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Multicenter Study
. 2020 May;267(5):1527-1535.
doi: 10.1007/s00415-020-09730-6. Epub 2020 Feb 7.

Utility of the Parkinson's disease-Cognitive Rating Scale for the screening of global cognitive status in Huntington's disease

Saul Martinez-Horta  1   2   3   4   5 , Andrea Horta-Barba  1   2   3   5 , Jesús Perez-Perez  1   2   3   4   5 , Frederic Sampedro  1   2   3   4 , Natascia de Lucia  5   6 , Giuseppe De Michele  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 , Pedro Garcia-Ruiz  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 , Jesus Ruiz-Idiago  5   15   16 , Emilia J Sitek  5   17   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
Multicenter Study

Utility of the Parkinson's disease-Cognitive Rating Scale for the screening of global cognitive status in Huntington's disease

Saul Martinez-Horta et al. J Neurol. 2020 May.

Erratum in

  • Correction to: Utility of the Parkinson's disease-Cognitive Rating Scale for the screening of global cognitive status in Huntington's disease.
    Martinez-Horta S, Horta-Barba A, Perez-Perez J, Sampedro F, de Lucia N, De Michele G, Kehrer S, Priller J, Migliore S, Squitieri F, Castaldo A, Mariotti C, Mañanes V, Lopez-Sendon JL, Rodriguez N, Martinez-Descals A, Garcia-Ruiz P, Júlio F, Januário C, Delussi M, de Tommaso M, Noguera S, Ruiz-Idiago J, Sitek EJ, Nuzzi A, Pagonabarraga J, Kulisevsky J; Cognitive Phenotype Working Group of the European Huntington’s Disease Network. Martinez-Horta S, et al. J Neurol. 2021 Feb;268(2):740. doi: 10.1007/s00415-020-10305-8. J Neurol. 2021. PMID: 33399970 No abstract available.

Abstract

Background: Cognitive impairment is an essential feature of Huntington's disease (HD) and dementia is a predictable outcome in all patients. However, validated instruments to assess global cognitive performance in the field of HD are lacking.

Objectives: We aimed to explore the utility of the Parkinson's disease-Cognitive Rating Scale (PD-CRS) for the screening of global cognition in HD.

Methods: A multicenter cohort of 132 HD patients at different disease stages and 33 matched healthy controls were classified as having preserved cognition, mild cognitive impairment (HD-MCI) or dementia (HD-Dem) according to the Clinical Dementia Rating and Functional Independence Score. The PD-CRS and the Mini-Mental State Examination were administered. Receiver operating characteristic curve analysis was used to determine optimal cutoffs to differentiate patients according to their cognitive status.

Results: A PD-CRS cutoff score ≤ 81/82 was optimal to detect HD-MCI (sensitivity = 93%; specificity = 80%; area under the curve (AUC) = 0.940), and ≤ 63/64 was optimal to detect HD-Dem (sensitivity = 90%; specificity = 87%; AUC = 0.933). MMSE scores failed to show robust psychometric properties in this context.

Discussion: The PD-CRS is a valid and reliable instrument to assess global cognition in HD in routine clinical care and clinical trials.

Keywords: Cognition; Cognitive assessment; Huntington’s disease; Neuropsychology; Psychometrics.

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