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These conditions result from various genetic mutations affecting the structures and pathways involved in movement control, primarily the caudate and putamen, ultimately impairing the basal ganglia circuits involved in the regulation of movement, cognition, and behavior. This review focuses on the main forms of hereditary choreas, including Huntington's disease, neuroacanthocytosis syndromes, Huntington's disease phenocopies, benign hereditary chorea, and other less common genetic disorders presenting with chorea. We discuss the clinical, genetic, and pathophysiological features of each condition, alongside key aspects of phenomenology, examination, and complementary tests\u2014including laboratory findings\u2014to guide phenotype\u2010driven genetic testing. We detail the characteristic features of key disorders while also highlighting less common but emerging conditions. This review aims to assist neurologists in recognizing and diagnosing hereditary choreas efficiently, including guidance on the selection of appropriate genetic tests, thereby reducing diagnostic delays, informing accurate counseling, and facilitating access to disease\u2010specific interventions and clinical trials.<\/jats:p>","DOI":"10.1111\/ene.70357","type":"journal-article","created":{"date-parts":[[2025,10,17]],"date-time":"2025-10-17T09:51:51Z","timestamp":1760694711000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Update on Genetic Chorea"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9835-0484","authenticated-orcid":false,"given":"Jes\u00fas","family":"P\u00e9rez\u2010P\u00e9rez","sequence":"first","affiliation":[{"name":"Movement Disorders Unit, Neurology Department Sant Pau Hospital  Barcelona Spain"},{"name":"Medicine Department Universitat Aut\u00f2noma de Barcelona (UAB)  Barcelona Spain"},{"name":"Center for Biomedical Network Research on Neurodegenerative Diseases (CIBERNED) Instituto de Salud Carlos III  Madrid Spain"},{"name":"Institut de Recerca Sant Pau (IR SANT PAU)  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4864-5548","authenticated-orcid":false,"given":"Gonzalo","family":"Olmedo\u2010Saura","sequence":"additional","affiliation":[{"name":"Movement Disorders Unit, Neurology Department Sant Pau Hospital  Barcelona Spain"},{"name":"Medicine Department Universitat Aut\u00f2noma de Barcelona (UAB)  Barcelona Spain"},{"name":"Center for Biomedical Network Research on Neurodegenerative Diseases (CIBERNED) Instituto de Salud Carlos III  Madrid Spain"},{"name":"Institut de Recerca Sant Pau (IR SANT PAU)  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0125-7249","authenticated-orcid":false,"given":"Sa\u00fcl","family":"Mart\u00ednez\u2010Horta","sequence":"additional","affiliation":[{"name":"Movement Disorders Unit, Neurology Department Sant Pau Hospital  Barcelona Spain"},{"name":"Medicine Department Universitat Aut\u00f2noma de Barcelona (UAB)  Barcelona Spain"},{"name":"Center for Biomedical Network Research on Neurodegenerative Diseases (CIBERNED) Instituto de Salud Carlos III  Madrid Spain"},{"name":"Institut de Recerca Sant Pau (IR SANT PAU)  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4427-0574","authenticated-orcid":false,"given":"Sara","family":"Bernal","sequence":"additional","affiliation":[{"name":"Medicine Department Universitat Aut\u00f2noma de Barcelona (UAB)  Barcelona Spain"},{"name":"Institut de Recerca Sant Pau (IR SANT PAU)  Barcelona Spain"},{"name":"Genetics Deparment Sant Pau Hospital  Barcelona Spain"},{"name":"Center for Biomedical Network Research on Rare Diseases (CIBERER) Instituto de Salud Carlos III  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3248-704X","authenticated-orcid":false,"given":"Javier","family":"Pagonabarraga","sequence":"additional","affiliation":[{"name":"Movement Disorders Unit, Neurology Department Sant Pau Hospital  Barcelona Spain"},{"name":"Medicine Department Universitat Aut\u00f2noma de Barcelona (UAB)  Barcelona Spain"},{"name":"Center for Biomedical Network Research on Neurodegenerative Diseases (CIBERNED) Instituto de Salud Carlos III  Madrid Spain"},{"name":"Institut de Recerca Sant Pau (IR SANT PAU)  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4870-1431","authenticated-orcid":false,"given":"Jaime","family":"Kulisevsky","sequence":"additional","affiliation":[{"name":"Movement Disorders Unit, Neurology Department Sant Pau Hospital  Barcelona Spain"},{"name":"Medicine Department Universitat Aut\u00f2noma de Barcelona (UAB)  Barcelona Spain"},{"name":"Center for Biomedical Network Research on Neurodegenerative Diseases (CIBERNED) Instituto de Salud Carlos III  Madrid Spain"},{"name":"Institut de Recerca Sant Pau (IR SANT PAU)  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,10,17]]},"reference":[{"key":"e_1_2_14_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/CON.0000000000000763"},{"key":"e_1_2_14_3_1","first-page":"865","article-title":"Clinical Neurophysiology of Muscle Jerks: Myoclonus, Chorea, and Tics","volume":"39","author":"Marsden C. 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