Epilepsy Research Centre, Department of Medicine, The University of Melbourne, Austin Health, Heidelberg, Victoria, Australia.
Epilepsy Research Centre, Department of Medicine, The University of Melbourne, Austin Health, Heidelberg, Victoria, Australia; Department of Paediatrics, The University of Melbourne, Parkville, Victoria, Australia; The Florey Institute of Neuroscience and Mental Health, Heidelberg, Victoria, Australia; Department of Neurology, Royal Children's Hospital, Parkville, Victoria, Australia.
Eur J Paediatr Neurol. 2018 May;22(3):532-535. doi: 10.1016/j.ejpn.2017.12.003. Epub 2017 Dec 19.
Epilepsy with myoclonic absences is a rare generalized epilepsy syndrome with distinctive seizures. Two unrelated children had mild developmental impairment and onset of myoclonic-absences at 3 and 8 years. Seizures were characterized by bilateral 3 Hz myoclonic jerks superimposed on tonic abduction of the upper limbs. Events lasted 10-60 s, and complex gestural automatisms were often observed; in one case, a boy undid his seatbelt and attempted to exit a moving vehicle. Post-ictally, both children immediately regained awareness without recollection of their actions. Ictal EEGs showed 3 Hz generalized spike-wave. Complex automatisms have not been described in myoclonic absence seizures. This generalized seizure type can be confused with focal seizures when these ictal behaviours occur.
肌阵挛失神癫痫是一种罕见的具有特征性发作的全面性癫痫综合征。两名无关联的儿童存在轻度发育障碍，分别在 3 岁和 8 岁时出现肌阵挛失神发作。发作表现为双侧 3Hz 肌阵挛性抽搐，上肢呈强直性外展。发作持续 10-60s，常伴有复杂的姿势性自动症；在其中一例中，男孩解开安全带并试图离开正在行驶的车辆。发作后，两名儿童均立即恢复意识，对其行为无记忆。发作期脑电图显示 3Hz 全面性棘慢波。在肌阵挛失神发作中尚未描述复杂的自动症。当这些发作行为出现时，这种全身性发作类型可能与局灶性发作相混淆。
