Emily Sanrey1,2, Valérie Macioce3, Victoria Gonzalez4,5, Laura Cif4,5, Fabienne Cyprien4,5, Emilie Chan Seng4,5, Philippe Coubes4,5, Gaetan Poulen4,5. 1. Unité Pathologies Cérébrales Résistantes, Département de Neurochirurgie, Institut génomique Fonctionnelle, Hôpital Gui de Chauliac, INSERM, CNRS, Univ Montpellier, CHU Montpellier, 80 Avenue Augustin Fliche, 34 295, Montpellier Cedex 5, France. e-sanrey@chu-montpellier.fr. 2. Unité de Recherche Sur Les Mouvements Et Comportements Anormaux, Montpellier, France. e-sanrey@chu-montpellier.fr. 3. Clinical Research and Epidemiology Unit, CHU Montpellier, Univ Montpellier, Montpellier, France. 4. Unité Pathologies Cérébrales Résistantes, Département de Neurochirurgie, Institut génomique Fonctionnelle, Hôpital Gui de Chauliac, INSERM, CNRS, Univ Montpellier, CHU Montpellier, 80 Avenue Augustin Fliche, 34 295, Montpellier Cedex 5, France. 5. Unité de Recherche Sur Les Mouvements Et Comportements Anormaux, Montpellier, France.
Abstract
OBJECTIVE: Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder associated with motor, psychiatric and cognitive deterioration over time. To date, Continuous Electrical Neuromodulation (CEN) of the globus pallidus internus (GPi) has been reported to improve chorea but little is known about cognitive progression in these patients. We propose to examine CEN impact on expected cognitive decline throughout long-term neuropsychological assessment of a cohort of HD patients. METHOD: 13 consecutive HD patients underwent GPi neuromodulation between January 2008 and February 2019. Over a 5-year follow-up period, they received systematic pre- and post-operative assessment according to the existing protocol in our unit. The main outcome measure was the total score obtained on the Mattis Dementia Rating Scale (MDRS) as an indicator of global cognitive function. RESULTS: Chorea decreased in all patients postoperatively with a mean improvement of 56% despite disease progression over time, according to previous studies. Moreover we found that the global cognitive profile of HD patients treated with CEN was stable during the first 3 years of treatment. CONCLUSION: We report an unexpected positive influence of GPi continuous electrical neuromodulation on the progression of global cognitive functioning in operated HD patients. This is the most important group of patients treated with this method to our knowledge whatever the sample size remains small. This result provides promising evidence of GPi-CEN efficacy not only in reducing chorea, but also in delaying cognitive decline in HD patients operated at an early stage of the disease.
OBJECTIVE:Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder associated with motor, psychiatric and cognitive deterioration over time. To date, Continuous Electrical Neuromodulation (CEN) of the globus pallidus internus (GPi) has been reported to improve chorea but little is known about cognitive progression in these patients. We propose to examine CEN impact on expected cognitive decline throughout long-term neuropsychological assessment of a cohort of HDpatients. METHOD: 13 consecutive HDpatients underwent GPi neuromodulation between January 2008 and February 2019. Over a 5-year follow-up period, they received systematic pre- and post-operative assessment according to the existing protocol in our unit. The main outcome measure was the total score obtained on the Mattis Dementia Rating Scale (MDRS) as an indicator of global cognitive function. RESULTS:Chorea decreased in all patients postoperatively with a mean improvement of 56% despite disease progression over time, according to previous studies. Moreover we found that the global cognitive profile of HDpatients treated with CEN was stable during the first 3 years of treatment. CONCLUSION: We report an unexpected positive influence of GPi continuous electrical neuromodulation on the progression of global cognitive functioning in operated HDpatients. This is the most important group of patients treated with this method to our knowledge whatever the sample size remains small. This result provides promising evidence of GPi-CEN efficacy not only in reducing chorea, but also in delaying cognitive decline in HDpatients operated at an early stage of the disease.
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