Acta Neurol Scand 2001: 103: 367–378 Copyright # Munksgaard 2001

Printed in UK. All rights reserved

ACTA NEUROLOGICA
SCANDINAVICA
ISSN 0001-6314

Behaviour in frontotemporal dementia,

Alzheimer’s disease and vascular dementia
Bathgate D, Snowden JS, Varma A, Blackshaw A, Neary D. Behaviour
in frontotemporal dementia, Alzheimer’s disease and vascular dementia.
Acta Neurol Scand 2001: 103: 367–378. # Munksgaard 2001.
Objectives – The study aimed to increase understanding of behavioural
changes in frontotemporal dementia (FTD) and identify features that
best differentiate FTD from Alzheimer’s disease (AD) and cerebrovas-
cular dementia (CvD). Methods – A semi-structured questionnaire was
administered to carers of 30 FTD, 75 AD and 34 CvD patients. Results –
Behavioural changes that strongly discriminated FTD from AD and to a
lesser extent CvD were loss of emotions and insight, selfishness,
disinhibition, personal neglect, gluttony and sweet food preference,
wandering, motor and verbal stereotypies, loss of pain, echolalia and
mutism. Irritability, hyposexuality and hypersomnia did not discrimi-
nate. Emotional, eating and stereotyped behaviours correctly classified
95% of patients using regression analysis. Conclusions – Behavioural
characteristics accurately differentiate FTD from AD and CvD. The
findings highlight the particular importance of affective change in FTD,
and underline the role of the frontotemporal lobes in emotion.
D. Bathgate1, J. S. Snowden,
A. Varma, A. Blackshaw, D. Neary
Department of Neurology, Manchester Royal Infirmary
1
Now at Department of Neurology, Middlesborough
General Hospital
Key words: frontotemporal dementia; Alzheimer’s
disease; vascular dementia; behaviour; affect;
hyperorality; stereotypies; pain
J.S. Snowden, Cerebral Function Unit, Department of
Neurology, Manchester Royal Infirmary, Manchester
M13 9WL, UK
Accepted for publication February 16, 2001

Frontotemporal dementia (FTD) (1–4) is the most
common clinical syndrome of frontotemporal lobar
degeneration. It is associated with a non-Alzheimer
pathology (5–7), the distinct histological character-
istics of which have been outlined in the Lund-
Manchester consensus statement (8). Mutations in
the tau gene on chromosome 17 have been identified
in some familial cases (9, 10).
The salient characteristics of FTD are profound
alterations in personality and social conduct, and
behavioural alterations constitute core and suppor-
tive symptoms laid out in diagnostic criteria (8, 11).
These include decline in social interpersonal con-
duct, impaired regulation of personal conduct,
emotional blunting, loss of insight, decline in
hygiene and grooming, mental rigidity and inflex-
ibility, distractibility and impersistence, hyperorality
and dietary changes, perseverative and stereotyped
behaviour and utilization behaviour. There is
currently little published information about the
prevalence of these individual symptoms in FTD.
There is also little data regarding diagnostic
specificity. In contrast to FTD, Alzheimer’s disease
(AD) is defined principally in terms of its cognitive
characteristics: alterations in memory, visuospatial
functions and language, and it is these changes in
instrumental functions that provide the basis for
clinical diagnosis (12). A diagnosis of cerebrovas-
cular dementia (CvD) relies heavily on the presence
of vascular risk factors and on findings on
neurological examination (13, 14). The respective
emphasis on cognitive and neurological data in the
diagnosis of these two common forms of dementia
might lead to putative behavioural symptoms being
overlooked. Indeed, since CvD affects subcortical
white matter, which has strong connections to the
frontal lobes, it might be anticipated that patients
with dementia due to subcortical vascular disease
ought to share some of the behavioural character-
istics of FTD.
Although a number of valuable comparative
studies of behaviour in FTD and other dementias
(15–22) have been published, these have typically not
been designed to address the range of unusual
behaviours encountered in FTD. Behavioural fea-
tures specified in consensus criteria are currently
broad, covering a spectrum of potential forms of
behavioural alteration. The symptom of dietary

