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July 2008 Volume 109, Number 1
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Long-term follow-up of Huntington disease treated by bilateral deep brain stimulation of the internal globus pallidus
Case report Brigitte Biolsi, M.D.1, Laura Cif, M.D.1,2, Hassan El Fertit, M.D.1, Santiago Gil Robles, M.D.1, and Philippe Coubes, M.D., Ph.D.1,2 1Department of Neurosurgery, University Hospital Guy de Chauliac; and 2Research Unit on Movement Disorder, Institut National de la Santé et de la Recherche Médicale U661, Centre National de la Recherche Scientifique UMR 5203, Montpellier, France Abbreviations used in this paper: DBS = deep brain stimulation; GPi = internal globus pallidus; HD = Huntington disease; UHDRS = Unified Huntington's Disease Rating Scale. 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. DOI: 10.3171/JNS/2008/109/7/0130 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. KEYWORDS:deep brain stimulation; globus pallidus; Huntington disease.
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