Related Experiment Video
Updated: Aug 8, 2026
Development of a Virtual Reality Assessment of Everyday Living Skills
Published on: April 23, 2014
Measuring the functional impact of cognitive impairment in Huntington's disease
Andrea Horta-Barba1,2,3,4,5, Saül Martínez-Horta1,2,3,4,5, Jesus Pérez-Pérez1,2,3,4,5
1Movement Disorders Unit, Neurology Department, Hospital de la Santa Creu i Sant Pau, Mas Casanovas 90, 08041, Barcelona, Spain.
Insights
A new Huntington's disease (HD) questionnaire, the HD-CFRS, effectively measures functional decline linked to cognitive impairment. This tool aids clinicians and researchers in assessing HD patients, particularly those with mild cognitive impairment or dementia.
Area of Science:
- Neuroscience
- Clinical Neurology
- Psychometrics
Background:
- Huntington's disease (HD) presents with diverse cognitive, behavioral, and motor symptoms.
- A validated tool to specifically assess the impact of cognitive impairment on functional capacity in HD is currently lacking.
Purpose of the Study:
- To develop and validate a brief questionnaire, the Huntington's Disease-Cognitive Functional Rating Scale (HD-CFRS), for assessing functional aspects sensitive to cognitive impairment in HD.
Main Methods:
- The HD-CFRS was developed and validated in 78 symptomatic Huntington's disease mutation carriers.
- Informant ratings were collected to assess agreement with patient self-reports.
- Objective cognitive measures were administered, and participants were categorized into cognitively preserved (HD-NC), mild cognitive impairment (HD-MCI), or dementia (HD-Dem) groups.
Main Results:
- The HD-CFRS demonstrated concurrent validity and internal consistency across all cognitive groups.
- Informants reported greater functional impairment than patients in the HD-MCI and HD-Dem groups.
- HD-CFRS scores strongly correlated with objective cognitive assessment measures.
Conclusions:
- The HD-CFRS is a brief, reliable instrument for measuring functional deficits related to cognitive impairment in Huntington's disease.
- This scale is valuable for both clinical practice and research in HD management.
Background:
Patients with Huntington's disease (HD) exhibit a variable predominance of cognitive, behavioral and motor symptoms. A specific instrument focusing on the impact of cognitive impairment in HD over functional capacity is lacking.
Objective:
To address the need for a brief and specifically developed HD questionnaire able to capture functional aspects suspected to be sensitive to cognitive impairment.
Methods:
We developed and validated the "Huntington's Disease-Cognitive Functional Rating Scale" (HD-CFRS) in 78 symptomatic carriers of the Huntington's disease mutation. We also administered the HD-CFRS to a knowledgeable informant to measure the level of agreement. To explore the association between HD-CFRS scores and participants' cognitive status, we administered objective measures of cognition. Participants were classified as cognitively preserved (HD-NC), as having mild cognitive impairment (HD-MCI), or as having dementia (HD-Dem).
Results:
The HD-CFRS showed concurrent validity and internal consistency in the three groups. HD carriers and informants in the HD-NC group obtained similar HD-CFRS scores. However, in patients with mild cognitive impairment and dementia, informers reported greater functional impairment than HD participants. The HD-CFRS total score showed strong correlations with measures assessing cognition.
Conclusions:
These findings support the utility of the HD-CFRS as a brief and reliable instrument to measure functional defects associated with cognitive impairment in HD. We believe this questionnaire could be a useful tool both for clinical practice and research.
More Related Videos
09:06Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
Published on: June 9, 2018
07:26Characterizing the Relationship Between Eye Movement Parameters and Cognitive Functions in Non-demented Parkinson's Disease Patients with Eye Tracking
Published on: September 26, 2019
