Skip to main content
Top
Published in: Journal of Neurology 5/2020

01-05-2020 | Dementia | Original Communication

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

Authors: Saul Martinez-Horta, Andrea Horta-Barba, Jesús Perez-Perez, Frederic Sampedro, Natascia de Lucia, Giuseppe De Michele, Stefanie Kehrer, Josef Priller, Simone Migliore, Ferdinando Squitieri, Anna Castaldo, Caterina Mariotti, Veronica Mañanes, Jose Luis Lopez-Sendon, Noelia Rodriguez, Asunción Martinez-Descals, Pedro Garcia-Ruiz, Filipa Júlio, Cristina Januário, Marianna Delussi, Marina de Tommaso, Sandra Noguera, Jesus Ruiz-Idiago, Emilia J. Sitek, Angela Nuzzi, Javier Pagonabarraga, Jaime Kulisevsky, Cognitive Phenotype Working Group of the European Huntington’s Disease Network

Published in: Journal of Neurology | Issue 5/2020

Login to get access

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.
Appendix
Available only for authorised users
Literature
4.
go back to reference Tabrizi SJ, Langbehn DR, Leavitt BR, Roos RA, Durr A, Craufurd D, Kennard C, Hicks SL, Fox NC, Scahill RI, Borowsky B, Tobin AJ, Rosas HD, Johnson H, Reilmann R, Landwehrmeyer B, Stout JC (2009) Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data. Lancet Neurol 8(9):791–801. https://doi.org/10.1016/S1474-4422(09)70170-XCrossRefPubMedPubMedCentral Tabrizi SJ, Langbehn DR, Leavitt BR, Roos RA, Durr A, Craufurd D, Kennard C, Hicks SL, Fox NC, Scahill RI, Borowsky B, Tobin AJ, Rosas HD, Johnson H, Reilmann R, Landwehrmeyer B, Stout JC (2009) Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data. Lancet Neurol 8(9):791–801. https://​doi.​org/​10.​1016/​S1474-4422(09)70170-XCrossRefPubMedPubMedCentral
6.
go back to reference Dogan I, Eickhoff SB, Schulz JB, Shah NJ, Laird AR, Fox PT, Reetz K (2013) Consistent neurodegeneration and its association with clinical progression in Huntington's disease: a coordinate-based meta-analysis. Neuro-degener Dis 12(1):23–35. https://doi.org/10.1159/000339528CrossRef Dogan I, Eickhoff SB, Schulz JB, Shah NJ, Laird AR, Fox PT, Reetz K (2013) Consistent neurodegeneration and its association with clinical progression in Huntington's disease: a coordinate-based meta-analysis. Neuro-degener Dis 12(1):23–35. https://​doi.​org/​10.​1159/​000339528CrossRef
9.
go back to reference Sampedro F, Martinez-Horta S, Perez-Perez J, Horta-Barba A, Lopez-Mora DA, Camacho V, Fernandez-Leon A, Gomez-Anson B, Carrio I, Kulisevsky J (2019) Cortical atrophic-hypometabolic dissociation in the transition from premanifest to early-stage Huntington's disease. Eur J Nucl Med Mol Imaging 46(5):1111–1116. https://doi.org/10.1007/s00259-018-4257-zCrossRefPubMed Sampedro F, Martinez-Horta S, Perez-Perez J, Horta-Barba A, Lopez-Mora DA, Camacho V, Fernandez-Leon A, Gomez-Anson B, Carrio I, Kulisevsky J (2019) Cortical atrophic-hypometabolic dissociation in the transition from premanifest to early-stage Huntington's disease. Eur J Nucl Med Mol Imaging 46(5):1111–1116. https://​doi.​org/​10.​1007/​s00259-018-4257-zCrossRefPubMed
12.
go back to reference Tabrizi SJ, Reilmann R, Roos RA, Durr A, Leavitt B, Owen G, Jones R, Johnson H, Craufurd D, Hicks SL, Kennard C, Landwehrmeyer B, Stout JC, Borowsky B, Scahill RI, Frost C, Langbehn DR (2012) Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data. Lancet Neurol 11(1):42–53. https://doi.org/10.1016/S1474-4422(11)70263-0CrossRefPubMed Tabrizi SJ, Reilmann R, Roos RA, Durr A, Leavitt B, Owen G, Jones R, Johnson H, Craufurd D, Hicks SL, Kennard C, Landwehrmeyer B, Stout JC, Borowsky B, Scahill RI, Frost C, Langbehn DR (2012) Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data. Lancet Neurol 11(1):42–53. https://​doi.​org/​10.​1016/​S1474-4422(11)70263-0CrossRefPubMed
13.
17.
