Rasmussen症候群の1例

A case of Rasmussen's syndrome

　片眼性のぶどう膜炎を伴うRasmussen症候群の1例を経験した。症例は25歳の男性，1歳時に左側半身に及ぶ知覚運動性の部分痙攣発作があり，次第に持続性部分てんかんに進展した。頭部CT検査で右大脳半球に皮質優位の著明な萎縮がみられた。4歳時の眼底検査では，右眼に以前のぶどう膜炎を示唆する軽度の硝子体混濁，視神経萎縮，静脈周囲炎がみられた。各種抗痙攣薬に抵抗し，右半球の萎縮が次第に進行したが，右眼のぶどう膜炎の再燃はなかった。

A 4-year-old male was referred to us for Rasmussen syndrome. He had a history of left-sided somatosensorimotor seizures at the age of 1 year which gradually developed into epilepsia partialis continua. Computed tomography showed severe cerebral cortical atrophy in the right hemisphere. The findings were compatible with the diagnosis of Rasmussen syndrome. The right eye showed mild vitreous opacity, optic atrophy and retinal periphlebitis suggesting longstanding uveitis. During the follow-up until the age of 25 years, seizures were resistant to anticonvulsant medications and the right cerebral atrophy progressed further. There has been no recurrence of uveitis.