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. 2023 Nov;270(11):5408-5417.
doi: 10.1007/s00415-023-11804-0. Epub 2023 Jul 18.

Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease: a comparison of assessment instruments

Andrea Horta-Barba  1   2   3   4   5 , Saul Martinez-Horta  1   2   3   4   5 , Jesús Pérez-Pérez  1   2   3   4   5 , Arnau Puig-Davi  2   3   4   5 , Natascia de Lucia  5   6 , Giuseppe de Michele  5   6 , Elena Salvatore  5   6 , Stefanie Kehrer  5   7 , Josef Priller  5   7 , Simone Migliore  8 , Ferdinando Squitieri  8 , Anna Castaldo  5   9 , Caterina Mariotti  5   9 , Veronica Mañanes  5   10 , Jose Luis Lopez-Sendon  5   10 , Noelia Rodriguez  5   11 , Asunción Martinez-Descals  5   11 , Filipa Júlio  5   12   13 , Cristina Januário  5   12   13 , Marianna Delussi  5   14 , Marina de Tommaso  5   14 , Sandra Noguera  5   15 , Jesús Ruiz-Idiago  5   15 , Emilia J Sitek  5   16   17 , Renata Wallner  18 , Angela Nuzzi  5 , Javier Pagonabarraga  1   2   3   4   5 , Jaime Kulisevsky  19   20   21   22   23 ; Cognitive Phenotype Working Group of the European Huntington’s Disease Network
Affiliations

Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease: a comparison of assessment instruments

Andrea Horta-Barba et al. J Neurol. 2023 Nov.

Abstract

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.

Keywords: Cognition; Dementia; Disease progression; Huntington’s disease; Mild cognitive impairment; Neuropsychology.

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Conflict of interest statement

Nothing to report.

Figures

Fig. 1
Fig. 1
Receiver operating characteristic (ROC) curves illustrating the discriminative properties of the PD-CRS, the MMSE and the UHDRS cogscore
Fig. 2
Fig. 2
Cognitive trajectory in terms of percentage of change with respect to baseline along follow-up visits for each cognitive instrument and group
See this image and copyright information in PMC

References

    1. Hart EP, Dumas EM, Giltay EJ, Middelkoop HAM, Roos RAC. Cognition in Huntington’s disease in manifest, premanifest and converting gene carriers over ten years. J Huntingtons Dis. 2013;2(2):137–147. doi: 10.3233/JHD-130059. - DOI - PubMed
    1. Zhang Y, Zhou J, Gehl CR, Long JD, Johnson H, Magnotta VA, et al. Mild cognitive impairment as an early landmark in Huntington’s disease. Front Neurol. 2021;12(July):1–10. - PMC - PubMed
    1. Duff K, Paulsen J, Mills J, Beglinger LJ, Moser DJ, Smith MM, et al. Mild cognitive impairment in prediagnosed Huntington disease. Neurology. 2010;75(6):500–507. doi: 10.1212/WNL.0b013e3181eccfa2. - DOI - PMC - PubMed
    1. Horta-Barba A, Martínez-Horta S, Pérez-Pérez J, Sampedro F, Puig-Davi A, Pagonabarraga J, et al. Measuring the functional impact of cognitive impairment in Huntington’s disease. J Neurol [Internet] 2022;269(7):3541–3549. doi: 10.1007/s00415-021-10955-2. - DOI - PMC - PubMed
    1. Martinez-Horta S, Sampedro F, Horta-Barba A, Perez-Perez J, Pagonabarraga J, Gomez-Anson B, et al. Structural brain correlates of dementia in Huntington’s disease. NeuroImage Clin [Internet] 2020;28(1):102415. doi: 10.1016/j.nicl.2020.102415. - DOI - PMC - PubMed

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