Authors:

Ball, S.L., Holland, A.J., Hon, J., Huppert, F.A., Treppner, P. & WATSON, P.C.

Reference:

Year of publication:

2006

CBU number:

6261

Abstract:

BACKGROUND: Research based on retrospective reports by carers suggests that the presentation of dementia in people with Downís syndrome may differ from that typical of Alzheimerís disease (AD) in the general population, with the earliest changes tending to be in personality or behaviour rather than in memory. This is the first long-term prospective study to test the hypothesis that such changes, which are more typical of dementia of frontal type (DFT) in the general population, mark the preclinical stage of AD in DS. METHODS: A previously identified population sample of older people with DS, first assessed in 1994 and followed-up 18 months later, were reassessed after a further 5 years. This study focuses on the 55 individuals who took part in the second follow-up. Dementia diagnosis was made using the modified CAMDEX informant interview and neuropsychological assessment was undertaken using the CAMCOG. Progression in clinical presentation was examined and degree of cognitive decline over time (on the CAMCOG and derived measures of executive function (EF) and memory) was compared across groups based on diagnosis and age: AD, DFT, personality/behaviour changes insufficient for a diagnosis of DFT (PBC), no diagnosis <50 years and no diagnosis 50+ years. RESULTS: Progression was observed from early changes in personality and behaviour to an increase in characteristics associated with frontal lobe dysfunction and/or a deterioration in memory, prior to the development of full AD. Individuals who met criteria for DFT were significantly more likely to progress to a diagnosis of AD over the following 5 years than those who did not and those with PBC were significantly more likely to progress to a more severe diagnosis (DFT or AD) than those without. In the 5 years prior to diagnosis, participants with PBC and DFT had shown a degree of global cognitive decline intermediate between those with no dementia and those with AD. Both these groups had shown a significant decline in EF but not in memory, while the AD group had shown significant decline on both measures, with a significantly greater degree of decline in memory. Older participants without informant reported changes showed a more generalised pattern of decline. CONCLUSIONS: These findings confirm that the early presentation of AD in DS is characterized by prominent personality and behaviour changes, associated with executive dysfunction, providing support for the notion that the functions of the frontal lobes may be compromised early in the course of the disease in this population. This has important implications for the diagnosis, treatment and management of dementia in people with DS.