**3. Results**

The number of people with Down syndrome living in the area is well known and their morbidity data was available for comparison. The participants with repeated ABS assessments were older than participants with single assessments. Dementia, medication use for challeng‐ ing behaviour, depression and epilepsy were more common among participants than among adults with Down syndrome living in the region.

#### *Alzheimer's disease*

Alzheimer's disease with dementia was diagnosed in 15 out of the 25 participants assessed repeatedly. Four of them died during the survey. The diagnosis was confirmed in ten partic‐ ipants by a neurologist and in five participants by the first author with competence in intel‐ lectual disability medicine. Other causes of dementia were excluded. Computerized tomographies of the brain were performed in ten of these participants. Eleven persons received medication for Alzheimer's disease (donepezile, galantamine or rivastigmine, in three participants combined with memantine). Early dementia was suspected in an additional four participants; they had increasing difficulties following instructions and performing their usual domestic work.

The number of living people with diagnosed and suspected dementia (fifteen persons among the participants and two not participating in this study) among adults with Down syndrome in the region gives prevalence's of dementia 38% (15 of 40 persons) in the age group 40 years and more, and 13% (two of 15 persons) in the age group 30-39 years. The prevalence estimate of dementia for the age group 30 years and more is thus 31% (17 of 55 persons).

#### *Medical problems*

Development, Numbers and Time, Domestic Activity, Prevocational/Vocational Activity, Self-Direction, Responsibility and Socialization. The ABS Manual reports that factor analysis has found three Part One factors: Personal Self-Sufficiency, Community Self-Sufficiency, and

Clinical evaluations were done by the principal investigator. These included interviews of the proxies, referrals for differential diagnostics and specialist consultations, and prescriptions and assessments of medications. Additional clinical data was drawn from the case records of the health centres, central hospital and service centre regarding all persons with Down syndrome in the region. The data of medical treatments for Alzheimer's disease, depression, behavioural problems, epilepsy, and other major health concerns possibly affecting adaptive behaviour were analyzed. The age at the time of the first observation of functional decline was

Informant ratings by ABS were scored and analysed. Total scores, scores for the ten subscales and three factors of ABS and changes of scores from the first to the last evaluation were counted. The ABS score changes as percentages per three years were calculated for subgroups of participants with and without Alzheimer's disease, depression, epilepsy, hypothyroidism, and

The ethical committee of the Kainuu Central Hospital approved the study and permission for combining data from medical and social records was given by the Ministry of Social and Health

The number of people with Down syndrome living in the area is well known and their morbidity data was available for comparison. The participants with repeated ABS assessments were older than participants with single assessments. Dementia, medication use for challeng‐ ing behaviour, depression and epilepsy were more common among participants than among

Alzheimer's disease with dementia was diagnosed in 15 out of the 25 participants assessed repeatedly. Four of them died during the survey. The diagnosis was confirmed in ten partic‐ ipants by a neurologist and in five participants by the first author with competence in intel‐ lectual disability medicine. Other causes of dementia were excluded. Computerized tomographies of the brain were performed in ten of these participants. Eleven persons received medication for Alzheimer's disease (donepezile, galantamine or rivastigmine, in three participants combined with memantine). Early dementia was suspected in an additional four participants; they had increasing difficulties following instructions and performing their usual

The number of living people with diagnosed and suspected dementia (fifteen persons among the participants and two not participating in this study) among adults with Down syndrome

antipsychotic medication use for challenging behaviour.

adults with Down syndrome living in the region.

Personal–Social Responsibility.

316 Pharmacology and Nutritional Intervention in the Treatment of Disease

calculated.

Affairs.

**3. Results**

*Alzheimer's disease*

domestic work.

