Please use this identifier to cite or link to this item: http://hdl.handle.net/2445/188634
Title: Longitudinal Clinical and Cognitive Changes Along the Alzheimer Disease Continuum in Down Syndrome
Author: Videla, Laura
Benejam, Bessy
Pegueroles, Jordi
Carmona Iragui, María
Padilla, Concepción
Fernández, Susana
Barroeta, Isabel
Altuna, Miren
Valldeneu, Silvia
Garzón, Diana
Ribas, Laia
Montal, Víctor
Arranz Martínez, Javier
Rozalem Aranha, Mateus
Alcolea, Daniel
Bejanin, Alexandre
Iulita, Maria Florencia
Videla Cés, Sebastià
Blesa González, Rafael
Lleó Bisa, Alberto
Fortea, Juan
Keywords: Síndrome de Down
Malaltia d'Alzheimer
Tests neuropsicològics
Down syndrome
Alzheimer's disease
Neuropsychological tests
Issue Date: 5-Aug-2022
Publisher: American Medical Association (AMA)
Abstract: IMPORTANCE Alzheimer disease (AD) is the main medical problem in adults with Down syndrome (DS). However, the associations of age, intellectual disability (ID), and clinical status with progression and longitudinal cognitive decline have not been established. OBJECTIVE To examine clinical progression along the AD continuum and its related cognitive decline and to explore the presence of practice effects and floor effects with repeated assessments. DESIGN, SETTING, AND PARTICIPANTS This is a single-center cohort study of adults (aged >18 years) with DS with different ID levels and at least 6 months of follow-up between November 2012 and December 2021. The data are from a population-based health plan designed to screen for AD in adults with DS in Catalonia. Spain. Individuals were classified as being asymptomatic, having prodromal AD, or having AD dementia. EXPOSURES Neurological and neuropsychological assessments. MAIN OUTCOMES AND MEASURES The main outcome was clinical change along the AD continuum. Cognitive decline was measured by the Cambridge Cognitive Examination for Older Adults With Down Syndrome and the modified Cued Recall Test. RESULTS A total of 632 adults with DS (mean [SD] age, 42.6 [11.4] years; 292 women [46.2%]) with 2847 evaluations (mean [SD] follow-up, 28.8 [18.7] months) were assessed. At baseline, there were 436 asymptomatic individuals, 69 patients with prodromal AD, and 127 with AD dementia. After 5 years of follow-up, 17.1% (95% CI, 12.5%-21.5%) of asymptomatic individuals progressed to symptomatic AD in an age-dependent manner (0.6% [95% CI, 0%-1.8%] for age <40 years; 21.1% [95% CI, 8.0%-32.5%] for age 40-44 years; 41.4% [95% CI, 23.1%-55.3%] for age 45.49 years; 57.5% [95% CI. 38.2%-70.8%] for age >= 50 years; P < .001), and 94.1% (95% CI. 84.6%-98.0%) of patients with prodromal AD progressed to dementia with no age dependency. Cognitive decline in the older individuals was most common among those who progressed to symptomatic AD and symptomatic individuals themselves. Importantly, individuals with mild and moderate ID had no differences in longitudinal cognitive decline despite having different performance at baseline. This study also found practice and floor effects, which obscured the assessment of longitudinal cognitive decline. CONCLUSIONS AND RELEVANCE This study found an association between the development of symptomatic AD and a high risk of progressive cognitive decline among patients with DS. These results support the need for population health plans to screen for AD-related cognitive decline from the fourth decade of life and provide important longitudinal data to inform clinical trials in adults with DS to prevent AD.
Note: Reproducció del document publicat a: https://doi.org/10.1001/jamanetworkopen.2022.25573
It is part of: JAMA Network Open, 2022, vol. 5, num. 8, p. e2225573
URI: http://hdl.handle.net/2445/188634
Related resource: https://doi.org/10.1001/jamanetworkopen.2022.25573
Appears in Collections:Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

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