Behavioral Deficits in Juvenile Onset Huntington’s Disease

Kathleen E Langbehn; Ashley M Cochran; Ellen van der Plas; Amy L Conrad; Eric Epping; Erin Martin; Patricia Espe-Pfeifer; Peg Nopoulos

doi:10.3390/brainsci10080543

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Behavioral Deficits in Juvenile Onset Huntington’s Disease

Journal article

Open access

Peer reviewed

Behavioral Deficits in Juvenile Onset Huntington’s Disease

Kathleen E Langbehn, Ashley M Cochran, Ellen van der Plas, Amy L Conrad, Eric Epping, Erin Martin, Patricia Espe-Pfeifer and Peg Nopoulos

Brain sciences, Vol.10(8), p.543

08/11/2020

DOI: 10.3390/brainsci10080543

PMID: 32796698

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Abstract

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.

Details

Title: Subtitle: Behavioral Deficits in Juvenile Onset Huntington’s Disease
Creators: Kathleen E Langbehn
Ashley M Cochran
Ellen van der Plas
Amy L Conrad
Eric Epping
Erin Martin
Patricia Espe-Pfeifer
Peg Nopoulos
Resource Type: Journal article
Publication Details: Brain sciences, Vol.10(8), p.543
DOI: 10.3390/brainsci10080543
PMID: 32796698
NLM abbreviation: Brain Sci
ISSN: 2076-3425
eISSN: 2076-3425
Grant note: DOI: 10.13039/100000065, name: National Institute of Neurological Disorders and Stroke, award: R01NS055903
Language: English
Date published: 08/11/2020
Academic Unit: Neurology; Psychiatry; Pediatric Psychology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Craniofacial Anomalies Research Center
Record Identifier: 9984070826302771

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