The Effect of Globus Pallidus Interna Deep Brain Stimulation on a Dystonia Patient with the GNAL Mutation Compared to Patients with DYT1 and DYT6open access
- Authors
- Ahn, Jong Hyeon; Kim, Ah Reum; Kim, Nayoung K. D.; Park, Woong-Yang; Kim, Ji Sun; Kim, Minkyeong; Park, Jongkyu; Lee, Jung-Il; Cho, Jin Whan; Cho, Kyung Rae; Youn, Jinyoung
- Issue Date
- May-2019
- Publisher
- 대한파킨슨병및이상운동질환학회
- Keywords
- Deep brain stimulation; GNAL; Dystonia; DYT25
- Citation
- Journal Of Movement Disorders, v.12, no.2, pp 120 - 124
- Pages
- 5
- Journal Title
- Journal Of Movement Disorders
- Volume
- 12
- Number
- 2
- Start Page
- 120
- End Page
- 124
- URI
- https://scholarworks.bwise.kr/sch/handle/2021.sw.sch/4556
- DOI
- 10.14802/jmd.19006
- ISSN
- 2005-940X
2093-4939
- Abstract
- Objective The aim of this study was to investigate the efficacy of globus pallidus interna deep brain stimulation (GPi-DBS) for treating dystonia due to the GNAL mutation. Methods We provide the first report of a dystonia patient with a genetically confirmed GNAL mutation in the Korean population and reviewed the literature on patients with the GNAL mutation who underwent GPi-DBS. We compared the effectiveness of DBS in patients with the GNAL mutation compared to that in patients with DYT1 and DYT6 in a previous study. Results Patients with the GNAL mutation and those with DYT1 had higher early responder rates (GNAT 5/5, 100%; DYT1, 7/7, 100%) than did patients with DYT6 (p = 0.047). The responder rates at late follow-up did not differ statistically among the three groups (p = 0.278). The decrease in the dystonia motor scale score in the GNAL group was 46.9% at early follow-up and 63.4% at late follow-up. Conclusion GPi-DBS would be an effective treatment option for dystonia patients with the GNAL mutation who are resistant to medication or botulinum toxin treatment.
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Collections - College of Medicine > Department of Neurology > 1. Journal Articles
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