The Global Deterioration Scale for Down Syndrome Population (GDS-DS): A Rating Scale to Assess the Progression of Alzheimer’s Disease

Emili Rodríguez-Hidalgo, Javier García-Alba, Ramon Novell and Susanna Esteba-Castillo ()
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Emili Rodríguez-Hidalgo: Specialized Service in Mental Health and Intellectual Disability, Institute of Health Assistance (IAS), Parc Hospitalari Martí i Julià, 17190 Girona, Spain
Javier García-Alba: Research and Psychology in Education Department, Complutense University of Madrid, 28040 Madrid, Spain
Ramon Novell: Specialized Service in Mental Health and Intellectual Disability, Institute of Health Assistance (IAS), Parc Hospitalari Martí i Julià, 17190 Girona, Spain
Susanna Esteba-Castillo: Specialized Service in Mental Health and Intellectual Disability, Institute of Health Assistance (IAS), Parc Hospitalari Martí i Julià, 17190 Girona, Spain

IJERPH, 2023, vol. 20, issue 6, 1-18

Abstract: The aim of this study is to adapt and validate the global deterioration scale (GDS) for the systematic tracking of Alzheimer’s disease (AD) progression in a population with Down syndrome (DS). A retrospective dual-center cohort study was conducted with 83 participants with DS (46.65 ± 5.08 years) who formed the primary diagnosis (PD) group: cognitive stability ( n = 48), mild cognitive impairment ( n = 24), and Alzheimer’s disease ( n = 11). The proposed scale for adults with DS (GDS-DS) comprises six stages, from cognitive and/or behavioral stability to advanced AD. Two neuropsychologists placed the participants of the PD group in each stage of the GDS-DS according to cognitive, behavioral and daily living skills data. Inter-rater reliability in staging with the GDS-DS was excellent (ICC = 0.86; CI: 0.80–0.93), and the agreement with the diagnosis categories of the PD group ranged from substantial to excellent with κ values of 0.82 (95% CI: 0.73–0.92) and 0.85 (95% CI: 0.72, 0.99). Performance with regard to the CAMCOG-DS total score and orientation subtest of the Barcelona test for intellectual disability showed a slight progressive decline across all the GDS-DS stages. The GDS-DS scale is a sensitive tool for staging the progression of AD in the DS population, with special relevance in daily clinical practice.

Keywords: global deterioration scale; rating scale; cognitive decline; cognitive testing; Alzheimer’s disease; dementia; down syndrome; intellectual disability (search for similar items in EconPapers)
JEL-codes: I I1 I3 Q Q5 (search for similar items in EconPapers)
Date: 2023
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