Electrical status epilepticus is a special electroencephalogram phenomenon,which means that the spike and slow waves are almost continuously emitted during the wake-sleep phases.Related concepts are epileptic encephalopa-thy with electrical status epilepticus during slow wave sleep,electrical status epilepticus in sleep,and subclinical electro-graphic seizures.The above related concepts are widely used in clinical practice,but there is a lack of unified criteria.There are abuses and misuses of these concepts.Clarifying related concepts is of great significance for scientific research and clinical practice.

Objective:To investigate the clinical and electrophysiological characteristics of patients with sudden unexpected death of epilepsy (SUDEP).Methods:Using "epilepsy" as the keyword, the relevant cases entered from October 2011 to March 2012 were searched in the database of the Electroencephalogram (EEG) Monitoring Center, Xijing Hospital, the Air Force Military Medical University. Telephone follow-up was conducted for all confirmed epilepsy patients, and for the death cases confirmed by telephone follow-up, the patients identified as consistent with SUDEP diagnosis were included in this study based on their past medical history, clinical data, death details, etc, and their clinical and neuroelectrophysiological characteristics were summarized and analyzed.Results:Among the 1 232 patients who underwent 24-hour video-EEG monitoring during the study period, 354 patients were successfully followed up by telephone interview, of whom 17 patients were died (4.8%), 12 individuals met the diagnosis of SUDEP (7 men, 5 women). The duration of the disease in 9 patients exceeded 10 years. Eight cases presented with focal-bilateral tonic clonic seizures. Nine patients were treated with anti-seizure drug monotherapy. All the 24-hour video EEG of 12 patients were abnormal. There were 8 occasions when the EEG occipital α background rhythm slowed down compared with the standard frequency of peers or was dominated by slow waves. Interictal epileptic discharge (IED) located in temporal lobe were found in 12 EEG records, of which 9 EEG records were found with frontal IED. One of the 12 cases received 24-hour video EEG twice within 6 years, and his EEG background rhythm was significantly slower and the IED region was expanded compared with the first EEG record. At the third year after reexamination of EEG, SUDEP developed in this patient.Conclusions:SUDEP patients have a long course of disease and bilateral tonic-clonic seizure. The interictal EEG shows occipital slow α activity and temporofrontal epileptiform discharges, which may increase the risk of SUDEP.

Objective:To explore the electro-clinical characteristics of sleep-related hypermotor epilepsy (SHE) in rapid eye movement (REM) stage.Methods:Five patients of SHE in REM stage were studied and followed up in the Electroencephalogram Monitoring Center, Department of Neurology, Xijing Hospital, the Air Force Military Medical University, from January 2016 to August 2021.Results:Among the 5 patients, there are 3 male patients, aged 21 to 46 years. A total of 23 seizures were monitored in 5 patients, of which 22 occurred in REM sleep and 1 occurred in non-REM Ⅲ sleep. Each attack lasted from 30 seconds to 1 minute, and was manifested as "hyperkinetic attack" during sleep, with or without disturbance of consciousness. There were no obvious abnormalities in electroencephalography during 13 attacks, with the focal sharp slow waves or slow waves during 9 attacks, and the focal slow waves occurrence at the end of the 10 attacks.Conclusion:Most of the hypermotor epileptic seizures in REM stage started from awakening reaction, and the interictal discharges occured in waking and non-REM sleep stage, which is necessary to distinguish from the REM sleep behavior disorder.

The concepts and diagnostic criteria such as "migralepsy" and "ictal epileptic headache" have been proposed by International Headache Society in succession, and have been widely used in clinical practice. However, the authors believe that these diagnostic criteria are worth discussing. Headache can be a symptom of epileptic seizures, or be a state after seizures. Migraine and epilepsy can be comorbid, while migralepsy may be a form of focal epilepsy essentially from the professional perspectives of epilepsy. All medical points of views and thoughts need to be focused, so the sub-professional groups of epilepsy and headache should strengthen the dialogue and consultation, in order to formulate the diagnostic criteria of epilepsy and migraine scientifically.

