**5. Discussion**

0

45 50 55 60 65 **Age, years**

**Figure 3.** Adaptive behaviour in two participants with different clinical course of diagnosed Alzheimer's disease: par‐ ticipant 1 with slow progression of dementia (clinical case data given in text), participant 2 with mosaic trisomy of

For most of the younger and many of older participants too, changes in ABS scores were minimal (Figure 1). Slight improvement in scores at ages 38-40 was seen in an participant after a change of residence, coupled with increasing exercise, weight loss and improved fitness. The highest scores with slight improvement at ages 38-43 were seen in a participant in spite of many treated health problems, including a slight permanent visual impairment, hypothyr‐

The ABS scores declined in most participants with Alzheimer´s disease (Figure 2). Improve‐ ments in ABS scores were seen in two elderly participants. In the first participant (Figure 2), the improvement at age 42 to 43 associated to the change of residence and medical treatment of Alzheimer's disease with response for two years before advancing deterioration later. Epilepsy and loss of mobility after a fall resulting in a hip fracture, and poor visual acuity

The improvement of the other participant (Figure 3) during the treatment of confirmed Alzheimer's disease, after a decline at age 48 to 49 lasted five years before further deterioration. Contributing factors to the functional improvement were the removal of cataracts resulting in improved visual acuity, active participation in activities with support of the carers, and

**4. Late onset of dementia with mosaic trisomy of chromosome 21**

oidism, fractures associated with osteoporosis and bronchial asthma.

contributed to the loss of independent functioning.

50

100

150

**ABS-RC:2 part 1 scores**

chromosome 21 and late onset of dementia

200

250

300

320 Pharmacology and Nutritional Intervention in the Treatment of Disease

We report experiences of a long term prospective clinical follow-up of adults with Down syndrome. The participants in this study were adults with Down syndrome and behavioural changes as perceived by carers. Adults without behavioural or mood changes observed by their proxies were not actively recruited and thus this group is not represented in this survey. The participants represent adults with Down syndrome with observed change of mood, behaviour or performance causing concern in their proxies.

Depression and, among participants in their forties and older, Alzheimer's dementia were the most common underlying reasons for the behavioural change. The number of participants with diagnosed and suspected dementia gave estimates of prevalence comparable to published epidemiological studies. Most people with Down syndrome and diagnosed Alzheimer's dementia in this population participated in this study. A change of behaviour or adjustment had been noticed by their proxies before the diagnosis of Alzheimer's dementia.

The current coping skills of the participants were assessed repeatedly using Adaptive Behav‐ iour Scale - Residential and Community, Part I (Nihira *et al.* 1993). Earlier research supports its feasibility in scientific studies of ageing and dementia in people with intellectual disabilities. The clinical use of ABS to monitor ageing and dementia from the early non-symptomatic phase to the advanced stages at various levels of abilities proved to be possible and helpful for the clinician. A decline in ABS scores was seen in most participants after their early forties. This supported the suspicion of Alzheimer's disease, led to differential diagnostic assessments and also helped in monitoring the progression of the disease.

Adaptive behaviour can be assessed by ABS in adults with intellectual disability at all phases of ageing and dementia. This informant-based method overcame many of the problems of cognitive based measures. For example, no cooperation or communication skills of the person to be evaluated were needed for this assessment. Direct evaluations of cognitive functions were not possible in this study due to the limited neuropsychological resources available. Stable scores in clinically stable participants between repeated evaluations supported the reliability of ABS, when used by proxy informants. The informants with a close and long familiarity to their proxies observed and reported subtle changes in daily life and completed adaptive behaviour questionnaires without obvious difficulty.

A careful evaluation of the life situation and comprehensive assessment of physical and mental health is necessary when carers describe a decline in everyday functioning - that is a difficulty in accomplishing daily tasks which the individual would normally complete with ease (Ball et al. 2006). This is also needed in the elderly with established Alzheimer's disease because of common co-morbidities. In the case of a rapid deterioration of function, the underlying reasons should be assessed even when Alzheimer's disease has been diagnosed and treatment started. Visual acuity and hearing should be regularly monitored in all adults with Down syndrome because of high prevalence's of visual impairment and hearing loss. Alzheimer's disease does not protect from any other disease or disability. Other treatable conditions, including hypo‐ thyroidism, visual impairment and hearing loss, may be found.

