二卵性双生児に発症したモヤモヤ病

MOYAMOYA DISEASE IN FRATERNAL TWINS

　二卵性双生児のモヤモヤ病を経験した。兄は2歳6カ月で発症し多発性脳梗塞のため重篤な神経学的後遺症を残した。両側encephalomyo synangiosisを5歳時に施行した。弟は5歳5カ月で発症したが特に神経学的後遺症は残さなかった。両側Superficial temporal artery-Middle cerebral artery anasto—mosis encephalomyo synangiosisを施行した。この兄弟とその妹は同一のHLA typeを有していた。遺伝的および環境要因がモヤモヤ病の病態に複雑に絡み合っているのではないかと考えられた。

The authors have reported here fraternal twins of moyamoya disease. The one has the onset at the age of two years and six months. Then he had suffered from multiple cerebral infarction and resulting in severe neurological deficits. Now he has right hemiparesis, left homonymous hemian-opsia, aphasia and mental retardation. The ence-phalomyo synangiosis was done to the boy bila-terally at the age of five years. The other one has the onset at the age of five years and five months. He had good physical and neurologi-cal development. The Superficial temporal artery- Middle cerebral artery anastomosis and Encepha-lomyo synangiosis were done bilaterally. Now his development has no problems. The twins and their younger sister all have the same HLA type. The hereditary and environmental factors may be completely related to the pathogenesis of this dis-ease.