Journal of Intellectual Disability Research

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    pp ‒  

Blackwell Science, LtdOxford, UKJIRJournal of Intellectual Disability Research-Blackwell Publishing Ltd, Original ArticleCAMDEX informant interview for use with adults with DSS. L. Ball et al.

The modified CAMDEX informant interview is a valid and

reliable tool for use in the diagnosis of dementia in adults
with Down’s syndrome
S. L. Ball,1 A. J. Holland,1 F. A. Huppert,2 P. Treppner,1 P. Watson,3 & J. Hon1
1 Section of Developmental Psychiatry, Department of Psychiatry, University of Cambridge, Cambridge, UK
2 Department of Psychiatry, University of Cambridge, Cambridge, UK
3 MRC – Cognition and Brain Sciences Unit, Cambridge, UK

Abstract the  at Time , and  of the  at Time , were

found to meet clinical criteria for AD. Forty-one
Background Dementia because of Alzheimer’s dis-
scored above floor on the CAMCOG at Time  and
ease (AD) commonly affects older adults with
were included in the analysis of cognitive decline.
Down’s syndrome (DS). Methods are needed, with
Concurrent validity was established by comparing
established concurrent and predictive validity, to
diagnosis at Time  with independent evidence of
facilitate the diagnostic assessment of dementia,
objective decline on cognitive tasks since Time .
when it is complicated by pre-existing intellectual
Predictive validity was established by examining how
disabilities (ID). We report on the reliability and
accurately diagnosis at Time  predicted both cogni-
validity of a modified version of the Cambridge
tive decline and future diagnosis. Inter-rater reliabil-
Examination for Mental Disorders of the Elderly
ity was determined by comparing the level of
(CAMDEX) informant interview, for use when
agreement between two raters.
assessing people with DS suspected as having
Results CAMDEX-based diagnosis of AD was
dementia.
shown to be consistent with objectively observed cog-
Methods As part of a previous epidemiological study
nitive decline (good concurrent validity) and to be a
of older people with DS, the CAMDEX informant
good predictor of future diagnosis. Although num-
interview was used to determine the prevalence of
bers are small, some support is also provided for the
dementia. The  people with DS included at that
accuracy with which diagnosis predicts cognitive
time (Time ) had also completed the Cambridge
decline. Inter-rater reliability was good with
Cognitive Examination (CAMCOG), the neuropsy-
Kappa > . for % of items and >. for all items.
chological assessment from the CAMDEX schedule.
Conclusions The use of the modified CAMDEX
Fifty-six were assessed again  years later (Time ).
informant interview enables the structured collection
Based on the CAMDEX informant interview, nine of
of diagnostic information, so that a valid and a reli-
able diagnosis of dementia can be made in those with
pre-existing ID, using established diagnostic criteria.
Correspondence: Sarah L. Ball, Section of Developmental
Psychiatry, Douglas House, b Trumpington Road, Cambridge Keywords Alzheimer’s disease, CAMDEX, Down’s
CB AH, UK (e-mail: slb@cam.ac.uk). syndrome, informant interview, reliability, validity
©  Blackwell Publishing Ltd
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S. L. Ball et al. • CAMDEX informant interview for use with adults with DS
Introduction
The diagnosis of dementia, when it affects people
with Down’s syndrome (DS), is made more compli-
cated by the presence of pre-existing intellectual dis-
ability (ID) (Aylward et al. ). However, the
principal that the diagnosis of dementia requires evi-
dence of a progressive deterioration in memory, in a
number of other cognitive domains, and in daily liv-
ing skills is the same for this group as it is for the
general population. Standardized data collection and
diagnostic procedures validated for the differential
diagnosis of apparent decline in people with DS are
therefore needed. Accurate and consistent diagnosis
is essential for both clinical practice and research.
This will be increasingly important as more effective
treatments become available and as the prevalence of
dementia increases with improved life expectancy.
