下肢遠位筋優位の筋萎縮，知覚障害に加え，中枢前庭障害を認めたCharcot-Marie-Tooth病が疑われた中枢・末梢神経変性症の1例

A Case of Peripheral and Central Neuropathy Patient with Muscle Atrophy and Sensory Disturbance in Distant Part of the Lower Extremity

　はじめに

　進行性の神経疾患の中には，大きく分けて遺伝性のものと単発性のものとがあり，中には末梢神経系，中枢神経系の両者に病変を生じるものも少なくない。多彩な症候を呈する疾患にさらに外的因子が加わると，ときに確定診断が困難となる。

　今回，Charcot-Marie-Tooth病を疑わせる遠位筋優位の筋萎縮，知覚障害を呈したが，本疾患のみでは説明できない所見を認めた症例を経験したので，神経耳科学的所見を中心に報告し，鑑別診断についての考察を加えた。

A 40-year-old man showed a marked atrophy of the lower extremity. The patient has a history of headinjury, alcohol habituation and diabetes but no family his-tory. Not only peripheral nerve degeneration but also brain atrophy were revealed by the neuro-physiological and neuro-radiological examinations. Neuro-otologial examinations showed saccadic pursuit, disturbed OKN, gaze evoked nystagmus, prolonged latency of VEMP, and enhancement of caloric responses under the light. Although possible diagnoses were discussed, it was diffi-cult to diagnose any one of the known diseases.