A five year follow-up study of dementia in persons with Down's syndrome: early symptoms and patterns of deterioration

Abstract

Objectives: To investigate the development of dementia over a five year follow up period in a population of females with Down's syndrome; to examine age at onset and duration of dementia in the population; to document the clinical features of dementia and to highlight scores on functional and cognitive rating scales at diagnosis of dementia and at the onset of complete dependency.Method: A five year follow-up study of 80 female subjects on prevalence of dementia, early clinical features of dementia and patterns of scoring on rating scales at diagnosis and end-stage dementia was completed. Results: Over the five year study period the number of subjects diagnosed with dementia rose from seven (8.75%) to 35 (43.75%). Age related prevalence figures showed that dementia was more common with increasing age. The earliest recognisable symptoms of dementia were memory loss, spatial disorientation and loss of independence especially in the area of personal hygiene. These findings were confirmed by the rating scales used in the study.Conclusions: The earliest recognisable clinical features of dementia include memory loss and increased dependency. The results of this study should facilitate earlier diagnosis of dementia in DS.

Down's syndrome and dementia

“…Deterioration in memory, learning and orientation tend to be the first signs, and these symptoms are often accompanied by increased dependence (Cosgrave et al, 2000). Personality change is often associated with early involvement of the frontal lobes (Holland et al, 2000).…”

Review of donepezil, rivastigmine, galantamine and memantine for the treatment of dementia in Alzheimer's disease in adults with Down syndrome: implications for the intellectual disability population

“…The onset of DAD has been reported in individuals with Down syndrome aged as young as 30 years, with a dramatic increase in prevalence rates over the subsequent decades; for example 54.5% for age range 60-69 years (Prasher, 1995). Although the detection of early symptoms of DAD may at times be difficult in individuals with intellectual disabilities, the clinical picture seen is not too dissimilar to that of the general population (Lai and Williams, 1989;Cosgrave et al, 2000). Insidious progression of memory impairment, personality change, dysfunction in language and motor skills, onset seizures and behavioural abnormalities, marked loss of self-care skills and in weight is typical.…”

Alzheimer’s Disease in Down Syndrome and Intellectual Disability: A Review

“…The authors commented that their rates, which are considerably lower than estimates from other countries, may have underestimated the true prevalence due to the use of very strict diagnostic criteria, a cross-sectional design and not prospectively examining longitudinal changes. In a follow-up study of the same sample (Cosgrave, Tyrrell, McCarron, Gill & Lawlor, 2000), the authors reported a greatly increased prevalence of 43.8%, and rates were also reported according to age group (see Table 1). The same research group has recently reported similar rates of dementia among a sample of 285 people with Down syndrome (see Table 1).…”

“…Because of the particular challenges of working with people with dementia, and the potentially high number of service users affected, the need to plan for appropriate, specialised service provision is quite evident. Cosgrave et al (2000) reported that end-stage dementia was often associated with total dependency for activities of daily living, total loss of mobility, seizures, incontinence, total loss of awareness of surroundings and a need for greatly increased nursing care. They suggested that specialised hospice-type units would be most suitable for people with these needs.…”

“…Here the emphasis is on maintenance of physical health, safety, appropriate level of stimulating activities and reduction of agitation. Counselling and support for carers and families will also be required (Cosgrave et al, 2000). Medication can help to treat agitation, psychosis and depression, and behavioural approaches are effective in increasing functional independence (Doody et al, 2001).…”