The Effect of Globus Pallidus Interna Deep Brain Stimulation on a Dystonia Patient with the GNAL Mutation Compared to Patients with DYT1 and DYT6
Journal of Movement Disorders
;
: 120-124, 2019.
Article
in English
| WPRIM
| ID: wpr-765849
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 (GNAL, 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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Botulinum Toxins
/
Follow-Up Studies
/
Deep Brain Stimulation
/
Dystonia
/
Globus Pallidus
Type of study:
Observational study
/
Prognostic study
Limits:
Humans
Language:
English
Journal:
Journal of Movement Disorders
Year:
2019
Type:
Article
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