[Multiple cerebrovascular occlusive disease associated with neurofibromatosis].

Multiple cerebrovascular occlusive disease is rarely seen in patients with neurofibromatosis. Two cases of such lesions are presented and literatures dealing with the clinical and angiographical aspects of this occlusive disease are reviewed. Case 1; A 38-year-old normotensive man had sudden onset of vomiting, left hemiparesis and disturbance of consciousness, one day before the admission. He had family history of neurofibromatosis, and examination showed café au lait spots over the body. CT scans revealed a subcortical hematoma in the right temporal lobe. Angiogram revealed multiple occlusive lesions of the cerebral arteries, including occlusions of the right internal carotid artery (ICA) at the distal end, middle (MCA) and anterior (ACA) cerebral artery at the proximal portion, and stenosis of the left ICA and ACA. Abnormal vascular networks at the base of the brain were also seen bilaterally. Decompressive craniectomy, removal of the hematoma and bilateral ventricular drainage were performed. Postoperative course was excellent. Angiogram performed five and a half years later, during which time without any surgical procedures, demonstrated no apparent angiographic differences from the previous one. Case 2; A 29-year-old woman without family history of neurofibromatosis presented with sudden onset left hemiparesis. Café au lait spots were found over the body. A CT scan revealed small infarctions in the territory of the right MCA, and angiogram demonstrated multiple occlusive lesions of the cerebral arteries, including stenosis of the bilateral ICA, the left MCA, both ACAs at the proximal portion, and the right posterior cerebral artery, and occlusions of the right MCA.(ABSTRACT TRUNCATED AT 250 WORDS)