{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T20:58:15Z","timestamp":1777669095273,"version":"3.51.4"},"reference-count":36,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,2,8]],"date-time":"2018-02-08T00:00:00Z","timestamp":1518048000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Cyprus School of Molecular Medicine"},{"name":"Cyprus Institute of Neurology and Genetics"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical Genetics"],"published-print":{"date-parts":[[2018,3]]},"abstract":"<jats:p>Huntington disease (HD) is most prevalent among populations of western European descent and isolated populations where founder effects may operate. The aim of this study was to examine the epidemiology of HD in Cyprus, an island in southern Europe with extensive western European colonization in the past. All registered HD patients in the Cyprus, since 1994, were included. Detailed pedigrees and clinical information were recorded and maps, showing the geographic distribution of HD, were constructed. Requests for genetic testing were also examined. The project identified 58 clinically manifested cases of HD belonging to 19 families. The 16 families of Cypriot origin were concentrated in a confined geographical cluster in southeast Cyprus. In 2015, prevalence of symptomatic HD was 4.64\/100\u2009000 population, while incidence was 0.12\/100\u2009000 person\u2010years<jats:bold>.<\/jats:bold> Prevalence displayed a marked increase during the past 20\u2009years. Disease characteristics of HD patients were similar to those reported in western European populations. Lastly, the uptake of predictive and\/or prenatal testing was limited. HD disease characteristics, incidence and prevalence in Cyprus were comparable to western European populations. Together with the geographical clustering observed, these results support the possibility for a relatively recent founder effect of HD in Cyprus, potentially of western European origin.<\/jats:p>","DOI":"10.1111\/cge.13168","type":"journal-article","created":{"date-parts":[[2017,11,6]],"date-time":"2017-11-06T10:58:52Z","timestamp":1509965932000},"page":"656-664","source":"Crossref","is-referenced-by-count":17,"title":["Epidemiology of Huntington disease in Cyprus: A 20\u2010year retrospective study"],"prefix":"10.1111","volume":"93","author":[{"given":"C.A.","family":"Demetriou","sequence":"first","affiliation":[{"name":"Neurology Clinic D The Cyprus Institute of Neurology and Genetics  Nicosia Cyprus"},{"name":"The Cyprus School of Molecular Medicine The Cyprus Institute of Neurology and Genetics  Nicosia Cyprus"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Heraclides","sequence":"additional","affiliation":[{"name":"Department of Primary Care and Population Health University of Nicosia Medical School  Nicosia Cyprus"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Salafori","sequence":"additional","affiliation":[{"name":"The Cyprus School of Molecular Medicine The Cyprus Institute of Neurology and Genetics  Nicosia Cyprus"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.A.","family":"Tanteles","sequence":"additional","affiliation":[{"name":"Clinical Genetics Clinic The Cyprus Institute of Neurology and Genetics  Nicosia Cyprus"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"Christodoulou","sequence":"additional","affiliation":[{"name":"The Cyprus School of Molecular Medicine The Cyprus Institute of Neurology and Genetics  Nicosia Cyprus"},{"name":"Neurogenetics Department The Cyprus Institute of Neurology and Genetics  Nicosia Cyprus"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Y.","family":"Christou","sequence":"additional","affiliation":[{"name":"Neurology Clinic D The Cyprus Institute of Neurology and Genetics  Nicosia Cyprus"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Zamba\u2010Papanicolaou","sequence":"additional","affiliation":[{"name":"Neurology Clinic D The Cyprus Institute of Neurology and Genetics  Nicosia Cyprus"},{"name":"The Cyprus School of Molecular Medicine The Cyprus Institute of Neurology and Genetics  Nicosia Cyprus"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,2,8]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-7506-5410-4.00008-6"},{"issue":"1","key":"e_1_2_7_3_1","first-page":"71","article-title":"Huntington's disease: the discovery of the Huntingtin gene","volume":"1","author":"Chial H","year":"2008","journal-title":"Nat Educ"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90585-E"},{"key":"e_1_2_7_5_1","first-page":"S1","article-title":"Huntington's disease: a clinical review","author":"Gudesblatt M","year":"2011","journal-title":"Neurol Rev"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-12-381328-2.00014-6"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-5-40"},{"key":"e_1_2_7_8_1","series-title":"Molecular and Cell Biology of Human Diseases Series","first-page":"1","volume-title":"Molecular and Cell Biology of Neuropsychiatric Diseases","author":"Craufurd D","year":"1994"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.66.1.52"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1152\/jn.00762.2011"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1159\/000443738"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjopen-2015-009070"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/ejhg.2010.229"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.25075"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2010.01603.x"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2012-304636"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0308679101"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1080\/13506120802676948"},{"issue":"1","key":"e_1_2_7_19_1","first-page":"24","article-title":"High frequency of Friedreich's ataxia carriers in the Paphos district of Cyprus","volume":"28","author":"Zamba\u2010Papanicolaou E","year":"2009","journal-title":"Acta Myol"},{"key":"e_1_2_7_20_1","volume-title":"The Genetic Heritage of Cypriots through Special Topics of Genetics","author":"Deltas C","year":"2014"},{"key":"e_1_2_7_21_1","unstructured":"Statistical Service Republic of Cyprus. 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