In the general population, a diagnosis of dementia
is reached on the basis of informant-based and objec-
tive evidence of progressive deterioration in a per-
son’s cognitive abilities and functional skills. The
operational definitions generally accepted are those
outlined in the Diagnostic and Statistical Manual of
Mental Disorders (DSM-IV, American Psychiatric
Association ) and the International Classifica-
tion of Diseases (ICD-, World Health Organization
). Clinical evidence can be evaluated against the
same criteria in order to reach a diagnosis of demen-
tia in a person with DS, provided that it is recognized
that, in this case, the diagnosis of dementia requires
a decline in functioning relative to the individual’s
baseline level, rather than impairment relative to a
‘normal’ level of performance. In the non-learning
disabled population, deterioration can generally be
inferred from the observation of a low level of perfor-
mance relative to population norms. In the DS pop-
ulation it is particularly important to establish change
explicitly, as cognitive impairment and functional dis-
abilities may result from the underlying ID, rather
than to the development of dementia. Neuropsycho-
logical assessment, although useful for providing evi-
dence of decline when used longitudinally, cannot
differentiate at a single point in time between impair-
ment because of dementia and that because of ID.
The diagnosis of dementia therefore requires inter-
views with carers or relatives that have known the
person for a long period of time in order to establish
whether there has been a change from his or her best
©  Blackwell Publishing Ltd, Journal of Intellectual Disability Research , ‒
     
level of performance. Carer reports are also required
in order to document the impact of any cognitive
decline on daily functioning, this being necessary to
meet diagnostic criteria for dementia.
A number of observer-rated scales have been devel-
oped specifically for diagnosing AD in people with
ID. Deb & Braganza () compared rates of
dementia diagnosis among  adults with DS using
the Dementia Questionnaire for Persons with Mental
Retardation (DMR) (Evenhuis ), the Dementia
Scale for Down’s Syndrome (DSDS) (Gedye ),
and the Mini Mental State Examination (MMSE)
against the clinician’s diagnosis. The authors con-
cluded that observer-rated scales were more useful
than direct neuropsychological tests and found good
sensitivity (. for DMR, . for DSDS) and spec-
ificity (. for DMR, . for DSDS) when com-
pared with clinician’s diagnosis. As expected, the
neuropsychological test (the MMSE) failed to sepa-
rate out cognitive impairment because of ID from
that because of dementia.
The DMR (Evenhuis ) consists of  ques-
tions for which the response from the informant is
either ‘normally yes’ (), ‘sometimes’ () or ‘normally
no’ (). Two totals are calculated, the ‘sum of cogni-
tive scores’ (questions on short-term and long-term
memory, spatial and temporal orientation) and the
‘sum of social scores’ (questions on speech, practical
skills, mood, activity and interest). Different cut-off
scores are provided for the diagnosis of dementia in
people with different levels of ID. However, the DMR
ideally requires repeat measures over time for the
presence of a change in function to be identified and
it does not map directly onto strict diagnostic criteria
for dementia, such as DSM-IV and ICD-.
The DSDS (Gedye ) consists of questions in
three categories – early-, middle- and late-stage
dementia. This instrument does place emphasis on
change, making a distinction between newly occur-
ring behaviours and ones that are typical of the per-
son’s normal behaviour. The diagnostic process
differs from that described above and is based on
reaching a global numerical threshold at each level of
severity, rather than making an overall judgement
about the presence or not of a deterioration in the
particular domains that are known to deteriorate with
dementia.
Ideally a diagnostic assessment procedure is
required that provides a structure to the diagnostic
 Journal of Intellectual Disability Research
613
S. L. Ball et al. • CAMDEX informant interview for use with adults with DS
process, mapping onto standard diagnostic criteria
and thereby ensuring that a reliable and valid judge-
ment can be made as to whether dementia is present
or not. A diagnosis of dementia (specifically AD)
should both reflect and, more importantly, predict a
progressive decline in cognitive and functional ability.
