Publications Authored By Johnny Hon
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.
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.
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.