{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T07:47:09Z","timestamp":1782978429303,"version":"3.54.5"},"reference-count":24,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,11,1]],"date-time":"2022-11-01T00:00:00Z","timestamp":1667260800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100005725","name":"CHDI Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005725","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000025","name":"National Institute of Mental Health","doi-asserted-by":"publisher","award":["P50\u2010HD103556"],"award-info":[{"award-number":["P50\u2010HD103556"]}],"id":[{"id":"10.13039\/100000025","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["K23\u2010NS117736"],"award-info":[{"award-number":["K23\u2010NS117736"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["R01\u2010NS055903"],"award-info":[{"award-number":["R01\u2010NS055903"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["R01\u2010NS094387"],"award-info":[{"award-number":["R01\u2010NS094387"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000098","name":"NIH Clinical Center","doi-asserted-by":"publisher","award":["S10\u2010OD025025"],"award-info":[{"award-number":["S10\u2010OD025025"]}],"id":[{"id":"10.13039\/100000098","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2023,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Juvenile\u2010onset Huntington's disease (JOHD) is a rare form of Huntington's disease (HD) characterized by symptom onset before the age of 21\u2009years. Observational data in this cohort is lacking.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Quantify measures of disease progression for use in clinical trials of patients with JOHD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Participants who received a motor diagnosis of HD before the age of 21 were included in the Kids\u2010JOHD study. The comparator group consisted of children and young adults who were at\u2010risk for inheriting the genetic mutation that causes HD, but who were found to have a CAG repeat in the non\u2010expanded range (gene non\u2010expanded [GNE]).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Data were obtained between March 17, 2006, and February 13, 2020. There were 26 JOHD participants and 78 GNE participants who were comparable on age (16.03 vs. 14.43, respectively) and sex (53.8% female vs. 57.7% female, respectively). The mean annualized decrease in striatal volume in the JOHD group was \u22123.99% compared to \u22120.06% in the GNE (mean difference [MD], \u22123.93%; 95% confidence intervals [CI], [\u22124.98 to \u22122.80], FDR\u2009&lt;\u20090.0001). The mean increase in the Unified Huntington's Disease Rating Scale Total Motor Score per year in the JOHD group was 7.29 points compared to a mean decrease of \u22120.21 point in the GNE (MD,\u00a07.5; 95% CI, [5.71\u20139.28], FDR\u2009&lt;\u20090\u00b70001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These findings demonstrate that structural brain imaging and clinical measures in JOHD may be potential biomarkers of disease progression for use in clinical trials. Collaborative efforts are required to validate these results in a larger cohort of patients with JOHD. \u00a9 2022 International Parkinson and Movement Disorder Society.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mds.29251","type":"journal-article","created":{"date-parts":[[2022,11,1]],"date-time":"2022-11-01T10:43:31Z","timestamp":1667299411000},"page":"113-122","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Longitudinal Clinical and Biological Characteristics in Juvenile\u2010Onset Huntington's Disease"],"prefix":"10.1002","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0985-7268","authenticated-orcid":false,"given":"Jordan L.","family":"Schultz","sequence":"first","affiliation":[{"name":"Department of Psychiatry Carver College of Medicine at the University of Iowa  Iowa City Iowa USA"},{"name":"Department of Neurology Carver College of Medicine at the University of Iowa  Iowa City Iowa USA"},{"name":"Division of Pharmacy Practice and Sciences University of Iowa College of Pharmacy  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7803-4679","authenticated-orcid":false,"given":"Douglas R.","family":"Langbehn","sequence":"additional","affiliation":[{"name":"Department of Psychiatry Carver College of Medicine at the University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hend M.","family":"Al\u2010Kaylani","sequence":"additional","affiliation":[{"name":"Department of Psychiatry Carver College of Medicine at the University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen","family":"van der Plas","sequence":"additional","affiliation":[{"name":"Department of Psychiatry Carver College of Medicine at the University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy R.","family":"Koscik","sequence":"additional","affiliation":[{"name":"Department of Psychiatry Carver College of Medicine at the University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric A.","family":"Epping","sequence":"additional","affiliation":[{"name":"Department of Psychiatry Carver College of Medicine at the University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia B.","family":"Espe\u2010Pfeifer","sequence":"additional","affiliation":[{"name":"Department of Psychiatry Carver College of Medicine at the University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erin P.","family":"Martin","sequence":"additional","affiliation":[{"name":"Department of Psychiatry Carver College of Medicine at the University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David 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