In this paper, using longitudinal diagnostic and
neuropsychological data from a previous prevalence
and ongoing longitudinal study of older people with
DS (Holland et al. ), we report on the validity
and reliability of a modified version of the informant
interview of the Cambridge Examination for Mental
Disorders of the Elderly (CAMDEX) (Roth et al.
) as a tool for the diagnosis of dementia in people
with DS.
Methods
Approval for the prevalence and longitudinal studies,
from which the data used in this paper are taken, was
obtained from the Cambridge Local Research Ethics
Committee. We have used data from two of the
assessment procedures employed as part of these past
and ongoing studies: the modified CAMDEX infor-
mant interview and the Cambridge Cognitive Exam-
ination (CAMCOG) neuropsychological test battery
(Holland et al. ).
Participants
At Time ,  participants ( females and  males)
made up a population sample of all individuals with
DS living in one health district (population  ),
who were over the age of  years on  July .
Eligible participants were identified first through
written communication, and then by direct contact,
with local care-providers for people with ID and with
community health teams. Each person with DS and
his/her main carer were then contacted and the con-
sent of the person with DS and/or the agreement of
his/her main carer was sought. Fifty-six of this origi-
nal  took part in a follow-up study approximately
 years later (Time ). At the time of the second
assessment nine participants or their carers did not
wish to be interviewed again and nine of the partici-
pants had died, five of whom had suffered from AD
prior to their death (see Table ).
©  Blackwell Publishing Ltd, Journal of Intellectual Disability Research , ‒
     
Table 1 Sample attrition and progression in diagnosis over  years
Diagnosis at Diagnosis at
Time 1 n at Time 1 Time 2 n at Time 2
Not AD 65 Not AD 45
AD 7
Deceased 4
Refused 9
AD 9 Not AD 0
AD 4
Deceased 5
Refused 0
AD, Alzheimer’s disease.
The CAMDEX
The CAMDEX was originally developed in  as
a standardized instrument for the diagnosis of mental
disorder in the elderly general population, with par-
ticular reference to the early detection of dementia
(Roth et al. ). It was subsequently published by
Cambridge University Press (Roth et al. ) and a
revised version approved in  (Roth et al. ).
The schedule includes an informant interview, an
interview with the participant, an objective examina-
tion of cognitive function (CAMCOG), a standard-
ized schedule for recording observations, and a
physical examination and information on laboratory
investigations.
The informant interview provides a means for col-
lecting information in a structured manner about
those areas of function that are likely to change with
the onset of dementia or of any other mental disorder.
It includes several questions about the informant’s
observations on each of the following: the person’s
memory, general mental and intellectual functioning,
judgement, general performance, specific higher cor-
tical functions, and personality, as well as the pres-
ence or not of specific symptoms and relevant
previous medical and family history. Its validity and
reliability in the general elderly population have been
shown to be good with, for example, information on
memory and mental functioning from the informant
interview highly correlated with objectively measured
decline (Neri et al. ). On measures of inter-rater
reliability in the general population the correlation
between total scores obtained by the two raters has
 Journal of Intellectual Disability Research
614
S. L. Ball et al. • CAMDEX informant interview for use with adults with DS
been found to be high (r = ., P < .) as has the
level of agreement on individual items (median phi
coefficient = ., range = .–.) (Roth et al.
).
Diagnostic assessment (modified CAMDEX
informant interview)
This is a two-step procedure: the first requiring the
collection of information pertinent to the diagnosis
of dementia and the second, the use of this informa-
tion to determine whether the necessary clinical cri-
teria are met for the diagnosis of dementia. For the
first of these steps, the CAMDEX informant inter-
view has been modified for use in our previous stud-
ies. This modification took into account the fact that
cognitive and functional abilities may already be
impaired, because of the person’s pre-existing ID. If
a particular problem is said to be present in answer
to one of the questions (e.g. ‘Does he or she have
difficulty in remembering recent events?’), the infor-
mant is then asked whether this is a change (i.e. a
deterioration), or whether this is something that has
always been a problem. There must be evidence of
deterioration in that particular function (e.g. mem-
ory), as observed by the informant, if that symptom
is then to be scored as being present.
