TY - JOUR
T1 - Cognitive decline in Huntington's disease expansion gene carriers
AU - REGISTRY Investigators of the European Huntington's Disease Network6
AU - Baake, Verena
AU - Reijntjes, Robert H.A.M.
AU - Dumas, Eve M.
AU - Thompson, Jennifer C.
AU - Roos, Raymund A.C.
AU - Bentivoglio, Anna Rita
AU - Biunno, Ida
AU - Bronzova, Juliana
AU - Dunnett, Stephen B.
AU - Frich, Jan
AU - Giuliano, Joe
AU - Illarioshkin, Sergey
AU - Illmann, Torsten
AU - Klempír, Jirí
AU - Landwehrmeyer, G. Bernhard
AU - Levey, Jamie
AU - McLean, Tim
AU - Nielsen, Jørgen E.
AU - Päivärinta, Markku
AU - Pålhagen, Sven
AU - Tabrizi, Sarah J.
AU - Vandenberghe, Wim
AU - Uhrova, Tereza
AU - Bernard, Tomáš
AU - Betz, Sabrina
AU - Come, Adrien
AU - Capodarca, Selene
AU - Wildson, Sébastien Charpentier
AU - Da Silva, Vieira
AU - Di Renzo, Martina
AU - Finisterra, Ana Maria
AU - Fullam, Ruth
AU - Genoves, Camille
AU - Gilling, Mette
AU - Handley, Olivia J.
AU - Hvalstedt, Carina
AU - Koppers, Kerstin
AU - Lamanna, Claudia
AU - Laurà, Matilde
AU - Descals, Asunción Martínez
AU - Münkel, Kristina
AU - Mütze, Lisanne
AU - Oehmen, Martin
AU - Padieu, Helene
AU - Paterski, Laurent
AU - Koivisto, Susana Pro
AU - Rindal, Beate
AU - Røren, Niini
AU - Salgueiro, Ana
AU - Minster, Sara
N1 - Publisher Copyright:
© 2017 Elsevier Ltd. All rights reserved.
PY - 2017/10/1
Y1 - 2017/10/1
N2 - Background: In Huntington's Disease (HD) cognitive decline can occur before unequivocal motor signs become apparent. As cognitive decline often starts early in the course of the disease and has a progressive nature over time, cognition can be regarded as a key target for symptomatic treatment. The specific progressive profile of cognitive decline over time is unknown. Objective: The aim of this study is to quantify the progression of cognitive decline across all HD stages, from pre-motormanifest to advanced HD, and to investigate if CAG length mediates cognitive decline. Methods: In the European REGISTRY study 2669 HD expansion gene carriers underwent annual cognitive assessment. General linear mixed models were used to model the cognitive decline for each cognitive task across all disease stages. Additionally, a model was developed to evaluate the cognitive decline based on CAG length and age rather than disease stage. Results: There was significant cognitive decline on all administered tasks throughout premotormanifest (close to estimated disease onset) participants and the subsequent motormanifest participants from stage 1 to stage 4. Performance on the Stroop Word and Stroop Color tests additionally declined significantly across the two pre-motormanifest groups: far and close to estimated disease onset. The evaluation of cognition performance in relation to CAG length and age revealed a more rapid cognitive decline in participants with longer CAG length than participants with shorter CAG length over time. Conclusion: Cognitive performance already shows decline in pre-motormanifest HD gene expansion carriers and gradually worsens to late stage HD. HD gene expansion carriers with certain CAG length have their own cognitive profile, i.e., longer CAG length is associated with more rapid decline.
AB - Background: In Huntington's Disease (HD) cognitive decline can occur before unequivocal motor signs become apparent. As cognitive decline often starts early in the course of the disease and has a progressive nature over time, cognition can be regarded as a key target for symptomatic treatment. The specific progressive profile of cognitive decline over time is unknown. Objective: The aim of this study is to quantify the progression of cognitive decline across all HD stages, from pre-motormanifest to advanced HD, and to investigate if CAG length mediates cognitive decline. Methods: In the European REGISTRY study 2669 HD expansion gene carriers underwent annual cognitive assessment. General linear mixed models were used to model the cognitive decline for each cognitive task across all disease stages. Additionally, a model was developed to evaluate the cognitive decline based on CAG length and age rather than disease stage. Results: There was significant cognitive decline on all administered tasks throughout premotormanifest (close to estimated disease onset) participants and the subsequent motormanifest participants from stage 1 to stage 4. Performance on the Stroop Word and Stroop Color tests additionally declined significantly across the two pre-motormanifest groups: far and close to estimated disease onset. The evaluation of cognition performance in relation to CAG length and age revealed a more rapid cognitive decline in participants with longer CAG length than participants with shorter CAG length over time. Conclusion: Cognitive performance already shows decline in pre-motormanifest HD gene expansion carriers and gradually worsens to late stage HD. HD gene expansion carriers with certain CAG length have their own cognitive profile, i.e., longer CAG length is associated with more rapid decline.
KW - Cognition
KW - Huntington's disease
KW - Longitudinal
KW - REGISTRY
UR - https://www.scopus.com/pages/publications/85027997207
U2 - 10.1016/j.cortex.2017.07.017
DO - 10.1016/j.cortex.2017.07.017
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C2 - 28843844
AN - SCOPUS:85027997207
SN - 0010-9452
VL - 95
SP - 51
EP - 62
JO - Cortex
JF - Cortex
ER -