Long-term follow-up of Huntington disease treated by bilateral deep brain stimulation of the internal globus pallidus

Case report

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Deep brain stimulation is now accepted as a safe and efficient treatment for movement disorders including selected types of dystonia and dyskinesia. Very little, however, is known about its effect on other movement disorders, particularly for “choreic” movements. Huntington disease is a fatal autosomal-dominant neurodegenerative disorder characterized by movement disorders, progressive cognitive impairment, and psychiatric symptoms. Bilateral chronic stimulation of the internal globus pallidus was performed to control choreic movements in a 60-year-old man with a 10-year history of Huntington disease. Chronic deep brain stimulation resulted in remarkable improvement of choreic movements. Postoperative improvement was sustained after 4 years of follow-up with a marked improvement in daily quality of life.

Abbreviations used in this paper: DBS = deep brain stimulation; GPi = internal globus pallidus; HD = Huntington disease; UHDRS = Unified Huntington's Disease Rating Scale.

Article Information

Address correspondence to: Philippe Coubes, M.D., Ph.D., Service de Neurochirurgie, Unité de Recherche sur les Mouvements Anormaux, Hôpital Gui de Chauliac, 80 Ave Augustin Fliche, 34295 Montpellier cedex 05, France. email: p-coubes@chu-montpellier.fr.

© AANS, except where prohibited by US copyright law.

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    Axial T1-weighted MR image showing the artifacts of the 2 leads.

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