Objective:To investigate the clinical characteristics and electroencephalogram (EEG) of epilepsy patients with breach rhythm, improve clinical understanding of breach rhythm and avoid over-interpretation.Methods:Twelve epilepsy patients with breach rhythm who visited the Department of Neurology, Xijing Hospital, the Air Force Military Medical University from January 2016 to January 2017 were collected retrospectively. The clinical data, including etiology, clinical manifestations, EEG features and prognosis were summarized, and outpatient and telephone follow-up was performed for at least three years.Results:The clinical data of 12 patients with epilepsy with breach rhythm were collected, including eight males and four females, aged 36-78 years. After analysis, it was found that brain trauma was the most common cause of breach rhythm. Among them, two cases of skull defect were not repaired, eight cases were repaired with skull titanium mesh, one case was repaired with skull polymethylmethacrylate, and one case was repaired with skull polyetheretherketone. The distribution of the breach rhythm in 12 patients was consistent with the abnormal area of the skull. The breach rhythm can be expressed as high amplitude and fast frequency, or low amplitude and slow frequency and appear individually (similar to sharp waves, spikes). On the basis of pleomorphic slow waves, 10 patients were mixed with sharp waves and spike waves, and imaging confirmed that they had brain damage in corresponding parts. All of the 12 patients had a history of seizures, with tonic-clonic seizures and (or) focal seizures being the most common. Three patients with breach rhythm had no clinical seizures for more than five years, and had been taking antiepileptic drugs for epileptic spikes on EEG, and they were given reduction and discontinuation of the drugs and were seizure-free for three years during follow up.Conclusions:Skull repair is a common cause of breach rhythm, and repair materials with different resistances cause different waveforms and frequencies. Breach rhythm, epileptiform discharge and other pathological slow-wave activities can exist at the same time. Breach rhythm is a benign variant phenomenon which needs no special treatment.

癫痫是临床上最常见的神经系统疾病之一,流行病学调查显示,活动性癫痫的平均患病率为7.2‰.大多数癫痫患者的寿命不受影响,但由于癫痫 猝 死(sudden unexpected death in epilepsy, SUDEP)的存在,癫痫也是一种潜在致死性疾病[1].SUDEP是指癫痫患者突然发生的、意外的、有或无目击者、非外伤或溺水所致的死亡,伴或不伴癫痫发作,必须排除癫痫持续状态,尸检未发现结构性或中毒性致死因素[2].SUDEP是癫痫的致命并发症,占癫痫患者死亡的7.5％～17％[3].一项有关SUDEP的回顾性研究认为,呼吸心跳骤停多发生在癫痫发作后的1～3 min内,如在此时间内给予有效的心肺复苏,即可降低SUDEP发生风险[4].患者在心跳/呼吸骤停后成功复苏,该事件被归类为Near-SUDEP.现结合文献,对我科在长程视频脑电监测过程中出现的一例Near-SUDEP事件进行回顾性分析,以强化对SUDEP事件的认识,探讨预防处理方法.

Objective:To explore the electro-clinical characteristics of Jeavons syndrome complicated with non-convulsive status epilepticus (NCSE).Methods:Three patients of Jeavons syndrome complicated with NCSE during electroencephalogram (EEG) monitoring in the EEG Monitoring Center, Department of Neurology, Xijing Hospital in 2018 were studied and followed up.Results:Among the three patients, there are two female patients, one male patient, aged 12 to 24 years. Generalized spike-wave discharges and impaired consciousness were recorded during video-EEG monitoring, which lasted for 8 minutes to 6.5 hours and evolved in generalized tonic clonic seizure (GTCS), and did not recur after receiving correct anti-epileptic drugs.Conclusion:NCSE can also occur in Jeavons syndrome, and NCSE in patients with Jeavons syndrome ends with GTCS, suggesting that early detection and timely termination of NCSE in patients with Jeavons syndrome may avoid the occurrence of GTCS.

Objective:To discuss the clinical and electrophysiological characteristics of familial cortical myoclonic tremor with epilepsy (FCMTE) with fixation-off sensitivity (FOS).Methods:The clinical and electrophysiological characteristics of four patients diagnosed as FCMTE with FOS in the Electroencephalography (EEG) Monitoring Center of Xijing Hospital from May 2016 to December 2017 were studied and followed up.Results:The four patients were all female. The age was ranged from 29 to 67 years. The course was from six to 30 years, and the follow-up time was at least two years. The tremors and jerks occurred to the four patients frequently when the eyes were closed, which prevented their falling a sleep, and three of them had generalized tonic-clonic seizure occasionally. The FOS was monitored in the all four patients, and the photosensitivity occured to the three of them.Conclusions:The fixation-off sensitive trail during EEG monitoring is helpful to find the FCMTE with FOS. It is necessary to determine the potential clinical significance of FOS and photosensitivity coexisting in patients with FCMTE.

新年伊始,Dorothee Kasteleijn-Nolst Trenite 教授向我推荐了她的新著——《光敏性在癫痫中的重要性》.这是由多位作者共同合作出版的综合性著作,我非常荣幸的阅读了此书,书中内容几乎涉及光敏性的所有方面.尽管过去已经有光敏性癫痫的专著,但据我所知,目前尚无一本由世界各地多位临床医生、科学家和癫痫专家分享关于光敏性经验的著作.

