Department of Clinical Neurophysiology, Necker-Enfants Malades Hospital, APHP, Paris, France.
Reference Center for Rare Epilepsies, Department of Pediatric Neurology, Necker-Enfants Malades Hospital, APHP, Paris, France, Department of Pediatric Neurology, Necker-Enfants Malades Hospital, APHP, Paris, France.
Epileptic Disord. 2021 Feb 1;23(1):191-200. doi: 10.1684/epd.2021.1240.
Epileptic myoclonus (EM) is reported in many paediatric epilepsies from neonatal period to adolescence. Myoclonus can be the only seizure type or may occur among others, independently or in combination as a single ictal event. We report two children presenting with absences associated with myoclonus, predominating on one side, in a setting of two different types of absence seizures and two different electro-clinical syndromes. Patients were explored with long-duration video-EEG coupled to surface EMG polygraphy. EEG was visually analysed and complemented by jerk-locked back-averaging. Two types of seizure, encompassing myoclonus and absence, were identified: myoclonic absences in the context of epilepsy with myoclonic absences and atypical absences with atonic component (negative myoclonus) in the context of encephalopathy related to status epilepticus during slow sleep (ESES). In the latter case, rhythmic upper limb jerking, mimicking positive myoclonus, corresponded to recovery of muscular tone after each negative myoclonus. Due to the rhythmic recovery of muscle tone, subsequent rhythmic negative myoclonus may exhibit a similar clinical picture to that of rhythmic positive myoclonus. Video-EEG recording coupled to EMG polygraphy is essential in order to precisely characterize motor manifestations during seizures with myoclonus [Published with video sequences].
癫痫性肌阵挛（EM）在新生儿期至青春期的许多儿科癫痫中均有报道。肌阵挛可为唯一的发作类型，也可独立或以单一发作事件的形式与其他类型发作共同出现。我们报告了两名儿童在两种不同类型的失神发作和两种不同的电临床综合征背景下出现与肌阵挛相关的失神发作，且肌阵挛主要发生在一侧。通过与表面肌电图描记仪相连的长程视频-EEG 对患者进行了探索。对 EEG 进行了视觉分析，并通过抽搐锁定后平均补充分析。确定了两种包含肌阵挛和失神的发作类型：在癫痫伴肌阵挛性失神和非典型失神伴无力成分（负性肌阵挛）的背景下，在与癫痫持续状态相关的慢睡眠期（ESES）的脑病背景下发生肌阵挛性失神。在后一种情况下，模仿阳性肌阵挛的上肢节律性抽搐对应于每次负性肌阵挛后肌肉张力的恢复。由于肌肉张力的有节奏恢复，随后的有节奏的负性肌阵挛可能表现出与有节奏的阳性肌阵挛相似的临床特征。视频-EEG 记录与肌电图描记仪相结合对于精确描述伴有肌阵挛的发作期间的运动表现至关重要[附有视频序列]。
