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West syndrome: A study of 26 patients receiving short-term therapy
Epilepsy & Behavior ( IF 2.6 ) Pub Date : 2021-01-01 , DOI: 10.1016/j.yebeh.2020.107235
Sebastián Fortini 1 , Gabriela Reyes Valenzuela 2 , Lorena Fasulo 3 , Roberto Caraballo 2
Affiliation  

OBJECTIVE We describe the electroclinical characteristics of a series of 26 patients with idiopathic West syndrome (WS), who had an excellent response to treatment with vigabatrin (VGB) and corticosteroids alone or in combination. METHODS Evaluating the records of 178 patients with WS studied at Garrahan Hospital, Niño Jesús Hospital, and Clínica San Lucas between January 2005 and June 2017, we selected 26 patients that met the inclusion criteria of idiopathic WS. The inclusion criteria for idiopathic WS were (1) no personal history of disease, (2) normal neurological examination and neurodevelopment, (3) symmetric spasms in clusters not preceded by any other type of seizure, (d) symmetric hypsarrhythmia, (e) normal electroencephalogram (EEG) background, e.g., normal sleep EEG pattern, (f) normal magnetic resonance imaging (MRI) recording, (g) normal neurometabolic and genetic studies, and (h) at least 2 years of follow-up. RESULTS Fifteen boys and 11 girls met the inclusion criteria of idiopathic WS. The current age of the children ranges between 2 years 10 months and 12 years 10 months. Age at first epileptic spasms (ES) ranged from 4 to 11 months, with a mean age of 7 and a median of 7.5 months, respectively; ES were in clusters, bilateral and symmetrical in all cases. Spasms were flexor in nine (34.7%), mixed flexor-extensor in 15 (57.7%), and extensor in three (7.6%). In all patients the EEG showed typical pattern of hypsarrhythmia. CONCLUSION These patients with idiopathic WS who have an excellent response to initial treatment should be treated for a short period of time with adrenocorticotropic hormone (ACTH) and VGB alone or in combination.

中文翻译:

West 综合征:一项对 26 名接受短期治疗的患者的研究

目的 我们描述了一系列 26 名特发性 West 综合征 (WS) 患者的电临床特征,这些患者对单独或联合使用氨己烯酸 (VGB) 和皮质类固醇治疗有很好的反应。方法 评估 2005 年 1 月至 2017 年 6 月在 Garrahan 医院、Niño Jesús 医院和 Clínica San Lucas 研究的 178 名 WS 患者的记录,我们选择了 26 名符合特发性 WS 纳入标准的患者。特发性 WS 的纳入标准是 (1) 无个人病史,(2) 神经系统检查和神经发育正常,(3) 对称性痉挛,之前没有任何其他类型的癫痫发作,(d) 对称性心律失常,(e)正常脑电图 (EEG) 背景,例如正常睡眠 EEG 模式,(f) 正常磁共振成像 (MRI) 记录,(g) 正常的神经代谢和遗传研究,以及 (h) 至少 2 年的随访。结果 15 名男孩和 11 名女孩符合特发性 WS 的纳入标准。孩子们目前的年龄在2岁10个月到12岁10个月之间。首次癫痫性痉挛 (ES) 的年龄范围为 4 至 11 个月,平均年龄分别为 7 个月和中位数为 7.5 个月;ES 在所有情况下都是成簇的、双边的和对称的。痉挛为屈肌 9 例 (34.7%),屈伸混合肌痉挛 15 例 (57.7%),伸肌 3 例 (7.6%)。在所有患者中,脑电图显示典型的心律失常模式。结论 这些对初始治疗反应良好的特发性 WS 患者应短期使用促肾上腺皮质激素 (ACTH) 和 VGB 单独或联合治疗。
更新日期:2021-01-01
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