ArticleBrain and behavior2021
Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross-sectional study.
Article in Brain and behavior, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 1 of them a synthesis that pooled it.
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12 citing papers in PubMed, 1 synthesis or guideline pooled it, 18 citations in OpenAlex.
- Emotion recognition in people with Huntington's disease: A comprehensive systematic review.Journal of Huntington's disease · 2026Pooled it
- Lipid Composition Drives Mutant Huntingtin Dimerization and Membrane Association: Insights from Computational Simulations.Molecules (Basel, Switzerland) · 2026Article
- Reliability and validity of the Huntington's Disease Everyday Functioning (Hi-DEF): A patient-reported measure of cognitive capacity on daily functioning in Huntington's disease.Journal of Huntington's disease · 2025Article
- Pathomechanisms of behavioral abnormalities in Huntington disease: an update.Journal of neural transmission (Vienna, Austria : 1996) · 2024Review
- The validation of the Italian version of multiple sclerosis neuropsychological screening questionnaire in Huntington's disease.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2023Article
- Effects of Physical Activity on Cognitive Functioning: The Role of Cognitive Reserve and Active Aging.Brain sciences · 2023Article
- Using a Clinical Formulation to Understand Psychological Distress in People Affected by Huntington's Disease: A Descriptive, Evidence-Based Model.Journal of personalized medicine · 2022Article
- Sleep Quality and Related Clinical Manifestations in Huntington Disease.Journal of personalized medicine · 2022Article
- Self-Reported Social Relationship Capacities Predict Motor, Functional and Cognitive Decline in Huntington's Disease.Journal of personalized medicine · 2022Article
- More than Just a Brain Disorder: A Five-Point Manifesto for Psychological Care for People with Huntington's Disease.Journal of personalized medicine · 2022Article
- Another Perspective on Huntington's Disease: Disease Burden in Family Members and Pre-Manifest HD When Compared to Genotype-Negative Participants from ENROLL-HD.Brain sciences · 2021Article
- Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross-sectional study.Brain and behavior · 2021Article
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Authors and funding
10 authors at 5 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionBehavioral and cognitive changes can be observed across all Huntington disease (HD) stages. Our multicenter and retrospective study investigated the association between cognitive and behavioral scale scores in manifest HD, at three different yearly timepoints.
methodsWe analyzed cognitive and behavioral domains by the Unified Huntington's Disease Rating Scale (UHDRS) and by the Problem Behaviors Assessment Short Form (PBA-s), at three different yearly times of life (t0 or baseline, t1 after one year, t2 after two years), in 97 patients with manifest HD (mean age 48.62 ± 13.1), from three ENROLL-HD Centers. In order to test the disease progression, we also examined patients' motor and functional changes by the UHDRS, overtime.
resultsThe severity of apathy and of perseveration/obsession was associated with the severity of the cognitive decline (p < .0001), regardless of the yearly timepoint. The score of irritability significantly and positively correlated with perseveration errors in the verbal fluency test at t0 (r = .34; p = .001), while the psychosis significantly and negatively correlated with the information processing speed at t0 (r = -.21; p = .038) and significantly and positively correlated with perseveration errors in the verbal fluency test at t1 (r = .35; p < .0001). The disease progression was confirmed by the significant worsening of the UHDRS-Total Motor Score (TMS) and of the UHDRS-Total Functional Capacity (TFC) scale score after two-year follow-up (p < .0001).
conclusionAlthough the progression of abnormal behavioral manifestations cannot be predicted in HD, the severity of apathy and perseveration/obsessions are significantly associated with the severity of the cognitive function impairment, thus contributing, together, to the disease development and to patients' loss of independence, in addition to the neurological manifestations. This cognitive-behavior pattern determines a common underlying deficit depending on a dysexecutive syndrome.
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