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Sturge-Weber二氏综合征是先天性疾患,似属显性遗传。临床上以皮肤血管痣、搐搦发作、精神症状、眼球突出、痉挛性偏瘫及半身萎缩为特征。颅骨片于枕部示片状或双层波状钙化灶。气脑造影示相应侧大脑半球脑沟增宽及脑室扩大。病理方面皮肤及脑膜有血管痣,脑皮质有钙化灶,神经细胞减少及胶质细胞增生。脑电图常有一侧波幅降低或尖波慢波灶。神经精神症状系脑膜血管瘤压迫所致。本病散见于世界各种族。作者将1936～1978年所见之22例作了临床分析。并附3例病历摘要。
Sturge-Weber’s syndrome is a congenital disorder that seems to be dominantly inherited. Clinically, vascular nevus, convulsions attack, mental symptoms, prominent eye, spastic hemiplegia and half body atrophy characterized. Skull piece in the occipital showed flake or double corrugated calcification. Cerebral angiography showed the corresponding side of the cerebral hemisphere sulci widening and ventricular enlargement. Pathological aspects of the skin and meninges vascular nevus, calcification of the cerebral cortex, decreased nerve cells and glial cell proliferation. EEG side of the volatility is often reduced or sharp wave slow wave stove. Neuropsychiatric symptoms caused by meningeal angioma compression. The disease scattered in all races in the world. The authors made a clinical analysis of 22 cases seen in 1936-1978. Attached 3 cases of medical records summary.