Aging & Mental Health, November 2003; 7(6): 431–437

Diagnostic instruments for dementia in older people with intellectual

disability in clinical practice

A. STRYDOM & A. HASSIOTIS

Department of Psychiatry and Behavioural Sciences, University College London, London, UK

Abstract
There is a need for simple and reliable screening instruments for dementia in the intellectual disability (ID) population that
can also be used to follow their progress, particularly if they are being treated with anti-dementia drugs. Commonly used
tests for the general population such as the Mini Mental State Examination (MMSE) are not appropriate for many people
with ID. This paper is a literature review of alternative instruments that have been used in research or recommended by
experts since 1991 and have the potential to be used as screening instruments. Two types of tests have been identified: those
administered to informants, and those that rely on direct assessment of the individual. The most promising informant rated
screening tool in most adults with ID including Down syndrome (DS) diagnosis is the Dementia Questionnaire for Persons
with Mental Retardation (DMR). However, sensitivity in single assessments is variable and cut-off scores need further
optimisation. In those with DS, the Dementia Scale for Down Syndrome (DSDS) has good specificity but mediocre
sensitivity. The Test for Severe Impairment and Severe Impairment Battery are two direct assessment tools that show
promise as screening instruments, but need further evaluation.

Introduction Reed & Corbett, 1991). Deb and Braganza (1999)

Diagnosis of dementia in people with pre-existing
cognitive deficits is complex and comprehensive
assessment with validated instruments is essential
(Aylward et al., 1997). Burt & Aylward (2000)
suggested a comprehensive test battery that included
tests for informant report and tests for direct assess-
ment to assist the diagnosis of dementia, particularly
when used sequentially. Their recommendations
aimed to improve international consensus in the
use of these tests in research. Since then, assessment
of cognitive function in older people with ID has
become an important issue with the availability of
anti-dementia drugs. Guidelines for the use of these
drugs, such as those of the National Institute for
Clinical Excellence (NICE, 2001) in the UK,
recommend symptom measurement and follow-up
of treatment response with tools such as the Mini
Mental State Examination (MMSE; Folstein,
Folstein & McHugh, 1975), which is also widely
used in the general population to screen for dementia.
The MMSE has been evaluated in adults with ID, but
found to be unsuitable for those with moderate or
greater degrees of ID or reading difficulties (Sturmey,
for example, could only apply the MMSE in 55% of
their patients.
Because of the limitations of the MMSE and other
short screening instruments in the ID population,
researchers have been investigating or developing
alternatives. The present review focuses on tests
that measure cognitive impairment or symptoms
associated with dementia in people with ID and are
suitable for use in clinical practice. We were looking
for tests that were appropriate in adults with mild to
severe ID, with or without Down syndrome (DS), in
hospital or community settings and for use by a
range of clinicians. We were particularly interested
in scales or tests that had the potential to be used
as screening instruments.
Method
We undertook a computerized search of Medline,
Embase and Psychinfo for English language journal
papers published between 1991 and 2002. We used
the search terms mental retardation, intellectual
disabilities and learning disability or disorders and

Correspondence to: Andre Strydom, Lecturer in the Psychiatry of Intellectual Disability, Department of Psychiatry and
Behavioural Sciences, University College London (UCL), Wolfson Building, 48 Riding House Street, London, W1N 8AA,
UK. E-mail: a.strydom@ucl.ac.uk

Received for publication 18th April 2002. Accepted 12th March 2003.