367
Bathgate et al.
change, for example, could encompass overeating as
well as food avoidance, food fads as well as
indiscriminate eating, preference for sweet and for
savoury foods. There is currently little data regard-
ing the degree of consistency and precise form of
behavioural changes within an FTD cohort.
Behavioural symptoms incorporated within cur-
rent diagnostic criteria might, moreover, not be fully
comprehensive. Clinical interview with patients’
relatives sometimes elicits an unusual symptom in
an individual patient, such as a failure to respond to
pain. Whether such anecdotal reports have diag-
nostic significance needs to be determined by
systematic enquiry.
The purpose of the study was to explore more
fully the nature of behavioural changes in classical
FTD and to determine the discriminating value of
behavioural characteristics in the differentiation of
FTD from two common forms of dementia, AD and
subcortical CvD. The findings ought to provide
information about the relative importance of
individual symptoms cited in current diagnostic
criteria as well as extend understanding of beha-
vioural changes in FTD.
Methods
Subjects
The subjects were consecutive referrals to a specialist
clinic for dementia with a clinical diagnosis of FTD,
AD or CvD. The diagnosis was based on historical
information, neurological examination and neuro-
psychological assessment and supported by findings
on structural (magnetic resonance) and functional
(SPECT) imaging. Patients in whom the clinical
diagnosis was equivocal or in whom mixed pathol-
ogy was suspected were excluded from the study.
Subsequent follow-up of subjects has reinforced the
clinical diagnoses in all cases.
Patients with FTD exhibited profound break-
down in personality and social conduct, in the
context of physical well-being. Neurological signs
were absent or limited to primitive reflexes and/or
mild akinesia and rigidity. Neuropsychological
investigation disclosed executive deficits indicative
of frontal lobe dysfunction in the absence of
visuospatial impairment and brain imaging revealed
atrophy and impaired function of the frontal and
anterior temporal regions. All patients fulfilled
criteria for prototypical FTD (11). Patients with
other clinical syndromes of frontotemporal lobar
degeneration, that is, semantic dementia and pro-
gressive aphasia (11) were not included in the study.
Patients with AD exhibited memory impairment
and deficits in perceptuo-spatial skills and/or
language, in the context of physical well-being.
368
Neurological examination was normal or disclosed
mild akinesia, rigidity or myoclonus. Structural
imaging showed atrophy and SPECT imaging
revealed characteristic posterior hemisphere changes
in the temporoparietal regions (23). The pattern of
cognitive change mirrored that of more than 80
pathologically verified cases of AD seen in this
centre. All patients fulfilled NINCDS-ADRDA
criteria for AD (12).
In patients with cerebrovascular dementia, neuro-
logical examination disclosed focal signs, suggestive
of cerebrovascular disease such as hemianopia,
lower facial weakness, dysarthria, hemisensory
deficit, hemiparesis or extensor plantar response.
All patients exhibited a subcortical pattern of
dementia (24) on neuropsychological examination
and had a Hachinski ischaemia scale score (13)
greater than 4. Patients fulfilled criteria for vascular
dementia (14).
Demographic details of patients at the time of
questionnaire administration are shown in Table 1.
Patients were relatively youthful, reflecting the
pattern of referral to the neurological department
clinic. However, FTD patients were slightly younger
than AD patients (t=2.5, P=0.01), who in turn
were younger than the CvD patients (t=3.4,
P=0.001). The groups were well matched for illness
duration at the time of assessment. Mini Mental
State Examination (MMSE) (25) scores were lower
in the AD group than the FTD (t=2.8, P=0.006)
and CvD (t=4.1, P=0.000) groups.
Informant-based behavioural questionnaire
A semi-structured questionnaire schedule was con-
structed, covering the following domains: basic and
social emotions, social behaviour, response to
sensory stimuli, eating and other oral behaviours,
wandering behaviour, sexuality, sleep pattern, repe-
titive behaviours, compulsions and rituals, environ-
mental dependency, memory and spatially-related
behaviours, delusions and hallucinations (see
Appendix).
Table 1. Demographic features
FTD AD CvD
Number 30 75 34
Males 15 36 22
Females 15 39 12
Age 59* 65* 69*
Illness duration 4.2 4.7 4.3
MMSE 21 16* 21
* Differed significantly from other groups.
MMSE=Mini Mental State Examination.
 Behaviour in frontotemporal dementia

