Tags

Type your tag names separated by a space and hit enter

KCNT1-positive epilepsy of infancy with migrating focal seizures successfully treated with nonnarcotic antitussive drugs after treatment failure with quinidine: A case report.
Brain Dev. 2020 Sep; 42(8):607-611.BD

Abstract

BACKGROUND

Epilepsy of infancy with migrating focal seizures (EIMFS) is one of the early-onset epileptic encephalopathies resistant to antiepileptic drugs, therefore carrying an extremely poor neurodevelopmental outcome. KCNT1, encoding for a sodium-activated potassium channel (KCa4.1 channel), has recently been reported as the major gene responsible for EIMFS. Since gain of function is the only type of mutation identified in patients with EIMFS, quinidine, a partial antagonist of KCa4.1 channel, is considered as a potential candidate for targeted treatment of EIMFS. However, treatment results reported so far vary from seizure-free state to no response, and cardiac side effect remains a challenge for dose titration and long-term treatment.

CASE REPORT

Our case was an infant diagnosed with EIMFS with confirmed mutation in KCNT1 gene. Quinidine therapy was started as early as 9 months old. Within the first month of treatment, the number of seizures reduced to about one third. However, seizure-free state was not obtained and his neuropsychological development remained severely delayed. After 16 months of treatment, quinidine had to be discontinued because of cardiac side effects. At 27 months of age, however, his seizures suddenly stopped and he remained seizure-free for five days. This coincided with the prescription of tipepidine, a commonly used antitussive, administered for his persistent cough. Reduction in seizure frequency was also observed with dextromethorphan, another conventional antitussive drug. Although the relation between these treatments and his symptom improvement is a matter of elucidation, there is a possibility that these nonnarcotic antitussive drugs might play a role in the treatment of EIFMS.

Authors+Show Affiliations

Department of Pediatrics, Tsuchiura Kyodo General Hospital, Ibaraki, Japan.Department of Pediatrics, Tsuchiura Kyodo General Hospital, Ibaraki, Japan. Electronic address: shiraken@hotmail.co.jp.Department of Pediatrics, Tsuchiura Kyodo General Hospital, Ibaraki, Japan.Department of Pediatrics, Tsuchiura Kyodo General Hospital, Ibaraki, Japan.Department of Pediatrics, Tsuchiura Kyodo General Hospital, Ibaraki, Japan.Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan.Department of Neurosurgery, National Center of Neurology and Psychiatry, Tokyo, Japan.Department of Human Genetics, Yokohama City University Graduate School of Medicine, Kanagawa, Japan.Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan.College of Nursing and Nutrition, Shukutoku University, Tokyo, Japan.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

32505479

Citation

Takase, Chihiro, et al. "KCNT1-positive Epilepsy of Infancy With Migrating Focal Seizures Successfully Treated With Nonnarcotic Antitussive Drugs After Treatment Failure With Quinidine: a Case Report." Brain & Development, vol. 42, no. 8, 2020, pp. 607-611.
Takase C, Shirai K, Matsumura Y, et al. KCNT1-positive epilepsy of infancy with migrating focal seizures successfully treated with nonnarcotic antitussive drugs after treatment failure with quinidine: A case report. Brain Dev. 2020;42(8):607-611.
Takase, C., Shirai, K., Matsumura, Y., Watanabe, T., Watanabe, A., Hirasawa-Inoue, A., Mizuguchi, T., Matsumoto, N., Sugai, K., & Hayashi, M. (2020). KCNT1-positive epilepsy of infancy with migrating focal seizures successfully treated with nonnarcotic antitussive drugs after treatment failure with quinidine: A case report. Brain & Development, 42(8), 607-611. https://doi.org/10.1016/j.braindev.2020.05.002
Takase C, et al. KCNT1-positive Epilepsy of Infancy With Migrating Focal Seizures Successfully Treated With Nonnarcotic Antitussive Drugs After Treatment Failure With Quinidine: a Case Report. Brain Dev. 2020;42(8):607-611. PubMed PMID: 32505479.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - KCNT1-positive epilepsy of infancy with migrating focal seizures successfully treated with nonnarcotic antitussive drugs after treatment failure with quinidine: A case report. AU - Takase,Chihiro, AU - Shirai,Kentaro, AU - Matsumura,Yu, AU - Watanabe,Tomohiro, AU - Watanabe,Akimitsu, AU - Hirasawa-Inoue,Ayaka, AU - Mizuguchi,Takeshi, AU - Matsumoto,Naomichi, AU - Sugai,Kenji, AU - Hayashi,Masaharu, Y1 - 2020/06/03/ PY - 2019/10/07/received PY - 2020/05/01/revised PY - 2020/05/03/accepted PY - 2020/6/9/pubmed PY - 2020/6/9/medline PY - 2020/6/8/entrez KW - Epilepsy of infancy with migrating focal seizures KW - KCNT1 KW - Nonnarcotic antitussive drugs KW - Quinidine SP - 607 EP - 611 JF - Brain & development JO - Brain Dev. VL - 42 IS - 8 N2 - BACKGROUND: Epilepsy of infancy with migrating focal seizures (EIMFS) is one of the early-onset epileptic encephalopathies resistant to antiepileptic drugs, therefore carrying an extremely poor neurodevelopmental outcome. KCNT1, encoding for a sodium-activated potassium channel (KCa4.1 channel), has recently been reported as the major gene responsible for EIMFS. Since gain of function is the only type of mutation identified in patients with EIMFS, quinidine, a partial antagonist of KCa4.1 channel, is considered as a potential candidate for targeted treatment of EIMFS. However, treatment results reported so far vary from seizure-free state to no response, and cardiac side effect remains a challenge for dose titration and long-term treatment. CASE REPORT: Our case was an infant diagnosed with EIMFS with confirmed mutation in KCNT1 gene. Quinidine therapy was started as early as 9 months old. Within the first month of treatment, the number of seizures reduced to about one third. However, seizure-free state was not obtained and his neuropsychological development remained severely delayed. After 16 months of treatment, quinidine had to be discontinued because of cardiac side effects. At 27 months of age, however, his seizures suddenly stopped and he remained seizure-free for five days. This coincided with the prescription of tipepidine, a commonly used antitussive, administered for his persistent cough. Reduction in seizure frequency was also observed with dextromethorphan, another conventional antitussive drug. Although the relation between these treatments and his symptom improvement is a matter of elucidation, there is a possibility that these nonnarcotic antitussive drugs might play a role in the treatment of EIFMS. SN - 1872-7131 UR - https://www.unboundmedicine.com/medline/citation/32505479/KCNT1-positive_epilepsy_of_infancy_with_migrating_focal_seizures_successfully_treated_with_nonnarcotic_antitussive_drugs_after_treatment_failure_with_quinidine:_A_case_report L2 - https://linkinghub.elsevier.com/retrieve/pii/S0387-7604(20)30141-8 DB - PRIME DP - Unbound Medicine ER -
Try the Free App:
Prime PubMed app for iOS iPhone iPad
Prime PubMed app for Android
Prime PubMed is provided
free to individuals by:
Unbound Medicine.