For the second step in both these studies, the clin-
ical information from the informant interview was
presented to A.J.H. (psychiatrist) and F.A.H. (psy-
chologist), blind to the previous diagnostic status, to
neuropsychological test performance, and to the age
and gender of the person with DS. A judgement was
then made as to whether the person had deteriorated
in each of the cognitive and functional domains in
which change is necessary in order to meet the crite-
ria for the diagnosis of dementia, or of any other
psychiatric disorder. A consensus diagnosis was
reached by rating these findings against the opera-
tional criteria for a CAMDEX, ICD- and DSM IV
diagnosis, also taking into account relevant labora-
tory information (e.g. thyroid function). All diag-
noses reported in this paper are based on CAMDEX
criteria.
Neuropsychological assessment (CAMCOG)
The CAMCOG neuropsychological test battery,
which forms part of the CAMDEX (Roth et al. ;
©  Blackwell Publishing Ltd, Journal of Intellectual Disability Research , ‒
     
Huppert et al. ), was completed at Time  and
at Time  by all participants who were able to do so.
This was used to obtain objective evidence of change
in the areas of cognitive function that characteristi-
cally decline with dementia in the general elderly
population. Hon et al. () found that older par-
ticipants with DS (>  years) performed significantly
worse than younger ones on a slightly modified ver-
sion of the CAMCOG. Scores on the CAMCOG
subscales for orientation, language, memory, praxis,
attention/calculation, abstract thinking and percep-
tion, as well as the total score, have been shown to
differentiate significantly between normal individuals
and those with dementia (Huppert et al. ).
Validity
As there is currently no ‘gold standard’ tool for the
diagnosis of dementia in people with DS, the tradi-
tional approach to establishing the validity of diag-
nostic instruments has been to compare findings with
the judgements of clinicians. This is potentially prob-
lematic, as clinicians tend to make these clinical deci-
sions using broadly the same assessment methods as
the instruments that are developed and thus high
levels of agreement are likely, whether or not the
assessments are valid. If, for example, dementia is
over-diagnosed by clinicians generally and over-
diagnosed when using a particular diagnostic tool to
the same degree, a high level of agreement between
the two would be a false indicator for the validity
of the test.
In the case of dementia (specifically AD), one
important test of the validity of a diagnostic tool is
whether it corresponds with independent evidence of
progressive decline prior to diagnosis (concurrent
validity). The concurrent validity of the CAMDEX
informant interview was established by examining the
agreement between informant interview-based diag-
nosis and objective measurement of decline on cog-
nitive tasks over the preceding  years.
An even stronger test of validity is whether the
diagnosis predicts the course of the illness (predictive
validity). If it does not, then clearly the diagnosis is
incorrect, as AD is a progressive brain disorder. The
predictive validity of the informant interview was
examined by determining the degree to which diag-
nosis at Time  predicts both subsequent decline on
cognitive tasks and diagnosis at Time .
 Journal of Intellectual Disability Research
615
S. L. Ball et al. • CAMDEX informant interview for use with adults with DS
Not AD at Time 1
No decline Decline* No decline
Not AD at Time 2 31 1 –
AD at Time 2 2 4 – 3
Total 33 5 –
AD, Alzheimer’s disease.
* Decline by  standard deviation below the mean change in score.
In order to cross-tabulate diagnosis with cognitive
decline, it is necessary that decline is quantified. In
the analysis of both concurrent and predictive valid-
ity, decline is quantified as a decrease in CAMCOG
score of more than one standard deviation (.
points) from the mean change of the group (-.),
that is, . points. Fifty-five participants completed
the CAMCOG at both Time  and Time . However,
in order to avoid floor effects, those participants who
scored less than . on the CAMCOG at Time 
were excluded from the analysis, on the basis that
they were unable to decline to the specified degree.
