肌阵挛失张力癫痫48例治疗及预后随访

目的　总结肌阵挛失张力癫痫（MAE）的治疗、脑电图（EEG）演变及预后，为选择治疗方案及预后评估提供参考。方法　对2005年11月至2010年12月北京大学第一医院儿科收集的MAE患儿48例，随访其治疗用药、疗效、EEG演变及预后。结果　48例中，男41例，女7例。随访时间8个月至5年5个月。应用抗癫痫药物（AEDs）发作控制42例（87.5%），其中单用或联合应用丙戊酸38例，联合应用拉莫三嗪25例、左乙拉西坦10例、氯硝西泮9例、托吡酯8例。应用促肾上腺皮质激素（ACTH）发作控制2例。在应用AEDs和ACTH发作控制的44例中，36例EEG全导棘慢波、多棘慢波于病程7～49个月时消失，30例EEG于病程7～44个月时恢复正常，6例遗留背景θ节律。随访发现，有认知损伤11例（22.9%）。病程中出现癫痫持续状态、强直发作、EEG持续痫样放电及发作未控制与出现认知损伤相关（P < 0.05或0.01）。结论　丙戊酸和拉莫三嗪对MAE疗效好，ACTH对少数难治性MAE有效；MAE发作控制后，EEG全导棘慢波、多棘慢波发放首先消失，背景θ节律恢复较晚；合理选择AEDs治疗，多数MAE患儿预后良好，仅少数出现认知损伤。

The treatment and prognosis of myoclonic atonic epilepsy.

Objectives　To summarize the treatment，EEG evolution and prognosis of myoclonic atomic epilepsy（MAE），and to provide a reference for selection of treatment program and evaluation of prognosis. Methods　MAE patients were diagnosed in Pediatric Department of Peking University First Hospital from November 2005 to December 2010. The treatment effects，EEG evolutions，and prognosis of MAE patients were followed up. Results　In 48 MAE patients，41 were male and 7 were female. The patients were followed up from 8 months to 5 years and 5 months. Seizures were controlled by antiepileptic drugs（AEDs）in 42 patients （87.5%）.The AEDs included valproate （38 cases），lamotrigine （25 cases），levetiracetam （10 cases），clonazepam （9 cases），and topiramate （8 cases）.The seizures of 2 patients were controlled by adrenocorticotropic hormone（ACTH）.In 44 seizure-free patients，the interictal generalized spike and wave discharges （GSWDs） disappeared in 36 patients in their course of 7 to 49 months，the EEG returned normal in 30 patients in their course of 7 to 44 months，θ rhythm existed in 6 cases. Cognitive decline were observed in 11 cases （22.9%）. Status epilepticus，tonic seizures，uncontrolled seizures，and continuous EEG discharges were associated with cognitive decline. Conclusion　Valproate and lamotrigine are more effective on MAE compared with other AEDs.After seizures have been controlled，the interictal GSWDs disappear first，and the background θ rhythm disappear later.The majority of MAE patients obtain a better prognosis by rational choice of AEDs for the treatment.