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Article

Behavioral Deficits in Juvenile Onset Huntington’s Disease

1
Department of Psychiatry, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA
2
Stead Family Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA
3
Department of Neurology, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA
*
Authors to whom correspondence should be addressed.
Brain Sci. 2020, 10(8), 543; https://doi.org/10.3390/brainsci10080543
Received: 14 July 2020 / Revised: 7 August 2020 / Accepted: 7 August 2020 / Published: 11 August 2020
(This article belongs to the Special Issue Juvenile Onset Huntington's Disease)
Reports of behavioral disturbance in Juvenile-Onset Huntington’s 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’s 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 < 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 < 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. View Full-Text
Keywords: Huntington’s disease; behavioral regulation; executive function; trinucleotide repeat disorder Huntington’s disease; behavioral regulation; executive function; trinucleotide repeat disorder
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MDPI and ACS Style

Langbehn, K.E.; Cochran, A.M.; van der Plas, E.; Conrad, A.L.; Epping, E.; Martin, E.; Espe-Pfeifer, P.; Nopoulos, P. Behavioral Deficits in Juvenile Onset Huntington’s Disease. Brain Sci. 2020, 10, 543. https://doi.org/10.3390/brainsci10080543

AMA Style

Langbehn KE, Cochran AM, van der Plas E, Conrad AL, Epping E, Martin E, Espe-Pfeifer P, Nopoulos P. Behavioral Deficits in Juvenile Onset Huntington’s Disease. Brain Sciences. 2020; 10(8):543. https://doi.org/10.3390/brainsci10080543

Chicago/Turabian Style

Langbehn, Kathleen E., Ashley M. Cochran, Ellen van der Plas, Amy L. Conrad, Eric Epping, Erin Martin, Patricia Espe-Pfeifer, and Peg Nopoulos. 2020. "Behavioral Deficits in Juvenile Onset Huntington’s Disease" Brain Sciences 10, no. 8: 543. https://doi.org/10.3390/brainsci10080543

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