{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,8]],"date-time":"2025-12-08T22:29:12Z","timestamp":1765232952568,"version":"build-2065373602"},"reference-count":17,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2020,8,26]],"date-time":"2020-08-26T00:00:00Z","timestamp":1598400000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["R01NS055903"],"award-info":[{"award-number":["R01NS055903"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>Patients with adult-onset Huntington\u2019s Disease (AOHD) have been found to have dysfunction of the autonomic nervous system that is thought to be secondary to neurodegeneration causing dysfunction of the brain\u2013heart axis. However, this relationship has not been investigated in patients with juvenile-onset HD (JOHD). The aim of this study was to compare simple physiologic measures between patients with JOHD (n = 27 participants with 64 visits) and participants without the gene expansion that causes HD (GNE group; n = 259 participants with 395 visits). Using data from the Kids-JOHD study, we compared mean resting heart rate (rHR), systolic blood pressure (SBP), and diastolic blood pressure (DBP) between the JOHD and GNE groups. We also divided the JOHD group into those with childhood-onset JOHD (motor diagnosis received before the age of 13, [n = 16]) and those with adolescent-onset JOHD (motor diagnosis received at or after the age of 13 [n = 11]). We used linear mixed-effects models to compare the group means while controlling for age, sex, and parental socioeconomic status and including a random effect per participant and family. For the primary analysis, we found that the JOHD group had significant increases in their rHR compared to the GNE group. Conversely, the JOHD group had significantly lower SBP compared to the GNE group. The JOHD group also had lower DBP compared to the GNE group, but the results did not reach significance. SBP and DBP decreased as disease duration of JOHD increased, but rHR did not continue to increase. Resting heart rate is more sensitive to changes in autonomic function as compared to SBP. Therefore, these results seem to indicate that early neurodegenerative changes of the central autonomic network likely lead to an increase in rHR while later progression of JOHD leads to changes in blood pressure. We hypothesize that these later changes in blood pressure are secondary to neurodegeneration in brainstem regions such as the medulla.<\/jats:p>","DOI":"10.3390\/brainsci10090589","type":"journal-article","created":{"date-parts":[[2020,8,26]],"date-time":"2020-08-26T09:05:37Z","timestamp":1598432737000},"page":"589","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Autonomic Changes in Juvenile-Onset Huntington\u2019s Disease"],"prefix":"10.3390","volume":"10","author":[{"given":"Jordan L.","family":"Schultz","sequence":"first","affiliation":[{"name":"Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USA"},{"name":"Department of Neurology, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8810-1903","authenticated-orcid":false,"given":"Peg C.","family":"Nopoulos","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USA"},{"name":"Department of Neurology, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USA"},{"name":"Department of Pediatrics, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,8,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington\u2019s disease chromosomes. The Huntington\u2019s Disease Collaborative Research Group","volume":"72","author":"MacDonald","year":"1993","journal-title":"Cell"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1016\/S1474-4422(13)70088-7","article-title":"Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington\u2019s disease in the TRACK-HD study: Analysis of 36-month observational data","volume":"12","author":"Tabrizi","year":"2013","journal-title":"Lancet Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1193","DOI":"10.1016\/S1474-4422(14)70238-8","article-title":"Prediction of manifest Huntington\u2019s disease with clinical and imaging measures: A prospective observational study","volume":"13","author":"Paulsen","year":"2014","journal-title":"Lancet Neurol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1136\/jnnp.72.6.726","article-title":"Autonomic nervous system function in Huntington\u2019s disease","volume":"72","author":"Andrich","year":"2002","journal-title":"J. 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Dis."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"e1939","DOI":"10.1212\/WNL.0000000000007355","article-title":"Brain structure in juvenile-onset Huntington disease","volume":"92","author":"Tereshchenko","year":"2019","journal-title":"Neurology"},{"key":"ref_14","first-page":"e1021","article-title":"Abnormal brain development in child and adolescent carriers of mutant huntingtin","volume":"93","author":"Langbehn","year":"2019","journal-title":"Neurology"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1135","DOI":"10.1016\/S1474-4422(15)00177-5","article-title":"Treating the whole body in Huntington\u2019s disease","volume":"14","author":"Carroll","year":"2015","journal-title":"Lancet Neurol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1161\/01.HYP.38.3.549","article-title":"Role of the medulla oblongata in hypertension","volume":"38","author":"Colombari","year":"2001","journal-title":"Hypertension"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1111\/bpa.12115","article-title":"Huntington\u2019s disease (HD): Degeneration of select nuclei, widespread occurrence of neuronal nuclear and axonal inclusions in the brainstem","volume":"24","author":"Rub","year":"2014","journal-title":"Brain Pathol."}],"container-title":["Brain Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-3425\/10\/9\/589\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T10:06:44Z","timestamp":1760177204000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-3425\/10\/9\/589"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,8,26]]},"references-count":17,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2020,9]]}},"alternative-id":["brainsci10090589"],"URL":"https:\/\/doi.org\/10.3390\/brainsci10090589","relation":{},"ISSN":["2076-3425"],"issn-type":[{"type":"electronic","value":"2076-3425"}],"subject":[],"published":{"date-parts":[[2020,8,26]]}}}