ISSN 1360–7863 print/ISSN 1364–6915 online/03/060431–07 ß Taylor & Francis Ltd

DOI: 10/108013607860310001594682
432 A. Strydom & A. Hassiotis

combined it with searches for dementia, Alzheimer’s
disease and diagnostic, psychological or psychometric
test or instrument. We selected abstracts and papers
that recommended or reviewed instruments used in
the diagnosis of dementia, that evaluated such
instruments, and that reported use of such instru-
ments in clinical or research settings. We excluded
instruments that did not measure the cognitive
impairments of dementia directly or as reported by
informants. Instruments that concern the diagnosis of
psychiatric disorders such as the mini PAS-ADD
(Prosser et al., 1998) may be useful in the differential
diagnosis of dementia, but were not included in
this review because these do not specifically mea-
sure cognitive function or symptoms of dementia.
Screening tests had to be brief and easy to perform,
inexpensive, and acceptable to individuals (Dixon,
Munro & Silcocks, 1997). We determined the ease
of use of a test by its description, and selected tests
that were simple to administer and interpret, and
took no more than 30 minutes to administer. We
selected scales or tests that could be applied in
settings with limited resources and completed by
any competent clinician. On this basis, we excluded
neuro-psychological test batteries that were too
comprehensive to be used as screening tests. These
included the Cambridge Cognitive Examination
(CAMCOG) (Hon et al., 1999); Planning, Attention,
Simultaneous and Successive processes (PASS)
(Das & Mishra, 1995), Rivermead Behavioural
Memory Test (Hon et al., 1998) as well as a number
of other test batteries (Burt et al., 1998; Crayton et al.,
1998; McDaniel et al., 1998).
We extracted the following information about
remaining tests: description of the test; details of
population in which it was evaluated (level of
ID, hospital or community status, and diagnosis of
Down syndrome); reliability and validity as well as
sensitivity and specificity figures. We supplemented
our initial searches with further searches using the
names of the selected tests.
Results
Instruments that measured cognitive impairment or
symptoms of dementia, were easy to use, and had
been applied in the diagnosis and assessment of
dementia in people with ID are summarized in
Table 1. The properties of instruments that appeared
suitable for use in screening for dementia in adults
with ID are discussed in the following section.
Cognitive instruments administered to informants
. Dementia questionnaire for persons with Mental
Retardation (DMR; Evenhuis, 1992).

TABLE 1. Cognitive impairment instruments for dementia diagnosis in adults with ID in clinical settings
Test
A. Administered to informant
Dementia Questionnaire
for mentally retarded
persons (DMR)
IQCODE
Dementia Scale for
Down Syndrome
Early signs of dementia checklist Visser et al., 1997
B. Direct assessment of individual
Selective reminding test
Mini Mental State Exam
Cued recall test
IBR Evaluation of Mental Status
Dementia Rating Scale
Visual memory computer test
Down Syndrome Mental State
Examination
Test for Severe Impairment
Visual memory test
Severe Impairment Battery
Delayed Match-to-sample test
Fuld (modified)
Developed by Used/evaluated by
Evenhuis, 1992 Roeden & Zitman, 1995; Evenhuis, 1996;
Prasher, 1997; Deb & Braganza, 1999;
Burt et al., 1998, 1999; Prasher et al., 2002
Jorm, 1994 Schultz et al., 1998
Gedye, 1995 Burt et al., 1998; Deb & Braganza, 1999;
Huxley et al., 2000; Devenny, 2000;
Temple et al., 2001
Visser et al., 1997; Hoekman & Maaskant, 2002
Buschke, 1973 Devenny et al., 1996, 2000
Folstein et al., 1975 Sturmey et al., 1991; Nelson et al., 1995;
Prasher et al., 1997; Deb & Braganza, 1999;
Hon et al., 1999
Buschke, 1984 Devenny et al., 2000, 2002
Wisniewski & Hill, 1985, Devenney et al., 1992, 1996; Burt et al., 1998
described in Burt et al., 1998
Mattis, 1988 McDaniel & McLaughlin, 2000;
Das et al., 1995
Sahakian et al., 1988 Crayton et al., 1998
Haxby, 1989 Cosgrave et al., 1998; Tyrrel et al., 1996
Albert & Cohen, 1992 Cosgrave et al., 1998; Tyrrel et al., 1996;
Cosgrave et al., 1999
Devenny et al., 1992 Devenny et al., 1996; Burt et al., 1998
Saxton et al., 1993 Witts & Elders, 1998; Prasher et al., 2002;
McKenzie et al., 2002
Dalton & McMurray, 1995, Hoekman & Maaskant, 2002
from Hoekman &
Maaskant, 2002
Burt & Aylward, 2000; Temple et al., 2001

