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Approach to Preventive Epilepsy Treatment in Tuberous Sclerosis Complex and Current Clinical Practice in 23 Countries
Pediatric Neurology ( IF 3.2 ) Pub Date : 2020-11-05 , DOI: 10.1016/j.pediatrneurol.2020.11.003
Monika Słowińska 1 , Katarzyna Kotulska 2 , Sylwia Szymańska 1 , Steven L Roberds 3 , Carla Fladrowski 4 , Sergiusz Jóźwiak 1
Affiliation  

Tuberous sclerosis complex (TSC) is associated with a high risk of early-onset epilepsy and developmental delay. Recently, EEG monitoring in infants with TSC and preventive antiepileptogenic treatment have been proposed to improve epilepsy and neurodevelopmental outcome. We explored how recent studies and recommendations regarding EEG monitoring and preventive epilepsy treatment have influenced the clinical practice of epilepsy management among children with TSC.

Methods

A survey on the epilepsy management approach in infants with TSC was sent by e-mail to 165 clinicians who actively participated in TSC international research conferences in years 2016 - 2019. Additionally, the e-mail addresses of TSC referral centers were collected from national TSC organizations. The survey was also distributed in the American Epilepsy Society newsletter. Only responses from centers providing neurological care for children with TSC were included in the study.

Results

Sixty-one responses from 23 countries were analyzed. Sixty respondents answered questions concerning infants, and 57 of 60 respondents (95%) perform at least one EEG study before epilepsy onset and 42 (70.0%) conduct regular EEG monitoring. Most of the clinicians perform video EEG (42/61, 68.8%). Overall, 51.7% of respondents, mostly from Europe, Australia, and South America, endorse preventive antiepileptic treatment in infants with TSC. Vigabatrin is a preferred drug in patients younger than two years old for both focal (61.7%) and generalized (56.7%) seizures.

Conclusions

Despite the lack of published results of randomized trials, the concepts of preseizure EEG monitoring and epilepsy prevention are already being implemented in the majority of surveyed centers.



中文翻译:

结节性硬化症的预防性癫痫治疗方法和 23 个国家的当前临床实践

结节性硬化症 (TSC) 与早发性癫痫和发育迟缓的高风险相关。最近,已提出对 TSC 婴儿进行脑电图监测和预防性抗癫痫治疗,以改善癫痫和神经发育结果。我们探讨了最近关于 EEG 监测和预防性癫痫治疗的研究和建议如何影响 TSC 儿童癫痫管理的临床实践。

方法

通过电子邮件向 2016-2019 年积极参加 TSC 国际研究会议的 165 名临床医生发送了一份关于 TSC 婴儿癫痫管理方法的调查。此外,TSC 转诊中心的电子邮件地址来自国家 TSC组织。该调查还发布在美国癫痫学会通讯中。该研究仅包括为患有 TSC 的儿童提供神经学护理的中心的回应。

结果

分析了来自 23 个国家的 61 份答复。60 名受访者回答了有关婴儿的问题,60 名受访者中的 57 名 (95%) 在癫痫发作前至少进行了一项脑电图研究,42 名 (70.0%) 进行了定期脑电图监测。大多数临床医生执行视频脑电图 (42/61, 68.8%)。总体而言,51.7% 的受访者(主要来自欧洲、澳大利亚和南美洲)支持对 TSC 婴儿进行预防性抗癫痫治疗。对于 2 岁以下的局灶性 (61.7%) 和全身性 (56.7%) 癫痫患者,氨己烯酸是首选药物。

结论

尽管缺乏已发表的随机试验结果,但大多数接受调查的中心已经实施了癫痫发作前脑电图监测和癫痫预防的概念。

更新日期:2020-12-11
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