目的 探索B超引导下胎儿脑电监测方法.方法 通过招募志愿者,对18例经B超确定胎儿头部紧贴于孕妇腹壁的胎儿给予脑电监测,B超引导下进行盘状电极安放及脑电信号采集.通过自身对照及健康志愿者对照等方法确定采集到的为胎儿脑电信号.出生后进行随访观察脑发育情况.结果 孕妇月龄越大,胎儿EEG波形越接近新生儿EEG;同部位胎儿脑电功率图空间分布与早产儿及新生儿头皮脑电功率图空间分布相同.随访时间为3～5 y,1例引产,另17例均在随访中,现年龄3岁6个月～4岁6个月,目前没有发现脑发育异常.结论 B超引导下胎儿脑电监测具有可行性,需要更大的样本及相关实验进一步验证采集的信号确定为脑电信号,为未来建立胎儿脑电图图谱提供可靠方法.

Objective:To discuss the clinical and electrophysiological characteristics of propriospinal myoclonus (PSM).Methods:The clinical and electrophysiological characteristics of four patients diagnosed as PSM in the Electroencephalography Monitoring Center, Department of Neurology, Xijing Hospital, the Air Force Military Medical University from April 2018 to July 2019 were studied. All patients were accorded with diagnostic criteria of PSM that international classification of sleep disorders-3 edition recommended and were followed up.Results:There were three males and one female in the four patients. The age of onset was ranged from 43 to 55 years. The course was from eight months to three years, and the follow-up time was from three months to one year. The clinical features of the four patients were characteristically paroxysmal tic or shaking of the neck, trunk or limbs, with short duration and great frequency. All patients accepted 24-hour video electroencephalography monitoring. No epileptic discharge was recorded during the monitoring. The burst activity of deltoid, quadriceps or rectus abdominis muscle was monitored by surface electromyography at the onset of myoclonus. All patients were treated with clonazepam. Three patients had an obvious curative effect and one patient had no effect.Conclusions:The clinical manifestation of PSM is similar to seizures. There is no epileptic discharge, and only the burst activity of muscles is monitored at the onset. Most patients have significant effect on clonazepam.

目的 总结桥本脑脊髓病的临床特点,探讨非惊厥性癫痫持续状态的诊治标准.方法 报道1例以"反复发作性精神行为异常"为主诉患者的临床诊治经过,文献检索桥本脑脊髓病和NCSE的研究进展.结果 该例患者表现为反复发作性精神行为异常,同步脑电图可见弥漫性慢波背景,左侧前额、额、前颞、中线较多量单发尖慢复合波及三相波发放;给予静脉推注地西泮处理,临床症状无明显改善,EEG频率较背景脑波节律有增快.进一步查甲状腺过氧化物酶抗体(TPO-Ab)>1300.00U/ml(正常值:0-78U/ml),甲状腺球蛋白抗体(TG)>500.00U/ml(正常值:0-60U/ml).综合临床表现及相关检查结果,诊断考虑"桥本脑脊髓病",给予糖皮质激素治疗后临床症状及异常脑电均明显好转.结论 对于反复发作性精神行为异常的患者,即使同步脑电图出现了不同于背景的变化,NCSE不是唯一的需要考虑的诊断,尤其是对地西泮静推效果不好时,积极寻找病因,避免误诊及漏诊.

目的 探讨肌阵挛失神癫痫(epilepsy with myoclonic absences,EMA)的临床和电生理特点.方法 对我院脑电监测中心2018-2019年收治的3例EMA患者的临床和电生理特点进行总结和随访研究.结果 3例患者均为男性.2例儿童期起病,1例成年起病.1例表现为肌阵挛失神和全面性强直阵挛发作,2例表现为肌阵挛失神发作.3例发作期脑电图异常放电均不是标准的3Hz棘慢复合波,发作期棘慢复合波频率波动在2.5～3.5 Hz.2例间断闪光刺激诱发试验阳性,其中1例表现为光阵发性反应(1～60 Hz阳性),1例表现为光阵发性反应及光惊厥性反应.睁闭眼试验3例均为阴性.过度换气试验2例阳性,1例阴性.治疗给予丙戊酸钠缓释片,2例有效,1例仍有发作.结论 EMA多见于儿童,但也有较大年龄发病者.以肌阵挛失神为主要发作类型.EMA发作期脑电图可以是2.5～3.5 Hz棘慢复合波.EMA患者光敏性可为低频、高频均敏感.其肌阵挛失神发作容易被过度换气所诱发.治疗使用丙戊酸钠或丙戊酸钠联合拉莫三嗪,通常有效,存在认知功能损害,或伴随其他发作,特别是全面性强直阵挛发作的患者,可能预后不良.

Breach rhythm was firstly described in 1979, and is considered as a rare benign variant of electroencephalogram. The etiology, electroencephalogram features and evaluation of breach rhythm, and so on, are discussed in this article.