Table 2. Affect and social behaviour

Odds ratios (with 95% confidence intervals)

Frequency of symptom (%) for pairwise group comparisons
FTD AD CvD FTD vs AD FTD vs CvD CvD vs AD

Loss of basic emotions 97 39 47 47.6 (4.4–45.0) 21.6 (2.5–187.8) 1.4 (0.7–3.9)

Exaggerated emotional display 37 49 50 0.5 (0.2–1.3) 0.2 (0.1–1.0) 1.2 (0.5–3.1)
Loss of embarrassment 90 47 53 13.5 (3.4–52.5) 16.9 (2.6–109.5) 1.3 (0.5–3.1)
Irritability 59 71 68 0.5 (0.2–1.1) 0.7 (0.2–2.2) 0.9 (0.4–2.3)
Aggression 47 43 50 1.1 (0.5–2.6) 0.7 (0.2–2.2) 1.5 (0.6–3.5)
Insightfulness 23 85 79 0.0 (0.0–0.1) 0.1 (0.0–0.3) 0.6 (0.2–1.9)
Excessive worrying 13 40 39 0.2 (0.1–0.8) 0.2 (0.1–0.9) 0.9 (0.4–2.1)
Selfishness 87 47 62 6.8 (2.1–21.9) 5.2 (1.2–22.1) 1.7 (0.7–4.0)
Disinhibition 73 27 35 8.3 (2.8–24.7) 4.1 (1.2–14.1) 1.7 (0.7–4.5)
Social avoidance 30 20 35 1.5 (0.6–4.1) 0.7 (0.2–2.2) 2.4 (0.9–6.5)
Seeks out social contact 23 23 26 1.0 (0.4–3.0) 1.0 (0.3–3.6) 1.4 (0.5–3.6)
Neglect of hygiene 87 44 50 9.0 (2.7–29.3) 5.7 (1.4–23.3) 1.3 (0.5–3.0)
Loss of interest 90 49 56 8.6 (2.3–31.2) 6.0 (1.4–26.3) 1.3 (0.5–3.1)

Procedure
The questionnaire was administered to primary
carers of patients, normally the spouse or partner.
It was emphasized that a ‘‘symptom’’ should
reflect a notable change from the patient’s
premorbid state and not a long-standing character
trait. At the beginning of the interview the
informant was asked to recall the onset of the
illness to permit demarcation of the period of
illness for which questions applied. Great care was
taken by the interviewer to ensure that each
symptom being probed was understood. The same
illustrative examples were given at each interview,
ensuring consistency of presentation and of
information given. Responses for each question
were coded as 1 or 0, representing the presence or
absence of notable change from the premorbid
state.
Interviews were conducted by a single interviewer
(D.B.), who was blind to diagnosis. (The informant
was advised at the beginning of the interview that the
patient’s clinical diagnosis should not be disclosed.)
The interviewer had no clinical contact with the
patient, either prior to or during the course of the
interview. A second blind interviewer (A.B.) was
present for 19 interviews and coded responses
independently to allow measures of inter-rater
reliability to be determined.
Analysis
Data analyses were carried out using the SPSS-PC
software package. For each individual item of the
Behavioural Questionnaire age and gender adjusted
odds ratios were calculated for pairwise group
comparisons, using logistic regression models.
Items that yielded a significant increase or decrease
in odds in the group comparisons were entered into a
stepwise logistic regression analysis to determine the
degree to which behavioural characteristics sepa-
rated FTD from non-FTD.
Results
Tables 2–8 show the frequency of behavioural
changes for the three diagnostic groups.
Frequencies refer to the percentage of the sample
in whom the behavioural abnormality was reported
to be present, an abnormality being defined as a
notable change from the patient’s premorbid state.
The tables also show odds ratios, adjusted for age
and gender, together with 95% confidence intervals
(CI) for pairwise group comparisons. A ratio of 1
indicates that a behavioural characteristic has not
changed the odds of a patient having the target
disease (the first disease in the comparison) relative
to the reference disease (the second disease in the
comparison). A ratio greater than 1 indicates an

Table 3. Sensory behaviours

Frequency of symptom Odds ratios (with 95% confidence intervals)

(%) for pairwise group comparisons
FTD AD CvD FTD vs AD FTD vs CvD CvD vs AD

Loss of awareness of pain 41 4 9 15.8 (3.9–64.3) 6.1 (1.3–28.9) 3.0 (0.5–18.4)

Loss of smell 38 17 22 3.2 (0.9–11.5) 1.8 (0.4–8.8) 1.6 (0.5–5.3)
Exaggerated sensory response 33 42 26 0.6 (0.2–1.5) 1.3 (0.4–4.6) 0.5 (0.2–1.2)
Exaggerated heat/cold response 7 20 9 0.3 (0.1–1.3) 0.9 (0.1–9.8) 0.3 (0.1–1.0)

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Bathgate et al.

