A randomized, controlled, double-blind, crossover trial of zonisamide in myoclonus-dystonia
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Abstract
Objective: To evaluate the efficacy and safety of zonisamide in patients with myoclonus-dystonia.
Methods: We conducted a randomized, double-blind, placebo-controlled crossover trial of zonisamide (300 mg/d) in 24 patients with myoclonus-dystonia. Each treatment period consisted of a 6-week titration phase followed by a 3-week fixed-dose phase. The periods were separated by a 5-week washout period. The co–primary outcomes were action myoclonus severity (section 4 of the Unified Myoclonus Rating Scale [UMRS 4]) and myoclonus-related functional disability (UMRS 5). Secondary outcomes included dystonia severity, assessed with the movement and disability subscales of the Burke-Fahn-Marsden-Dystonia Rating Scale (BFM), the Clinical Global Impression–Improvement scale (CGI), and safety measures. Wilcoxon signed-rank tests for paired data were used to analyze treatment effects.
Results: Twenty-three patients (11 men, 12 women) were analyzed in the intention-to-treat analysis. Zonisamide significantly improved both action myoclonus (median improvement [95% confidence limits] −5 [−9.25 to −1.44], p = 0.003) and myoclonus-related functional disability (median improvement [95% confidence limits] −2 [−2.58 to −2.46], p = 0.007) compared to placebo. Zonisamide also significantly improved dystonia (BFM movement) compared to placebo (median improvement [95% confidence limits] −3 [−8.46 to 0.03], p = 0.009). No difference was found between zonisamide and placebo with respect to the CGI (median improvement [95% confidence limits] −1 [−1.31 to 0.09], p = 0.1). Zonisamide was well-tolerated.
Conclusions: Zonisamide is well-tolerated and effective on the motor symptoms of myoclonus-dystonia.
Classification of evidence: This study provides Class I evidence that zonisamide improves myoclonus and related disability in patients with myoclonus-dystonia.
GLOSSARY
- AE=
- adverse events;
- BFM=
- Burke-Fahn-Marsden Dystonia Rating Scale;
- CGI=
- Clinical Global Impression–Improvement Scale;
- GPi-DBS=
- deep brain stimulation of the internal globus pallidum;
- ITT=
- intention-to-treat;
- M-D=
- myoclonus-dystonia syndrome;
- UMRS=
- Unified Myoclonus Rating Scale
Footnotes
↵* These authors contributed equally to this work.
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Supplemental data at Neurology.org
- Received July 29, 2015.
- Accepted in final form January 25, 2016.
- © 2016 American Academy of Neurology
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