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.


  • 1

    Cif LEl Fertit HVayssiere NHemm SHardouin EGannau A: Treatment of dystonic syndromes by chronic electrical stimulation of the internal globus pallidus. J Neurosurg Sci 47:52552003

  • 2

    Coubes PCif LEl Fertit HHemm SVayssiere NSerrat S: Electrical stimulation of the globus pallidus internus in patients with primary generalized dystonia: long-term results. J Neurosurg 101:1891942004

  • 3

    Coubes PRoubertie AVayssiere NHemm SEchenne B: Treatment of DYT1-generalised dystonia by stimulation of the internal globus pallidus. Lancet 355:222022212000

  • 4

    Coubes PVayssiere NEl Fertit HHemm SCif LKienlen J: Deep brain stimulation for dystonia. Surgical technique. Stereotact Funct Neurosurg 78:1831912002

  • 5

    Hebb MOGarcia RGaudet PMendez IM: Bilateral stimulation of the globus pallidus internus to treat choreathetosis in Huntington's disease: technical case report. Neurosurgery 58:E3832006

  • 6

    Huntington Study Group: Unified Huntington's Disease Rating Scale: reliability and consistency. Mov Disord 11:1361421996

  • 7

    Limousin PKrack PPollak PBenazzouz AArdouin CHoffmann D: Electrical stimulation of the subthalamic nucleus in advanced Parkinson's disease. N Engl J Med 339:110511111998

  • 8

    Moro ELang AEStrafella APPoon YYArango PMDagher A: Bilateral globus pallidus stimulation for Huntington's disease. Ann Neurol 56:2902942004

  • 9

    Peppe APierantozzi MBassi AAltibrandi MGBrusa LStefani A: Stimulation of the subthalamic nucleus compared with the globus pallidus internus in patients with Parkinson disease. J Neurosurg 101:1952002004

  • 10

    Vidailhet MVercueil LHoueto JLKrystkowiak PLagrange CYelnik J: French SPIDY Study Group. Bilateral, pallidal, deep-brain stimulation in primary generalised dystonia: a prospective 3 year follow-up study. Lancet Neurol 6:2232292007


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