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Time to diagnosis in youngonset dementia as compared with lateonset
D. van Vliet, M. E. de Vugt, C. Bakker, Y. A. L. Pijnenburg, M. J. F. J. VernooijDassen, R. T. C. M. Koopmans and F. R. J.
Psychological Medicine / Volume 43 / Issue 02 / February 2013, pp 423 432
DOI: 10.1017/S0033291712001122, Published online: 28 May 2012
Link to this article: http://journals.cambridge.org/abstract_S0033291712001122
How to cite this article:
D. van Vliet, M. E. de Vugt, C. Bakker, Y. A. L. Pijnenburg, M. J. F. J. VernooijDassen, R. T. C. M. Koopmans and F. R. J.
Verhey (2013). Time to diagnosis in youngonset dementia as compared with lateonset dementia. Psychological Medicine,
43, pp 423432 doi:10.1017/S0033291712001122
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Time to diagnosis in young-onset dementia as
compared with late-onset dementia
D. van Vliet1, M. E. de Vugt1*, C. Bakker2,3, Y. A. L. Pijnenburg4, M. J. F. J. Vernooij-Dassen3,5,6,7,
R. T. C. M. Koopmans3,5and F. R. J. Verhey1
1School for Mental Health and Neuroscience, Alzheimer Centre Limburg, Maastricht University Medical Centre, Maastricht, The Netherlands
2Florence, Mariahoeve, Centre for Specialized Care in Young-Onset Dementia, The Hague, The Netherlands
3Department of Primary and Community Care: Centre for Family Medicine, Geriatric Care and Public Health, Radboud University Nijmegen,
Medical Centre, Nijmegen, The Netherlands
4Alzheimer Centre and Department of Neurology, VU University Medical Centre Amsterdam, Amsterdam, The Netherlands
5Alzheimer Centre Nijmegen, Radboud University Nijmegen, Medical Centre, Nijmegen, The Netherlands
6Kalorama Foundation, Beek-Ubbergen, The Netherlands
7Scientific Institute for Quality of Healthcare, Radboud University Nijmegen, Medical Centre, Nijmegen, The Netherlands
Background. The extent to which specific factors influence diagnostic delays in dementia is unclear. Therefore, the
aim of the present study was to compare duration from symptom onset to diagnosis for young-onset dementia (YOD)
and late-onset dementia (LOD) and to assess the effect of age at onset, type of dementia, gender, living situation,
education and family history of dementia on this duration.
Method. Data on 235 YOD and 167 LOD patients collected from caregivers from two prospective cohort studies were
used. Multiple linear regression analysis was performed.
Results. The duration between symptom onset and the diagnosis of YOD exceeded that of LOD by an average of 1.6
years (2.8 v. 4.4 years). Young age and being diagnosed with frontotemporal dementia were related to increases in
the time to diagnosis. Subjects with vascular dementia experienced shorter time to diagnosis.
Conclusions. There is a need to raise special awareness of YOD to facilitate a timely diagnosis.
Received 23 January 2012; Revised 15 April 2012; Accepted 24 April 2012; First published online 28 May 2012
Key words: Diagnostic delay, early onset dementia, presenile, time to diagnosis, young onset dementia.
The timely diagnosis of dementia is increasingly re-
garded as a prerequisite for beginning adequate
treatment, planning for the future and coping with the
prognosis (Vernooij-Dassen et al. 2005; Salloway &
Correia, 2009). However, diagnosis is often delayed
(Salloway & Correia, 2009). The time from the pres-
entation of an individual’s first symptoms to a de-
mentia diagnosis has been found to be 1 year on
average (Wilkinson et al. 2004), but delays of up to
5 years have also been reported (Holzer & Warshaw,
2000; Bamford et al. 2007). Some of the barriers to early
diagnosis in primary care are failure to recognize the
slowly evolving symptoms, lack of confidence to make
and disclose a diagnosis, negative attitudes towards
diagnosis, for example, because of a lack of support
resources for the person with dementia or potential
risks of an early diagnosis (Iliffe & Manthorpe, 2004;
Iliffe et al. 2006). Long delays may be particularly true
for young-onset dementia (YOD) due to its lower
prevalence and larger variety of aetiologies (Mendez,
2006; Ridha & Josephs, 2006; Rossor et al. 2010). YOD
may also present differently from late-onset dementia
(LOD), with more marked neuropsychiatric symp-
toms than cognitive symptoms in frontotemporal de-
mentia (FTD) and several other rare aetiologies
typically occurring at a young age (Mendez, 2006;
Rosness et al. 2008; Kelley et al. 2009). Non-recognition
can have serious consequences for younger people
and their families, such as tension with family mem-
bers living in the same house (often children) and a
higher chance of divorce or job loss (van Vliet et al.
