U.O.C. di Neuropsichiatria Infantile, Dipartimento ad Attività Integrata Materno Infantile - AOUI di Verona, Verona, Italy, PhD program Applied Sciences of Life and Health, University of Verona, Verona, Italy.
U.O.C. di Neuropsichiatria Infantile, Dipartimento ad Attività Integrata Materno Infantile - AOUI di Verona, Verona, Italy, Centro Ricerca per le Epilessie in età Pediatrica (CREP), Azienda Ospedaliera Universitaria di Verona, Verona, Italy.
Epileptic Disord. 2022 Apr 1;24(2):387-396. doi: 10.1684/epd.2021.1402.
Febrile status epilepticus evolves from a febrile seizure (FS) in 5% of cases. Its prompt recognition is challenging, especially when motor manifestations are absent or subtle. We describe the ictal electroclinical features of non-convulsive febrile status epilepticus (NCFSE) following an apparently concluded FS, initially misinterpreted as postictal obtundation and in some way mimicking the described "non-epileptic twilight state". We present an electroclinical study of 18 children, collected in our unit, who presented with NCFSE after an apparently resolved FS, longitudinally followed for one year to seven years and nine months (mean: four years and three months). The age at first NCFSE ranged between one year and two months and five years and eight months (mean: two years and six months). Patients were examined after spontaneous or rectal diazepam-induced resolution of a FS, while showing persisting impairment of awareness. A lack of responsiveness to painful stimulation, abnormal posturing and aphasia were present in all cases, variably associated with perioral cyanosis, hypersalivation, automatisms, gaze deviation and other lateralizing signs; eyes were open. The EEG recording started 20 to 140 minutes after the apparent resolution of the FS and was invariably characterized by delta or theta-delta pseudorhythmic activity, mainly involving the fronto-temporal regions, with hemispheric predominance in two thirds of the cases. The electroclinical condition, lasting 25 to 210 minutes, quickly recovered after intravenous diazepam. Follow-up revealed normal neurodevelopment and EEG in almost all patients (learning disability emerged in three). In five subjects, NCSE relapsed (twice in two). None presented afebrile seizures. Our series highlights the electroclinical features of focal NCFSE. Distinctive elements are a lack of reactivity, cyanosis, lateralizing clinical and EEG signs, and resolution clearly tied to intravenous benzodiazepine administration.
热性惊厥后发展为热性惊厥持续状态（FS）的比例为 5%。其及时识别具有挑战性，特别是当运动表现缺失或不明显时。我们描述了一种看似结束的 FS 后出现的无惊厥性热性惊厥持续状态（NCFSE）的发作期电临床特征，最初被误诊为发作后意识模糊，在某种程度上模仿了描述的“非癫痫性黄昏状态”。我们对在我院接受治疗的 18 例出现 NCFSE 的儿童进行了电临床研究，这些儿童在一次看似已结束的 FS 后出现 NCFSE，随后进行了 1 年至 7 年 9 个月（平均：4 年 3 个月）的纵向随访。首次出现 NCFSE 的年龄在 1 岁 2 个月至 5 岁 8 个月（平均：2 岁 6 个月）之间。在 FS 自发或直肠地西泮诱导缓解后，患儿表现出持续性意识障碍，进行检查。所有患儿均存在对疼痛刺激无反应、异常姿势和言语障碍，不同程度地伴有口周发绀、流涎过多、自动症、凝视偏斜和其他偏侧化体征；患儿的眼睛是睁开的。EEG 记录在 FS 明显缓解后 20 至 140 分钟开始，始终表现为 delta 或 theta-delta 假性节律性活动，主要累及额颞区，三分之二的病例表现为半球优势。电临床状况持续 25 至 210 分钟，在静脉注射地西泮后迅速恢复。随访发现几乎所有患儿的神经发育和 EEG 均正常（3 例患儿出现学习障碍）。5 例患儿出现 NCFSE 复发（2 例患儿复发 2 次）。这些患儿均未出现无热惊厥。本研究系列强调了局灶性 NCFSE 的电临床特征。其显著特征为反应缺失、发绀、具有临床和 EEG 定位意义的体征，以及与静脉内苯二氮䓬类药物给药明显相关的缓解。
