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; Susanna Esteba-Castillo

doi:10.3390/ijerph20065096

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

Int J Environ Res Public Health. 2023 Mar 14;20(6):5096. doi: 10.3390/ijerph20065096.

Authors

Emili Rodríguez-Hidalgo^{1

2}, Javier García-Alba³, Ramon Novell^{1

2}, Susanna Esteba-Castillo^{1

2}

Affiliations

¹ Specialized Service in Mental Health and Intellectual Disability, Institute of Health Assistance (IAS), Parc Hospitalari Martí i Julià, 17190 Girona, Spain.
² Neurodevelopmental Group [Girona Biomedical Research Institute]-IDIBGI, Institute of Health Assistance (IAS), Parc Hospitalari Martí i Julià, 17190 Girona, Spain.
³ Research and Psychology in Education Department, Complutense University of Madrid, 28040 Madrid, Spain.

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: Alzheimer’s disease; cognitive decline; cognitive testing; dementia; down syndrome; global deterioration scale; intellectual disability; rating scale.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Alzheimer Disease* / epidemiology
Cohort Studies
Disease Progression
Down Syndrome* / epidemiology
Humans
Reproducibility of Results
Retrospective Studies

Grants and funding

This research was funded by the Spanish Government, grant number PI12/02019, PSI-2014-53524-P. The APC was funded by S.E.-C,’s SESMDI research start-up funds.