title: Deep brain stimulation of the internal pallidum in Huntington's disease patients: clinical outcome and neuronal firing patterns.
abstract: Deep brain stimulation (DBS) of the internal globus pallidus (GPi) could treat chorea in Huntington's disease patients. The objectives of this study were to evaluate the efficacy of GPi-DBS to reduce abnormal movements of three patients with Huntington's disease and assess tolerability. Three non-demented patients with severe pharmacoresistant chorea underwent bilateral GPi-DBS and were followed for 30, 24, and 12 months, respectively. Primary outcome measure was the change of the chorea and total motor scores of the Unified Huntington's Disease Rating Scale between pre- and last postoperative assessments. Secondary outcome measures were motor changes between ventral versus dorsal and between on- and off- GPi-DBS. GPi neuronal activities were analyzed and compared to those obtained in patients with Parkinson's disease. No adverse effects occurred. Chorea decreased in all patients (13, 67 and 29%) postoperatively. Total motor score decreased in patient 2 (19.6%) and moderately increased in patients 1 and 3 (17.5 and 1.7%), due to increased bradykinesia and dysarthria. Ventral was superior to dorsal GPi-DBS to control chorea. Total motor score increased dramatically off-stimulation compared to ventral GPi-DBS (70, 63 and 19%). Cognitive and psychic functions were overall unchanged. Lower mean rate and less frequent bursting activity were found in Huntington's disease compared to Parkinson's disease patients. Ventral GPi-DBS sustainably reduced chorea, but worsened bradykinesia and dysarthria. Based on these results and previous published reports, we propose to select non-demented HD patients with severe chorea, and a short disease evolution as the best candidates for GPi-DBS.
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Groupe Hospitalier Pitié-Salpêtrière, 47 boulevard de l'Hôpital, 75013, Paris, France.|Sorbonne Université, UPMC Univ Paris 06, UMR S 1127, CNRS UMR 7225, ICM, 75013, Paris, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Delorme
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Groupe Hospitalier Pitié-Salpêtrière, 47 boulevard de l'Hôpital, 75013, Paris, France. alister.rogers@icloud.com.|Sorbonne Université, UPMC Univ Paris 06, UMR S 1127, CNRS UMR 7225, ICM, 75013, Paris, France. alister.rogers@icloud.com.|Neurosurgery department, Groupe Hospitalier Pitié-Salpêtrière, Brain and Spine Institute, CHU Pitié-Salpêtrière, 47, Bd de L'Hôpital, 75651, Paris Cedex 13, France. alister.rogers@icloud.com.
authors.forename: Alister
authors.initials: A
authors.lastname: Rogers
authors.affiliation: Sorbonne Université, UPMC Univ Paris 06, UMR S 1127, CNRS UMR 7225, ICM, 75013, Paris, France.
authors.forename: Brian
authors.initials: B
authors.lastname: Lau
authors.affiliation: Sorbonne Université, UPMC Univ Paris 06, UMR S 1127, CNRS UMR 7225, ICM, 75013, Paris, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: Francisque
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Groupe Hospitalier Pitié-Salpêtrière, 47 boulevard de l'Hôpital, 75013, Paris, France.|Sorbonne Université, UPMC Univ Paris 06, UMR S 1127, CNRS UMR 7225, ICM, 75013, Paris, France.
authors.forename: Marie-Laure
authors.initials: ML
authors.lastname: Welter
authors.affiliation: Sorbonne Université, UPMC Univ Paris 06, UMR S 1127, CNRS UMR 7225, ICM, 75013, Paris, France.|Centre de Neuroimagerie de Recherche, Institut du Cerveau et de la Moelle épinière, 75013, Paris, France.
authors.forename: Sara Fernandez
authors.initials: SF
authors.lastname: Vidal
authors.affiliation: Sorbonne Université, UPMC Univ Paris 06, UMR S 1127, CNRS UMR 7225, ICM, 75013, Paris, France.
authors.forename: Jérôme
authors.initials: J
authors.lastname: Yelnik
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Groupe Hospitalier Pitié-Salpêtrière, 47 boulevard de l'Hôpital, 75013, Paris, France.|Sorbonne Université, UPMC Univ Paris 06, UMR S 1127, CNRS UMR 7225, ICM, 75013, Paris, France.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Durr
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Groupe Hospitalier Pitié-Salpêtrière, 47 boulevard de l'Hôpital, 75013, Paris, France.|Sorbonne Université, UPMC Univ Paris 06, UMR S 1127, CNRS UMR 7225, ICM, 75013, Paris, France.
authors.forename: David
authors.initials: D
authors.lastname: Grabli
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Groupe Hospitalier Pitié-Salpêtrière, 47 boulevard de l'Hôpital, 75013, Paris, France.|Sorbonne Université, UPMC Univ Paris 06, UMR S 1127, CNRS UMR 7225, ICM, 75013, Paris, France.|Neurosurgery department, Groupe Hospitalier Pitié-Salpêtrière, Brain and Spine Institute, CHU Pitié-Salpêtrière, 47, Bd de L'Hôpital, 75651, Paris Cedex 13, France.
authors.forename: Carine
authors.initials: C
authors.lastname: Karachi
mesh_terms: D000328:Adult; D046690:Deep Brain Stimulation / Q000379:methods*; D005260:Female; D005917:Globus Pallidus / Q000502:physiology; D006801:Humans; D006816:Huntington Disease / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009043:Motor Activity; D016896:Treatment Outcome
keywords: Chorea; Deep brain stimulation; Electrophysiolgy; Huntington’s disease; Internal pallidum