Department of Paediatric Neurology, P D Hinduja Hospital, Veer Savarkar Marg, Mahim (west), Mumbai 400016, Maharashtra, India.
Epilepsy Behav. 2012 Nov;25(3):381-5. doi: 10.1016/j.yebeh.2012.08.028. Epub 2012 Oct 24.
The aims of this study were to assess the cognitive and behavioral problems of patients with Epilepsy with Electrical Status Epilepticus in slow sleep (ESES) and related syndromes and to review their EEG (electroencephalography) findings and treatment options.
Fourteen patients with ESES were evaluated and treated in 2010. Nine children had continuous spike and wave during slow-wave sleep (CSWS)/ESES syndrome, 3 had Atypical BECTS (benign epilepsy with centrotemporal spikes), 1 had Opercular syndrome, and 1 had Landau-Kleffner syndrome. The duration of ESES ranged from 6 to 52 months. Eleven (91%) children had behavioral issues, most prominent being hyperactivity. Seven of the 13 children (53%) showed evidence of borderline to moderate cognitive impairment. A total of 28 EEG findings of ESES were analyzed for SWI (spike-wave index). Antiepileptic drugs received by the patients included valproate, clobazam, levetiracetam, and others. Eleven patients had been treated with oral steroids and it was found to be efficacious in seven (63%).
Disabilities caused by ESES affect multiple domains. Patients with an SWI>50% should be followed up frequently with neuropsychological assessments. Steroids appear to be effective, although there is a need to standardize the dose and duration of treatment.
本研究旨在评估电持续状态癫痫性电静息(ESES)及相关综合征患者的认知和行为问题,并回顾其脑电图(EEG)表现和治疗选择。
2010 年评估并治疗了 14 例 ESES 患者。9 例儿童患有慢波睡眠期持续棘慢波(CSWS)/ESES 综合征,3 例患有非典型 BECTS(良性癫痫伴中央颞区棘波),1 例患有脑桥小脑脚综合征,1 例患有 Landau-Kleffner 综合征。ESES 持续时间为 6 至 52 个月。11 例(91%)患儿存在行为问题,以多动最为突出。13 例患儿中有 7 例(53%)存在边缘至中度认知障碍证据。共分析了 28 例 ESES 的 EEG 发现,以计算棘慢波指数(SWI)。患儿接受的抗癫痫药物包括丙戊酸钠、氯巴占、左乙拉西坦等。11 例患儿接受了口服类固醇治疗,其中 7 例(63%)有效。
ESES 引起的残疾影响多个领域。SWI>50%的患者应定期进行神经心理学评估。类固醇似乎有效,但需要标准化治疗剂量和时间。