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Within the expanding domain of genetic ataxias, knowledge of regional epidemiology is scarce, particularly outside of North America and Europe. Understanding the epidemiology of genetic ataxias, together with deep phenotyping and knowledge of the appropriate ancillary studies, is crucial for the development and deployment of diagnostic testing strategies. This review offers a comprehensive, data\u2010driven overview of 2932 articles with regional epidemiological estimates and the occurrence and prevalence of 548 genes associated with ataxia across 122 countries. Regional differences in epidemiology and phenotypic spectra are highlighted, driving an approach that incorporates complementary diagnostic test results and how these may inform cost\u2010effective, region\u2010specific genetic testing. All data are also publicly available as an online database, the MDSGene Global Genetic Ataxia Resource, accessible at h<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"https:\/\/www.mdsgene.org\/ataxia.html\">ttps:\/\/www.mdsgene.org\/ataxia.html<\/jats:ext-link>. A phenotype\u2010guided, tailored, and sequential testing approach is proposed, based on regional prevalence, to assist clinicians worldwide in diagnosing individuals with presumed genetic ataxia, of which at least 45 causes are treatable. This approach is particularly important in underserved regions, but also in developed countries where health systems limit access to genetic testing, improving the cost\u2010effectiveness and feasibility of genetic testing in these areas. Future screening studies in high\u2010income settings should adopt a more comprehensive approach, integrating broader genetic testing that covers the full range of genetic ataxias. Capacity building for genetic screening in underserved regions, particularly in Africa, South America, and the Middle East, should be prioritized. \u00a9 2025 International Parkinson and Movement Disorder Society.<\/jats:p>","DOI":"10.1002\/mds.30302","type":"journal-article","created":{"date-parts":[[2025,7,19]],"date-time":"2025-07-19T06:53:15Z","timestamp":1752907995000},"page":"1805-1820","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Unravelling the Global Tapestry of Genetic Ataxias: Epidemiology and Genetic Testing Approaches"],"prefix":"10.1002","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3383-8566","authenticated-orcid":false,"given":"Malco","family":"Rossi","sequence":"first","affiliation":[{"name":"Secci\u00f3n de Movimientos Anormales, Departamento de Neurolog\u00eda Fleni  Buenos Aires Argentina"},{"name":"Instituto Fleni\u2010CONICET (INEU) Buenos Aires  Buenos Aires 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Investiga\u00e7\u00e3o e Inova\u00e7a\u00e3o em Sa\u00fade Universidade do Porto  Porto Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1672-8894","authenticated-orcid":false,"given":"Jos\u00e9 Luiz","family":"Pedroso","sequence":"additional","affiliation":[{"name":"Department of Neurology, Ataxia Unit Universidade Federal de S\u00e3o Paulo  S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheng\u2010Han","family":"Kuo","sequence":"additional","affiliation":[{"name":"Department of Neurology Columbia University Medical Center  New York New York USA"},{"name":"Initiative of Columbia Ataxia and Tremor Columbia University Irving Medical Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chi\u2010Ying R.","family":"Lin","sequence":"additional","affiliation":[{"name":"Alzheimer's Disease and Parkinson's Disease Centers, Department of Neurology Baylor 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