Ventricular hemorrhage at an early stage of moyamoya disease--case report.

A 19-year-old male presented with intraventricular hemorrhage manifesting as sudden onset of headache. Angiography showed mild stenotic changes in the distal internal carotid artery and proximal anterior cerebral artery only on the right. The anterior choroidal artery and lenticulostriate arteries appeared dilated, and an aneurysm-like shadow was seen in the distal right anterior choroidal artery. He was discharged without treatment. Eighteen months later, he presented with a second intraventricular hemorrhage manifesting as sudden occipitalgia, vomiting, and nausea. He had hyperreflexia of the left extremities and paresthesia of the left upper extremity. Angiography showed marked progression of the vascular abnormalities bilaterally. Moyamoya vessels were also present. He received bilateral encephalo-duro-arterio-myo-synangiosis with good results. Moyamoya disease may cause hemorrhage even at an early stage.