Of the 25 participants eleven (44%) had experienced long periods of depression. Thirteen (52%) had received antipsychotic medication mainly for behavioural problems, six of them already during early adulthood and eight for behavioural problems with dementia. Eleven persons were treated for hypothyroidism. Varying degrees of visual impairment were common, and three had cataracts. Recurrent faints were seen in eight, with falls causing fractures in three persons. Epilepsy was diagnosed in eight persons.

Among participants with Alzheimer's disease (n=15), antipsychotic medication had been used for eleven (73%), depression had been diagnosed in ten (67%), thyroid disease in ten (67%), and epilepsy in seven (47%) participants. Among participants without Alzheimer's disease (n=10), antipsychotic medication had been used for two (20%), depression had been diagnosed in one (10%), thyroid disease in four (40%), and epilepsy in one participant (10%). Among eleven participants with depression (N=11), antipsychotic medication had been used for seven (64%), Alzheimer's disease had been diagnosed in ten (91%), thyroid disease in 6 (55%), and epilepsy in five (45%) participants.

#### *Informant observations*

A decline of daily functioning was observed by informants in regards to 19 of 25 persons, starting at the ages of 37-51 years with a mean of 44.9 and SD +/- 4 years in persons with full trisomy of chromosome 21. In addition, there was one participant with mosaic trisomy of chromosome 21 whose decline started only at the age of 60 (Figure 3, participant 2).

#### *Adaptive Behavior Scale (ABS) scores*

The mean ages, ABS total scores at first and last assessments and the calculated percentages of score changes per three years in subgroups of participants are presented in Table 1. The mean ABS total scores for the 25 participants with multiple assessments declined from 161 to 126 (21.8%) during the mean 3.0 years between the first and last assessments. The decline of ABS total scores associated very strongly to Alzheimer's disease: there was no decline in the mean ABS total scores in the group of participants with no suspected or confirmed Alzheimer's disease. The mean rates of ABS score change were higher in participant groups with Alzheim‐ er's disease (33.6% in three years), and depression (32.0%) compared to participants without these conditions (0.6% and 13.9% respectively). The participants treated with medication for Alzheimer's disease had lower mean rates of score declines compared to untreated patients, 31% and 40% declines in three years respectively. The mean rates of change were almost similar in groups of persons with and without epilepsy, antipsychotic medication, and hypothyroid‐ ism. (Table 1)


The biggest mean declines were seen in the subscales Domestic Activity, Responsibility and Self-Direction, to 35.2, 48.8 and 49.7%, respectively. The slightest changes were seen in the domains of Economic Activity, Physical Development and Language Development (Table 2). The mean changes of scores for the ABS factors Personal Self-Sufficiency, Community Self-Sufficiency and Personal–Social Responsibility were 22.2, 27.9 and 36.8%, respectively.

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The ABS scores remained stable in nine and improved in three persons. A progressive decline of ABS scores was seen in 13 of 25 (52%) participants after their early forties. The direction, amount and rate of change of ABS scores varied from an increase of 27% within a year in the youngest participant recovering from deep depression to 90% decline during seven years in

> 35 40 45 50 55 **Age, years**

40 45 50 55 60 **Age, years**

0

**Figure 1.** Adaptive behaviour in 9 participants without clinical Alzheimer's disease

0

**Figure 2.** Adaptive behaviour in 12 participants with diagnosed Alzheimer's disease

50

100

150

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

200

250

300

50

100

150

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

200

250

300

*Individual changes in ABS scores*

an ageing participant.

**Table 1.** Ages, ABS-RC:2 total scores and score changes in subgroups of participants with Down syndrome.


**Table 2.** ABS-RC:2 subscale mean scores and their change during the prospective follow-up of 25 participants with Down syndrome.

The biggest mean declines were seen in the subscales Domestic Activity, Responsibility and Self-Direction, to 35.2, 48.8 and 49.7%, respectively. The slightest changes were seen in the domains of Economic Activity, Physical Development and Language Development (Table 2). The mean changes of scores for the ABS factors Personal Self-Sufficiency, Community Self-Sufficiency and Personal–Social Responsibility were 22.2, 27.9 and 36.8%, respectively.