2011). Timely recognition makes it possible to adjust to
the consequences of the disease; therefore, it is im-
portant to gain insight into the factors associated with
time to diagnosis and to what extent they influence the
duration of that period. Young age (Luscombe et al.
1998; Rossor et al. 2010) and a diagnosis of FTD
(Rosness et al. 2008) have both been associated with
* Address for correspondence: M. E. de Vugt, Ph.D., Maastricht
University Medical Centre, School for Mental Health and
Neuroscience/Alzheimer Centre Limburg, Department of Psychiatry
and Psychology, PO Box 5800, 6202 AZ Maastricht, The Netherlands.
Psychological Medicine (2013), 43, 423–432.
f Cambridge University Press 2012
diagnostic difficulties in separate studies, so their in-
dependent roles are unknown. We hypothesize that
younger age is related to longer time to diagnosis in-
dependent of the influence of a diagnosis of FTD.
Furthermore, it is assumed that a high education level
and female gender are associated with a shorter time
to diagnosis based on studies of help-seeking behav-
iour (Tijhuis et al. 1990; Mackenzie et al. 2006;
Tedstone Doherty & Kartalova-O’Doherty, 2010).
Likewise, it is hypothesized that a family history of
dementia is related to the length of time to diagnosis
(Ramakers et al. 2009) because such a history would
promote the earlier recognition of symptoms. In ad-
dition, recognition of dementia by general prac-
titioners in patients living alone has been found to be
reduced; thus we expect this to be related to increased
time to diagnosis (Pentzek et al. 2009). The aim of the
present study is to compare the duration of the period
from symptom onset to diagnosis for YOD and LOD
and to investigate the influence of age of onset, de-
mentia type, gender, education, living situation and
family history of dementia on the time to diagnosis.
This study is part of a Dutch prospective cohort study,
the Needs in Young onset Dementia (NeedYD) study,
which has been described in more detail elsewhere
(van Vliet et al. 2010). A total of 215 participants with
YOD were recruited who had an age at onset of less
than 65 years. They were included in the study to-
gether with their primary caregivers. Patients were
consecutive referrals from university medical centres
(n=56), regional hospitals (n=10) and regional com-
munity mental health services (n=20); they applied to
participate (n=14) or were recruited through special-
ized day-care facilities (n=115). The diagnoses were
made on the basis of clinical, neuropsychological and
neuroimaging data according to the criteria from the
Diagnostic and Statistical Manual of Mental Disorders,
fourth edition, text revision (DSM-IV-TR; APA, 2000)
for dementia and the Dutch consensus guidelines
(CBO, 2005) that include internationally accepted cri-
teria for the subtype diagnoses (McKhann et al. 1984;
Erkinjuntti, 1994; Neary et al. 1998; Mesulam et al.
2003; McKeith, 2006). Clinical diagnoses were checked
against clinical files for every patient and against
medical hospital records when available. The ex-
clusion criteria were: (1) unreliability on the part of the
informant (if the informant and person with dementia
did not have contact with each other at least once per
week); (2) lack of informed consent of the participant;
(3) living in a nursing home; or (4) dementia caused by
human immunodeficiency virus (HIV), traumatic
brain injury, Down’s syndrome, Huntington’s chorea
or alcohol dementia. Since these diagnoses are as-
sociated with a range of specific problems, which
would have obscured the impact of a younger age at
dementia onset, these diagnoses were excluded.