Table 4. Eating and vegetative behaviours

Frequency of symptom Odds ratios (with 95% confidence intervals)

(%) for pairwise group comparisons
FTD AD CvD FTD vs AD FTD vs CvD CvD vs AD

Preference for sweet foods 50 19 26 4.6 (1.7–12.0) 2.2 (0.7–7.2) 1.3 (0.5–3.6)
Preference for savoury foods 0 3 0 – – –
Food fads 17 19 21 0.8 (0.3–13.6) 0.6 (0.1–2.6) 1.1 (0.4–3.2)
Loss of discrimination 46 18 18 4.0 (1.5–11.0) 4.5 (1.2–17.4) –
Overeats 70 23 35 7.8 (2.8–21.4) 3.6 (1.1–11.9) 1.9 (0.7–4.9)
Eats continually if food present 43 7 15 13.0 (3.7–46.1) 6.2 (1.4–26.4) 1.8 (0.5–7.3)
Steals food from others’ plates 30 1 9 29.8 (3.3–266.8) 2.8 (0.6–13.7) 11.6 (1.1–121.3)
Seeks out food 33 5 9 7.4 (2.0–27.2) 3.1 (0.6–16.2) 1.4 (0.3–7.3)
Crams food 50 1 18 80.6 (9.2–707.2) 4.9 (1.3–18.5) 12.3 (1.4–110.1)
Increased alcohol consumption 13 11 0 1.2 (0.3–4.4) – –
Increased smoking 100# 17 27 – – –
Oral exploration of objects 13 3 15 6.6 (1.1–39.4) 0.8 (0.2–3.8) 6.1 (1.1–34.7)
Wandering 67 25 38 6.7 (2.5–17.8) 3.2 (1.0–10.6) 2.1 (0.8–5.2)
Pacing 60 24 35 4.4 (1.7–11.1) 2.7 (0.8–8.6) 1.7 (0.7–4.3)
Hyposexuality 58 52 48 1.2 (0.5–3.1) 0.9 (0.2–3.0) 1.1 (0.4–2.6)
Hypersexuality 19 15 6 1.3 (0.4–4.5) 3.1 (0.4–22.3) 0.5 (0.1–2.8)
Hyposomnia 20 19 21 1.0 (0.3–2.9) 1.0 (0.2–4.0) 1.3 (0.4–3.7)
Hypersomnia 47 36 35 1.2 (0.5–3.0) 2.0 (0.6–6.5) 1.2 (0.5–3.1)
#
There were only 3 smokers in the FTD cohort so that the 100% increase in smoking in that group needs to be viewed with caution.