Forty-one participants remained in the analysis. Of
the  participants who scored below this threshold
at baseline none had received a diagnosis of AD on
the basis of informant reported decline. All had mod-
erate to profound ID. This observation in itself
indicates that the use of absolute scores on such
neuropsychological tests for making dementia diag-
noses is problematic.
Concurrent validity
We examined the degree to which a diagnosis of AD
(made at Time ) was associated with objective
decline in CAMCOG performance between Times 
and . Diagnosis was made using information from
the informant interview alone, without reference to
neuropsychological test performance or previous
diagnosis. Diagnostic category at Time  (‘AD’ vs.
‘not AD’) was cross-tabulated with decline in CAM-
COG score (‘decline’ vs. ‘no decline’) defined as
above. Only data for the  participants who scored
>. on the CAMCOG at baseline assessment are
included in this analysis.
©  Blackwell Publishing Ltd, Journal of Intellectual Disability Research , ‒
     
Table 2 Cross-tabulation of diagnosis at
AD at Time 1 Time  with decline* in the Cambridge
Examination for Mental Disorders of the
Elderly (CAMCOG) score since Time 
Decline* Total
– 32
9
3 41
Predictive validity
Predicting cognitive decline
We examined the accuracy with which an informant
interview-based diagnosis of AD at Time  predicted
decline in CAMCOG performance over the following
 years. Diagnostic category at Time  (‘AD’ vs. ‘not
AD’) was examined in relation to subsequent decline
in CAMCOG score (‘decline’ vs. ‘no decline’), as
defined above. Only data for the  participants who
scored >. on the CAMCOG at baseline assess-
ment were included in this analysis.
Predicting diagnosis
In order to investigate the stability of diagnosis over
time, and to provide additional evidence for predic-
tive validity, we examined the accuracy with which
CAMDEX-based diagnosis at Time  predicted diag-
nostic category at Time . Time  diagnoses were
made blind to diagnoses at Time . Data for  par-
ticipants were included in this analysis. The carers of
 of these were interviewed at Times  and . For a
diagnosis of AD to be valid, it must be followed by
deterioration rather than by recovery. On the basis
that they had clearly deteriorated following diagnosis,
we have also included data for the five participants
with AD at Time  who had died before the Time 
assessment (n =  in total).
Inter-rater reliability
For the analysis of inter-rater reliability, data were
collected on a subset of  people with DS (eight
males,  females, mean age  years, range –).
 Journal of Intellectual Disability Research
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S. L. Ball et al. • CAMDEX informant interview for use with adults with DS
Four were diagnosed with AD. The informants were
either relatives () or paid carers (), who had
known the participants for a mean of  years. All but
two of these carers had known the participants for
more than  years. One had had  months of direct
contact and one only  weeks, but both had obtained
detailed information from colleagues spanning over
several years. The responses of the  informants
were rated simultaneously and independently by a
psychiatrist (P.T.) who conducted the informant
interview, and a psychologist (S.L.B.) who observed.
For each participant the ratings were compared for
all items in the interview. The level of agreement
between interviewers for each item was calculated by
means of the Kappa coefficient.
Results
Diagnoses
At Time , nine out of the  participants received a
diagnosis of AD. At Time ,  of the remaining 
participants received a diagnosis of AD. Of these, four
had been diagnosed with AD at Time  and seven
were new cases since baseline. Five of the nine diag-
nosed with AD at Time  had died before Time . All
were still suffering from AD at the time of their
deaths.
Concurrent validity
Diagnosis at Time  was cross-tabulated with decline
on CAMCOG since Time . This revealed that seven
out of the nine individuals diagnosed with AD at
Time  (%) had shown a decline of >. points
between the assessments at Times  and . In com-
parison, only one of the  individuals without AD
at Time  (%) had shown a decline of >. points
(see Table ). Informant interview-based diagnosis
was shown to have a specificity of . [% confi-
dence interval (CI) = .–. using Wilson’s
method, Wilson ] when compared with objec-
tively measured decline (as described above).
