OBJECTIVE Continuous spikes and waves during sleep (CSWS) is an epileptic encephalopathy characterized by generalised epileptiform activity and neurocognitive dysfunction. Causes and outcome are diverse and treatment is mainly empirical. METHODS Retrospective descriptive analysis of clinical and EEG data of children with CSWS diagnosed between 1998 and 2018 at the University Hospital Heidelberg. RESULTS Ninety-five children were included with a median age at diagnosis of 5.4 years. A structural/metabolic aetiology was found in 43.2%, genetic alterations in 17.9%, while it remained unknown in 38.9%. The proportion of patients with genetic aetiology increased from 10.3% (1998-2007) to 22.8% (2008-2018). On average, each patient received 5 different treatments. CSWS was refractory in >70% of cases, steroids and neurosurgery were most effective. No difference was observed between children with CSWS or Near-CSWS (Spike-Wave-Index 40-85%). CONCLUSIONS Our cohort confirms CSWS as an age-dependent epileptic encephalopathy. Structural brain abnormalities were most frequent, but genetic causes are increasingly identified. More specific criteria for the diagnosis and treatment goals should be elaborated and implemented based on evidence. SIGNIFICANCE This study is the largest monocentric observational study on treatment effects in children with CSWS, providing data for diagnostic and therapeutic decisions.

中文翻译：

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95例儿童睡眠期间持续尖峰波（CSWS）的临床谱及治疗结果

目的 睡眠期间的持续尖峰和波 (CSWS) 是一种癫痫性脑病，其特征是全身性癫痫样活动和神经认知功能障碍。原因和结果多种多样，治疗主要是经验性的。方法 对 1998 年至 2018 年在海德堡大学医院诊断为 CSWS 儿童的临床和脑电图数据进行回顾性描述性分析。结果 包括 95 名儿童，诊断时的中位年龄为 5.4 岁。43.2% 的人发现结构/代谢病因，17.9% 的人发现基因改变，而 38.9% 的人仍然未知。遗传病因患者的比例从10.3%（1998-2007）增加到22.8%（2008-2018）。平均而言，每位患者接受了 5 种不同的治疗。CSWS 在 70% 以上的病例中难治，类固醇和神经外科手术最有效。在患有 CSWS 或近 CSWS（尖峰波指数 40-85%）的儿童之间没有观察到差异。结论 我们的队列证实 CSWS 是一种年龄依赖性癫痫性脑病。结构性脑异常是最常见的，但越来越多地确定遗传原因。应根据证据制定和实施更具体的诊断和治疗目标标准。意义 本研究是最大的关于 CSWS 儿童治疗效果的单中心观察性研究，为诊断和治疗决策提供数据。应根据证据制定和实施更具体的诊断和治疗目标标准。意义 本研究是最大的关于 CSWS 儿童治疗效果的单中心观察性研究，为诊断和治疗决策提供数据。应根据证据制定和实施更具体的诊断和治疗目标标准。意义 本研究是最大的关于 CSWS 儿童治疗效果的单中心观察性研究，为诊断和治疗决策提供数据。