A total of 173 subjects with LOD were used as a
comparison group. They were selected from a 2-year
follow-up cohort study, the Maastricht Study of
Behaviour in Dementia (MAASBED) study (Aalten
et al. 2006), overall using the same design, assessment
measures and diagnostic criteria. The NeedYD study
protocol was based on the methods of the MAASBED
study to be able to compare outcomes. Both the
MAASBED and NeedYD studies are prospective stu-
dies, with assessments every 6 months during 2 years.
Inclusion criteria and recruitment differed somewhat
between the studies. In the MAASBED study, patients
with dementia were included irrespective of their di-
agnostic subtype and they were consecutively enrolled
from the Memory Clinic of the Maastricht University
Medical Centre (MUMC+) or from the Regional
Institute for Community Mental Health Care (RIAGG)
in Maastricht. The MAASBED cohort included 26
YOD patients added to the YOD group in the present
study. For the current study, the data collected from
the primary caregivers of the patients in the NeedYD
and MAASBED studies were used.
The study protocol was approved by the Medical
Ethics Committee of the University Medical Centre
Maastricht. The written informed consent of the sub-
jects or their legal representatives was obtained prior
to the investigation. During a semi-structured inter-
view with the patient as well as the primary caregiver
the following questions were asked: ‘Which diagnosis
was given to you? Do you know what type of de-
mentia you have?’, followed by the question: ‘In
which year did the very first symptoms or complaints
occur?’. The interviewer was instructed to ask whe-
ther there were any earlier signs or symptoms. In ad-
dition, a semi-structured open-ended interview with
the primary caregiver was tape-recorded including the
following questions: ‘When did you first notice
something was wrong with your family member?’,
‘What did you notice?’, ‘Can you describe the period
prior to diagnosis?’, ‘What did you think was
wrong?’, ‘Which problems did you experience during
this period?’. Again the interviewer was instructed to
encourage caregivers to elaborate on their answers
and to think of any other signs or symptoms that
occurred. The year of the earliest sign or symptom,
either in the cognitive, behavioural or functional
424D. van Vliet et al.
domain, was recorded as the year of symptom onset.
The moment that caregivers consciously noticed
changes mostly occurred later than the moment that
was indicated with hindsight. In these cases, the year
that was indicated with hindsight was noted asthe year
of symptom onset. Subjects’ medical records were used
to determine the year in which each diagnosis was es-
tablished. The duration (in years) between symptom
onset and diagnosis was calculated by subtracting the
year of symptom onset from the year of diagnosis.
Demographic data were collected that indicated
age, gender, education level divided into eight cat-
egories of the Dutch educational system ranging from
(1) primary school to (8) the completion of university-
level education and family history of dementia, which
was determined to exist if any family member had had
dementia. The Global Deterioration Scale (GDS) was
used to assess the severity of the dementia (Reisberg
et al. 1982).
First, a sample-size calculation was performed for the
group comparisons and the regression analysis, with a
medium effect size, an a level of 0.05 and power of
0.80, which showed that the sample size should be at
least 64 in each group for the group comparisons and
103 for the regression analysis. Analyses were per-
formed using SPSS software, version 17.0 (SPSS, Inc.,
USA). To assess descriptive group characteristics, x2
tests and Mann–Whitney U tests were used as appro-
priate. For comparisons of duration to diagnosis be-
tween groups (YOD and LOD), Mann–Whitney U tests
were performed. These non-parametric tests were
used, because the distribution of the dependent vari-
able was skewed or because the variance of the de-
pendentvariable was not
groups. A multiple linear regression analysis was
performed to assess the relationship between group
(YOD v. LOD), type of dementia, living arrangements
(alone v. not alone), education, gender and family
history of dementia (the independent variables) and
the estimated duration from symptom onset to diag-
nosis in years (the dependent variable). The data were
log-transformed because of the heteroscedastic vari-
ance of the residuals of the variable: duration from
symptom onset to diagnosis. The types of dementia
were divided into four categories and dummy-coded
with Alzheimer’s disease (AD) as the reference group:
(1) AD; (2) FTD; (3) vascular dementia (VaD) and
mixed dementia (AD and VaD); and (4) other.
