基于癫痫患者SEEG的发作期双侧同步性肌张力障碍的临床研究

The clinical study of seizures presenting as bilateral synchronized dystonia based on SEEG of patients with epilepsy

目的研究癫痫患者双侧同步性肌张力障碍发作的临床及电生理特征。方法回顾性分析2016年1月1日-2022年9月1日在广东三九脑科医院及深圳市第二人民医院进行手术评估的患者,收集16例发作期表现为双侧同步性肌张力障碍的癫痫患者,均有详细的病史记录、头皮脑电图(均记录到惯常发作2~3次)、头部MRI及头部PET检查,均进行立体定向脑电图(stereoelectroencephalography,SEEG)置入(其中8例双侧植入,8例单侧植入,均记录到惯常发作2~3次)。结果发作期双侧同步性肌张力障碍多见于颞岛叶癫痫,根据SEEG监测结果,颞岛附加症9例,岛叶癫痫2例,颞叶内侧型癫痫5例,颞岛附加症及岛叶癫痫均以双侧同步性肌张力障碍为首发或早期症状,颞叶内侧型癫痫出现双侧同步性肌张力障碍继发于自动运动或复杂运动,SEEG显示产生双侧同步性肌张力障碍时颞叶内侧结构及岛叶均参与发作。结论发作期双侧同步性肌张力障碍可见于颞叶内侧型癫痫、岛叶癫痫和颞岛叶癫痫附加症,颞叶内侧结构及岛叶同时参与发作是产生双侧同步性肌张力障碍的必要条件。

Objective To investigate the clinical and electrophysiological features of seizures presenting as bilateral synchronized dystonia.Methods A retrospective analysis was performed for patients who underwent surgical evaluation in Guangdong 999 Brain Hospital and Shenzhen Second People's Hospital from January 1,2016 to September 1,2022,and 16 patients with seizures presenting as bilateral synchronized dystonia were enrolled for analysis,among whom there were 11 male patients and 5 female patients,with a mean age of 26±8.97 years and a mean age of onset of 12.6±5.9 years.All patients had detailed records of medical history,scalp VEEG(2-3 times of habitual seizures),head MRI,and heat PET examination.All patients underwent stereotactic electroencephalography(SEEG)implantation(bilateral implantation in 8 patients and ipsilateral implantation in 8 patients),with 2-3 times of habitual seizures observed in all patients.Results Seizures presenting as bilateral synchronized dystonia were often observed in temporal epilepsy,insular epilepsy,or temporal plus epilepsy,and based on SEEG monitoring results,there were 9 patients with temporal-insular plus epilepsy,2 patients with insular epilepsy,and 5 patients with medial temporal lobe epilepsy(MTLE).Bilateral synchronized dystonia was the first or early manifestation of temporal-insular plus epilepsy and insular epilepsy,and in medial temporal lobe epilepsy,bilateral synchronized dystonia was the secondary symptom of automatism or complex motor.SEEG showed that both the medial temporal lobe and the insular lobe were involved in seizures presenting as bilateral synchronized dystonia.Conclusion Seizures presenting as bilateral synchronized dystonia is often observed in temporal-insular plus epilepsy,insular epilepsy,and MTLE,and the involvement of the medial temporal lobe and the insular lobe is required for bilateral synchronized dystonia.