2次運動ニューロン病変を偶然伴う筋ジストロフィーの1例

MUSCULAR DYSTROPHY ASSOCIATED WITH COINCIDENTAL LOWER MOTOR NEURON INVOLVEMENT

I．緒言

　きわめて稀に，2種の筋病変が同じ筋または筋群を侵すことがある。かかる症例は神経筋系の病態を考察する上に種々の示唆を与える。筋病変の組合せには多くの可能性があるが，報告がもつとも多いのは，急性灰白髄炎による神経原性筋萎縮に筋ジストロフィーが生じた症例で，その逆の症例がそれについでいる。しかし，それらの貴重な症例についての検索が十分でないのは遺憾である。

　本論文の目的は，2次運動ニューロン病変を伴う筋ジストロフィーの1例を報告し，これまでの文献例を概観することである。

One case of progressive muscular dystrophy as-sociated with coincidental lower motor neuron in-volvement had been reported. The patient was the facio-scapulo-humeral type developed in a male with left paretic leg due to poliomyelitis. The results of the clinical examination were conflicting. The elec-tromyographic findings showed that the left leg was compatible with neurogenic amyotrophy while the right one was compatible with dystrophy. The electromyogram may reflect preceeding muscle in-volvement. Histologically, it was observed in the Rectus femoris on the left side groups of small fibers in the area showing dystrophic changes of the same degree as the right side. Enzyme activities in biopsy materials were measured but extremely difficult to discuss.

The contingent association of more than two different muscle involvements is interesting to note. As far as we know, 13 cases of muscular dystro-phy have been reported which developed in the victims of poliomyelitis, while 6 were affected by the latter disease after the onset of dystrophy.