increase in odds for the target disease and less than
1 a decrease in odds. Ratios that differed from 1
with 95% confidence were considered significant and
are in bold.
Affect and social behaviour
A loss of the capacity to demonstrate basic emotions
was virtually ubiquitous in FTD (Table 2), whereas it
was reported in less than half of patients with other
dementias. Loss of emotion greatly increased the
odds of FTD relative to both AD and CvD. A
subsequent analysis of individual emotional states
(not shown in the table) indicated that the loss of
affect in FTD was not confined to a single emotion.
Loss of specific emotions significantly increased the
odds of FTD compared to AD: loss of happiness
(odds ratio=5.3, CI=2.0–14.3), sadness (odds
ratio=17.6, CI=5.5–56.7), fear (odds ratio=12.4,
CI=4.4–35.2), anger (odds ratio=14.1, CI=
4.4–45.0), surprise (odds ratio=7.2, CI=2.8–19.0)
and disgust (odds ratio=13.3, CI=4.6–39.0).
Comparisons between FTD and CvD also revealed
a greater likelihood of loss of specific emotions in
FTD: for happiness (odds ratio=3.8, CI=1.1– 13.7),
sadness (odds ratio=30.0, CI=4.6–159.0), fear
(odds ratio=22.1, CI=4.0–122.6), anger (odds
ratio=15.7, CI=3.1–79.0), surprise (odds ratio=
7.3, CI=2.0–26.4) and disgust (odds ratio=9.0,
CI=2.4–33.8). Comparisons between AD and CvD
revealed no influence of emotion on likelihood of
diagnosis.
Affective change in FTD patients included
changes in social emotions. Loss of feelings of
embarrassment was almost universal in FTD, but
not in other groups. FTD patients were more likely
than other patients to be selfish, showing a lack of
sympathy with and empathy for others. They were
also more likely than other patients to exhibit
disinhibited behaviours, to show a lack of concern
for personal hygiene and a general loss of interest in
activities. The presence of these behavioural features
significantly increased the likelihood of a diagnosis
of FTD compared to both AD and CvD. There were
no differences between the groups in the tendency
either to avoid or seek out social contact.
Insightfulness, determined by the patients’
demonstration of anxiety, concern or loss of
confidence, when confronted by tasks that they
can no longer accomplish, was present less often in
FTD than in other groups. The presence of
insightfulness greatly decreased the odds of a patient
having FTD compared to both AD and CvD.
Similarly, the tendency to worry excessively
decreased the likelihood of FTD compared to AD
and CvD.
Irritability and aggression were present in a
relatively high proportion of patients with FTD.
However, they were present also in other groups,
and group comparisons revealed no significant
effects. Affective and social behavioural symptoms
did not significantly influence the odds of CVD
compared to AD.
Sensory behaviours
A lack of appropriate response to painful stimuli
was reported in approaching half of the FTD group

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 Behaviour in frontotemporal dementia

Table 5. Repetitive behaviours, compulsions and rituals

Frequency of symptom Odds ratios (with 95% confidence intervals)

(%) for pairwise group comparisons
FTD AD CvD FTD vs AD FTD vs CvD CvD vs AD

Simple motor stereotypies 60 39 26 2.8 (1.1–6.9) 3.1 (1.0–9.8) 0.6 (0.2–1.4)

Complex motor routines 27 5 6 4.8 (1.2–18.7) 2.6 (0.4–15.7) 1.9 (0.3–12.4)
Paces fixed route 29 3 3 15.9 (3.1–80.0) 10.8 (1.2–96.8) 1.5 (0.1–18.5)
Verbal stereotypies 50 16 21 5.6 (2.1–15.5) 4.0 (1.1–14.1) 1.2 (0.4–3.5)
Verbal perseverations 17 3 5 2.5 (0.6–11.0) 6.9 (0.4–115.4) 0.4 (0.0–4.8)
Repetitive themes 30 24 32 1.3 (0.5–3.4) 0.6 (0.2–1.9) 1.4 (0.6–3.6)
Counts objects 13 4 15 2.8 (0.5–14.5) 0.5 (0.1–2.7) 5.5 (1.2–25.7)
Aligns objects 37 24 21 2.1 (0.8–5.4) 2.9 (0.8–10.7) 0.6 (0.2–1.8)
Adherence to daily routine 13 15 6 0.9 (0.2–3.2) 3.5 (0.4–28.9) 0.3 (0.1–1.4)
Arranges belongings in same way 7 12 3 0.6 (0.1–3.1) 2.4 (0.1–56.1) 0.2 (0.0–1.4)
Overconcern with cleanliness 3 8 0 0.4 (0.0–3.8) – –
Clockwatching 27 13 21 2.2 (0.7–6.7) 1.2 (0.3–4.7) 1.5 (0.5–4.4)
Excessive checking 13 19 24 0.6 (0.2–2.2) 0.4 (0.1–2.1) 1.2 (0.4–3.3)
Excessive attention to detail 17 9 15 1.8 (0.5–6.3) 1.3 (0.3–6.0) 1.8 (0.5–6.8)
Upset if routine disrupted 27 15 24 2.1 (0.7–6.0) 1.2 (0.3–4.5) 1.5 (0.6–4.8)
Needs to do things immediately 40 25 38 1.9 (0.8–4.9) 1.1 (0.4–3.6) 2.0 (0.8–4.9)
Unusual toileting routines 30 8 3 5.2 (1.5–17.8) 9.8 (1.1–89.4) 0.3 (0.0–2.4)
Superstitious rituals 20 1 3 14.7 (1.6–139.1) 5.6 (0.5–58.9) 5.9 (0.3–123.4)