The DMR was developed as a screening instru-
ment in The Netherlands in the 1980s, and has
been evaluated in adults with ID including those
with DS. It is widely used in research and clinical
practice throughout Europe and the UK. It has
50 items and eight sub-scales (short-term mem-
ory, long-term memory, spatial and temporal
orientation, speech, practical skills, mood, activity
and interest and behavioural disturbance). The
sub-scales are grouped into a cognitive score
(mostly short- and long-term memory) and social
score (the rest of the sub-scales). Cut-off scores
are available for a single assessment and differ-
ences in repeat assessments, and cut-offs are
adjusted for the level of intellectual disability
(Evenhuis, 1992). Evenhuis (1996) later made
slight modifications to cut-off scores. Evenhuis
(1992) investigated inter-rater reliability and
internal consistency, which was satisfactory. Parti-
cipants with dementia scored significantly higher
on most sub-scales. Burt et al., (1999) compared
reported everyday orientation items in the DMR
with direct assessment by the orientation items of
the IBR Evaluation of Mental Status—a test based
on the MMSE (Burt et al., 1998). They found
. Down Syndrome Dementia Scale (DSDS; Gedye,
excellent internal consistency of these DMR items
and good agreement between these instruments.
Most of these items were robust regardless of the
cause of ID, gender, age or IQ. They concluded
that most informants could accurately predict the
performance of adults with ID on direct orienta-
tion assessment. Deb and Braganza (1999) found
good agreement between the DMR and Dementia
Scale for Down Syndrome (DSDS) in adults with
DS, as well as a high positive correlation between
the scores of these instruments (Table 2). In
contrast, Hoekman and Maaskant (2002) found
poor agreement between DMR, a dementia
checklist and the Delayed Match-to-Sample test.
When used in single assessments, dementia is
suspected when the cognitive score is  7 and/or
social score  10. Evenhuis (1992, 1996) reported
sensitivity and specificity separately for both
scores, which varied considerably (Table 2).
Prasher (1997) has shown that specificity was
unacceptable using these criteria in adults with
DS. If the criteria were modified to a combination
TABLE 2. Characteristics of the DMR on single assessments of adults with ID with and without DS
Down syndrome or not
Evenhuis (1992) Non-DS
DS
Evenhuis (1996) Non-DS
DS
Prasher (1997) Non-DS
DS—unmodified score
DS—modified score
Deb & Braganza (1999) Non-DS
DS
Hoekman & Maaskant (2002) Both DS and non-DS
Dementia instruments in ID 433
of the two scores (at or above the cut-off in
cognitive AND social scores) the specificity im-
proved, and sensitivity remained acceptable
(Table 2). In general, the psychometric properties
are more impressive for people with DS than for
people with other causes of ID. The instrument
tended to miss vascular dementia, and false
positive results were due to organic illness,
depression and sensory disability (Evenhuis,
1996). Non-demented individuals who presented
with behavioural problems may also have false
positive results (Prasher, 1997). These factors
partially account for the variation in performance
in these studies. In addition, UK studies have used
semi-structured interviews with informants
whereas Dutch studies have used ratings by direct
carers, which may account for the improved
sensitivity and specificity in UK studies. The
study by Hoekman and Maaskant (2002) has
particular methodological problems, such as using
a consensus diagnosis of dementia rather than a
clinical assessment of mental state and cognition,
a small number of participants with dementia and
the exclusion of those with severe dementia.
1995).
This instrument has only been used in people with
DS. The DSDS consists of 60 items, and was
developed to detect dementia in adults in the
severe to profound range of mental retardation. It
relies on change in function after acquiring a
baseline. Although the manual requires a char-
tered psychologist to gather information on
changes in behaviours from two informants it
has been used in clinical practice and in screen-
ing by other mental health professionals (Deb &
Braganza, 1999; Huxley, Prasher & Haque, 2000).
The questions are divided into three categories
indicating early, middle and late stages of demen-
tia and can generate information about the stage of
dementia through various scores. It also allows for
a differential diagnosis of dementia with depres-
sion, hypothyroidism, and visual and hearing
impairment (Deb & Braganza, 1999). Sensitivity
and specificity in single assessments in people
with DS are given in Table 3. As with the DMR,
Sensitivity (%) Specificity (%)
91–100 65–97
- -
57–100 39–85
83–100 80–81
- -
91.5 47
82 82
- -
92 92
77 67
434 A. Strydom & A. Hassiotis
TABLE 3. Sensitivity and specificity of the Down
Syndrome Scale for Dementia in single assessments on