#### *Individual changes in ABS scores*

**Participants (number of persons) Age of participants at**

318 Pharmacology and Nutritional Intervention in the Treatment of Disease

**ABS-RC:2 subscale Scores, first assessment**

Participants with repeated assessments

(25)

Down syndrome.

Participants with single assessments (17) 36.5 (36.5) 174 (174) males (10) 32.3 (32.3) 188 (188) females (7) 42.4 (42.4) 155 (155)

**first (last) assessment**

males (11) 43.8 (47.0) 135 (117) -12.4 females (14) 48.4 (51.5) 193 (137) -28.4 Alzheimer`s disease (AD, 15) 49.7 (52.9) 165 (106) -33.6 AD, no medication (4) 47.8 (50.8) 155 (93) -40.0 AD, medication (11) 50.6 (53.9) 170 (112) -31.0 No AD (10) 40.0 (43.1) 154 (155) +0.6 Depression (11) 47.6 (50.5) 159 (112) -32.0 No depression (14) 44.4 (47.8) 162 (136) -13.9 Epilepsy (8) 50.6 (54.5) 193 (141) -23.0 No epilepsy (17) 43.5 (46.5) 145 (118) -18.7 Antipsychotic medication (13) 49.0 (52.0) 168 (132) -21.7 No antipsychotic medication (12) 42.3 (45.7) 152 (119) -19.3 Hypothyroidism (11) 45.8 (48.7) 152 (125) -19.4 No hypothyroidism (14) 45.8 (49.1) 166 (126) -21.5

**Table 1.** Ages, ABS-RC:2 total scores and score changes in subgroups of participants with Down syndrome.

**Table 2.** ABS-RC:2 subscale mean scores and their change during the prospective follow-up of 25 participants with

**Mean (SD)**

Domestic Activity 9.6 (6.1) 5.8 (6.1) -64.8 Responsibility 5.0 (3.0) 3.3 (3.1) -51.2 Self-Direction 12.1 (5.9) 8.0 (6.9) -50.3 Prevocational/Vocational Activity 5.8 (3.1) 4.0 (3.6) -44.0 Numbers and Time 5.4 (3.4) 3.8 (3.8) -41.1 Independent Functioning 64.8 (24.3) 52.3 (32.0) -23.8 Language Development 21.0 (9.4) 17.2 (8.9) -22.0 Economic Activity 3.6 (3.3) 3.2 (3.5) -12.5 Physical Development 18.4 (3.5) 16.0 (5.5) -15.0 Economic Activity 3.6 (3.3) 3.2 (3.5) -12.5

45.8 (48.8) 161 (126)

**ABS total scores, means at first (last) assessment**

**Scores, last assessment**

**Mean (SD) Score change, %**

**ABS score change percentage per three**

**years, %**


The ABS scores remained stable in nine and improved in three persons. A progressive decline of ABS scores was seen in 13 of 25 (52%) participants after their early forties. The direction, amount and rate of change of ABS scores varied from an increase of 27% within a year in the youngest participant recovering from deep depression to 90% decline during seven years in an ageing participant.

**Figure 1.** Adaptive behaviour in 9 participants without clinical Alzheimer's disease

**Figure 2.** Adaptive behaviour in 12 participants with diagnosed Alzheimer's disease

stabilization of mood and behaviour. This participant had a long history of hypothyroidism, depression and challenging behaviour and he had long-term antidepressant and antipsychotic

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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,

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

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

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

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

had been noticed by their proxies before the diagnosis of Alzheimer's dementia.

medication, and successful treatment of late onset epilepsy.

behaviour or performance causing concern in their proxies.

also helped in monitoring the progression of the disease.

behaviour questionnaires without obvious difficulty.

**5. Discussion**

**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 chromosome 21 and late onset of dementia