but was rare in other groups (Table 3). Loss of pain
response significantly increased the odds of FTD
compared to both AD and CvD. A loss of sense of
smell was numerically more frequent in FTD. An
exaggerated or inappropriate response to sensory
stimuli was reported in a proportion of patients in all
groups. A change in response to heat and cold was
reported numerically most often in AD. However,
none of these behavioural features altered the odds
of diagnosis to a significant degree.
Eating and oral behaviours
Alterations in eating behaviour were reported more
frequently in FTD than in other dementias
(Table 4). An altered preference for sweet foods,
and a range of gluttonous behaviours including
indiscriminate eating, overeating, eating continually
while food remains present, stealing food from
others’ plates, seeking out food and cramming food
increasd the odds of FTD compared to AD and to a
slightly lesser extent CvD.
Only 3 (10%) FTD patients, 11 (32%) CVD
patients and 18 (24%) of AD patients smoked
cigarettes prior to the onset of illness. Odds ratios
were not calculated for change in smoking habits,
because this behavioural feature could potentially be
documented in so few cases. The figure of 100%
increase in smoking in FTD is based on 3 patients
only so should be viewed with caution. Nevertheless,
the figure is consistent with the view that hyperoral
behaviour in FTD may extend beyond the realm of
food consumption. Oral exploration of inanimate
objects was relatively rare although it increased
diagnostic odds for both FTD and CvD compared
to AD.
Other vegetative behaviours
Wandering and pacing were reported more fre-
quently in FTD than in other groups (Table 4).
Wandering significantly increased the diagnostic
odds of FTD compared to AD and CvD. Pacing
increased the odds of FTD compared to AD. Loss of
interest in sexual activity and increased somnolence

Table 6. Environmental dependency

Frequency of symptom Odds ratios (with 95% confidence intervals)

(%) for pairwise group comparisons
FTD AD CvD FTD vs AD FTD vs CvD CvD vs AD

Hoarding 57 41 24 1.6 (0.7–4.0) 4.0 (1.2–13.5) 0.6 (0.2–1.4)

Touches or handles objects 40 23 26 2.1 (0.8–5.5) 1.5 (0.5–5.0) 1.2 (0.5–3.4)
Echolalia 33 4 12 14.6 (3.4–63.0) 5.0 (1.1–22.0) 2.6 (0.5–13.6)
Echopraxia 3 1 6 2.6 (0.1–45.7) 0.6 (0.0–8.2) 6.5 (0.5–78.3)
Utilization phenomena 0 0 0 – – –
Reads aloud notices 33 21 18 1.7 (0.6–4.5) 1.6 (0.4–6.3) 0.9 (0.3–2.6)

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Bathgate et al.

Table 7. Cognitively-mediated behaviours

Frequency of symptom Odds ratios (with 95% confidence intervals)

(%) for pairwise group comparisons
FTD AD CvD FTD vs AD FTD vs CvD CvD vs AD

Mislays objects 53 89 76 0.2 (0.1–0.5) 0.4 (0.1–1.4) 0.5 (0.1–1.4)

Lost in familiar surroundings 23 41 45 0.5 (0.2–1.5) 0.4 (0.1–1.6) 1.4 (0.6–3.4)
Disoriented in own home 7 31 24 0.2 (0.0–0.8) 0.2 (0.0–1.4) 0.8 (0.3–2.0)
Fails to recognize objects 17 19 12 0.8 (0.3–2.7) 1.8 (0.4–8.8) 0.6 (0.2–2.1)
Difficulty locating objects 7 56 15 0.0 (0.0–0.2) 0.4 (0.1–2.7) 0.2 (0.1–0.5)
Uses wrong words 23 45 35 0.4 (0.1–0.9) 0.3 (0.1–1.2) 0.7 (0.3–1.8)
Mutism 23 5 3 6.0 (1.5–23.4) 33.0 (2.1–523.3) 0.6 (0.1–6.5)

