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A randomized, controlled, double-blind, crossover trial of zonisamide in myoclonus-dystonia

Elodie Hainque 1 Marie Vidailhet 1 Nathalie Cozic 2 Fanny Charbonnier-Beaupel 2 Stephane Thobois 3 Christine Tranchant 4 Vanessa Brochard 1 Gérald Glibert Sophie Drapier 5, 6 Eugénie Mutez 7 Anne Doe de Maindreville Thibaud Lebouvier Cécile Hubsch 8, 1 Bertrand Degos 2, 8 Cécilia Bonnet 8 David Grabli 8 André-Pierre Legrand Aurélie Méneret 1 Jean-Philippe Azulay 9 Anne Bissery Noel Zahr 10 Fabienne Clot 11, 12 Alain Mallet Sophie Dupont 13, 1 Emmanuelle Apartis 14, 1 Jean-Christophe Corvol 1, 15 Emmanuel Roze 1, 16
1 ICM - Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute
2 CHU Pitié-Salpêtrière [AP-HP]
3 CNC - Institut des sciences cognitives Marc Jeannerod - Centre de neuroscience cognitive - UMR5229
4 Pôle Tête-Cou-CETD
5 Comportement et noyaux gris centraux = Behavior and Basal Ganglia [Rennes]
6 Service de Neurologie [Rennes]
7 JPArc - Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer - U1172 Inserm - U837
8 Département des maladies du système nerveux
9 TIMONE - Hôpital de la Timone [CHU - APHM]
10 UF pharmacologie et pharmacogénétique
11 Neurologie et thérapeutique expérimentale
12 Service de Génétique Cytogénétique et Embryologie [CHU Pitié-Salpêtrière]
13 NEMESIS-CRICM - Equipe NEMESIS - Centre de Recherches de l'Institut du Cerveau et de la Moelle épinière
CRICM - Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière
14 CHU Saint-Antoine [AP-HP]
15 CIC Pitié Neurosciences
16 Service de neurologie 1 [CHU Pitié-Salpétrière]
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
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https://hal-univ-rennes1.archives-ouvertes.fr/hal-01302410
Contributor : Laurent Jonchère <>
Submitted on : Thursday, April 14, 2016 - 11:21:47 AM
Last modification on : Thursday, January 14, 2021 - 11:30:19 AM

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UNIV-RENNES1 | CNRS | UPMC | ICM | CNGC | UR1-UFR-SVE | UNIV-LYON1 | UNIV-RENNES | SORBONNE-UNIVERSITE | SU-MEDECINE | UDL | UNIV-LILLE

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Elodie Hainque, Marie Vidailhet, Nathalie Cozic, Fanny Charbonnier-Beaupel, Stephane Thobois, et al.. A randomized, controlled, double-blind, crossover trial of zonisamide in myoclonus-dystonia. Neurology, American Academy of Neurology, 2016, 86 (18), pp.1729-35. ⟨10.1212/WNL.0000000000002631⟩. ⟨hal-01302410⟩

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