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Its management is challenging, consisting mainly of off\u2010label treatments.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>The International Parkinson and Movement Disorder Society commissioned a task force to review and evaluate the evidence of available therapies for HD gene expansion carriers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We followed the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. Eligible randomized controlled trials were identified via an electronic search of the CENTRAL, MEDLINE, and EMBASE databases. All eligible trials that evaluated one or more of 33 predetermined clinical questions were included. Risk of bias was evaluated using the Cochrane Risk of Bias tool. A framework was adapted to allow for efficacy and safety conclusions to be drawn from the balance between the GRADE level of evidence and the importance of the benefit\/harm of the intervention.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty\u2010two eligible studies involving 17 interventions were included, providing data to address 8 clinical questions. These data supported a likely effect of deutetrabenazine on motor impairment, chorea, and dystonia and of tetrabenazine on chorea. The data did not support a disease\u2010modifying effect for premanifest and manifest HD. There was no eligible evidence to support the use of specific treatments for depression, psychosis, irritability, apathy, or suicidality. Similarly, no evidence was eligible to support the use of physiotherapy, occupational therapy, exercise, dietary, or surgical treatments.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Data for therapeutic interventions in HD are limited and support only the use of VMAT2 inhibitors for specific motor symptoms. \u00a9 2021 International Parkinson and Movement Disorder Society<\/jats:p><\/jats:sec>","DOI":"10.1002\/mds.28855","type":"journal-article","created":{"date-parts":[[2021,11,29]],"date-time":"2021-11-29T07:17:12Z","timestamp":1638170232000},"page":"25-35","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":47,"title":["An <scp>MDS<\/scp> Evidence\u2010Based Review on Treatments for Huntington's Disease"],"prefix":"10.1002","volume":"37","author":[{"given":"Joaquim J.","family":"Ferreira","sequence":"first","affiliation":[{"name":"Laboratory of Clinical Pharmacology and Therapeutics, Faculdade de Medicina Universidade de Lisboa  Lisbon Portugal"},{"name":"Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes  Lisbon Portugal"},{"name":"CNS \u2010 Campus Neurol\u00f3gico  Torres Vedras Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5567-7938","authenticated-orcid":false,"given":"Filipe B.","family":"Rodrigues","sequence":"additional","affiliation":[{"name":"Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes  Lisbon Portugal"},{"name":"CNS \u2010 Campus Neurol\u00f3gico  Torres Vedras Portugal"},{"name":"UCL Huntington's Disease Centre, UCL Queen Square Institute of Neurology University College London  London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7802-1897","authenticated-orcid":false,"given":"Gon\u00e7alo S.","family":"Duarte","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Pharmacology and Therapeutics, Faculdade de Medicina Universidade de Lisboa  Lisbon Portugal"},{"name":"Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes  Lisbon Portugal"},{"name":"Centro de Estudos de Medicina Baseada na Evid\u00eancia, Faculdade de Medicina Universidade de Lisboa  Lisbon Portugal"},{"name":"Centro Hospitalar Universit\u00e1rio Lisboa Norte  Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiago A.","family":"Mestre","sequence":"additional","affiliation":[{"name":"Parkinson disease and Movement Disorders Centre, Division of Neurology, Department of Medicine, The Ottawa Hospital Research Institute The University of Ottawa Brain and Mind Research Institute  Ottawa Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne\u2010Catherine","family":"Bachoud\u2010Levi","sequence":"additional","affiliation":[{"name":"National Centre of Reference for Huntington's Disease, Neurology Department Henri Mondor Hospital, Assistance Publique\u2010H\u00f4pitaux de Paris  Cr\u00e9teil France"},{"name":"Neuropsychologie Interventionelle Lab INSERM U955 E01B, PSL University  Paris France"},{"name":"Universit\u00e9 Paris Est Cr\u00e9teil  Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna Rita","family":"Bentivoglio","sequence":"additional","affiliation":[{"name":"Istituto di Neurologia Universit\u00e0 Cattolica del Sacro Cuore  Rome Italy"},{"name":"Movement Disorder Unit, Fondazione Policlinico Universitario A. 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