Education was recoded as low (1–4) or high (5–8). The
collinearity between the predictors was explored
using phi-correlations. The potential influence of out-
liers was determined. In order to detect outliers, which
are data points of the dependent variable (i.e. time to
diagnosis) that differ largely from the values predicted
by the model, the standardized residuals (residual/
estimated standard deviation of the residual) were
assessed. As a general rule, 99.9% of standardized re-
siduals (or z scores) should lie between x3.29 to 3.29,
and 3 is generally used as an approximation. The a
level was set at 0.05 for the analyses. In addition, out-
liers were detected by visual inspection of the data.
For these cases it was checked whether the year of
symptom onset that was noted by the researcher cor-
responded with the tape-recorded interviews. In one
case, this resulted in correction of the year of symptom
The overall study group consisted of 414 patients (241
with YOD and 173 with LOD). Information on the
duration between symptom onset and diagnosis was
not available for six YOD and six LOD patients (year
of diagnosis was missing in one case, year of symptom
onset in the other 11 cases). These patients were simi-
lar to the group with complete data with regard of age
at study entry (U=2291.5, p=0.78), gender (p=0.25,
Fisher’s exact test), education (p=0.47, Fisher’s exact
test), distribution of diagnoses (p=0.96, Fisher’s exact
test) and group (YOD v. LOD) (x2=0.34, p=0.56).
Thus, 402 patients with complete data were included
in this study. The mean estimated age at onset was for
the YOD group 54.8 years (S.D.=5.9) and for the LOD
group 75.8 years (S.D.=5.9). Table 1 shows the age at
diagnosis and the sample characteristics of both
groups at study entry. The patients of the YOD group
were more frequently male, they had a higher edu-
cation and they more often had a family member with
dementia. In addition there were significant differ-
ences in the distribution of diagnoses between groups,
with more FTD and ‘other’ dementias in the YOD
group. The dementia was more severe in the YOD
group based on GDS scores. Mini-mental state exam-
ination (MMSE) scores were missing in a large sample
and therefore less reliable. Spouses were most often
the primary caregiver in the YOD group, while in LOD
children were primary caregiver in the largest pro-
portion of cases. In the LOD group there were also
more patients living alone than in the YOD group.
Duration from symptom onset to diagnosis
The group comparison between YOD and LOD of es-
timated duration from symptom onset to diagnosis
showed a significant difference, with an average time
Time to diagnosis in young-onset dementia425
to diagnosis of 4.4 years (S.D.=3.1, range 0.5–18.0) in
the YOD group and an average time to diagnosis of
2.8 years (S.D.=2.1, range 0.2–10.0) in the LOD group
For each dementia subtype, the period was longer
in the YOD group than in the LOD group, with
significant differences for those with AD (U=6212.5,
p<0.001) and VaD (U=353.0, p=0.006). Differences
between young- and late-onset FTD or ‘other’
dementias could not be assessed because of the
small sample sizes, especially in the LOD group
Table 1. Demographic variables at study entry of the YOD and LOD groups
YOD (n=235)LOD (n=167)Test valuep
Mean age at diagnosis, years (S.D.)
Mean age at study entry, years (S.D.)
Gender, % female
Low (levels 1 and 2)
Medium (levels 3–5)
High (levels 6–8)
Family history of dementia, % 49.6
Living alone, %
Caregiver relationship with patient, %
Dementia severity (GDS score), %
Mild (scores 3 and 4)
Moderate (score 5)
Severe (scores 6 and 7)
Mean MMSE score (S.D.)c
YOD, young-onset dementia; LOD, late-onset dementia; S.D., standard deviation; AD, Alzheimer’s disease; FTD,
frontotemporal dementia; VaD, vascular dementia and mixed dementia; PA, progressive aphasia; DLB, dementia with
Lewy bodies; PDD, Parkinson’s disease dementia; MELAS, mitochondrial encephalomyopathy, lactic acidosis and
stroke-like episodes; TE, toxic encephalopathy; MSA, multiple system atrophy; GDS, Global Deterioration Scale; MMSE,
mini-mental state examination.
aEducation level ranged from the category (1) primary school to (8) the completion of university-level education.
bBased on the categories: AD, FTD, VaD, other dementias.
cReasons for missing MMSE scores: refusal, cognitive problems, e.g. language problems, emotional reactions or lack of
motivation during testing.
