Moyamoya disease initially presenting visual field defect

Progressive narrowing of distal carotid arteries and the development of com pensatory fine networks are the characteristic findings of moyamoya disease . Cerebral infarction in moyamoya disease is due to a decreased blood flow and shows an uneven distribution in the distal bed of the anterior and midd le cerebral arteries. The progression of disease in the posterior circulati on follows that in the anterior circulation. Posterior circulation symptoms due to cerebral infarction usually occur in the advanced stage of the dise ase and follow the anterior circulation symptoms. We encountered an unusual case of moyamoya disease which initially presented with a transient visual field defect. One month later our patient developed blindness and her cere bral angiography showed advanced moyamoya. disease.