**Author details**

**References**

T. Määttä1\*, T. Tervo-Määttä2

, A. Taanila3,4, M. Kaski5

Adaptive Behaviour Change and Health in Adults with Down Syndrome: A Prospective Clinical Follow-Up Study

\*Address all correspondence to: tuomo.maatta@kainuu.fi

1 Kainuu Social and Health Care Joint Authority, Finland

3 Institute of Health Sciences, University of Oulu, Finland

5 The Rinnekoti Research Centre, Espoo, Finland

*ty Research* 48, 611-620.

*Psychological Society* 47, 1-29.

*ciency Research* 35, 512-520.

2 Department of Teacher Education, University of Oulu, Finland

4 Unit of General Practice, University Hospital of Oulu, Finland

6 Department of Child Neurology, University of Helsinki, Finland

*International Journal of Geriatric Psychiatry* 21, 661-673.

[1] Ball, S. L., Holland, A. J., Hon, J., Huppert, F. A., Treppner, P., & Watson, P. C. (2006). Personality and behaviour changes mark the early stages of Alzheimer's disease in adults with Down's syndrome: Findings from a prospective population-based study.

[2] Ball, S. L., Holland, A. J., Huppert, F. A., Treppner, P., Watson, P., & Hon, J. (2004). The modified CAMDEX informant interview is a valid and reliable tool for use in the diagnosis of dementia in adults with Down's syndrome. *Journal of Intellectual Disabili‐*

[3] Ball, S. L., Holland, A. J., Treppner, P., Watson, P. C., & Huppert, F. A. (2008). Execu‐ tive 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. *The British Journal of Clinical Psychology / the British*

[4] Bhaumik, S., Collacott, R. A., Garrick, P., & Mitchell, C. (1991). Effect of thyroid stim‐ ulating hormone on adaptive behaviour in Down's syndrome. *Journal of Mental Defi‐*

[5] Brugge, K. L., Nichols, S. L., Salmon, D. P., Hill, L. R., Delis, D. C., Aaron, L., et al. (1994). Cognitive impairment in adults with Down's syndrome: Similarities to early

cognitive changes in Alzheimer's disease. *Neurology* 44, 232-238.

and M. Iivanainen5,6

http://dx.doi.org/10.5772/57461

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The decline of the ABS total scores associated strongly to Alzheimer's disease; therefore the described declines in the ABS subscale scores probably reflect changes attributable to Alz‐ heimer's disease. The relative rates of change among of the subscales of ABS differed. The scores of the domains Domestic Activity, Responsibility, Self-Direction and Vocational Activity declined more, as compared to other domains including Independent Functioning and Physical Activities. This may reflect the early impairment of frontal lobe functions among people with Down syndrome (Holland *et al.* 2000), including executive dysfunction in the development of Alzheimer's disease (Ball *et al.*2008).

Individual differences of the functional skills assessed by ABS scores were considerable. Decline of skills with ageing started at very different ages among participants in this study. The improvements of adaptive behaviour seen in several participants highlight the need for careful assessment of treatable medical conditions and possibilities for supporting the maintenance of functional independence. Stabilization of ABS scores was seen during medi‐ cation for Alzheimer's disease in one participant for up to five years. Studies using population based representative larger samples would be needed to further analyse factors that represent potential confounders affecting adaptive behaviour more vigorously.

A considerable proportion of people with Down syndrome do not develop clinical dementia at all (Coppus et al. 2006). People with Down syndrome differ in their individual biological and genetic risk of dementia (Zigman & Lott 2007; Prasher et al. 2008; Patel et al. 2008). Better understanding of genetic and environmental influences and medical conditions contributing to these differences is needed.

Repeated prospective assessments overcome memory errors compared to retrospective evaluations. Short questionnaires of adaptive behaviour change may be sufficient for screening purposes (Prasher *et al.* 2004). However, repeated assessments are needed for the confirmation of dementia and evaluation of interventions. The possibility of performing an assessment in various settings without special professional expertise is a benefit of this approach.

The informant-based assessments of coping skills for daily living may prove practical and useful for the follow up of ageing and dementia from the early non-symptomatic phase to the advanced stages at various levels of abilities. The authors suggest repeated assessments of adaptive behaviour and careful clinical evaluations to detect treatable medical conditions in adults with Down syndrome.
