先天性両側顔面筋麻痺症状群？—症例報告

CONGENTAL FACIAL DIPLEGIA SYNDROME?: A CASE REPORT

　von Graefeが1880年に始めて，先天性両側顔面筋麻痺症状群について報告し，それ以来，Harlan^4），Chisolum^1），Moebiusio^10），Henderson^5）及びSprofkin^11）等によつて本症状群が報告された。しかるに我が国では，谷・荒木^12）によつて最近報告された1例があるに過ぎないようである。この様に本症状群は極めて稀な疾患であるもののようで，現在までの報告は100例にもみたないといわれている。

　一方，進行性筋ジストロフィーは，1891年にErb^3）がその概念を明瞭に確立して以来，多くの研究者によつて知見が得られており，我が国に於いても，本疾患が筋肉の自律神経支配の障害によることを明らかにした呉・冲中教授^7）8）の業蹟がある。もともと後者は可成多く見られるものであるが，出生時代既に症状を示すものは極めて稀であつて，Dekaban^2）によると数例の報告があるのみという。

A 7-years & 2 months old boy was admi-tted to the Neuropsychiatric Clinic of Hiro-saki University Hospital with chief complaints of difficulty in closing eyes and atrophy of body musculatur.

There were no abnormalities at birth. At the 3rd. day, half-open lips and difficulty in closing eyes even during sleep were first noticed. He could not suck so sufficiently, that he was given milk with spoon. His faci-al expression was scanty. He began to walk after 15 months old although he was unusu ally clumsy and frequently suf feed falls. At 4 years old, protrusion of sternum, winging scapula and deficient growth of body muscles were noticed. On one hand, the manifesta-tion on his face have persisted without pro-gression, but on the other hand, atrophy of musculatur has been progressing very slowly.

In clinical examination, mask-like face with half-open lips and scanty facial expre-ssion, difficulty in closing eyes, and muscular atrophy in the scaptlo-humeral region in severe degree and in the gluteal in moderate, were found. He could not raise his arms above his shoulder level, his gait was stagge-ring. The muscles of the distal regions of his extremities were not so affected.