Bilateral deep brain stimulation of the pallidum for myoclonus-dystonia due to ε-sarcoglycan mutations: a pilot study.

Abstract : OBJECTIVE: To assess the efficacy of bilateral deep brain stimulation of the internal pallidum in patients with myoclonus-dystonia due to genetically proved ε-sarcoglycan (SGCE-M-D) deficiency. DESIGN: Patients with documented SGCE-M-D undergoing bilateral deep brain stimulation of the internal pallidum were recruited. Standardized assessments of M-D were videorecorded before surgery and 6 to 9 months and 15 to 18 months after surgery, using the movement and disability subscales of the Burke-Fahn-Marsden Dystonia Rating Scale and the Unified Myoclonus Rating Scale. The analysis was based on blinded evaluation of the recordings. SETTING: Movement disorder unit in a university hospital in Paris. PATIENTS: Five consecutive patients with documented SGCE-M-D. MAIN OUTCOME MEASURES: Myoclonus and dystonia scores at follow-up. RESULTS: The median myoclonus score decreased from 76 before surgery (range, 38-116) to 10 at 6 to 9 months after surgery (range, 6-31). The median dystonia score decreased from 30.0 before surgery (range, 18.5-53.0) to 4.5 after surgery (range, 3.5-16.0). Disability was also improved and symptoms remained stable between the postoperative evaluations. No adverse effects occurred. CONCLUSIONS: Bilateral deep brain stimulation of the internal pallidum is safe and highly effective in this homogeneous population of patients with SGCE-M-D. This therapeutic option should therefore be considered for patients with severe, drug-resistant forms of the disorder.
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Archives of Neurology -Chigago-, American Medical Association, 2011, 68 (1), pp.94-8. 〈10.1001/archneurol.2010.338〉
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Contributeur : Olivier Colliot <>
Soumis le : jeudi 28 février 2013 - 18:40:01
Dernière modification le : lundi 10 décembre 2018 - 01:19:05

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Julie Azoulay-Zyss, Emmanuel Roze, Marie-Laure Welter, Soledad Navarro, Jérôme Yelnik, et al.. Bilateral deep brain stimulation of the pallidum for myoclonus-dystonia due to ε-sarcoglycan mutations: a pilot study.. Archives of Neurology -Chigago-, American Medical Association, 2011, 68 (1), pp.94-8. 〈10.1001/archneurol.2010.338〉. 〈hal-00795732〉

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