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Perampanel for nonepileptic myoclonus in Angelman syndrome.

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Title: Perampanel for nonepileptic myoclonus in Angelman syndrome.
Authors: Kawano, Osamu Browse this author
Egawa, Kiyoshi Browse this author →KAKEN DB
Shiraishi, Hideaki Browse this author →KAKEN DB
Keywords: Angelman syndrome
Involuntary movement
Issue Date: May-2020
Publisher: Elsevier
Journal Title: Brain & development
Volume: 42
Issue: 5
Start Page: 389
End Page: 392
Publisher DOI: 10.1016/j.braindev.2020.02.007
PMID: 32164978
Abstract: Background: Angelman syndrome (AS) is a neurodegenerative disorder caused by functional loss of the maternal ubiquitin-protein ligase 3A gene. Nonepileptic myoclonus, also described as tremulous movement, often occurs during puberty and increasesin adulthood. The involuntary movement in AS has not been defined patho-physiologically and the drugs used such as levetiracetamand piracetamare not alwayseffective. Recently, the alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionate (AMPA) receptorantagonist, perampanel (PER), was used to alleviate myoclonus in progressive myoclonus epilepsy. Herein, we tested the efficacyof PER for nonepileptic myoclonus.Methods and results: Four patients with AS, aged from 20 to 40 years at the beginning of treatment, were enrolled in our study.All patients reported disruption to their daily lives from the myoclonus movement. They experienced mild to moderate improvementwith the starting dose of 2 mg. The dose was increased to 4 mg in one patient to achieve sufficient efficacy, while two had their dosereduced to 1 mg due to dizziness or possible exacerbation of myoclonus. The last patient continued to take the starting dose. Follow-up over 16?20 months revealed a significant reduction in the severity of nonepileptic myoclonus in all patients.Conclusion: Our study suggests that PER could be one of the promising drugs for nonepileptic myoclonus in AS.O2020 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
Rights: © 2020. This manuscript version is made available under the CC-BY-NC-ND 4.0 license
Type: article (author version)
Appears in Collections:北海道大学病院 (Hokkaido University Hospital) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 白石 秀明

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