Efficacy of perampanel for controlling seizures and improving neurological dysfunction in a patient with dentatorubral-pallidoluysian atrophy (DRPLA)

Shiraishi, Hideaki; Egawa, Kiyoshi; Ito, Tomoshiro et al.

Epilepsy & Behavior Case Reports, 2017, 8, 44-46

Permalink : https://hdl.handle.net/2115/68215

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アクセス権
open access
URI
https://hdl.handle.net/2115/68215
タイトル
Efficacy of perampanel for controlling seizures and improving neurological dysfunction in a patient with dentatorubral-pallidoluysian atrophy (DRPLA)
著者
Shiraishi, Hideaki ; Egawa, Kiyoshi ; Ito, Tomoshiro ; Kawano, Osamu ; Asahina, Naoko ; Kohsaka, Shinobu
e-Rad_Researcher
80374411
著者名
Shiraishi, Hideaki
著者名
Egawa, Kiyoshi
著者名
Ito, Tomoshiro
著者名
Kawano, Osamu
著者名
Asahina, Naoko
著者名
Kohsaka, Shinobu
言語
英語
キーワード
Perampanel
Dentatombral-pallidoluysian atrophy
DRPLA
Progressive myoclonic epilepsy
Child
発行日
2017
出版者
Elsevier
収録物名
Epilepsy & Behavior Case Reports
巻(号)
8
ページ
44-46
抄録
We administered perampanel (PER) to a bedridden 13-year-old male patient with dentatorubral-pallidoluysian atrophy (DRPLA). The DRPLA diagnosis was based on the presence of a CAG trinucleotide repeat in the ATN1 gene. The patient experienced continuous myoclonic seizures and weekly generalized tonic-clonic seizures (GTCs). PER stopped the patient's myoclonic seizures and reduced the GTCs to fragmented clonic seizures. The patient recovered his intellectual abilities and began to walk again with assistance. We suggest that PER be considered as one of the key drugs used to treat patients with DRPLA.
資源タイプ
学術雑誌論文
出版タイプ
Version of Record
権利情報
Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International
PISSN
2213-3232
関連情報 (isIdenticalTo)
出版社版DOI
https://doi.org/10.1016/j.ebcr.2017.05.004