Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease: a comparison of assessment instruments
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Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
Published on: June 9, 2018
Andrea Horta-Barba1,2,3,4,5, Saul Martinez-Horta1,2,3,4,5, Jesús Pérez-Pérez1,2,3,4,5
1Department of Medicine, Autonomous University of Barcelona (UAB), Bellaterra, Spain.
Insights
The Parkinson's Disease-Cognitive Rating Scale (PD-CRS) effectively identifies mild cognitive impairment and dementia in Huntington's disease (HD). It reliably tracks cognitive changes over time in HD patients.
Area of Science:
- Neurology
- Neuroscience
- Genetics
Background:
- Huntington's disease (HD) is characterized by progressive cognitive decline.
- Current methods for classifying cognitive severity and monitoring progression in HD are insufficient.
- Reliable tools are needed to assess cognitive status in HD patients.
Purpose of the Study:
- To evaluate the utility of the Parkinson's Disease-Cognitive Rating Scale (PD-CRS) in classifying cognitive status in HD.
- To assess the sensitivity and reliability of PD-CRS in monitoring cognitive progression.
- To establish cutoff scores for PD-CRS to differentiate cognitive categories in HD.
Main Methods:
- Data from 180 gene-carriers (33 premanifest HD, 147 manifest HD) were analyzed.
- Participants were classified into normal cognition, mild cognitive impairment (MCI), and dementia groups.
- PD-CRS, MMSE, and UHDRS cogscore were administered at baseline and 6- and 12-month follow-ups.
Main Results:
- PD-CRS demonstrated excellent sensitivity and specificity for identifying MCI and dementia.
- PD-CRS captured cognitive progression and conversion between cognitive categories across all groups.
- The scale effectively identified different cognitive trajectories over time.
Conclusions:
- PD-CRS is a valid and reliable instrument for diagnosing MCI and dementia in HD.
- PD-CRS accurately reflects cognitive decline and progression in Huntington's disease.
- The scale shows significant sensitivity to change in cognitive status in HD patients.
Background:
Progressive cognitive decline is an inevitable feature of Huntington's disease (HD) but specific criteria and instruments are still insufficiently developed to reliably classify patients into categories of cognitive severity and to monitor the progression of cognitive impairment.
Methods:
We collected data from a cohort of 180 positive gene-carriers: 33 with premanifest HD and 147 with manifest HD. Using a specifically developed gold-standard for cognitive status we classified participants into those with normal cognition, those with mild cognitive impairment, and those with dementia. We administered the Parkinson's Disease-Cognitive Rating Scale (PD-CRS), the MMSE and the UHDRS cogscore at baseline, and at 6-month and 12-month follow-up visits. Cutoff scores discriminating between the three cognitive categories were calculated for each instrument. For each cognitive group and instrument we addressed cognitive progression, sensitivity to change, and the minimally clinical important difference corresponding to conversion from one category to another.
Results:
The PD-CRS cutoff scores for MCI and dementia showed excellent sensitivity and specificity ratios that were not achieved with the other instruments. Throughout follow-up, in all cognitive groups, PD-CRS captured the rate of conversion from one cognitive category to another and also the different patterns in terms of cognitive trajectories.
Conclusion:
The PD-CRS is a valid and reliable instrument to capture MCI and dementia syndromes in HD. It captures the different trajectories of cognitive progression as a function of cognitive status and shows sensitivity to change in MCI and dementia.
