であり，すでに古くから数多くの研究がなされたが，発病の本態および治療に関する根本問題はまだ解決されていない状態である。

　われわれは最近当科に入院した進行性筋ジストロフィー患者6名の症例を経験し，ATP-Na塩を中心に治療をかさねつつ，臨床的に長期の経過観察ならび筋電図学的知見に病理組織学的考察を試みた。

We have clinically observed the progress of 6 patients of Progressive Muscular Dystrophy recently accomodated in this clinic and found that they had dysfunction of the autonomic nervous system mild deficiency of adrenocorti-cal function and anomalies in the excretion of creatin and creatinine and the contents of protein in fractins serum, as already pointed out by some authors, and that the strength of affected muscles was not recovered, in the cases with severe clincal symptoms.

In EMG, low amplitude NMU voltage was observed in nearly all the cases, complex NMU voltage in 4 cases and fibrillation voltage in 1 case.

Besides, tendency to grouping was manifest, the diagram of discharge intervals showed slow fluctuation and the histogram failed to show normal distribution.

Histopathological examination of the effe-cted parts revealed existence of fatty de-generation, vacuolation, atrophy, swelling, collapse and connective-tissue formation of the muscle fibers, lowered definition of end-plates of motor nerves, thickening of the con-nective capsules of muscle spindles and swel-ling and rupture at the central parts with aggregated nuclei of the end-plates of motor nerve.

Some discussions were made on the mecha-nism of occurrence of the above mentioned EMG anomalies, based on the histopathologi-cal findings.