Published 08/01/2010
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Long-term follow-up of dyt1 dystonia patients treated by deep brain stimulation: an open-label study

G.Collod-Beroud , M.Claustres , V.Gonzalez , L.Cif , S.Tuffery-Giraud , P.Coubes , X.Vasques , P.Ravel , B.Biolsi , H.Elfertit

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Long-term efficacy of internal globus pallidus deep-brain stimulation in DYT1 dystonia and disease progression under DBS was studied. Twenty-six patients of this open-label study were divided into two groups: with single bilateral GPi lead, with a second bilateral GPi lead implanted owning to subsequent worsening of symptomatology. Dystonia was assessed with the Burke Scale. Appearance of new symptoms and distribution according to body region were recorded. In the whole cohort, significant decreases in motor and disability subscores were observed at 1 year and maintained up to 10 years. Group B showed worsening of the symptoms. At 1 year, there were no significant differences between Groups A and B; at 5 years, a significant difference was found for motor and disability scores. Within Group B, four patients exhibited additional improvement after the second DBS surgery. In the 26 patients, significant difference was found between the number of body regions affected by dystonia preoperatively and over the whole follow-up. DBS efficacy in DYT1 dystonia can be maintained up to 10 years. New symptoms appear with long-term follow-up and may improve with additional leads in a subgroup of patients. O 2010 Movement Disorder Society
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The primary aims of this study were to observe the durability of the initial response to pallidal stimulation and the evolution of the DYT1 motor phenotype during DBS. The inclusion criteria were as follows: genetically con-rmed DYT1 mutation; segmental or generalized dys-tonia; follow-up post-DBS of at least 3 years; normal neurological examination except for dystonia; absence of severe psychiatric disorders or other comorbidity increasing the surgical risks or compromising the clini-cal lack of response to pharmacological treatments. The mean preoperative scores were compared with the mean worst scores with DBS to check for the response to stimulation.
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