失张力发作:一独立的癫癎类型或癫癎综合征?

Atonic seizures: An independent epilepsy type or epilepsy syndrome?

目的：探讨失张力发作的临床特点及治疗。方法：对２例患者的临床表现、辅助检查及对抗癫 药物治疗的反应进行观察。结果：２例均儿童起病，以突发短暂全身肌张力丧失伴神志不清为唯一临床表现，不伴智能损害及其它类型的癫 发作，发作期 ＥＥＧ：１例为３～４ Ｈｚ棘慢波、多棘慢波，另１例为低幅快波伴脑电抑制。发作间期ＥＥＧ及头颅ＣＴ均正常。单用鲁米那治疗均显效而丙戊酸钠无效。结论：该２例仅表现为失张力发作的患者临床特点有别于Ｌｅｎｎｏｘ－Ｇａｓｔａｕｔ综合征和Ｄｏｏｓｅ综合征。此２例病例可否独立诊断为“失张力性癫 ”或“失张力性癫 综合征”，值得探讨。

Objectives: To explore the clinical features and treatment of epilepsy with atonic seizures. Methods The clinical manifestation, auxiliary test and the response to antiepileptic drugs were observed in two patients with only atonic seizures. Results: Both patients were childhood-onset, only showing sudden loss of muscles tone with loss of consciousness accompanied by neither intelligence disturbance nor other form of seizures. Ictal EEG showed generalized synchronous 3-4Hz spike and waves or multispike and waves in one of the patients, and fast waves with low ampiltude in the other. Both interictal EEG and CT scan were normal. Monotherapy with luminal showed significant effect in the two patients while valprate showed no effect. Conclusions: The clinical manifestation of atonic seizures in the two patients appeared to be characteristic and different from those of atonic seizures in Lennox-Gastaut syndrome and Doose syndrome. Our findings suggest it might be worth further exploring that if these patients with only atonic seizures could be regarded as an independent diagnosis of epilepsy or epileptic syndrome.