Although sensitivity was calculated to be ., the
small number of participants showing cognitive
decline (n = ) is reflected in a wide confidence inter-
val (% CI = .–., Wilson ), making it
difficult to draw clear conclusions from this result.
However, a decline of >. points on the CAMCOG
©  Blackwell Publishing Ltd, Journal of Intellectual Disability Research , ‒
     
since Time  is significantly more likely to have
occurred in those diagnosed with AD at Time  than
in those without a diagnosis of AD (P < .,
Fisher’s Exact). The odds of a participant having
shown a decline of >. points are at least  times
greater if he/she has been diagnosed with CAMDEX
AD than if he/she has not (odds ratio = ., %
CI = .–.). This is a conservative estimate
based on the lower bound of the confidence interval
for the odds ratio.
A detailed look at ‘false positive’ and ‘false nega-
tive’ cases provides some further support for the con-
current validity of the informant measure. The two
individuals diagnosed as having AD at Time  who
had not shown decline in CAMCOG score since
Time  had been diagnosed with only mild AD. One
had shown decline on memory and praxis subscales
(- and -, respectively) but improvements in other
areas of cognition, and the other showed decline on
the praxis subscale only (-). This observation would
suggest that these two people probably do have AD,
but as the diagnosis was made early in the course of
the disease, the level of cognitive decline over the
previous  years was insufficient to meet the ‘decline’
criteria we set for this study. Further follow-up
assessments are required to confirm whether or not
this is the case.
The one participant in the group of  considered
not to have AD, who had in fact showed decline on
the CAMCOG between Times  and , was found to
be suffering from a serious physical illness at the time
of the CAMCOG assessment at Time . This explains
her impaired performance on the task at that time.
Thus, even though she showed cognitive decline
when the two CAMCOG scores were compared, the
absence of a diagnosis of AD was correct.
Predictive validity
Predicting cognitive decline
Only three of the nine participants who were diag-
nosed with AD at Time  were subsequently able to
participate in cognitive assessment at Time . Five
had died and one was too ill to be assessed. The
findings from this part of the study, although sup-
portive of predictive validity of the diagnostic assess-
ment, are limited because of the small numbers of
people diagnosed as having AD at Time  that could
be re-assessed at Time .
 Journal of Intellectual Disability Research
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S. L. Ball et al. • CAMDEX informant interview for use with adults with DS
Cross-tabulation of diagnosis at Time  with
decline on CAMCOG over the subsequent  years
revealed that all three (%) showed a decline of
>. points compared with only five out of  (%)
of participants not diagnosed with AD at baseline. A
decline of >. points on the CAMCOG was signif-
icantly more likely to occur in those with AD at
baseline than in those without (P < ., Fisher’s
Exact). However, although this lends support to the
predictive validity of the CAMDEX, the number of
participants with AD is too small to enable us to
estimate accurately the magnitude of this difference
in likelihood of cognitive decline.
A detailed look at ‘false positive’ and ‘false nega-
tive’ cases provides further support for the validity
of the informant measure. Of the five participants
considered not to have AD at Time  but who
subsequently showed cognitive decline, four were
diagnosed with AD at Time  (blind to previous
diagnostic data and CAMCOG scores). This
reflected new incidences of AD, as would be expected
during the course of the study (discussed in more
detail below). The one participant showing decline,
but not receiving a diagnosis of AD at Time , was
severely affected by physical illness (as described
under ‘Concurrent validity’).
Predicting diagnosis
Cross-tabulation of diagnosis at Time  with diagno-
sis  years later at Time  revealed that all nine par-
ticipants diagnosed with AD at baseline (%) were
either still suffering from AD () or had died follow-
ing the disease () by Time . Of the  individuals
not diagnosed with AD at Time , however, only
seven (%) had ‘switched diagnoses’ and were diag-
nosed as having AD at Time  (see Table ). Those
diagnosed with AD at Time  were significantly more
likely to be diagnosed with AD at Time  than those
Table 3 Cross-tabulation of diagnosis at Time  with diagnosis at Time 
Not AD at Time 2
Not AD at Time 1 45
AD at Time 1 – 4
Total 45
AD, Alzheimer’s disease.
