Sparagana Steven P, Delgado Mauricio R, Batchelor Lori L, Roach E Steve
Department of Child Neurology, Texas Scottish Rite Hospital for Children, Dallas 75219, USA.
Arch Neurol. 2003 Sep;60(9):1286-9. doi: 10.1001/archneur.60.9.1286.
Epilepsy is a common neurologic complication of tuberous sclerosis complex (TSC) and it is often refractory to treatment. Therefore, treating physicians are often reluctant to discontinue antiepileptic drugs (AEDs) in individuals with TSC who have attained seizure remission. To our knowledge, seizure remission and AED discontinuation in children with TSC has not been studied.
To characterize seizure remission and AED discontinuation in children with TSC.
Retrospective medical record and neuroimaging analysis of 15 children with TSC and epilepsy who had seizure remission, with a subsequent trial of discontinuation of AED treatment.
The seizure remission rate for the group of patients with TSC and epilepsy was 14.2%. From the group of 15 patients who had a remission, the absolute relapse rate was 26.7% after a mean follow-up of 5 years 7 months. Patients with sustained remission were more likely to have normal intelligence and only a few cortical or subcortical lesions on neuroimaging.
The proportion of children with TSC and epilepsy who achieve seizure remission is small. Nevertheless, some do attain seizure remission, and AEDs may be successfully discontinued. Mild cerebral involvement is a general clinical marker for seizure remission. The relapse rate in those who have undergone a trial of discontinuation of AED therapy is comparable with the rate in the general pediatric population with epilepsy.
癫痫是结节性硬化症（TSC）常见的神经系统并发症，且往往难以治疗。因此，治疗医生通常不愿在已实现癫痫发作缓解的TSC患者中停用抗癫痫药物（AEDs）。据我们所知，尚未对TSC患儿的癫痫发作缓解及AEDs停用情况进行研究。
描述TSC患儿的癫痫发作缓解及AEDs停用情况。
对15例TSC合并癫痫且已实现癫痫发作缓解的患儿进行回顾性病历及神经影像学分析，随后进行AED治疗停药试验。
TSC合并癫痫患者组的癫痫发作缓解率为14.2%。在15例实现缓解的患者组中，平均随访5年7个月后，绝对复发率为26.7%。持续缓解的患者更有可能智力正常，且神经影像学检查仅发现少数皮质或皮质下病变。
TSC合并癫痫且实现癫痫发作缓解的患儿比例较小。然而，仍有一些患儿实现了癫痫发作缓解，且AEDs可能成功停用。轻度脑受累是癫痫发作缓解的一般临床标志。接受AED治疗停药试验的患儿复发率与一般癫痫患儿群体的复发率相当。