adults with DS
Sensitivity (%) Specificity (%)
Huxley et al., (2000) 58 96
Deb & Braganza (1999) 85 89
the instrument performs better with repeat
assessments. There is good agreement between
the DSDS and the DMR, and a high positive
correlation between overall scores. The difference
in sensitivity between the two studies may be due
to fewer people with severe or profound ID in
Huxley et al.’s sample. Deb and Braganza (1999)
did not find all the items useful, and pointed out
the practical problems of using only chartered
psychologists and interviewing two caregivers.
. Other measures
A few researchers have developed checklists to
identify those with symptoms of dementia in
long-term follow-up studies (Cosgrave et al., 2000;
Visser et al., 1997). These are easy to use and can
detect early symptoms of dementia. The Early
Signs of Dementia Checklist (Visser et al., 1997) is
a list of 37 questions with binary scores. It scores
the early clinical signs of mental deterioration and
was found to have very good internal consistency
and inter-rater reliability (Visser et al., 1997).
There appears to be a more comprehensive
version, consisting of 64 questions, which was
used by Hoekman and Maaskant (2002) in a
concurrent validity and sensitivity study. They
found poor agreement with other instruments, but
reasonable sensitivity and specificity when com-
pared with expert opinion, though the study had
methodological problems mentioned earlier. The
Short Informant Questionnaire on Cognitive
Decline in the Elderly (IQCODE) (Jorm, 1994)
has been evaluated by Schultz et al., (1998) for use
in people with ID. In this population, they found
mediocre test-retest reliability, poor inter-rater
reliability and poor correlation with current
mental status. It probably requires modification
before clinical use. Adaptive behaviour appears to
correlate well with poorer cognitive function, and
instruments that measure this have been found to
be sensitive to dementia status (Cosgrave et al.,
2000). This is usually measured by informant
report and a number of instruments are available.
However, we could not find any that has been
adapted to specifically screen for dementia.
Direct cognitive assessment of an individual
. Test for Severe Impairment (TSI) (Albert &
Cohen, 1992).
The TSI is a short cognitive test with 24 items,
each of which is answered correctly or incorrectly,
and tests a broad range of cognitive functions. It
was designed for people in the general population
whose MMSE score is less than 10, and most
persons with moderate and severe ID should be
able to score on it unless they are in an advanced
stage of dementia (Cosgrave et al., 2000). The
TSI tests the following areas, with four items in
each: motor performance, language production,
language comprehension, memory, conceptu-
alisation, and general knowledge. It takes 10
minutes to administer. Only eight of the 24 points
on the TSI require a verbal answer, which is
beneficial when testing people with poor verbal
ability (Cosgrave et al., 1999). The TSI has been
validated in the DS population, and reliability
and validity assessed by Cosgrave et al., (1998).
They established satisfactory convergent validity
and better concurrent validity in comparison with
the Down Syndrome Mental Status Examination,
a neuropsychological test battery (Haxby, 1989).
The Cronbach’s alpha coefficient, inter-rater
reliability and test-retest reliability was excellent,
even in those with severe ID. They are developing
cut-off scores for single tests and change scores in
an ongoing longitudinal study. This test has only
been evaluated in people with DS.
. Severe Impairment Battery (SIB) (Saxton et al.,
1993).
This was also originally developed as a diagnostic
tool for those with severe dementia in the general
population. It consists of 39 items measuring
aspects of cognition such as social interaction,
memory, orientation, language, attention, praxis
and language. Witts and Elders (1998), have
shown that it has adequate test-retest reliability
and criterion validity against the Vineland Adap-
tive Behaviour Scales. Discriminant validity has
been demonstrated by McKenzie et al., (2002)
for cognitive decline in those with DS. It has also
been used by Prasher et al., (2002) to follow
people with DS and AD in a randomised trial of
donepezil. No sensitivity or specificity information
is available.
. Other measures
Other direct rating instruments to detect dementia
include the Dementia Rating Scale (Mattis, 1988),
Evaluation of Mental Status (described by
Burt et al., 1998) and Down Syndrome Mental
Status Examination (Haxby, 1989) (Table 1). The
Cued Recall Test (Buschke, 1984) has been used
by a group of researchers in the USA (Devenny
et al., 2000; 2002) to follow people with Down
syndrome at risk of developing dementia. They
reported good sensitivity and specificity in this
group (Devenny et al., 2002). Simple instruments
developed by Dalton and McMurray (Delayed

