Singhal Nilika Shah, Sullivan Joseph E
Department of Neurology, University of California, P.O. Box 0114, 505 Parnassus Avenue, M-798, San Francisco, CA 94143-0114, USA.
ISRN Neurol. 2014 Jan 30;2014:619079. doi: 10.1155/2014/619079. eCollection 2014.
Continuous spike and wave during slow wave sleep (CSWS) is an epileptic encephalopathy that presents with neurocognitive regression and clinical seizures, and that demonstrates an electroencephalogram (EEG) pattern of electrical status epilepticus during sleep, as defined by the Commission on Classification and Terminology of the International League Against Epilepsy 1989. CSWS is an age-related condition, typically presenting in children around 5 years of age, with clinical seizures which progress within 2 years to a severe epileptic encephalopathy. The pathophysiology of CSWS is not completely understood, but the corticothalamic neuronal network involved in sleep patterns is thought to be involved. Genetic predisposition and injury in early development are thought to play etiological roles. Treatment strategies have involved traditional anticonvulsants, hormonal therapies, and other newer techniques. Outcomes are fair, and the thought is that earlier diagnosis and intervention preserve neurocognitive development, as in the case of other epileptic encephalopathies. Further understanding of the mechanisms of CSWS may lead to improved therapeutic options and thus outcomes of children with CSWS.
慢波睡眠期持续棘慢波(CSWS)是一种癫痫性脑病,表现为神经认知功能倒退和临床发作,且脑电图(EEG)显示睡眠期癫痫持续状态模式,这是根据国际抗癫痫联盟分类和术语委员会1989年的定义。CSWS是一种与年龄相关的疾病,通常在5岁左右的儿童中出现,临床发作在2年内进展为严重的癫痫性脑病。CSWS的病理生理学尚未完全明确,但参与睡眠模式的皮质丘脑神经网络被认为与之相关。遗传易感性和早期发育损伤被认为在病因学上起作用。治疗策略包括传统抗惊厥药物、激素疗法和其他新技术。预后尚可,人们认为与其他癫痫性脑病一样,早期诊断和干预可保留神经认知发育。对CSWS机制的进一步了解可能会带来更好的治疗选择,从而改善CSWS患儿的预后。