Low-dose fenfluramine as an effective treatment option for 'atypical' Dravet syndrome

被引：0

作者：

Iguchi, Akihiro ^{[1
]}

Yamaguchi, Tokito ^{[1
]}

Yabe, Tomona ^{[1
]}

Miyashita, Mitsuhiro ^{[1
]}

Mizutani, Satoshi ^{[1
]}

Otani, Hideyuki ^{[1
]}

Miyata, Rie ^{[2
]}

Imai, Katsumi ^{[1
]}

机构：

[1] NHO Shizuoka Inst Epilepsy & Neurol Disorders, Natl Epilepsy Ctr, Dept Pediat, 886 Urushiyama,Aoi Ku, Shizuoka 4208688, Japan

[2] Tokyo Kita Med Ctr, Dept Pediat, 4-17-56 Akabanedai,Kita Ku, Tokyo 1150053, Japan

来源：

EPILEPSY & BEHAVIOR REPORTS | 2024年 / 28卷

关键词：

Dravet syndrome; Fenfluramine; Periventricular nodular heterotopia; Atypical; Low-dose; SEVERE MYOCLONIC EPILEPSY; INFANTS;

D O I：

10.1016/j.ebr.2024.100714

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Dravet syndrome (DS) is characterized by recurrent convulsive seizures, including status epilepticus, and intellectual disability as a comorbidity. Seizures associated with DS are commonly resistant to antiseizure medications. Typical features of DS are recurrent episodes of status epilepticus, the presence of genetic mutations, and no abnormal magnetic resonance imaging (MRI) findings. Here, we report a rare case of DS in a 14-year-old girl who was negative for genetic mutations, had experienced status epilepticus only once, and had abnormal findings on brain MRI. Although our patient's case features are atypical of DS, they do not contradict the diagnostic criteria. Despite the difficulty in diagnosing DS because of the negative genetic testing results, we started our patient on fenfluramine (FFA). Long-term treatment with low-dose FFA effectively controlled our patient's seizures and resulted in cognitive and functional improvements.

引用

页数：4

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