{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T06:13:01Z","timestamp":1790748781880,"version":"4.1.0"},"reference-count":39,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2020,10,16]],"date-time":"2020-10-16T00:00:00Z","timestamp":1602806400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Genetics"],"published-print":{"date-parts":[[2021,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Huntington's disease is associated with motor, cognitive and behavioral dysfunction. Behavioral symptoms may present before, after, or simultaneously with clinical disease manifestation. The relationship between age of onset and behavioral symptom presentation and severity was explored using the Enroll\u2010HD database. Manifest individuals (n = 4469) were initially divided into three groups for preliminary analysis: early onset (&lt;30\u2009years; n = 479); mid\u2010adult onset (30\u201059\u2009years; n = 3478); and late onset (&gt;59\u2009years; n = 512). Incidence of behavioral symptoms reported at onset was highest in those with early onset symptoms at 26% (n = 126), compared with 19% (n = 678) for mid\u2010adult onset and 11% (n = 56) for late onset (\n                    <jats:italic>P<\/jats:italic>\n                    \u2009&lt;\u20090.0001). Refined analysis, looking across the continuum of ages rather than between categorical subgroups found that a one\u2010year increase in age of onset was associated with a 5.6% decrease in the odds of behavioral symptoms being retrospectively reported as the presenting symptom (\n                    <jats:italic>P<\/jats:italic>\n                    \u2009&lt;\u20090.0001). By the time of study enrollment, the odds of reporting severe behavioral symptoms decreased by 5.5% for each one\u2010year increase in reported age of onset. Exploring environmental, genetic and epigenetic factors that affect age of onset and further characterizing types and severity of behavioral symptoms may improve treatment and understanding of Huntington's disease's impact on affected individuals.\n                  <\/jats:p>","DOI":"10.1111\/cge.13857","type":"journal-article","created":{"date-parts":[[2020,10,5]],"date-time":"2020-10-05T23:49:30Z","timestamp":1601941770000},"page":"133-142","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Age of onset and behavioral manifestations in Huntington's disease: An\n                    <scp>Enroll\u2010HD<\/scp>\n                    cohort analysis"],"prefix":"10.1111","volume":"99","author":[{"given":"Megha","family":"Ranganathan","sequence":"first","affiliation":[{"name":"Division of Human Genetics, Department of Internal Medicine The Ohio State University College of Medicine  Columbus Ohio USA"},{"name":"Department of Medicine Memorial Sloan Kettering Cancer Center  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3371-753X","authenticated-orcid":false,"given":"Sandra K.","family":"Kostyk","sequence":"additional","affiliation":[{"name":"Department of Neurology The Ohio State University Wexner Medical Center  Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dawn C.","family":"Allain","sequence":"additional","affiliation":[{"name":"Division of Human Genetics, Department of Internal Medicine The Ohio State University College of Medicine  Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan A.","family":"Race","sequence":"additional","affiliation":[{"name":"Division of Biostatistics The Ohio State University  Columbus Ohio USA"},{"name":"Eli Lilly and Company, Design Hub\u2010Immunology Division  Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allison M.","family":"Daley","sequence":"additional","affiliation":[{"name":"Department of Neurology The Ohio State University Wexner Medical Center  Columbus Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,10,16]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90585-E"},{"issue":"20","key":"e_1_2_9_3_1","first-page":"1267","article-title":"Huntington's disease. 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