de Saint-Martin Anne, Rudolf Gabrielle, Seegmuller Caroline, Valenti-Hirsch Maria Paola, Hirsch Edouard
Pediatric Neurology, Department of Pediatrics, University Hospital of Strasbourg, Strasbourg, France; Referent Center for Rare Epilepsies, Associated, Strasbourg, France.
Epilepsia. 2014 Aug;55 Suppl 3:21-3. doi: 10.1111/epi.12724.
Epileptic encephalopathy with continuous diffuse spike-waves during slow-wave sleep (ECSWS) presents clinically with infrequent nocturnal focal seizures, atypical absences related to secondary bilateral synchrony, negative myoclonia, and atonic and rare generalized tonic-clonic seizures. The unique electroencephalography (EEG) pattern found in ECSWS consists of continuous, diffuse, bilateral spike-waves during slow-wave sleep. Despite the eventual disappearance of clinical seizures and EEG abnormalities by adolescence, the prognosis is guarded in most cases because of neuropsychological and behavioral deficits. ECSWS has a heterogeneous etiology (genetic, structural, and unknown). Because epilepsy and electroencephalography (EEG) abnormalities in epileptic encephalopathy with continuous diffuse spike-waves during slow-wave sleep (ECSWS) are self-limited and age related, the need for ongoing medical care and transition to adult care might be questioned. For adolescents in whom etiology remains unknown (possibly genetic) and who experience the disappearance of seizures and EEG abnormalities, there is rarely need for long-term neurologic follow-up, because often a relatively normal cognitive and social evolution follows. However, the majority of patients with structural and possibly "genetic syndromic" etiologies will have persistent cognitive deficits and will need suitable socioeducative care. Therefore, the transition process in ECSWS will depend mainly on etiology and its related features (epileptic active phase duration, and cognitive and behavioral evolution) and revolve around neuropsychological and social support rather than medical and pharmacologic follow-up.
慢波睡眠期持续弥漫性棘波的癫痫性脑病(ECSWS)临床上表现为夜间偶发局灶性发作、与继发性双侧同步相关的非典型失神发作、负性肌阵挛、失张力发作以及罕见的全身强直阵挛发作。ECSWS中发现的独特脑电图(EEG)模式包括慢波睡眠期持续、弥漫、双侧性棘波。尽管临床发作和EEG异常在青春期最终会消失,但由于神经心理和行为缺陷,大多数病例的预后仍不容乐观。ECSWS病因多样(遗传、结构及不明原因)。由于慢波睡眠期持续弥漫性棘波的癫痫性脑病(ECSWS)中的癫痫和脑电图(EEG)异常具有自限性且与年龄相关,因此可能会质疑是否需要持续的医疗护理以及向成人护理的过渡。对于病因不明(可能为遗传性)且发作和EEG异常消失的青少年,很少需要长期的神经科随访,因为通常随后会有相对正常的认知和社会发展。然而,大多数具有结构性病因以及可能“遗传综合征性”病因的患者将存在持续的认知缺陷,需要适当的社会教育护理。因此,ECSWS的过渡过程将主要取决于病因及其相关特征(癫痫活动期持续时间、认知和行为发展),并围绕神经心理和社会支持而非医疗和药物随访展开。