were common features of FTD, but they occurred
too in other dementias. Alterations in sexual and
sleep behaviours did not significantly alter diagnos-
tic odds in any group comparison.
Repetitive behaviours, compulsions and rituals
Repetitive behaviours were common in the FTD
group (Table 5). Simple motor stereotypies, com-
prising vocalizations such as grunting or humming,
facial movements such as lip smacking or limb
movements such as hand rubbing or foot tapping,
were reported in almost two thirds of the sample,
and significantly increased the odds of FTD
compared to AD and CvD. The odds of FTD
compared to AD were also increased by the presence
of complex repetitive motor routines. Repetitive
routines in FTD included the tendency to sing the
same song or recite the same rhyme over and over
again, to clap the same rhythm repetitively or dance
the same dance. The most commonly reported
individual behaviour, present in 25% of FTD
patients, was rhythm clapping. Other repetitive
behaviours that significantly increased the odds for
FTD compared to AD and (in most cases) CvD were
pacing a fixed route, producing the same word or
phrase repetitively (verbal stereotypy), engaging in
unusual toileting routines and the presence of
superstitious rituals, such as avoiding standing on
cracks between paving stones. Frankly obsessive
features, such as repeated checking and excessive
attention to detail, were rare and did not signifi-
cantly alter diagnostic odds. Clockwatching
occurred in a quarter of FTD patients and a
tendency to align objects in one third. Although
numerically more frequent than in other groups the
increase in odds for FTD was non-significant. A
tendency to count objects significantly increased the
odds for CvD compared to AD. No other repetitive,
compulsive or ritualistic behaviours altered diag-
nostic odds for CvD compared to AD.
Environmental dependency
Hoarding was a common feature in FTD (Table 6),
but it had diagnostic value only in comparison with
CvD, since hoarding was a relatively common
feature also of AD. Echolalia, present in one third
of FTD patients, significantly increased the odds of
FTD compared to AD and to a lesser extent CvD.
One third of FTD patients showed a tendency to
read public notices aloud, although the increased
odds for FTD compared to AD and CvD was not
significant. Frank utilization behaviour was not
reported in any patient.
Cognitively-mediated behaviours
Behaviours relating to memory and spatial dysfunc-
tion were numerically less common in FTD than in
other dementias, particularly AD (Table 7). The
tendency to mislay objects, to be disoriented within
the home and to have difficulty locating objects
placed in front of them significantly decreased the

Table 8. Hallucinations and delusions

Frequency of symptom Odds ratios (with 95% confidence intervals)

(%) for pairwise group comparisons
FTD AD CvD FTD vs AD FTD vs CvD CvD vs AD

Suspiciousness 30 44 50 0.6 (0.2–1.5) 0.4 (0.1–1.2) 1.2 (0.5–2.8)

Delusions of theft 10 25 35 0.4 (0.1–1.4) 0.2 (0.1–1.0) 1.3 (0.5–3.4)
False belief that people in home 10 20 21 0.6 (0.1–2.3) 0.5 (0.1–2.8) 1.1 (0.4–3.1)
Misidentification phenomena 30 41 29 0.6 (0.2–1.5) 0.9 (0.3–3.2) 0.7 (0.3–1.9)
Visual hallucinations/illusions 7 23 15 0.3 (0.1–1.2) 0.5 (0.1–3.1) 0.6 (0.2–1.8)
Auditory hallucinations/illusions 10 8 12 1.0 (0.2–4.7) 0.9 (0.1–5.9) 2.0 (0.5–8.4)

