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KMID : 0359719920100020242
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Journal of the Korean Neurological Association
1992 Volume.10 No. 2 p.242 ~ p.247
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Noyamoya Disease Associated with Neurofibromatosis-Case Report-
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Abstract
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Cerebrovascular lesions in neruofibromatosis are uncommon. Cerebral arterial occlusive diseases associated with neruofibromatosis have been documented in less than 40 patients in world literature. We report a 28-year-old male patient with
moyamoya
disease associated with neurofibromatosis. Neurofibromatosis manifestation consisted of multiple caf*-au-lait spots, depigmentation, neurofibromas, iris Lisch nodle, 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 multicocal 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 moyamoya disease with neurofibromatosis in Korean literature.
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KEYWORD
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