©  Blackwell Publishing Ltd, Journal of Intellectual Disability Research , ‒
     
without a baseline diagnosis of AD (P < .,
Fisher’s Exact).
The odds of a participant receiving a diagnosis of
AD at Time  are at least  times greater if he/she
was diagnosed with AD at Time  than if he/she was
not (odds ratio = ., % CI = .–.). This
is based on the lower bound of the confidence inter-
val for the odds ratio.
Based on the annual incidence rate estimated in
previous paper (Holland et al. ) of .%, one
would expect up to  new cases to develop over
 years in this population sample. However, with
sample attrition because of refusal (nine participants)
and death from causes other than AD (four partici-
pants), we would expect observed incidence to be
slightly lower than this. It is therefore likely that the
seven that were said not to have AD at Time  but
were found to have AD at Time  represent new
‘incidence’ cases rather than a failure to make the
diagnosis at baseline. This is supported by the fact
that of the seven, two had received a diagnosis of ‘age-
related cognitive decline’ (ARCD) and one had
received a diagnosis of ‘frontal type dementia’ (FTD)
at Time . Both of these diagnoses, we believe, fre-
quently precede the development of AD in people
with DS (see Holland et al. ,  for further
information).
Inter-rater reliability of the CAMDEX
informant interview
Analysis of individual items in the interview has
shown the agreement between raters to be excellent
with % of items falling within the ‘near perfect’
range (Kappa > .) and all items showing an agree-
ment of Kappa > . (substantial), as defined by
(Landis & Koch ). See Table  for a breakdown
of levels of agreement across the cognitive and func-
tional areas assessed.
AD at Time 2 Deceased following AD at Time 2 Total
7 – 52
5 9
11 5 61
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618
S. L. Ball et al. • CAMDEX informant interview for use with adults with DS
Table 4 Inter-rater reliability: agreement between raters for individual items
Part 1: items concerned with history of present difficulty
Personality
Memory
General mental functioning
Everyday activities
Health (delirium, depressed mood, sleep, paranoid features,
cerebrovascular problems)
Part 2: questions pertaining to participant’s past history
Part 3: questions pertaining to family history
Over all
Discussion
The necessity of disentangling decline from pre-
existing intellectual and functional impairments com-
plicates the diagnosis of dementia in people with DS.
However, these findings demonstrate that the CAM-
DEX informant interview, with careful modification
to ensure emphasis on change, enables sound diag-
noses to be made. Our findings suggest that the mod-
ified CAMDEX informant interview is a both valid
and reliable tool.
The concurrent validity of the instrument is very
good. Diagnosis based on the findings of the infor-
mant interview alone, blind to information regarding
neuropsychological test performance, was highly
consistent with expectations based on independent
objectively observed decline. As expected, diagnostic
category at Time  discriminated well between those
who had declined by the specified degree and those
who had not. Those with a diagnosis of AD were at
least eight times more likely to have shown decline in
neuropsychological test performance over the pre-
ceding  years than those without a diagnosis of AD.
However, although point estimates of sensitivity and
specificity for the CAMDEX informant interview
were shown to be high (. and ., respectively)
and comparable with the levels found for the DMR
and DSDS (Deb & Braganza ), the small num-
ber of participants with AD in the study, and result-
ing width of the % confidence interval for the
sensitivity score, mean that these should be inter-
preted with caution.
