肌阵挛失神发作伴复杂姿势自动症。

Myoclonic absence seizures with complex gestural automatisms.

机构信息

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.

出版信息

Eur J Paediatr Neurol. 2018 May;22(3):532-535. doi: 10.1016/j.ejpn.2017.12.003. Epub 2017 Dec 19.

DOI:10.1016/j.ejpn.2017.12.003

PMID:29325826

Abstract

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.

摘要

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。