结节性硬化症患儿癫痫预后的危险因素分析

Risk factoks for epileptic prognosis in children with tuberous sclerosis

目的探讨影响结节性硬化症(TSC)患儿进展为难治性癫痫的危险因素。方法根据近1年内有无癫痫发作及药物控制情况,将83例合并癫痫的TSC患儿分为难治性癫痫组和癫痫控制组,比较两组患儿的临床资料、脑电图(EEG)和头颅MRI检查差异,并通过多因素Logistics回归分析确定影响难治性癫痫进展的独立危险因素。结果与癫痫控制组患儿相比,难治性癫痫组患儿中,癫痫首次发作年龄<2岁、新生儿癫痫、智商评分<70、婴儿痉挛史、合并林-戈综合征、自闭症、癫痫持续状态、使用抗癫痫药物≥3种的患儿显著升高。而难治性癫痫组患儿的EEG呈全面型的患儿比例显著高于癫痫控制组,磁共振(MRI)显示皮质结节≥3个的患儿比例也显著高于癫痫控制组(P<0.001)。多因素Logistics回归分析显示癫痫首次发作年龄<2岁(OR=1.243,95%CI:1.114~1.672,P=0.036)、合并自闭症(OR=3.892,95%CI:1.241~9.351,P=0.008)和大脑皮质结节≥3个(OR=1.905,95%CI:1.101~3.288,P=0.021)是影响难治性癫痫的独立危险因素。结论癫痫首发年龄小于2岁、合并自闭症和脑皮质结节数目超过3个是影响TSC患儿难治性癫痫进展的独立危险因素,对于此类TSC患儿应早期采取干预措施以预防或延缓难治性癫痫的进展。

Objective To investigate the risk factors for affecting refractory epilepsy development in children with tuberous sclerosis complex(TSC).Methods 83 children with TSC and epilepsy was divided into refractory epilepsy group and epilepsy control group according to the presence or absence of seizures and drug control in the past year. The clinical characteristics, the results of EEG and brain MRI examination of the two groups were compared, and independent risk factors affecting refractory epilepsy development were determined by multivariate Logistic regression analysis. Results Compared with children in the epilepsy control group, the proportion of patient with the first episode of seizures less than 2 years old, neonatal epilepsy, IQ score <70, infantile spasm, combined Lennox-Gastaut syndrome, autism and status epilepticus, and the use of anti-epileptic drugs ≥ 3 species in refractory epilepsy group were significantly elevated. In the refractory epilepsy group, the general findings in electroencephalogram(EEG) was significantly higher than that of the epilepsy control group, and cortical tubers ≥ 3 in MRI was also significantly higher than that in the epilepsy control group, both with statistical significance(P<0.001). Multivariate Logistic regression analysis showed that the first episode of seizure less than 2 years old(OR=1.243, 95% CI: 1.114-1.672, P=0.036), and autism(OR=3.892, 95% CI: 1.241-9.351, P=0.008) and cortical tubers ≥ 3(OR=1.905, 95% CI: 1.101-3.288, P=0.021) were independent risk factors for refractory epilepsy development. Conclusion Epileptic seizures younger than 2 years old, accompained with autism and cortical tubers over three are independent risk factors for refractory epilepsy development in children with TSC. Early intervention should be taken to prevent or delay refractory epilepsy development in TSC patients with these risk factors.