レックリングハウゼン氏病に合併したくも膜下出血2例の報告—多発性脳動脈瘤に脳動静脈奇形を合併した1例と前交通動脈動脈瘤の1例

Two Cases of Subarachnoid Hemorrhage Associated with Neurofibromatosis type I : a case of multiple cerebral aneurysms and arteriovenous malformation, and another case of an anterior communicating artery aneurysm

I．はじめに

　von Recklinghausen氏病（neurofibrnmatosistype I）は神経皮膚症候群（neurocutaneous syn-drome）の一種で，第17染色体に異常のある常染色体優性遺伝疾患であり，1／3000の頻度でみられる^19）．このvon Recklinghausen氏病は主として，neurofibroma, café-au-lait spot, glial tumor，meningioma等の発生に示されるように外胚葉系の異常を示すが，その他，骨形成異常，腎嚢胞，膵嚢胞の発生に示されるように中胚葉系の異常も示す^15）．この中胚葉系の異常に属するものには血管病変もあるが，これは比較的稀である^{2,12,14,20）}．中枢神経系の血管障害の中では脳梗塞が多いが，今回われわれは稀なくも膜下出血の2例を経験したので，考察と共に報告する．

Two cases of subarachnoid hemorrhage associated with neurofibromatosis type I (von Recklinghausen'sdisease) are reported. A 30-year-old male patient (case 1) had been diagnosed as having neurofibromatosistype I due to neurofibroma and café-au-lait spot. He suffered from subarachnoid hemorrhage and angiogra-phy showed multiple aneurysms in the right and left middle cerebral arteries and left internal carotidartery. He also had arteriovenous malformation in the left temporal lobe. Case 2 was that of a 62-year-oldfemale patient with neurofibroma and café-au-lait spot. She suffered from subarachnoid hemorrhage andangiography showed an aneurysm in the anterior communicating artery. Both patients were dischargedwith no deficits after neck clipping, however they both suffered from large-sized hematoma in the punctur-ed site of the femoral artery after angiography. The cerebral aneurysms associated with neurofibromatosistype I are often multiple and may coexist with arteriovenous malformation.