[Neurofibromatosis with occlusion of the internal carotid artery: case report]

Vascular lesions of the central nervous system in neurofibromatosis (NF) are uncommon. Cerebral arterial occlusive diseases in NF have been documented in only 29 patients. This paper reports a case of a 16-year-old male having occlusion of the right internal carotid artery with NF. He was admitted with an episode of clonic convulsion and left hemiparesis at the age of five years. On admission, he had multiple café-au-lait spots, depigmentation and fibroma molle throughout his body. Neurological examination revealed left hemiparesis with hypereflexia and pathological reflex on the left side. CT scan showed brain atrophy of the right hemisphere and low density area in the white matter of the right frontal lobe. Cerebral angiography showed occlusion at the extracranial portion of the right internal carotid artery. He underwent STA-MCA bypass on the right side. On histological examination of a section of STA, the intimal layer was thickened by elastofibrosis. The mechanisms of arterial occlusion in patients with NF are discussed with review of the literature.