Category: Huntington's Disease
Objective: The aim is to obtain relevant information regarding the effectiveness of using Deep Brain Stimulation (DBS) as a treatment of Huntington’s disease (HD).
Background: HD is an autosomal dominant neurodegenerative disease with a wide range of symptoms, especially motor disorders with chorea, still in lack of treatment. DBS is a neurosurgical procedure based on electrical stimulation through implanted electrodes that has shown significant positive impact to treat movement disorders, thus, it might enrich HD treatment.
Method: Studies from Pubmed and Cochrane Library were searched using the descriptors “Huntington’s disease” and “deep brain stimulation”, whose publications had occurred from 2018, totalizing 46 studies. Then, they were submitted to inclusion and exclusion criteria, which favored randomized clinical trials and literature reviews. Seven studies were selected, among which there were five literature reviews, one randomized controlled trial and one meta-analysis.
Results: The 7 studies analyzed conflicting results. A meta-analysis of 18 studies published in 2020 concluded that DBS of internal globus pallidus (IGP) attenuated chorea in HD compared to observation at 30-month follow-ups, with no mitigation in other symptoms. A 2017 systematic review demonstrated that the benefit of IGP-DBS could be up to 60% by the Unified Huntington’s Disease Rating Scale (UHDRS) at a 3-year follow-up. Another 3 literature reviews also reinforced the mitigation of chorea in IGP-DBS. In contrast, one study did not perceive a significant difference; another detected an initial improvement (in the first 6 months), but a later equalization between the two groups (after 12 months); and another study reported worsening post-DBS chorea at a 4-year follow-up. The vast majority of studies showed some benefit in IGP-DBS, with the same stimulation on the external globus pallidus and subthalamic nucleus having greater evidence of non-efficacy.
Conclusion: Most of the analyzed studies suggest that IGP-DBS should be reserved for the individuals who present predominance of chorea as a symptom of HD, although a few other studies show different results. More studies are needed to consolidate the evidence that this stimulation improves the quality of life of patients.
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To cite this abstract in AMA style:
H. Rodrigues, G. Mantovani, M. da Rosa, V. de Souza, L. Aires, M. Fin, L. Lobato, R. Albuquerque, PL. da Silva, A. Hilbig. Effectiveness of deep brain stimulation in the treatment of Huntington’s disease [abstract]. Mov Disord. 2023; 38 (suppl 1). https://www.mdsabstracts.org/abstract/effectiveness-of-deep-brain-stimulation-in-the-treatment-of-huntingtons-disease/. Accessed June 15, 2025.« Back to 2023 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/effectiveness-of-deep-brain-stimulation-in-the-treatment-of-huntingtons-disease/