426D. van Vliet et al.
Factors related to time to diagnosis
The data for the variable ‘family history of dementia’
were missing in 19 cases. In most of these cases, the
person providing the information was not a spouse or
partner and may not have been aware of the family
history. This was significantly more frequent in the
group with missing data than in the group with com-
plete data (spouse v. other: p<0.001, Fisher’s exact
test). To determine the effect of family history of de-
mentia on time from symptom onset to diagnosis, a
regression analysis was performed with the predictors
group, type of dementia, living arrangements, gender
and education in the first block and ‘family history of
dementia’ in a second block. Adding this variable did
not significantly improve the model (DR2=0.00,
p=0.40); therefore, family history of dementia was left
out of the analyses so that the cases with missing data
for this variable could still be included.
The regression analysis (Table 3) with estimated
time from symptom onset to diagnosis as the depen-
dent variable and group, type of dementia, living ar-
rangements, gender and education as predictors
showed that having LOD [b=x0.20, t(395)=x3.81,
p<0.001] and being diagnosed with VaD as opposed
to AD [b=x0.13, t(395)=x2.70, p=0.007] were sig-
nificantly associated with a shorter time to diagnosis
and a diagnosis of FTD with a longer time to diagnosis
[b=0.12, t(395)=2.49, p=0.013], with group the most
important predictor. The model explained 10% of the
variance. A sensitivity analysis was performed ex-
cluding the YOD patients that were recruited in the
MAASBED study, which did not change the results. In
addition, the analyses were repeated, excluding the
FTD and ‘other’ diagnostic categories and results
t(324)=x5.93, p<0.001] and being diagnosed with
VaD as opposed to AD [b=x0.11, t(324)=x2.19,
p=0.029] were significantly associated with a shorter
time to diagnosis.
There were no outliers with a standardized residual
outside the range of ¡3. A post hoc regression analysis
of family history of YOD within the YOD group
showed that this was not a significant predictor of the
duration from symptom onset to diagnosis [b=x0.03,
Dementia severity around time of diagnosis
Post hoc we selected patients that were included
shortly after diagnosis and we excluded the FTD sub-
category to compare more homogeneous groups. Time
from symptom onset to diagnosis, GDS score and
MMSE score were compared between these sub-
samples of YOD (n=93) and LOD (n=164) patients.
Results confirmed our previous finding that a signifi-
cantly longer time from symptom onset to diagnosis
was found in the YOD group (4.6 years, S.D.=3.1)
compared with the LOD group (2.8 years, S.D.=2.1)
(U=4789.0, p<0.001). Groups did not differ in GDS
scores [x2(2)=5.5, p=0.065], which were categorized
into mild- (74.7% YOD, 71.3% LOD), moderate-
(18.7% YOD, 26.8% LOD) and severe-stage dementia
(6.6% YOD, 1.8% LOD). There was a significant dif-
ference in MMSE scores between groups, with a mean
score of 20.3 (S.D.=5.6, n=85) in the YOD group v. 18.1
(S.D.=4.5, n=158) in the LOD group [F(1)=10.9,
This study investigated the estimated time that elapses
between the appearance of the first symptoms of de-
mentia and moment of diagnosis using a relatively
large sample of both YOD and LOD patients. The
group comparisons showed that the duration of this
period in the YOD group took an average of 1.6 years
longer than in the LOD group. The average time from
symptom onset to diagnosis in the YOD group ex-
ceeded that of the LOD group for every dementia
subtype; it was statistically significant for AD and
The regression analysis showed that having YOD
was the most important predictor for an extended
period between symptom onset and diagnosis. A diag-
nosis of FTD independent of age at onset was also
Table 2. Estimated duration (years) from symptom onset to
diagnosis for each dementia subtype and test statistics for the
differences between young- and late-onset AD and VaD
AD, Alzheimer’s disease; VaD, vascular and mixed de-
mentia; YOD, young-onset dementia; LOD, late-onset de-
mentia; S.D., standard deviation; FTD, frontotemporal
Time to diagnosis in young-onset dementia427
associated with a longer time to diagnosis. These
findings can be explained by the typical clinical
characteristics of FTD, which include behavioural and
personality changes and an early loss of insight, both
of which can delay help-seeking behaviour (Passant
et al. 2005; Rosness et al. 2008; van Vliet et al. 2011).
