RT Journal
A1 Azoulay-Zyss J, Roze E, Welter M, et al
T1 Bilateral deep brain stimulation of the pallidum for myoclonus-dystonia due to ε-sarcoglycan mutations: A pilot study
JF Archives of Neurology
JO Archives of Neurology
YR 2011
FD January 1
VO 68
IS 1
SP 94
OP 98
DO 10.1001/archneurol.2010.338
UL http://dx.doi.org/10.1001/archneurol.2010.338
AB 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.