脊髓固有肌阵挛患者临床及神经电生理特征分析(附5例报告)

Clinical and nerve electrophysiological features of patients with propriospinal myoclonus:A report of 5 cases

目的总结脊髓固有肌阵挛(propriospinal myoclonus,PSM)患者的临床及神经电生理特征,提高对该病的认识。方法回顾性分析2019年7月—2021年9月就诊于我院脑电图监护中心的5例PSM患者的相关病例资料,总结其临床及神经电生理特点,并随访药物治疗的效果。结果5例患者均符合PSM诊断标准,其中男3例,女2例,均以躯体不自主肌阵挛为主要症状。PSM起病年龄28~50岁。3例为特发性PSM,2例为继发性PSM。5例患者均接受视频脑电图(V-EEG)和肌电图检查,发作期未见异常放电,仅见肌电伪差,肌电起源部位以腹部、躯干为主,肌电传导速度为1.4~13.7 m/s,肌电爆发时限为100~1000 ms。氯硝西泮等药物可以有效减少PSM患者的肌阵挛发作。结论了解PSM的临床及神经电生理特征是诊断该疾病的关键所在。V-EEG是鉴别诊断PSM的重要依据,表现为发作期无异常放电,仅可见肌电图上肌电爆发。氯硝西泮对PSM症状改善有较好的疗效。

Objective To investigate the clinical and nerve electrophysiological features of patients with propriospinal myoclonus(PSM),and to improve the understanding of this disease.[WTHX]Methods A retrospective analysis was performed for the medical records of five patients with PSM who were treated at Electroencephalogram Monitoring Center of our hospital from July 2019 to September 2021.Their clinical and nerve electrophysiological features were summarized,and they were followed up to observe the efficacy of drug treatment.[WTHX]Results All fivepatients met the diagnostic criteria for PSM,among whom there were three male patients and two female patients,with involuntary myoclonus of the body as the main symptom.The age of onset of PSM ranged from 28 to 50 years.There were three patients with idiopathic PSM and two with secondary PSM.All five patients underwent video electroencephalography(V-EEG)and electromyography examinations,and no abnormal discharges were observed du-ring the ictal period,while only electromyographic artifacts were observed.Myoelectricity mainly originated from the abdomen and the trunk,with a conduction velocity of 1.4-13.7 m/s and a burst duration of 100-1000 ms.Medications such as clonazepam could effectively reduce myoclonic seizuresin PSM patients.[WTHX]Conclusion Understanding the clinical and nerve electrophysiological features of PSM is the key to its diagnosis.V-EEG is an important basis for the differential diagnosis of PSM,characterized by the absence of abnormal discharges during the ictal period and the presence of myoclonic electromyographic bursts on electromyography.Clonazepam has a good therapeutic effect on the symptoms of PSM.