{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T20:20:12Z","timestamp":1785270012939,"version":"3.55.0"},"reference-count":25,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2020,8,8]],"date-time":"2020-08-08T00:00:00Z","timestamp":1596844800000},"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>Huntington\u2019s disease (HD) is a fatal neurodegenerative disease caused by the expansion of cytosine-adenine-guanine (CAG) repeats in the huntingtin gene. An increased CAG repeat length is associated with an earlier disease onset. About 5% of HD cases occur under the age of 21 years, which are classified as juvenile-onset Huntington\u2019s disease (JOHD). Our study aims to measure subcortical metabolic abnormalities in JOHD participants. T1-Rho (T1\u03c1) MRI was used to compare brain regions of 13 JOHD participants and 39 controls. Region-of-interest analyses were used to assess differences in quantitative T1\u03c1 relaxation times. We found that the mean relaxation times in the caudate (p &lt; 0.001), putamen (p &lt; 0.001), globus pallidus (p &lt; 0.001), and thalamus (p &lt; 0.001) were increased in JOHD participants compared to controls. Furthermore, increased T1\u03c1 relaxation times in these areas were significantly associated with lower volumes amongst participants in the JOHD group. These findings suggest metabolic abnormalities in brain regions previously shown to degenerate in JOHD. We also analyzed the relationships between mean regional T1\u03c1 relaxation times and Universal Huntington\u2019s Disease Rating Scale (UHDRS) scores. UHDRS was used to evaluate participants\u2019 motor function, cognitive function, behavior, and functional capacity. Mean T1\u03c1 relaxation times in the caudate (p = 0.003), putamen (p = 0.005), globus pallidus (p = 0.009), and thalamus (p = 0.015) were directly proportional to the UHDRS score. This suggests that the T1\u03c1 relaxation time may also predict HD-related motor deficits. Our findings suggest that subcortical metabolic abnormalities drive the unique hypokinetic symptoms in JOHD.<\/jats:p>","DOI":"10.3390\/brainsci10080533","type":"journal-article","created":{"date-parts":[[2020,8,10]],"date-time":"2020-08-10T05:07:23Z","timestamp":1597036043000},"page":"533","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Subcortical T1-Rho MRI Abnormalities in Juvenile-Onset Huntington\u2019s Disease"],"prefix":"10.3390","volume":"10","author":[{"given":"Alexander V.","family":"Tereshchenko","sequence":"first","affiliation":[{"name":"Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordan L.","family":"Schultz","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 Pharmacy Practice and Science, College of Pharmacy, University of Iowa, Iowa City, IA 52242, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ansley J.","family":"Kunnath","sequence":"additional","affiliation":[{"name":"Department of Cell Biology and Neuroscience, Rutgers University, New Brunswick, NJ 08854, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2415-1658","authenticated-orcid":false,"given":"Joel E.","family":"Bruss","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"}],"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, IA 52242, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8639-5354","authenticated-orcid":false,"given":"Vincent A.","family":"Magnotta","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USA"},{"name":"Department of Radiology, Carver College of Medicine at the University of Iowa, Iowa City, IA 52242, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"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 Pharmacy Practice and Science, College of Pharmacy, University of Iowa, Iowa City, IA 52242, USA"},{"name":"Department of Pediatrics, Stead Family Children\u2019s Hospital at the University of Iowa, Iowa City, IA 52242, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,8,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"91","DOI":"10.31887\/DCNS.2016.18.1\/pnopoulos","article-title":"Huntington disease: A single-gene degenerative disorder of the striatum","volume":"18","author":"Nopoulos","year":"2016","journal-title":"Dialogues Clin. 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