Moyamoya Disease Associated with Neurofibromatosis -A Case Report-

J Korean Neurol Assoc > Volume 10(2); 1992 > Article

Journal of the Korean Neurological Association 1992;10(2): 242-247.

다발성 신경섬유종과 동반된 Moyamoya 병 1예

김효경, 김종성,이명종,최지호

울산대학교 신경과, 피부과. 서울중앙병원 신경과, 피부과.

Moyamoya Disease Associated with Neurofibromatosis -A Case Report-

Hyo-Kyung Kim, M.D., Jong-Sung Kim, M.D., Myoung-Chong Lee, M.D., Jee-ho Choi, M.D.

Department of Neurology and Dermatology, College of Medicine, Ulsan University, Asan Medcial Center

Abstract

Cerebrovascular lesions in neurofibromatosis are uncommon. Cerebral arterial occlusive diseases associated with neurofibromatosis have been documented in less than 40 patients in world literature. We report a 28-year-old male patient with moyarnoya disease associated witb neurofibromatosis. Neurofibromatosis manifestation consisted of multiple cafe-au-lait spots, depigmentation, neurofibromas, iris Lisch nodule, axillary freckling and mental retardation. On admission with acute ischemic syndrome, focal neurologic deficits were decreased left sided pinprick sensation and bilaterally brisk knee jerks. Magnetic resonance imaging showed multifocal ischemic lesions in deep cerebral white matter and multifocal signal voids at the basal ganglia. Angiogram showed bilateral occlusion of distal internal carotid artery associated with moyamoya vessels. To our knowledge. This is a first case report of moyarnoya disease with neurofibromatosis in Korean literature