Dementia in Down's syndrome.
Down's syndrome is the most common genetic cause of learning difficulties, and individuals with this condition represent the largest group of people with dementia under the age of 50 years. Genetic drivers result in a high frequency of Alzheimer's pathology in these individuals, evident from neuroimaging, biomarker, and neuropathological findings, and a high incidence of cognitive decline and dementia. However, cognitive assessment is challenging, and diagnostic methods have not been fully validated for use in these patients; hence, early diagnosis remains difficult. Evidence regarding the benefits of cholinesterase inhibitors and other therapeutic options to treat or delay progressive cognitive decline or dementia is very scarce. Despite close similarities with late-onset Alzheimer's disease, individuals with Down's syndrome respond differently to treatment, and a targeted approach to drug development is thus necessary. Genetic and preclinical studies offer opportunities for treatment development, and potential therapies have been identified using these approaches.
Wolfson Centre for Age-Related Diseases, King's College London, London, UK. Electronic address: clive.ballard@kcl.ac.uk.
Clive
C
Ballard
Center for Neural Circuits and Behavior, School of Medicine, University of California San Diego, San Diego, CA, USA.
William
W
Mobley
Department of Molecular Neuroscience, University College London, London, UK.
John
J
Hardy
Wolfson Centre for Age-Related Diseases, King's College London, London, UK.
Gareth
G
Williams
Wolfson Centre for Age-Related Diseases, King's College London, London, UK.
Anne
A
Corbett
D015897:Comorbidity; D003704:Dementia / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism; D004314:Down Syndrome / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism; D006801:Humans