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橄欖核－橋腦－小腦萎縮症
Olivopontocerebellar Atrophy
摘要
橄欖核－橋腦－小腦萎縮症是一種少見的原發性神經系統退化性疾病，致病原因迄今不明。男女發生率相同。主要病理變化為腦幹及小腦萎縮。臨床症狀主要是以進行性失調性步態開始的小腦功能障碍。一般生化檢查、腦脊液檢查及腦電波檢查等皆無診斷價值。但腦部電腦斷層攝影可顯示橋腦、小腦萎縮以幫助診斷。此症迄今仍無特殊治療方法。本文就三軍總醫院近三年來發現的五個病例加以分析、討論。
並列摘要
Olivopontocerebellar atrophy (OPCA) is first created by Dejerine and Thomas in 1900, which comprises a series of heterogenereous diseases whose major common factor is the loss of neurons in the ventral portion of the pons, inferior olives and cerebellar cortex. This disorder affects either sex with approximately equal frequency in cases draw from the literature. OPCA usually begins with a disturbance of gait and balance. Cerebellar disturbances are the most outstanding clinical features of the disease. In general ancillary investigations, with the possible exception of pneumoencephalography, typically yield normal findings in OPCA. Recently, CT scan has added new possibilities with a noninvasive method for diagnosing OPCA. In all cases, atrophy of brainstem and cerebellum was found. Atrophy of the cerebellar hemispheres was equal to, or more marked to atrophy of the vermis. Neither curative nor adequate palliative therapy is available for OPCA. Five cases of OPCA was diagnosed in our hospital from May 1982 to March 1985. General clinical data, neurological findings and CT findings were analyzed.