Document Detail


Deep brain stimulation for Huntington's disease: long-term results of a prospective open-label study.
MedLine Citation:
PMID:  24702329     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Object To date, experience of globus pallidus internus (GPi) deep brain stimulation (DBS) in the treatment of Huntington's disease (HD) has been limited to a small number of case reports. The aim of this study was to analyze long-term motor outcome of a cohort of HD patients treated with GPi DBS. Methods Seven patients with pharmacologically resistant chorea and functional impairment were included in a prospective open-label study from 2008 to 2011. The main outcome measure was the motor section of the Unified Huntington's Disease Rating Scale. The primary end point was reduction of chorea. Results Patients underwent MRI-guided bilateral GPi implantation. The median duration of follow-up was 3 years. A significant reduction of chorea was observed in all patients, with sustained therapeutic effect; the mean improvement on the chorea subscore was 58.34% at the 12-month follow-up visit (p = 0.018) and 59.8% at the 3-year visit (p = 0.040). Bradykinesia and dystonia showed a nonsignificant trend toward progressive worsening related to disease evolution and partly to DBS. The frequency of stimulation was 130 Hz for all patients. DBS-induced bradykinesia was managed by pulse-width reduction or bipolar settings. Levodopa mildly improved bradykinesia in 4 patients. Regular off-stimulation tests confirmed a persistent therapeutic effect of DBS on chorea. Conclusions GPi DBS may provide sustained chorea improvement in selected HD patients with pharmacologically resistant chorea, with transient benefit in physical aspects of quality of life before progression of behavioral and cognitive disorders. DBS therapy did not improve dystonia or bradykinesia. Further studies including quality of life measures are needed to evaluate the impact of DBS in the long-term outcome of HD.
Authors:
Victoria Gonzalez; Laura Cif; Brigitte Biolsi; Sara Garcia-Ptacek; Anne Seychelles; Emily Sanrey; Irene Descours; Christine Coubes; Ana-Maria Ribeiro de Moura; Astrid Corlobe; Syril James; Thomas Roujeau; Philippe Coubes
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-4-4
Journal Detail:
Title:  Journal of neurosurgery     Volume:  -     ISSN:  1933-0693     ISO Abbreviation:  J. Neurosurg.     Publication Date:  2014 Apr 
Date Detail:
Created Date:  2014-4-7     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0253357     Medline TA:  J Neurosurg     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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