Objective To investigate the electro-clinical features of epilepsy with eye closure sensitivity (ECS).Methods The electroencephalograph database was searched using ECS during half a year period from January to June 2017 in Xijing Hospital.The duration of the follow-up was one year.Results Fifty-one patients diagnosed as epilepsy with ECS were investigated.Patients were classified into four epilepsy syndromes:33 with eyelid myoclonia with absences (EMA);13 with juvenile myoclonia epilepsy (JME);two with epilepsy with generalized tonic-clonic seizure on awakening and three with idiopathic occipital lobe epilepsy (IOE).The duration of the epileptiform discharges (EDs) triggered by eye closure (ECL) lasted more than five seconds in four patients with EMA and one patient with IOE.The EDs triggered by ECL were more frequent (85.2± 11.4 vs 37.5± 12.6,t=12.399,P=0.000) and lasting longer ((4.3± 1.9) s vs (2.3±0.8) s,t=3.585,P=0.001) in EMA than in JME.Conclusions ECS is common in EMA.The frequency and duration of the EDs triggered by ECL are helpful for identifying EMA and JME.

Objective To investigate the electroclinical,treatment response and prognosis of juvenile absence epilepsy (JAE).Methods Thirty-two patients diagnosed as JAE from Epilepsy Center of Xijing Hospital between January 2014 and August 2017 were investigated.All of them underwent 24-hour video-electroencephalography (EEG) recording.Clinical aspects,electroencephalographic features,and antiepileptic drugs (AEPs) received were reviewed.The follow-up time was 6-44 months,with an average of (18.6 ± 11.6) months.Results Five patients (15.6％) had family history of epilepsy.Four patients (12.5％) had history of febrile seizures.Twenty-six patients (81.3％) showed absence seizures,11 patients (34.4％) were hyperventilation test positive,five patients (15.6％) were intermittent flash stimulation test positive.All the 32 patients received AEDs treatments,21 cases (65.5％) with monotherapy,nine patients (28.1％) with two AEDs,two patients (6.3％) with three AEDs.The most commonly used AED was valproic acid,and the second was levetiracetam.At the end of follow-up,five patients (15.6％) had complete seizure free and 27 patients (84.4％) had poor seizure control.Conclusions It is necessary to give electroclinical study on more samples of JAE and establish detailed practical diagnostic criteria about JAE as early as possible.More than half of JAE patients seizure control were not ideal.The monotherapy of JAE was less effective than combined treatment.

Exploding head syndrome(EHS)is a rare sensory parasomnia and rarely reported in China, leading to serious misdiagnosis, mistreatment, and unnecessary testing. A detailed collection of clinical symptoms and longterm polysomnography-electroencephalography monitoring is very important for diagnosis and differential diagnosis of EHS. We diagnosed a patient with EHS according to the International classification of sleep disorders, third edition diagnostic criteria. The electro-clinical feature of the patient and relevant references were analyzed in order to improve the understanding of EHS, meanwhile to prevent unnecessary testing and avoid misdiagnosis and mistreatment.

癫痫是一种慢性神经系统疾病,随着人们对癫痫认识的不断深入,非药物治疗癫痫的方法越来越多,然而口服抗癫痫药物仍然是治疗癫痫最常用的方法. 但是对于以下问题:何时开始抗癫痫药物治疗、如何确定抗癫痫药物的服用剂量以及减停抗癫痫药物的依据等问题,临床观点不一,有些临床实践尚需进一步商榷.

Objective To investigate the clinical and electrophysiological aspects of the transient epileptic amnesia.Methods We studied the clinical and electrophysiological characteristics of 6 patients diagnosed as TEA(transient epileptic amnesia).Results The age of 6 patients was ranging from 34 to 74 years old.The seizure types of them had amnesia,automatism,sGTCS,or a brief period of unconsciousness.The epileptiform discharge in the temporal region(unilateral or bilateral)was recorded in 5 patients.The other one patient' EEG was normal.5 patients were treated with antiepileptic drugs.3 patients were seizure free for a year,and 2 patients had epileptic seizures.Conclusion TEA is often misdiagnosis or missed diagnoses.Although the majority of temporal lobe epilepsy is reported as resistant to AEDs,our study suggested that temporal lobe epilepsy with TEA responded well to AEDs.

Objective:To discuss the drug therapy of Non‐convulsive status epilepticus(NCSE)epi‐sodes .Methods:The clinical ,electroencephalographic (EEG )features and treatment of 2 patients with NCSE were analyzed .Results:The 2 paitents had the same ictal type .The ir abnormal discharges in the ic‐tal period were different and their symptoms and abnormal EEG were different to AEDs .Conclusion:The reason of different response to AEDs needs further observation ,and it may be related with the different ictal EEG of the patient with NCSE in the type of CPS .