VaD appeared to be diagnosed earlier in comparison
with AD. This trend may be a function of the more
abrupt onset of the disease when it was due to
cerebrovascular accidents, the presence of neurologi-
cal symptoms, or its potentially more fluctuating
course (Reilly et al. 2010). Higher education levels and
female gender were hypothesized to facilitate earlier
recognition and help-seeking behaviour (Tijhuis et al.
1990; Mackenzie et al. 2006; Tedstone Doherty &
Kartalova-O’Doherty, 2010). These effects may be
masked by other factors such as the failure of physi-
cians to recognize dementia or their providing a di-
agnosis other than one of dementia (van Vliet et al.
2011). This may have resulted in a prolonged time
from symptom onset to diagnosis, while presenting to
services could have taken place early. Unfortunately,
the moment presenting to services was not assessed in
our study. Contrary to our expectations, family history
of dementia and living situation did not predict time
to diagnosis. The finding that living situation is not
related to time to diagnosis may be explained by the
small number of patients living alone in the YOD
The age at onset effect
Although the findings regarding time to diagnosis in
YOD patients are supported by the previous literature,
this period was much longer than that previously
found: 3.3 years in the YO-AD group, 4.9 years in the
YO-FTD group and 1 year in the LOD group
(Wilkinson et al. 2004; Rosness et al. 2008). This dis-
crepancy could be a result of country-related differ-
ences or differences in the way the time between
symptom onset and diagnosis was estimated. Whereas
the former study (Rosness et al. 2008) used medical
records to determine both the moment of first symp-
toms and the diagnosis, the latter (Wilkinson et al.
2004) based the data regarding both the moment of
symptom onset and the moment of diagnosis on care-
giver recollections. In the current study, the infor-
mation on the moment of symptom onset was
collected through caregivers and the moment of diag-
nosis from medical records. The most important and
inevitable drawback of all these studies is the retro-
spective estimation of moment of symptom onset,
which alone could account for heterogeneous study
Dementia is a slowly progressing condition, in
which pathological changes generally occur over ex-
tended periods before clinical presentation (Braak &
Braak, 1997). The fact that it takes time to recognize
symptoms and diagnose them is therefore an inherent
feature of many dementia aetiologies. There are sev-
eral possible explanations for the longer time between
estimated symptom onset and diagnosis in YOD. The
finding that disease severity at time of diagnosis was
comparable between groups indicates that YOD care-
givers may notice the dementia symptoms in an earlier
stage of the disease process. This may be due to the
higher demands and expectations of younger people,
for example, at work. However, when it concerns VaD,
one may expect symptoms to be picked up by YOD
and LOD caregivers in a roughly similar stage of the
disease process, because of the more obvious signs of
VaD. Still, for the YO-VaD patients in the present
Table 3. Results from the multiple linear regression analysisa
Group, YOD v. LOD
AD v. FTD
AD v. VaD
AD v. other
Gender, male v. female
Education, low v. high
alone v. not alone
S.E., Standard error; YOD, young-onset dementia; LOD, late-onset dementia; AD,
Alzheimer’s disease; FTD, frontotemporal dementia; VaD, vascular and mixed
aAdjusted R2=0.10 (p<0.001).
*p<0.05, ** p<0.001.
428D. van Vliet et al.
study the time from symptom onset to diagnosis was
substantially longer than in the LO-VaD group.
