Cerebral Hemorrhage in Neurofibromatosis Type 1: one case

J Korean Neurol Assoc > Volume 16(5); 1998 > Article

Journal of the Korean Neurological Association 1998;16(5): 724-727.

다발성 신경섬유종증 제1형에서 발생한 뇌출혈 1예

정상욱, 배희준, 이승훈, 윤병우

서울대학교 의과대학 신경과학교실

Cerebral Hemorrhage in Neurofibromatosis Type 1: one case

Sang-Wuk Jeong, Hee-Joon Bae, Seung-Hoon Lee & Byung-Woo Yoon

Department of Neurology, College of Medicine, Seoul National University

Abstract

Cerebrovascular diseases associated with neurofibromatosis type 1 (NF-1) are not frequent. Ischemic stroke have been reported quite well in the literature, but cerebral hemorrhage associated with intracranial steno-occlusion were very rare and has not been reported in Korea. We report a 28-year-old woman with NF-1, who presented with cerebral hemorrhage. She had the history of multiple episodes of transient left hemiparesis. Sudden right occipital headache and left hemiparesis, followed by loss of consciousness, brought her to our emergency room and right thalamic hemorrhage with intraventricular extension was detected. Angiogram showed occlusion of the right supraclinoid internal carotid artery (ICA) and the left anterior cerebral artery, focal narrowing of the right petrous and the left supraclinoid ICA, and diffuse narrowing of the basilar artery. We discussed the genetic aspect and the pathophysiology of vascular occlusive disease in patients with NF-1. Key words: neurofibromatosis, intracerebral hemorrhage, Moyamoya disease