Volume 25, Issue 3 p. 289-299
Research Article

Long-term follow-up of DYT1 dystonia patients treated by deep brain stimulation: An open-label study

Laura Cif MD

Corresponding Author

Laura Cif MD

CHRU Montpellier, Hôpital Gui de Chauliac, Service de Neurochirurgie, Montpellier, France

INSERM, U661, Montpellier, France

Université de Montpellier 1, Montpellier, France

CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier, France

Service de Neurochirurgie, Hôpital Gui de Chauliac, 80, Avenue Augustin Fliche, Montepellier, 34295 Cedex 5, France===Search for more papers by this author
Xavier Vasques PhD

Xavier Vasques PhD

INSERM, U661, Montpellier, France

Université de Montpellier 1, Montpellier, France

CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier, France

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Victoria Gonzalez MD

Victoria Gonzalez MD

CHRU Montpellier, Hôpital Gui de Chauliac, Service de Neurochirurgie, Montpellier, France

INSERM, U661, Montpellier, France

Université de Montpellier 1, Montpellier, France

CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier, France

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Patrice Ravel PhD

Patrice Ravel PhD

Université de Montpellier 1, Montpellier, France

CNRS UMR5048, Centre de Biochimie Structurale, Montpellier, France

INSERM, U554, Montpellier, France

Université de Montpellier 2, Montpellier, France

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Brigitte Biolsi MD

Brigitte Biolsi MD

CHRU Montpellier, Hôpital Gui de Chauliac, Service de Neurochirurgie, Montpellier, France

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Gwenaelle Collod-Beroud PhD

Gwenaelle Collod-Beroud PhD

Université de Montpellier 1, Montpellier, France

INSERM, U827, Montpellier, France

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Sylvie Tuffery-Giraud PhD

Sylvie Tuffery-Giraud PhD

Université de Montpellier 1, Montpellier, France

INSERM, U827, Montpellier, France

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Hassan Elfertit MD

Hassan Elfertit MD

CHRU Montpellier, Hôpital Gui de Chauliac, Service de Neurochirurgie, Montpellier, France

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Mireille Claustres MD, PhD

Mireille Claustres MD, PhD

Université de Montpellier 1, Montpellier, France

INSERM, U827, Montpellier, France

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Philippe Coubes MD, PhD

Philippe Coubes MD, PhD

CHRU Montpellier, Hôpital Gui de Chauliac, Service de Neurochirurgie, Montpellier, France

INSERM, U661, Montpellier, France

Université de Montpellier 1, Montpellier, France

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First published: 24 February 2010
Citations: 126

Potential conflict of interest: Nothing to report

Abstract

Long-term efficacy of internal globus pallidus (GPi) deep-brain stimulation (DBS) in DYT1 dystonia and disease progression under DBS was studied. Twenty-six patients of this open-label study were divided into two groups: (A) with single bilateral GPi lead, (B) 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 (P < 0.0001) 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 (without subsequent worsening) 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 (P = 0.001) 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 (two patients). New symptoms appear with long-term follow-up and may improve with additional leads in a subgroup of patients. © 2009 Movement Disorder Society