Match-to-Sample test, from Hoekman &
Maaskant, 2002), Buschke (1973), Devenny
et al., (1992), and Fuld (Burt & Aylward, 2000)
have been used by researchers to assess memory
functions (Table 1). We could not find enough
information to recommend any of these as
diagnostic screening tools.
Discussion
Diagnosis of dementia in adults with ID requires a
change of baseline functioning, which exceeds that of
normal ageing (Aylward et al., 1997). The clinical
presentation may be confused with other conditions
such as sensory disability, hypothyroidism or depres-
sion. The perception of decline will also depend
on the environmental demands on the individual
(Aylward et al., 1997). In any comprehensive assess-
ment of a person with ID with possible memory loss,
it is therefore important to diagnose co-morbid
mental illness and to investigate adaptive func-
tioning. These issues have complicated the use
of instruments in diagnosing dementia in older
people with ID. The sequential use of assessments
is the best way to confidently diagnose dementia.
However, screening tests will have practical value to
quickly and cheaply identify those that need com-
prehensive assessment. We have identified tests that
have the potential to be used in this way.
Informant administered instruments
Informant-based instruments are often used to
complement assessments in the general elderly
population, but it is even more important in the
assessment of those with ID. Both the DSDS and
DMR have been thoroughly evaluated. The DMR is
the only instrument that has been used in adults with
DS and those without this diagnosis. However,
with single assessments, the sensitivity is variable
and clinical assessment to exclude other causes of
cognitive decline is essential. Longitudinal use gives
the most reliable results (Evenhuis, 1996). The use
of another screening instrument with the DMR has
been advocated (Hoekman & Maaskant, 2002). The
most suitable cut-off score combinations for single
completions, particularly for those without DS, are
not yet known. We suggest that the test’s perfor-
mance may be enhanced by plotting different cut-offs
with a receiver operating characteristic curve (Dixon,
Munro & Silcocks, 1997).
Direct assessment
Direct cognitive assessment of individuals with ID
and memory loss or cognitive and other changes
associated with dementia is more problematic.
Dementia instruments in ID 435
A floor effect in combination with test error and the
variability in the degree of intellectual impairment
limits the interpretation of single assessments with
screening tests (McDaniel et al., 1998). Other prob-
lems include deficits in language comprehension,
attention, and sensory motor functions. It is these
problems that make the Mini Mental State
Examination unsuitable for use as a screening
instrument in adults with ID. Although a good
number of promising tests are now available to use
instead of MMSE, most have not been properly
evaluated.
Given the problems in assessing cognition in
adults with ID, a combination of an informant-
based instrument and direct assessment may be
more useful than either on its own. This combined
approach has been shown to improve accuracy in
screening for dementia in the general population
(Mackinnon & Mulligan, 1998).
Conclusion
Our review suggests that when any of these instru-
ments are used as single assessment tools to screen
for dementia, the results have to be interpreted with
caution. The most promising screening tool in most
adults with ID regardless of DS diagnosis is the
Dementia Questionnaire for Persons with Mental
Retardation (DMR) (Evenhuis, 1992). The instru-
ment has good specificity but sensitivity varies and
optimal cut-offs need further evaluation. Although
some direct assessment tools show promise as
screening instruments, none have been sufficiently
evaluated for this function in adults with ID. This
highlights the need for further evaluation.
Measurement of change in cognitive function is
increasingly important with the advent of anti-
dementia drugs. There is not much longitudinal
work in this area partly because the use of these
drugs is relatively new in people with ID. It is also
important for instruments to be evaluated for its
sensitivity to treatment response.
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