Previous studies showed equivocal results. Similar or
higher MMSE scores in YOD as compared with LOD
around the time of diagnosis have been reported
(McMurtray et al. 2006; Papageorgiou et al. 2009).
However, this difference may be explained by the
higher proportion of YO-FTD-patients in these stu-
dies. A similar study, comparing only AD patients
showed that the YOD group was more cognitively
impaired than the LOD group at time of diagnosis
(Picard et al. 2011), while another study assessing only
FTD patients showed YO-FTD patients to be less cog-
nitively impaired than LO-FTD patients (Shinagawa
et al. 2008).
An additional explanation of the predictive value of
younger age at onset may be that people are less in-
clined to consider dementia as a possible source of
symptoms in young patients as has been suggested by
other researchers (Williams et al. 2001; Harris &
Keady, 2004; Werner et al. 2009). YOD also has a vastly
differential diagnosis: it includes not only atypical
dementias and rare sporadic or hereditary diseases
(Sampson et al. 2004; Ridha & Josephs, 2006), but also
psychiatric conditions. Some studies have reported the
relatively frequent occurrence of behavioural and
personality changes as a presenting sign in patients
with YOD (Mendez, 2006; Rosness et al. 2008; van
Vliet et al. 2011). Whereas behavioural problems in
older patients seem to trigger the timely and adequate
recognition of dementia by caregivers (Eustace et al.
2007), in younger people they may be more likely to be
explained in psychosocial terms. Our previous quali-
tative report on the experiences of YOD caregivers in
the period prior to diagnosis showed that most care-
givers did not attribute symptoms to dementia and
were likely to link behavioural changes to psycho-
logical causes (van Vliet et al. 2011). In line with this
notion, FTD caregivers have been found to experience
the failure to recognize the early stages of illness as
dementia as a major troublesome aspect (Chow et al.
Our previous study also showed that initial
non-dementia diagnoses in YOD cases are relatively
common and several caregivers felt that the medical
profession was not responsive when they sought help
(van Vliet et al. 2011). Another survey on YOD found
that a higher number of diagnostic problems, such as
misdiagnosis, was related to patient and caregiver’s
younger age (Luscombe et al. 1998). Persons suffering
from FTD are more likely to receive an initial diag-
nosis indicating that they have burnout or depression
than patients with AD, probably because FTD is char-
acterized by behavioural changes in conjunction with
a relatively preserved memory capacity (Rosness et al.
2008). These factors may therefore also contribute to a
longer duration from symptom onset to diagnosis in
YOD. In our study, the effect of age of onset on time to
diagnosis was relatively small, with only 10% of the
variance explained. We acknowledge that the model
proposed does not nearly describe the interplay of all
possible factors influencing time to diagnosis. Aspects
such as denial among patients (van Vliet et al. 2011),
type of diagnostic service (Cordery et al. 2002), patient
residing in a rural versus an urban area (Koller et al.
2010), contact rate with the primary care physician
(Eefsting et al. 1996), and primary care physician atti-
tude towards the early detection of dementia are im-
portant (Hansen et al. 2008) but unfortunately have not
been assessed in the current study. Furthermore, it
could be that age does not explain a greater part of
the variance because distinct factors related to older
age can cause a diagnostic delay in this age group. For
example, patients and their relatives as well as general
practitioners may be more likely to put down memory
problems to ‘old age’ in the elderly than in younger
people, or attribute them to co-morbidities or medi-
cation use (Cahill et al. 2006; Leung et al. 2011).
Strengths and limitations
The strength of the present study is that the factors
related to time to diagnosis were investigated and
compared in a relatively large sample of both YOD
and LOD subjects, whereas previous studies pre-
sented qualitative data and/or data from smaller stu-
dies focusing on one age group. Furthermore, different
diagnostic groups were compared. Some potential
limitations, however, also exist. Since dementia com-
monly has an insidious onset, it is by definition diffi-
cult to accurately time the onset (Ratnavalli et al. 2002).
