Executive dysfunction and its association with personality and behaviour changes in the development of Alzheimer's disease in adults with Down syndrome and mild to moderate learning disabilities

• Published in: British journal of clinical psychology 2008-03, Vol.47 (1), p.1-29
• Main Authors: Ball, Sarah L., Holland, Anthony J., Treppner, Peter, Watson, Peter C., Huppert, Felicia A.
• Format: Article
• Language: English
• Subjects: Adult; Adult and adolescent clinical studies; Aged; Alzheimer Disease - diagnosis; Alzheimer Disease - epidemiology; Alzheimer Disease - physiopathology; Alzheimer's disease; Behavioural changes; Biological and medical sciences; Chromosome aberrations; Clinical psychology; Cognition; Cognition Disorders - diagnosis; Cognition Disorders - epidemiology; Cognition Disorders - physiopathology; Comorbidity; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Disabled persons; Down syndrome; Down Syndrome - diagnosis; Down Syndrome - epidemiology; Down Syndrome - physiopathology; Down's syndrome; Executive function; Frontal Lobe - physiopathology; Humans; Illness; International Classification of Diseases - statistics & numerical data; Language Tests - statistics & numerical data; Learning; Learning disabled people; Learning Disorders - diagnosis; Learning Disorders - epidemiology; Learning Disorders - physiopathology; Medical genetics; Medical research; Medical sciences; Memory Disorders - diagnosis; Memory Disorders - epidemiology; Memory Disorders - physiopathology; Mental Disorders - diagnosis; Mental Disorders - epidemiology; Mental Disorders - physiopathology; Middle Aged; Neurology; Neuropsychological Tests - statistics & numerical data; Organic mental disorders. Neuropsychology; Personality; Personality Disorders - diagnosis; Personality Disorders - epidemiology; Personality Disorders - physiopathology; Prognosis; Psychology. Psychoanalysis. Psychiatry; Psychopathology. Psychiatry; Reproducibility of Results; Severity of Illness Index; Speech Disorders - diagnosis; Speech Disorders - epidemiology; Speech Disorders - physiopathology
• ISSN: 0144-6657; 2044-8260
• DOI: 10.1348/014466507X230967

Background Recent research suggests that preclinical Alzheimer's disease (AD) in people with Down syndrome (DS) is characterized by changes in personality/behaviour and executive dysfunction that are more prominent than deterioration in episodic memory. This study examines the relationship between executive dysfunction and the clinical and preclinical features of AD in DS. To determine the specificity of this relationship, performance on executive function (EF) measures is contrasted with performance on memory measures. Methods One hundred and three people with DS (mean age 49 years, range 36–72) with mild to moderate learning disabilities (LD) took part. Dementia diagnosis was based on the CAMDEX informant interview conducted with each participant's main carer. Reported changes in personality/behaviour and memory were recorded. Participants completed six EF and six memory measures (two of which also had a strong executive component) and the BPVS (as a measure of general intellectual ability). First, performance was compared between those with and without established dementia of Alzheimer's type (DAT), controlling for age and LD severity using ANCOVA. Next, the degree to which informant‐reported changes predicted cognitive test performance was examined within the non‐DAT group using multiple regression analyses. Results The DAT group (N = 25) showed a consistent pattern of impaired performance relative to the non‐DAT group (N = 78), across all measures. Within the non‐DAT group, number of informant‐reported personality/behaviour changes was a significant predictor of performance on two EF and two ‘executive memory’ tests (but not on episodic memory tests). Informant‐reported memory changes, however, were associated with impaired performance on a delayed recall task only. Conclusions These findings provide further evidence for a specific impairment in frontal‐lobe functioning in the preclinical stages of AD in DS. Implications for the assessment, diagnosis, and management of dementia in DS are discussed.