一家系にみられた先天性両側顔面神経麻痺—いわゆるMöbius症候群

A FAMILY OF CONGENITAL BILATERAL FACIAL PARALYSIS: MÖBIUS SYNDROME

　I．はじめに

　先天性両側顔面神経麻痺について最初に記載したのは1880年von Graefe^8）である。以後外国では本疾患についての報告は散見され，もつとも理解を容易にさせるのはHenderson（1939）^5）とDanis（1945）^1）のものであり，今日までおよそ125症例以上^3）が報告されているものと思われる。

　しかし，わが国での報告は稀で私たらの調べた範囲内では耳鼻科領域では昭和35年関西地方会における清水の1例^19）をみるにすぎない。

　最近，われわれは父親，第1子，第2子の3人にみられた先天性両側顔面神経麻痺の一家系に接したので若干の考察を加えここに報告する。

　A family of congenital, bilateral facial nerve paralysis was reported. Möbius syndrome was seen in the father (34 years old), the first child (5-year-old male), and the second child (4-month-old female). The etiology, heredity, diagnosis and treatment of this syndrome were discussed with the referential literature.

　The electromyogram was obtained from the m. frontalis, orbiculus oculi, and angulus oris of the father and the first child. No responses were recorded in the voluntary contraction of the m. frontalis and orbicularis oculi either in the father or the first child, and low voltage spikes were obtained from the orbiculus oris: from the left angulus oris of the father and bilaterally from the first child. It was of much clinical interest that these cases showed almost complete paralysis.