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01-02-2016 | Original Communication

Deep brain stimulation of the internal pallidum in Huntington’s disease patients: clinical outcome and neuronal firing patterns

Authors: Cécile Delorme, Alister Rogers, Brian Lau, Hélène Francisque, Marie-Laure Welter, Sara Fernandez Vidal, Jérôme Yelnik, Alexandra Durr, David Grabli, Carine Karachi

Published in: Journal of Neurology | Issue 2/2016

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.

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Title: Deep brain stimulation of the internal pallidum in Huntington’s disease patients: clinical outcome and neuronal firing patterns
Authors: Cécile Delorme
Alister Rogers
Brian Lau
Hélène Francisque
Marie-Laure Welter
Sara Fernandez Vidal
Jérôme Yelnik
Alexandra Durr
David Grabli
Carine Karachi
Publication date: 01-02-2016
Publisher: Springer Berlin Heidelberg
Published in: Journal of Neurology / Issue 2/2016
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-015-7968-0

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Deep brain stimulation of the internal pallidum in Huntington’s disease patients: clinical outcome and neuronal firing patterns

Abstract

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Abstract

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