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Original Investigation
November 2018

Prevalence of Aging, Dementia, and Multimorbidity in Older Adults With Down Syndrome

Author Affiliations
  • 1Sorbonne Université Faculté de Médecine, Hôpital Pitié-Salpêtrière–Assistance Publique Hôpitaux de Paris, Département de Rééducation Neurologique, Paris, France
  • 2Laboratoire d'Economie LEDa-LEGOS, Université Paris–Dauphine, PSL Research University, Paris, France
  • 3Global Brain Health Institute, University of California, San Francisco
  • 4Department of Neurology, Memory and Aging Center, University of California, San Francisco
  • 5Université Paris Est Faculté de Médecine, Hôpital Henri Mondor–Assistance Publique Hôpitaux de Paris, Centre de référence Maladie de Huntington et service de neurologie, Créteil, France
  • 6Département d'Etudes Cognitives, Ecole Normale Supérieure, PSL Research University et Inserm U955, Equipe E01 Neuropsychologie Interventionnelle, Paris, France
  • 7Departments of Psychiatry, Neurology and Epidemiology and Biostatistics, University of California, San Francisco
JAMA Neurol. 2018;75(11):1399-1406. doi:10.1001/jamaneurol.2018.2210
Key Points

Question  How common are chronic comorbidity conditions, dementia, and both among aging individuals with Down syndrome older than 45 years?

Findings  In this cross-sectional study of a Medicare sample of 878 older individuals with Down syndrome (age range, 45-89 years), nearly half of older individuals with Down syndrome had a diagnosis of dementia after age 65 years. Individuals with Down syndrome had high rates of age-related conditions, especially those with Down syndrome dementia.

Meaning  Early systematic screening of treatable multimorbidity during Down syndrome adulthood and repeated tracking of cognitive decline are needed to improve elderly and dementia care in DS.


Importance  As the life expectancy of people with Down syndrome (DS) has markedly increased over the past decades, older adults with DS may be experiencing a higher incidence of aging conditions. In addition to longevity, the amyloid precursor protein gene located on chromosome 21 places individuals with DS at a high risk for developing Alzheimer disease. Yet, few studies have determined prevalence of dementia and comorbidities among older people with DS.

Objective  To determine the prevalence of dementia and aging-related comorbidities in older adult individuals with DS.

Design, Setting, and Participants  Cross-sectional analysis of 2015 California Medicare claims data. We examined 1 year of cross-sectional Medicare claims data that included 100% of Californian Medicare beneficiaries enrolled in both Medicare Part A and B in 2015. Of these 3 001 977 Californian Medicare beneficiaries 45 years or older, 878 individuals were identified as having a diagnosis of DS. Data were analyzed between April 2017 and February 2018.

Main Outcomes and Measures  The frequency of DS dementia was assessed across different age categories. The number and frequency of 27 comorbidities were compared among individuals with DS with and without dementia and by age and sex groups.

Results  A total of 353 DS individuals (40%) were identified as having dementia diagnoses (mean, 58.7 years; 173 women [49%]) and 525 without dementia diagnoses (mean, 55.9 years; 250 women [48%]). The frequency of DS dementia among those 65 years or older rose to 49%. The mean number of comorbidities per individual increased with age in general. Comorbid conditions were more numerous among those with dementia compared with those with DS without dementia (mean, 3.4 vs 2.5, respectively), especially among those younger than 65 years. In particular, 4 treatable conditions, hypothyroidism, epilepsy, anemia, and weight loss, were much more frequent in DS dementia.

Conclusions and Relevance  Older Medicare beneficiaries in California with DS, especially those with dementia, have a high level of multimorbidity including several treatable conditions. While DS follow-up has long been confined to the pediatric sphere, we found that longevity in individuals with DS will necessitate complex adult and geriatric care. More evidenced-based and standardized follow-up could support better long-term comorbidity management and dementia care among aging adults with DS.