脳底動脈狭窄を伴った成人発症型類もやもや病の1例

A Case of Unilateral Atypical Moyamoya Disease of Adult Onset with Stenosis of the Basilar Artery

I．はじめに

　もやもや病および類もやもや病において，椎骨脳底動脈系にも病変が進展することは知られており，特に若年発症型もやもや病では椎骨動脈．脳底動脈にも狭窄・閉塞が進展する^3）ことがある，と言われている．一方，成人発症型もやもや病および類もやもや病では後大脳動脈狭窄・閉塞の報告^2,4,12）は散見されるものの，椎骨動脈・脳底動脈病変を伴った症例の報告はない．今回われわれは，脳室内出血で発症した脳底動脈狭窄を伴う成人発症型類もやもや病の1例を経験したので文献的考察を加え報告する．

A 29-year-old, 39-week-pregnant female who had headache and nausea was admitted to our hospital. She bore a baby son by natural delivery after several hours. After labor, her headache was continuous. Brain CT scan demonstrated intracerebral and intraventricular hemorrhage. After conservative treatment for two weeks, her only neurological deficiency was visual field defect. Angiography demonstrated that her left internal carotid artery had partial stenosis at the C2 portion. Her right internal carotid artery had stenosis at the C2 portion. Her right middle cerebral artery was occluded at the M, portion, and abnormal vascular networks had developed in the ganglionic region. Stenosis was also found in the basilar artery. We diagnosed her as being a case of adult-onset, unilateral, atypical Moyamoya disease with basilar artery stenosis. As our case was of adult-onset, and as she showed no ischemic signs, we did not think that reconstructive surgery was indicated. About the posterior circulation of Moyamoya or atyp-ical Moyamoya disease, it was reported that in cases of juvenile onset the vertebral, basilar or posterior cere-bral artery was sometimes stenosecl or occluded, but, in adult-onset cases, stensis or occlusion of the posterior cerebral artery would he an abnormality.

Our case is a very rare example of unilateral atypical Moyamoya disease of adult onset with basilar artery stenosis.