AP-HP, Hôpital Robert Debré, Service de Neurologie Pédiatrique, 75019, Paris, France; Université Paris Diderot, Sorbonne Paris Cité, INSERM UMR1141, 75019, Paris, France.
AP-HP, Hôpital Robert Debré, Service de Neurologie Pédiatrique, 75019, Paris, France.
Eur J Paediatr Neurol. 2018 Sep;22(5):837-844. doi: 10.1016/j.ejpn.2018.04.009. Epub 2018 Apr 27.
Epileptic encephalopathy with continuous spikes-and-waves during sleep (EE-CSWS) is a rare childhood epilepsy syndrome characterized by a regression in cognitive, behavioral and psychiatric functioning, seizures and a specific electroencephalographic pattern. An early recognition and an appropriate treatment might play a key role in the outcome of this epileptic encephalopathy. We conducted a case-control study to evaluate if there is any clinical or electroencephalographic sign suggestive of EE-CSWS after the first seizure. We retrospectively identified 10 EE-CSWS patients with available EEG recordings at time of the first seizure. We matched them with 10 controls from our first seizure clinics. All EEG recording were analyzed for the study. We did not find any clinical or EEG features that would suggest later development of EE-CSWS. As reported by others, the occurrence of multiple seizures types and a seizure worsening during the follow-up is more frequent in the cases than in the controls. These clinical criteria might be used as a red flag in clinical practice to identify the very few patients with EE-CSWS among the frequent patients with BECTS.
睡眠中持续棘慢波癫痫性脑病(EE-CSWS)是一种罕见的儿童癫痫综合征,其特征是认知、行为和精神功能退化、癫痫发作和特定的脑电图模式。早期识别和适当的治疗可能对这种癫痫性脑病的预后起着关键作用。我们进行了一项病例对照研究,以评估首次发作后是否存在任何提示 EE-CSWS 的临床或脑电图征象。我们回顾性地确定了 10 例首次发作时有可用脑电图记录的 EE-CSWS 患者,并与我们首次发作诊所的 10 例对照进行了匹配。所有脑电图记录均进行了分析。我们没有发现任何临床或脑电图特征可以提示以后会发展为 EE-CSWS。正如其他人所报道的,与对照组相比,病例组在随访期间出现多种癫痫发作类型和癫痫恶化的情况更为常见。这些临床标准可能在临床实践中被用作识别 BECTS 中极少数 EE-CSWS 患者的警示标志。