Baseline neuropsychological profiles in prion disease predict survival time.
Journal
Annals of clinical and translational neurology
ISSN: 2328-9503
Titre abrégé: Ann Clin Transl Neurol
Pays: United States
ID NLM: 101623278
Informations de publication
Date de publication:
09 2020
09 2020
Historique:
received:
08
05
2020
revised:
06
06
2020
accepted:
09
06
2020
entrez:
14
12
2020
pubmed:
15
12
2020
medline:
21
10
2021
Statut:
ppublish
Résumé
Few studies have captured the neuropsychological profile of sporadic Creutzfeldt-Jakob disease (sCJD) with neuropsychological testing, and little is known about cognitive predictors of survival. We characterized baseline neuropsychological performance in sCJD and investigated associations with survival. sCJD participants who completed the MMSE (n = 118), 61 sCJD of whom also completed a neuropsychological battery at baseline, and 135 age-matched healthy controls, were included. Composite scores of global cognition, memory, executive functions, visuospatial, and language were derived. Cox proportional hazard models estimated survival time, controlling for age and education. Additional models adjusted for Barthel Index and PRNP codon 129 polymorphism. sCJD participants performed significantly worse than controls on all cognitive tasks and composites with most showing very large effect sizes. The three tests showing the largest group differences were delayed verbal recall (Hedges'g = 4.08, P < 0.0001), Stroop Inhibition (Hedges'g = 3.14, P < 0.0001), and Modified Trails (Hedges'g = 2.94, P < 0.0001). Memory (95%) and executive functioning (87%) composites were most commonly impaired. Poorer global (HR = 0.65, P < 0.0001), visuospatial (HR = 0.82, P < 0.0001), and memory (HR = 0.82, P = 0.01) composites predicted shorter survival. Visuospatial cognition remained a significant predictor even after adjusting for all other cognitive composites; each standard deviation decrease in visuospatial cognition was associated with an 18% higher chance of death (HR = 0.82, P < 0.003). Global (HR = 0.68, P = 0.03) and visuospatial (HR = 0.82, P = 0.001) composites remained significant predictors after controlling for Barthel Index and codon 129. sCJD participants exhibit a broad range of cognitive impairments, with memory and executive functioning deficits in the vast majority. Neuropsychological assessment, particularly of visuospatial abilities, informs prognostication in sCJD.
Identifiants
pubmed: 33314770
doi: 10.1002/acn3.51115
pmc: PMC7480924
doi:
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
1535-1545Subventions
Organisme : NIA NIH HHS
ID : K23 AG061253
Pays : United States
Organisme : NIH/NIA
ID : K23AG021989
Pays : International
Organisme : NIA NIH HHS
ID : K24 AG045333
Pays : United States
Organisme : NIH/NIA
ID : K23AG058752
Pays : International
Organisme : NIA NIH HHS
ID : P30 AG062422
Pays : United States
Organisme : NIA NIH HHS
ID : R01 AG062562
Pays : United States
Organisme : NIH/NIA
ID : R56AG055619
Pays : International
Informations de copyright
© 2020 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.
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