Therefore we used the year of symptom onset of
which estimation variation will average out over
groups. Memory bias could be a factor in our study,
given that the moment when symptoms first appeared
was assessed retrospectively. The recruitment strategy
for the YOD patients differed from the strategy that
was used in the MAASBED study, which may have
also biased our results. While the LOD group from
the MAASBED study was recruited both from a
University Memory Clinic (MUMC) and from the
Regional Institute for Community Mental Health Care
(RIAGG) in Maastricht, the YOD patients were re-
cruited through more diverse settings across the
Netherlands including specialized day-care centres in
115 cases. The interval between diagnosis and study
entry was larger in the YOD group; therefore recall
bias could be more pronounced in this group. By
contrast, memory problems in the LOD caregivers
associated with their advanced age could have led to
Time to diagnosis in young-onset dementia 429
recall bias in this group. Also the fact that older people
may be more likely to dismiss memory problems as
‘just ageing’ may be a confounding factor. In addition,
the dementia diagnoses were not standardized, al-
though regular criteria were used. Finally, the FTD
and ‘other’ dementia subgroups were rather small
and may not be representative of the intended popu-
lations. Especially in the LOD group these subsamples
were small, because of the inherent differences be-
tween YOD and LOD, with more heterogeneous ae-
tiologies and a higher prevalence of FTD at a younger
age. This may have resulted in an overestimation of
the effect of FTD on time to diagnosis. In addition, the
outcomes of the ‘other’ dementia subgroup are by
definition hard to compare between YOD and LOD,
because this is not a homogeneous category. The re-
sults regarding time to diagnosis of these categories
should therefore be interpreted with caution and re-
plicated in larger homogeneous samples. However, a
post hoc analysis showed that including these categ-
ories did not unduly influence our results of the
impact of age at onset and having a diagnosis of VaD
on time to diagnosis.
As yet, it is not clear which factors cause the extended
time from symptom onset to diagnosis in YOD. Future
studies should therefore assess the influence of factors
such as denial among patients, misattribution of
symptoms, type of diagnostic service and primary
care physician attitude towards the early detection of
dementia on time to diagnosis in YOD and LOD
within large homogeneous diagnostic samples. In ad-
dition, our finding that FTD, independent of age at
onset, results in a delay in diagnosis should be re-
plicated in a larger sample of YOD as well as LOD
patients. This would provide important issues to focus
on when supporting patients and caregivers in the
diagnostic trajectory as well as starting points to fa-
cilitate timelier diagnoses in both groups.
The present study, together with our previous
qualitative report, shows that the time from symptom
onset to diagnosis is relatively long in YOD, that it
considered too long by many caregivers and that this
delay may result in detrimental psychosocial conse-
quences for YOD families (van Vliet et al. 2011). In
addition, it shows that not only in YO-FTD patients
time to diagnosis is longer than in their older coun-
terparts, but also in other YOD diagnostic categories.
Hence, we advocate that efforts should be made to
reach a more timely diagnosis in YOD patients.
Therefore, our recommendations are aimed at pro-
moting public awareness of YOD as well as increasing
knowledge among the medical profession. In addition,
the development and/or enhancement of specialized
diagnostic centres for the diagnosis of YOD are im-
portant, because a structured approach based on all
clinical features is required to diagnose YOD (Rossor
et al. 2010). These centres may in turn increase aware-
ness of this subgroup in primary care (Rosness et al.
2008). However, in promoting awareness of YOD we
must be alert to the risk of false-positive diagnoses,
which may even lead to more harmful consequences.
Previous research indicated that erroneously diag-
nosing psychiatric disease as dementia is common in
YOD (Marsden & Harrison, 1972; Ron et al. 1979);
therefore we emphasize the urgent need to maintain
or, better still, improve the standards of diagnostic
accuracy of YOD.
In conclusion, the present study underlines the need
to raise special awareness of YOD, to facilitate a timely
diagnosis, but also demonstrates that more research is
necessary focusing on the mechanisms that cause an
extended time between symptom onset and diagnosis.
This study was supported by a grant from the Dutch
Alzheimer’s Society and a grant from the Florence
Care Group in the Netherlands. We would like to
thank all research assistants for collecting the data.
Declaration of Interest
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