内頸動脈閉塞と悪性星細胞腫を合併したneurofibromatosisの1例

概要

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A 38-year-old female presented with headache and weakness of the left upper extremity. At the age of 9 years she had had several episodes of transient left hemiparesis, which had apparently been induced by crying. Her mother and one of her siblings had documented neurofibromatosis. Physical examination on admission disclosed cafe au lait spots and fibroma molle. Neurological examination revealed mild memory disturbance, bilateral papilledema, left hemiparesis, and left hemihypesthesia. Computed tomography showed a high-density mass between the frontal horns and dilatation of the lateral ventricle with periventricular low density. Cerebral angiography demonstrated occlusion of the right internal carotid artery and abnormal vascular networks resembling those of moyamoya disease. A diagnosis of neurofibromatosis with occlusion of the right internal carotid artery and malignant astrocytoma was established. Cerebrovascular lesions are uncommon in patients with neurofibromatosis. Only 34 cases of neurofibromatosis with occlusive cerebrovascular lesions have been reported in the literature. The clinical features and angiographic findings of this syndrome are discussed, with special reference to moyamoya disease.
日本脳神経外科学会の論文
1987-12-15