Frontiers in Neurology (Jan 2022)
Efficacy of Anti-seizure Medications, Quinidine, and Ketogenic Diet Therapy for KCNT1-Related Epilepsy and Genotype-Efficacy Correlation Analysis
- Zehong Lin,
- Zehong Lin,
- Zehong Lin,
- Tian Sang,
- Tian Sang,
- Tian Sang,
- Ying Yang,
- Ying Yang,
- Ying Yang,
- Yuan Wu,
- Yuan Wu,
- Yuan Wu,
- Yan Dong,
- Taoyun Ji,
- Taoyun Ji,
- Taoyun Ji,
- Yuehua Zhang,
- Yuehua Zhang,
- Yuehua Zhang,
- Ye Wu,
- Ye Wu,
- Ye Wu,
- Kai Gao,
- Kai Gao,
- Kai Gao,
- Kai Gao,
- Yuwu Jiang,
- Yuwu Jiang,
- Yuwu Jiang,
- Yuwu Jiang,
- Yuwu Jiang
Affiliations
- Zehong Lin
- Department of Pediatrics, Peking University First Hospital, Beijing, China
- Zehong Lin
- Beijing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases, Beijing, China
- Zehong Lin
- Children Epilepsy Center, Peking University First Hospital, Beijing, China
- Tian Sang
- Department of Pediatrics, Peking University First Hospital, Beijing, China
- Tian Sang
- Beijing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases, Beijing, China
- Tian Sang
- Children Epilepsy Center, Peking University First Hospital, Beijing, China
- Ying Yang
- Department of Pediatrics, Peking University First Hospital, Beijing, China
- Ying Yang
- Beijing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases, Beijing, China
- Ying Yang
- Children Epilepsy Center, Peking University First Hospital, Beijing, China
- Yuan Wu
- Department of Pediatrics, Peking University First Hospital, Beijing, China
- Yuan Wu
- Beijing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases, Beijing, China
- Yuan Wu
- Children Epilepsy Center, Peking University First Hospital, Beijing, China
- Yan Dong
- Department of Pediatrics, Third Affiliated Hospital of Zhengzhou University, Zhengzhou, China
- Taoyun Ji
- Department of Pediatrics, Peking University First Hospital, Beijing, China
- Taoyun Ji
- Beijing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases, Beijing, China
- Taoyun Ji
- Children Epilepsy Center, Peking University First Hospital, Beijing, China
- Yuehua Zhang
- Department of Pediatrics, Peking University First Hospital, Beijing, China
- Yuehua Zhang
- Beijing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases, Beijing, China
- Yuehua Zhang
- Children Epilepsy Center, Peking University First Hospital, Beijing, China
- Ye Wu
- Department of Pediatrics, Peking University First Hospital, Beijing, China
- Ye Wu
- Beijing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases, Beijing, China
- Ye Wu
- Children Epilepsy Center, Peking University First Hospital, Beijing, China
- Kai Gao
- Department of Pediatrics, Peking University First Hospital, Beijing, China
- Kai Gao
- Beijing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases, Beijing, China
- Kai Gao
- Children Epilepsy Center, Peking University First Hospital, Beijing, China
- Kai Gao
- Key Laboratory for Neuroscience, Ministry of Education/National Health and Family Planning Commission, Peking University, Beijing, China
- Yuwu Jiang
- Department of Pediatrics, Peking University First Hospital, Beijing, China
- Yuwu Jiang
- Beijing Key Laboratory of Molecular Diagnosis and Study on Pediatric Genetic Diseases, Beijing, China
- Yuwu Jiang
- Children Epilepsy Center, Peking University First Hospital, Beijing, China
- Yuwu Jiang
- Key Laboratory for Neuroscience, Ministry of Education/National Health and Family Planning Commission, Peking University, Beijing, China
- Yuwu Jiang
- Center of Epilepsy, Beijing Institute for Brain Disorders, Beijing, China
- DOI
- https://doi.org/10.3389/fneur.2021.834971
- Journal volume & issue
-
Vol. 12
Abstract
AimTo evaluate the efficacy of anti-seizure medications (ASMs), quinidine, and ketogenic diet therapy (KDT) for KCNT1-related epilepsy and to explore genotype-efficacy correlations.MethodsWe collected the data for KCNT1-related epilepsy cases from our hospital's medical records and the literature. In total, 50 patients received quinidine, 23 received classical KDT, and 15 received ASMs; all ASM data were from our hospital owing to the lack of detailed ASM data in the literature. The efficacy rates (ERs) of the treatments were compared; an ER that reduced the number of seizures by ≥50% was considered positive. Efficacy according to genotype was also assessed.ResultsThe ERs for the 30 patients at our hospital were 40, 26.7, 30, and 44.4% for all treatments, ASMs, quinidine, and KDT, respectively. For all patients (ours and those in previous reports), the overall ERs for quinidine and KDT were 26.0 and 43.5%, respectively (P = 0.135). The ERs for quinidine and KDT in functional domain variant-related epilepsy differed significantly (20.6 vs. 53.8%; P = 0.037).InterpretationKDT may be better at treating KCNT1-related epilepsy than quinidine; ASMs were the least effective. KDT is a viable treatment option for functional domain variant-related epilepsy.
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