{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T18:45:43Z","timestamp":1760985943198,"version":"build-2065373602"},"reference-count":19,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2020,8,11]],"date-time":"2020-08-11T00:00:00Z","timestamp":1597104000000},"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>Reports of behavioral disturbance in Juvenile-Onset Huntington\u2019s Disease (JOHD) have been based primarily on qualitative caregiver reports or retrospective medical record reviews. This study aims to quantify differences in behavior in patients with JOHD using informant- and self-report questionnaires. Informants of 21 children\/young adults (12 female) with JOHD and 115 children\/young adults (64 female) with a family history of Huntington\u2019s Disease, but who did not inherit the disease themselves (Gene-Non-Expanded; GNE) completed the Behavior Rating Inventory of Executive Function (BRIEF) and the Pediatric Behavior Scale (PBS). Mixed linear regression models (age\/sex adjusted) were conducted to assess group differences on these measures. The JOHD group had significantly higher scores, indicating more problems, than the GNE group on all BRIEF subscales, and measures of Aggression\/Opposition and Hyperactivity\/Inattention of the PBS (all p &lt; 0.05). There were no group differences in Depression\/Anxiety. Inhibit, Plan\/Organize, Initiate, and Aggression\/Opposition had significant negative correlations with Cytosine-Adenine-Guanine (CAG) repeat length (all p &lt; 0.05) meaning that individuals with higher CAG repeats scored lower on these measures. There was greater discrepancy between higher informant-vs. lower self-reported scores in the JOHD group, supporting the notion of lack of insight for the JOHD-affected group. These results provide quantitative evidence of behavioral characteristics of JOHD.<\/jats:p>","DOI":"10.3390\/brainsci10080543","type":"journal-article","created":{"date-parts":[[2020,8,11]],"date-time":"2020-08-11T09:28:57Z","timestamp":1597138137000},"page":"543","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Behavioral Deficits in Juvenile Onset Huntington\u2019s Disease"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6875-6320","authenticated-orcid":false,"given":"Kathleen E.","family":"Langbehn","sequence":"first","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ashley M.","family":"Cochran","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ellen","family":"van der Plas","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amy L.","family":"Conrad","sequence":"additional","affiliation":[{"name":"Stead Family Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eric","family":"Epping","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erin","family":"Martin","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patricia","family":"Espe-Pfeifer","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8810-1903","authenticated-orcid":false,"given":"Peg","family":"Nopoulos","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA"},{"name":"Stead Family Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA"},{"name":"Department of Neurology, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,8,11]]},"reference":[{"key":"ref_1","unstructured":"Quarrell, O.W.J., Brewer, H.M., Squitieri, F., Barker, R.A., Nance, M.A., and Landwehrmeyer, G.B. (2009). The clinical phenotype of juvenile Huntington\u2019s Disease. Juvenile Huntington\u2019s Disease and Other Trinucleotide Repeat Disorders, Oxford University Press."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"986","DOI":"10.1016\/S1474-4422(18)30294-1","article-title":"Biological and clinical manifestations of juvenile Huntington\u2019s disease: A retrospective analysis","volume":"17","author":"Fusilli","year":"2018","journal-title":"Lancet Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"223","DOI":"10.2310\/7010.2006.00055","article-title":"Clinical characteristics of childhood-onset (juvenile) Huntington disease: Report of 12 patients and review of the literature","volume":"21","author":"Afifi","year":"2006","journal-title":"J. Child. Neurol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"307","DOI":"10.2217\/nmt-2017-0019","article-title":"A survey-based study identifies common but unrecognized symptoms in a large series of juvenile Huntington\u2019s disease","volume":"7","author":"Moser","year":"2017","journal-title":"Neurodegener. Dis. Manag."