モヤモヤ病を合併したTurner症候群の1例

A Case of Turner's Syndrome Assoclated with Moyamoya Disease

I．はじめに

　Turner症候群は，単独X染色体を右し，女性型外性器，低身長，性腺萎縮による原発性無月経を伴う疾患である．本症候群に合併しやすい血管異常である大動脈縮窄症についてはよく知られているが，頭蓋内血管の異常が合併することは稀である．われわれはTurner症候群にモヤモヤ病を合併した1例を経験したので文献的考察を加えて報告する．

A case of Turner's syndrome associated with Moyamoya disease in a 10-year-old female is presented. This patient had experienced two periods of syncope and transient right hemiparesis in 2 years. She was short in stature. She had cubitus vargus and mental re-tardation. The serum LEI and FSH values were high. By chromosomal examination Turner's syndrome (karyotype : 45, X/46, X, i (Xq)) was diagnosed. An MRI (SE 2000/20) revealed abnormal vessels on the basal ganglia. Cerebral angiography showed occlusion of bi-lateral internal carotid arteries at the distal portion, bi-lateral posterior cerebral arteries at the proximal por-tion. Basal moyamoya well developed. EDAS (encepha-lo-duro-arterio-synangiosis) was performed bilaterally. We consider that this may be the first case report of Turner's syndrome associated with Moyamoya disease, and that there may be no relationship between Turner's syndrome and Moyamoya disease in this case.