睡眠相关过度运动性癫痫的研究进展

睡眠相关过度运动性癫痫（SHE）的主要临床表现为睡眠中发生的不对称强直或复杂的过度运动发作,且具有短暂、频繁、丛集发作的特点,其发病率约为1.8/100 000。由于SHE在睡眠中多发,易误诊为异态睡眠,临床上应注意鉴别。绝大多数SHE患者病因不明,可能的病因包括基因突变及结构异常,不同的病因可影响患者疾病严重程度及预后。SHE的治疗主要包括药物治疗和手术治疗,其中药物治疗首选卡马西平,经选择的难治性癫痫患者手术治疗预后良好。本文就SHE的诊断、发病机制、治疗及预后方面的研究进展进行综述,旨在增加临床医生对该病的认识。

Advances in sleep-related hypermotor epilepsy

Sleep-related hypermotor epilepsy (SHE), formerly known as nocturnal frontal epilepsy, is characterized by asymmetrical tonic or complex hypermotor seizures during sleep, with transient, frequent and clustering attack. The accurate incidence is not known but somehow low, which is estimated about 1.8/100 000. The differential diagnosis between SHE and parasomnias may be challenging due to possible similarities between the two sleep-related manifestations. In a majority of patients, the etiology is unknown. Identified etiologies are heterogeneous and structural abnormalities, which are involved in the severity and prognosis of SHE. In terms of treatment, it mainly includes pharmacological therapy and surgery. Carbamazepine seems to be the drug of choice in SHE patients, and epilepsy surgery provides excellent results in selected drug-resistant SHE cases. This review will focus on diagnosis, pathogenesis, treatment and prognosis of SHE, aiming to promote its early diagnosis and appropriate treatment.