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1001\/archneur.64.6.813","article-title":"Psychiatric and cognitive difficulties as indicators of juvenile Huntington disease onset in 29 patients","volume":"64","author":"Ribai","year":"2007","journal-title":"Arch. Neurol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1111\/j.1399-0004.2006.00624.x","article-title":"The personal experience of juvenile Huntington\u2019s disease: An interpretative phenomenological analysis of parents\u2019 accounts of the primary features of a rare genetic condition","volume":"69","author":"Smith","year":"2006","journal-title":"Clin. Genet."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"171","DOI":"10.3233\/JHD-180339","article-title":"Clinical Presentation and Features of Juvenile-Onset Huntington\u2019s Disease: A Systematic Review","volume":"8","author":"Cronin","year":"2019","journal-title":"J. Huntingt. Dis."},{"key":"ref_8","unstructured":"Quarrell, O.W.J., Brewer, H.M., Squitieri, F., Barker, R.A., Nance, M.A., and Landwehrmeyer, G.B. (2009). The treatment of juvenile Huntington\u2019s disease. Juvenile Huntington\u2019s Disease and Other Trinucleotide Repeat Disorders, Oxford University Press."},{"key":"ref_9","first-page":"140","article-title":"Correlation between magnetic resonance and genetic, clinical, neurophysiological and neuropsychological studies of patients with juvenile form of Huntington\u2019s disease","volume":"2","author":"Poniatowska","year":"2001","journal-title":"Case Rep. Clin. Pract. Rev."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1007\/s11920-017-0759-9","article-title":"Juvenile Huntington\u2019s Disease: Diagnostic and Treatment Considerations for the Psychiatrist","volume":"19","author":"Quigley","year":"2017","journal-title":"Curr. Psychiatry Rep."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1055\/s-2000-7461","article-title":"Huntington disease in children: Genotype-phenotype correlation","volume":"31","author":"Rasmussen","year":"2000","journal-title":"Neuropediatrics"},{"key":"ref_12","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_13","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1016\/j.dcn.2017.11.008","article-title":"Structural brain development: A review of methodological approaches and best practices","volume":"33","author":"Vijayakumar","year":"2018","journal-title":"Dev. Cogn. Neurosci."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Gioia, G.A., Isquith, P., Guy, S., and Kenworthy, L. (2000). Behavior Rating Inventory of Executive Function: Professional Manual, Psychological Assessment Resources, Inc.","DOI":"10.1037\/t73087-000"},{"key":"ref_15","unstructured":"Prinz, E. (1987). Assessing Child Behavior Problems in a Medical Setting: Development of the Pediatric Behavior Scale. Advances in Behavioral Assessment of Children and Families, JAI."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"267","DOI":"10.2217\/nmt.13.18","article-title":"Managing juvenile Huntington\u2019s disease","volume":"3","author":"Quarrell","year":"2013","journal-title":"Neurodegener. Dis. Manag."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1093\/clipsy.9.3.300","article-title":"Informant-reports of personality disorder: Relation to self-reports and future research directions","volume":"9","author":"Klonsky","year":"2002","journal-title":"Clin. Psychol. Sci. Pract."},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Quarrell, O., O\u2019Donovan, K.L., Bandmann, O., and Strong, M. (2012). The Prevalence of Juvenile Huntington\u2019s Disease: A Review of the Literature and Meta-Analysis. PLoS Curr., 4.","DOI":"10.1371\/4f8606b742ef3"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"257","DOI":"10.5334\/tohm.231","article-title":"The many facets of unawareness in huntington disease","volume":"4","author":"McCusker","year":"2014","journal-title":"Tremor Other Hyperkinet. Mov."}],"container-title":["Brain Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-3425\/10\/8\/543\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T09:59:16Z","timestamp":1760176756000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-3425\/10\/8\/543"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,8,11]]},"references-count":19,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2020,8]]}},"alternative-id":["brainsci10080543"],"URL":"https:\/\/doi.org\/10.3390\/brainsci10080543","relation":{},"ISSN":["2076-3425"],"issn-type":[{"type":"electronic","value":"2076-3425"}],"subject":[],"published":{"date-parts":[[2020,8,11]]}}}