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目的:探讨儿童结节性硬化症(TSC)并发神经系统损伤的相关临床特点。方法:回顾性分析29例TSC患儿的临床资料。结果:选择29例TSC患儿,随访时间为4个月～5年。有癫痫发作者占93.10%,主要表现为婴儿痉挛13例,强直性发作5例,强直-阵挛性发作5例,部分运动性发作2例,失神发作2例;智力障碍者19例,占65.52%,其中轻度10例,中重度9例;癫痫发作仍存在的患儿,其中重度智力障碍发生率明显增高,合并孤独症行为者亦增多。患儿头颅CT、MRI及脑电图检查有异常。结论:TSC神经系统损伤以癫痫发作和智力损伤为主,加强对TSC患儿神经系统症状进行有效的治疗可能是TSC患儿智能改善及提高生存质量的主要手段。
Objective: To investigate the clinical features of children with tuberous sclerosis (TSC) complicated by nervous system injury. Methods: A retrospective analysis of 29 cases of TSC in children with clinical data. Results: Twenty-nine TSC patients were selected and the follow-up time ranged from 4 months to 5 years. There were seizures accounted for 93.10%, mainly in 13 cases of infantile spasms, 5 cases of tonic episodes, 5 cases of tonic-clonic seizures, 2 cases of partial seizures, 2 cases of absence seizures; 19 cases of mental retardation, accounting for 65.52%, of which mild in 10 cases, moderate and severe in 9 cases; seizures still exist in children, including the incidence of severe mental retardation increased significantly, with autism increased. Children with skull CT, MRI and EEG abnormalities. CONCLUSIONS: TSC-induced nervous system injury is mainly caused by seizures and intellectual impairment. Strengthening the effective treatment of neurological symptoms in children with TSC may be the primary means of improving intelligence and improving quality of life in children with TSC.