肌阵挛失神性癫痫  

Epilepsy with myoclonic absences

作　　者：黎冰梅[1] 廖卫平[1] 易咏红[1] 何小诗[1] 王剑威[1] 

机构地区：[1]广州医学院神经科学研究所

出　　处：《罕少疾病杂志》2004年第4期25-27,共3页Journal of Rare and Uncommon Diseases

摘　　要：目的 探讨肌阵挛失神性癫痫的临床表现、脑电图特点、治疗及预后。 方法 对1例肌阵挛失神性癫痫患者的临床表现、发作间期、发作期及睡眠脑电图监测结果进行研究和随访。 结果 患者起病年龄为7岁,发作表现为失神伴严重的双侧节律性阵挛,常有强直性收缩。脑电图的特点是总伴随着双侧同步、对称、节律性的3 Hz棘慢波发放。患儿起病以后智能逐渐减退,丙戊酸钠加氯硝安定能控制发作。 结论 肌阵挛失神性癫痫是一种临床特征明确的癫痫综合征,临床上应重点与儿童失神性癫痫鉴别。Objective To explore the clinical manifestations, characteristics of EEG, treatment and prognosis of epilepsy with myoclonic absences. Methods The clinical data, interictal, ictal and sleep EEG of a case of epilepsy with myoclonic absences were studied and followed-up. Results The onset age of the patient was 7 years old. The seizure was characterized clinically by absences accompanied by severe bilateral rhythmical myoclonias, often associated with a tonic contraction. Ictal EEG always showed bilateral, synchronous and symmetrical discharge of rhythmical SW at 3Hz. The seizures were controlled under the treatment with VPA CR and clonazepam. Conclusion Epilepsy with myoclonic absences is a epileptic syndrome of definite clinical characteristics. It should be differentiated from childhood absence epilepsy in clinic.

关 键 词：肌阵挛失神性癫痫 痫性发作 儿童失神性癫痫 肌阵挛 脑电图 

分 类 号：R742.1[医药卫生—神经病学与精神病学] R741.044[医药卫生—临床医学]