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Published on: June 9, 2018
Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross-sectional study
Simone Migliore1, Giulia D'Aurizio2, Sabrina Maffi1
1Huntington and Rare Diseases Unit, Fondazione IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo, Italy.
Insights
In Huntington disease (HD), apathy and perseveration severity correlate with cognitive decline, impacting independence. This highlights a common dysexecutive syndrome underlying cognitive and behavioral changes in HD patients.
Area of Science:
- Neuroscience
- Neurology
- Psychiatry
Background:
- Huntington disease (HD) presents with progressive behavioral and cognitive changes across all stages.
- Understanding the interplay between cognitive and behavioral symptoms is crucial for managing HD progression and patient independence.
Purpose of the Study:
- To investigate the association between cognitive and behavioral scale scores in manifest Huntington disease (HD) over a two-year period.
- To examine how motor and functional changes correlate with cognitive and behavioral domains in HD patients.
Main Methods:
- A multicenter, retrospective study involving 97 patients with manifest HD.
- Cognitive and behavioral domains assessed using the Unified Huntington's Disease Rating Scale (UHDRS) and Problem Behaviors Assessment Short Form (PBA-s) at baseline, 1 year, and 2 years.
- Motor and functional changes evaluated by UHDRS over time to confirm disease progression.
Main Results:
- Apathy and perseveration/obsession severity significantly correlated with cognitive decline (p < .0001).
- Irritability linked to verbal fluency perseveration errors (t0), while psychosis correlated with processing speed (t0) and verbal fluency errors (t1).
- Confirmed disease progression through significant worsening of UHDRS motor and functional scores over two years (p < .0001).
Conclusions:
- Apathy and perseveration severity are significantly associated with cognitive impairment in HD, contributing to disease progression and loss of independence.
- The observed cognitive-behavior pattern suggests a common underlying dysexecutive syndrome in Huntington disease.
- While abnormal behavioral progression is unpredictable, understanding these associations aids in managing HD's impact on patients.
Introduction:
Behavioral 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.
Methods:
We 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.
Results:
The 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).
Conclusion:
Although 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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