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目的探讨结节性硬化症(tuberous sclerosis complex,TSC)的临床特征和治疗特点。方法对天津市儿童医院神经内科1995—2004年41例TSC患儿的临床表现、辅助检查及治疗进行了回顾性分析。结果41例患儿中,39例(95.1%)伴有癫发作,智力低下14例(34.1%),均有头CT/MRI特异性改变,脑电图异常31/39例(79.5%);抗癫药物治疗12/39例(30.8%)完全控制无发作,1例行室管膜下巨细胞星形细胞瘤切除术,1例行肾血管平滑肌脂瘤切除术。结论结节性硬化症临床表现多样,有较明显的年龄特征,抗癫治疗并不悲观。
Objective To investigate the clinical features and therapeutic characteristics of tuberous sclerosis complex (TSC). Methods The clinical manifestations, laboratory examinations and treatment of 41 children with TSC during 1995-2004 were retrospectively analyzed in Department of Neurology, Children’s Hospital of Tianjin. Results Of the 41 children, 39 (95.1%) had epileptic seizures and 14 (34.1%) had mental retardation. All patients had head CT / MRI specific changes. EEG abnormalities were found in 31/39 (79.5% ; Antiepileptic drug treatment in 12/39 cases (30.8%) completely controlled without seizures, 1 case of subependymal giant cell astrocytoma resection, 1 case of renal angiomyolipoma resection. Conclusion The clinical manifestations of tuberous sclerosis are diverse and have obvious age characteristics. The antiepileptic treatment is not pessimistic.