Successful use of Fenfluramine in super-refractory status epilepticus in a patient with tuberous sclerosis complex and Lennox-Gastaut syndrome

IF 1.8 Q3 CLINICAL NEUROLOGY
Thorsten Wildermann , Felicitas Becker , Sarah Jesse , Hartmut Baier , Jan Wagner
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引用次数: 0

Abstract

A 24-year-old female patient with pre-existing refractory epilepsy caused by tuberous sclerosis (TSC) and electroclinical features of Lennox-Gastaut syndrome (LGS) was referred to our hospital from an external clinic. Upon arrival, she presented with super-refractory status epilepticus (SRSE) since anaesthetics had already been used in the referring clinic. Despite various changes in ASM-treatment and continuous administration of anaesthetics for more than two weeks, SRSE could not be terminated. On treatment day 24, we started Fenfluramin (FFA) which was soon titrated to a dose of 0,7 mg/kg/day. A few days after beginning the treatment with FFA, EEG and clinical situation improved dramatically. The following 6 weeks of treatment went without reported seizures. This case illustrates the successful use of FFA in SRSE in TSC and LGS and, to the best of our knowledge, represents the first report of FFA in this clinical context.

芬氟拉明成功治疗一名结节性硬化症复合体和伦诺克斯-加斯塔特综合征患者的超难治性癫痫状态
一名 24 岁的女性患者从外院转诊至我院,她患有由结节性硬化症(TSC)引起的难治性癫痫,并具有伦诺克斯-加斯托特综合征(LGS)的电临床特征。由于转诊诊所已经使用了麻醉剂,她一到医院就出现了超级难治性癫痫状态(SRSE)。尽管对 ASM 治疗进行了各种更改,并持续使用麻醉剂超过两周,但仍无法终止 SRSE。在治疗的第 24 天,我们开始使用芬氟拉明(FFA),并很快将剂量调整为 0.7 毫克/千克/天。开始使用芬氟拉明治疗几天后,脑电图和临床症状都有了显著改善。在随后的 6 周治疗中,没有报告有癫痫发作。本病例说明了 FFA 在 TSC 和 LGS SRSE 中的成功应用,据我们所知,这是 FFA 在这种临床情况下的首次报告。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Epilepsy and Behavior Reports
Epilepsy and Behavior Reports Medicine-Neurology (clinical)
CiteScore
2.70
自引率
13.30%
发文量
54
审稿时长
50 days
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