自発眼振および低音難聴を伴つたCharcot-Marie-Tooth病の1例

A CASE OF CHARCOT-MARIE-TOOTH DISEASE WITH A CHARACTERISTIC SPONTANEOUS AND POSITIONAL NYSTAGMUS AND LOW-TONE DEAFNESS

　I．はじめに

　後天的に発来する中枢神経系の変性疾患においては眼球運動障害を伴うものが多い。

　たとえば，多発性硬化症では眼球運動障害，とくに眼振が特徴的な所見の一つであることはよく知られている。またオリーブ・橋・小脳萎縮症などでも病変が橋や小脳の眼運動系にまで及び眼振がしばしば発来する。

　A patient of typical Charcot-Marie-Tooth disease, male and aged 27, was examined both from the neurotological and audiological point of view. Examinations revealed various signs indicating central involvement such as bilateral gaze nystagmus, positional and positioning nystagmus of direction-changing type, optokinetic inversion in optokinetic pattern (OKP)-test, disturbed pursuit motion in eye-tracking test and so on. The involvement of spinal motor neurons is one of characteristic features of this disease, but the present report leads the authors to the impression that there are widely-spread degenerative changes in the central nervous system, especially in the oculomotor system in the brainstem.