©  Blackwell Publishing Ltd, Journal of Intellectual Disability Research , ‒
     
% of items with near % of items with substantial
perfect inter-rater inter-rater reliability
reliability (Kappa > 0.8) (Kappa 0.6–0.8)
88 12
100 –
91 8
93 7
91 8
93 7
95 5
100 –
91 9
Despite the small number of participants with AD
at baseline who survived to take part in the Time 
assessment, predictive validity was also shown to be
good. None of the AD diagnoses made at baseline
were reversed at Time . Those with a diagnosis of
AD at baseline were at least six times more likely to
be diagnosed with AD at Time  than those without
a baseline diagnosis of AD. The Time  diagnoses
were all made blind to knowledge of previous diag-
noses, thus ruling out potential bias. There is also
some support for the accuracy of the CAMDEX in
predicting cognitive decline, although numbers are
too small to draw any firm conclusions.
As expected, a number of participants developed
AD in the  year period between baseline and follow-
up assessment. These also showed decline in neurop-
sychological test performance.
Although the results of the study are highly sup-
portive of the validity of the informant interview, the
relatively small number of participants with AD in
the study limits both the strength of the conclusions
that can be drawn, and the degree to which validity
measures can be compared with those of other diag-
nostic tests. However, the CAMDEX informant
interview is currently the only tool for the assessment
of AD in DS to have been evaluated with regard to
predictive validity, and to use accepted criteria to
make AD diagnoses. This study is also the first in this
field to have demonstrated validity as measured
against objective evidence of neuropsychological
decline (a much stronger comparison than clinician’s
diagnosis). A minor drawback to this method is that
 Journal of Intellectual Disability Research
619
S. L. Ball et al. • CAMDEX informant interview for use with adults with DS
the difficulty level of the CAMCOG entailed the
exclusion of participants who did not score suffi-
ciently high above the floor of the test at baseline,
thus reducing numbers further and limiting the
degree to which results can be generalized to the DS
population as a whole.
In terms of the agreement between independent
ratings of individual items, the informant interview
was shown to have excellent inter-rater reliability. We
did not examine inter-informant reliability, as in our
earlier study we found that it was essential that the
informant had known the person for at least
 months and had direct experience of supporting
that person over that time. Any informant without
this experience was found not to be able to answer
the questions reliably. We thus determined that the
‘gold standard’ for informants must be that stated
above.
As described earlier, the diagnostic procedure used
in this and our previous studies has consisted of two
stages, the first being the collection of relevant infor-
mation (using the CAMDEX informant interview)
and the second being the rating of this information
against recognized diagnostic criteria. The CAM-
DEX informant interview standardizes the history-
taking procedure, enabling the clinician to extract
information on changes in cognition and behaviour
that can then be rated against the standard sets of
criteria used for the diagnosis of dementia (and its
subtypes) or other mental disorders in the general
population (i.e. ICD-, DSM-IV, Criteria for FTD,
criteria for Lewy body dementia, CAMDEX criteria,
or criteria for affective disorders, etc.). The infor-
mant-based diagnostic process is part of a compre-
hensive clinical assessment that includes, where
necessary, investigations that rule out disorders that
might mimic the decline seen in dementia (e.g.
hypothyroidism).
We recommend that where cognitive or behav-
ioural change affecting an older person with DS has
been observed, a proper assessment is essential. This
should include a full informant and patient interview
that identifies the nature and course of the change. It
should identify mental state abnormalities and social
factors that might inform the diagnostic process, and
where clinically indicated, investigations to exclude
other possible causes of decline. A neuropsychologi-
cal assessment can identify the person’s cognitive
strengths and weaknesses and provide a baseline
©  Blackwell Publishing Ltd, Journal of Intellectual Disability Research , ‒
     
against which subsequent decline can be measured.
The valid and reliable diagnosis of dementia and the
exclusion of other possible causes of observed decline
are the starting point for the development of appro-
priate support for the person concerned and his/her
family or other carers, as well as for the initiation of
treatment.
Acknowlegements
The most recent study has been funded by the
Down’s Syndrome Association with a grant from the
National Lotteries Community Fund. A.J.H. is sup-
ported by the Heath Foundation. We are most grate-
ful for their support and for the help we have received
from many people with DS and their families and
other carers.
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Accepted  February 