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结节性硬化症是以痉挛发作、智能障碍、皮脂腺腺瘤为三大特征的遗传性疾病。本病在大脑皮质及脑室壁有多数结节性病变,出现异型神经胶质细胞和异型神经细胞,伴有钙质沉着,X线检查时颅内有钙化。本文讨论智能障碍程度与痉挛发作,脑电图所见及临床症状之间的关系从附表可见仅病例9因年幼,尚未出现皮脂腺腺瘤。8例在1岁内有痉挛发作,1例1岁半有痉挛发作。初发时有全身性痉挛发作4例,有点头癫痫4例,有局灶型癫痫1例。在病程中发作类型有变化者4例,有癫痫持续状态者6例,其中反复癫痫持续者3例。 CT所见:9例皆有脑内钙化,钙化数为3～13个,大小为3～12mm,形状为圆形、卵圆形或不整
Tuberous sclerosis is a genetic disorder characterized by seizures, mental retardation and sebaceous adenomas. The disease in the cerebral cortex and ventricular wall has the most nodular lesions, there is abnormal glial cells and dysplasia, accompanied by calcific, X-ray examination intracranial calcification. This article discusses the degree of mental retardation and spasm seizures, EEG seen and the relationship between clinical symptoms can be seen from the table only 9 cases of young patients, has not yet appeared sebaceous adenoma. Eight patients had seizures within 1 year of age, and 1 patient had seizures at 1 year and a half. Initial episodes of systemic seizures in 4 cases, a little head epilepsy in 4 cases, 1 case of focal epilepsy. In the course of the onset of seizure type in 4 cases, 6 cases of status epilepticus, including 3 cases of persistent epilepsy. CT seen: 9 cases of intracranial calcification, the calcification number of 3 to 13, the size of 3 ~ 12mm, the shape of a round, oval or irregular