go back to reference Ringkobing SP, Larsen IU, Jorgensen K, Vinther-Jensen T, Vogel A (2019) Cognitive screening tests in huntington gene mutation carriers: examining the validity of the mini-mental state examination and the montreal cognitive assessment. J Huntington's Dis. https://doi.org/10.3233/JHD-190350CrossRef Ringkobing SP, Larsen IU, Jorgensen K, Vinther-Jensen T, Vogel A (2019) Cognitive screening tests in huntington gene mutation carriers: examining the validity of the mini-mental state examination and the montreal cognitive assessment. J Huntington's Dis. https://​doi.​org/​10.​3233/​JHD-190350CrossRef
21.
go back to reference Skorvanek M, Goldman JG, Jahanshahi M, Marras C, Rektorova I, Schmand B, van Duijn E, Goetz CG, Weintraub D, Stebbins GT, Martinez-Martin P (2018) Global scales for cognitive screening in Parkinson's disease: critique and recommendations. Mov Disord 33(2):208–218. https://doi.org/10.1002/mds.27233CrossRefPubMed Skorvanek M, Goldman JG, Jahanshahi M, Marras C, Rektorova I, Schmand B, van Duijn E, Goetz CG, Weintraub D, Stebbins GT, Martinez-Martin P (2018) Global scales for cognitive screening in Parkinson's disease: critique and recommendations. Mov Disord 33(2):208–218. https://​doi.​org/​10.​1002/​mds.​27233CrossRefPubMed
25.
go back to reference Hughes CP, Berg L, Danziger WL, Coben LA, Martin RL (1982) A new clinical scale for the staging of dementia. Br J Psychiatry 140:566–572CrossRef Hughes CP, Berg L, Danziger WL, Coben LA, Martin RL (1982) A new clinical scale for the staging of dementia. Br J Psychiatry 140:566–572CrossRef
26.
go back to reference Callaghan J, Stopford C, Arran N, Boisse MF, Coleman A, Santos RD, Dumas EM, Hart EP, Justo D, Owen G, Read J, Say MJ, Durr A, Leavitt BR, Roos RA, Tabrizi SJ, Bachoud-Levi AC, Bourdet C, van Duijn E, Craufurd D (2015) Reliability and factor structure of the Short Problem Behaviors Assessment for Huntington's disease (PBA-s) in the TRACK-HD and REGISTRY studies. J Neuropsychiatry Clin Neurosci 27(1):59–64. https://doi.org/10.1176/appi.neuropsych.13070169CrossRefPubMed Callaghan J, Stopford C, Arran N, Boisse MF, Coleman A, Santos RD, Dumas EM, Hart EP, Justo D, Owen G, Read J, Say MJ, Durr A, Leavitt BR, Roos RA, Tabrizi SJ, Bachoud-Levi AC, Bourdet C, van Duijn E, Craufurd D (2015) Reliability and factor structure of the Short Problem Behaviors Assessment for Huntington's disease (PBA-s) in the TRACK-HD and REGISTRY studies. J Neuropsychiatry Clin Neurosci 27(1):59–64. https://​doi.​org/​10.​1176/​appi.​neuropsych.​13070169CrossRefPubMed
32.
go back to reference Labuschagne I, Cassidy AM, Scahill RI, Johnson EB, Rees E, O'Regan A, Queller S, Frost C, Leavitt BR, Durr A, Roos R, Owen G, Borowsky B, Tabrizi SJ, Stout JC (2016) Visuospatial processing deficits linked to posterior brain regions in premanifest and early stage Huntington's disease. J Int Neuropsychol Soc 22(6):595–608. https://doi.org/10.1017/S1355617716000321CrossRefPubMed Labuschagne I, Cassidy AM, Scahill RI, Johnson EB, Rees E, O'Regan A, Queller S, Frost C, Leavitt BR, Durr A, Roos R, Owen G, Borowsky B, Tabrizi SJ, Stout JC (2016) Visuospatial processing deficits linked to posterior brain regions in premanifest and early stage Huntington's disease. J Int Neuropsychol Soc 22(6):595–608. https://​doi.​org/​10.​1017/​S135561771600032​1CrossRefPubMed
Metadata
Title
Utility of the Parkinson’s disease-Cognitive Rating Scale for the screening of global cognitive status in Huntington’s disease
Authors
Saul Martinez-Horta
Andrea Horta-Barba
Jesús Perez-Perez
Frederic Sampedro
Natascia de Lucia
Giuseppe De Michele
Stefanie Kehrer
Josef Priller
Simone Migliore
Ferdinando Squitieri
Anna Castaldo
Caterina Mariotti
Veronica Mañanes
Jose Luis Lopez-Sendon
Noelia Rodriguez
Asunción Martinez-Descals
Pedro Garcia-Ruiz
Filipa Júlio
Cristina Januário
Marianna Delussi
Marina de Tommaso
Sandra Noguera
Jesus Ruiz-Idiago
Emilia J. Sitek
Angela Nuzzi
Javier Pagonabarraga
Jaime Kulisevsky
Cognitive Phenotype Working Group of the European Huntington’s Disease Network
Publication date
01-05-2020
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 5/2020
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-020-09730-6

Other articles of this Issue 5/2020

Journal of Neurology 5/2020 Go to the issue