372

odds of FTD compared to AD. A difficulty in the
spatial location of objects also decreased the odds of
CvD compared to AD. Use of wrong words,
reported more commonly in AD than FTD,
decreased the odds of FTD compared to AD. By
contrast, mutism, reported most commonly in FTD,
increased the odds of FTD compared to both AD
and CvD.
Hallucinations and delusions
Suspiciousness, delusions of theft, the false belief of
the presence of others in the home, misidentification
phenomena and visual hallucinations and illusions
were numerically less common in FTD patients than
in other groups (Table 8). However, these relatively
small differences did not significantly alter diagnos-
tic odds.
Inter-rater reliability
There was high agreement between the two raters in
determining the presence or absence of symptoms
(mean Kappa statistic 0.97).
Effect of age and gender on symptoms
Examination of patterns of behavioural change in
younger compared to older patients within a group
(<mean vs >mean) and in men compared to
women did not reveal notable differences in pattern
of behavioural change as a function of age or gender.
Logistic regression analysis: FTD v non-FTD
Items that had yielded a significant increase or
decrease in odds ratio in the pairwise group
comparisons were entered into a stepwise logistic
regression analysis. Where variables were obviously
inter-correlated (e.g. loss of emotion, loss of capacity
for sadness) only the variable whose odds ratio
showed the largest difference from 1 was included, to
reduce the degree of multicollinearity. The criterion
for entry of variables was set at 0.05 and for removal
of variables set at 0.1. Five variables entered the
equation in the following order: insightfulness,
pacing a fixed route, loss of emotion, food cramming
and difficulty locating objects. The numerical size of
the parameter estimate at step 1 was x2.9, standard
error 0.5, and at step 5 estimates ranged from 4.7 to
x4.7, standard error 1.2–1.9. Insightfulness and a
difficulty locating objects contributed negatively to a
diagnosis of FTD, whereas loss of emotion, food
cramming, pacing a fixed route contributed posi-
tively. Using a classification cut-off of 0.5, these five
variables resulted in an overall accuracy of classifi-
cation of 95%. FTD patients were correctly classified
with an accuracy of 90% and AD and CvD patients
Behaviour in frontotemporal dementia
were correctly classified as non-FTD with an
accuracy of 96%. The single variable of insightful-
ness alone effectively classified 79% of FTD patients
and 83% of non-FTD. High levels of classification
accuracy could be achieved using other variable
combinations, the common feature being that they
included variables relating to a) emotion, b) altered
eating habits and c) repetitive behaviours.
Discussion
In FTD behavioural change is the overriding clinical
symptom. A number of behavioural symptoms show
very high sensitivity to FTD, being present in at least
50% of cases. These include loss of basic emotions
(e.g. fear) and social emotions (e.g. embarrassment),
together with selfishness, disinhibition, irritability,
neglect of personal hygiene, loss of interest/apathy,
gluttony, altered preference for sweet foods, wan-
dering, pacing, hyposexuality, motor and verbal
stereotypies, and hoarding. The most sensitive
feature, being present in virtually all patients, is
the loss of basic emotions, representing the capacity
to demonstrate happiness, sadness, fear, anger,
surprise and disgust. Also present in virtually all
patients is a failure to show embarrassment and a
general loss of interest in activities. Selfishness and
neglect of personal hygiene are reported in 87% of
patients.
Behaviours that are sensitive to FTD, as judged by
their prevalence of at least 50%, also typically show
significant diagnostic specificity. Those common
behaviours all increase the diagnostic odds of
FTD compared to AD and CvD, with the exception
of irritability and hyposexuality which occur with
relatively equal frequency in all three groups.
There are a number of additional behaviours that,
although present in only a proportion of FTD
patients, have diagnostic value. Loss of awareness of
pain was reported in 41% of FTD patients, yet only
rarely in other groups. In most cases, this loss had
come to light as a result of dramatic incidents:
patients had for example, stepped into a scalding
bath and shown no withdrawal reaction, leading to
burns requiring medical attention. A number of
eating-related behavioural changes increase the odds
of FTD in addition to the general features of
overeating and sweet food preference. These
included a tendency to continue eating for as long
as food is present, to steal food from others’ plates
and to cram food into the mouth. These features
may to some extent reflect environmental depen-
dency: stimulus-driven behaviour that cannot be
overridden by virtue of the impairment in goal-dir-
ected, purposive behaviour engendered by frontal
lobe dysfunction. However, the tendency in a third
373
Bathgate et al.
of FTD patients to seek out food, even when it is not
visibly present, suggests that environmental depen-
dency cannot be a sufficient explanation, and
suggests that more fundamental change to satiety
mechanisms may also be present. The altered
preference for sweet foods present in 50% of patients
supports the notion of fundamental dietary changes,
independent of environmental dependency. The
importance of dietary changes in FTD has been
highlighted by others (26).
Verbal stereotypies, comprising repetitive use of
the same word or phrase, and motor stereotypies,
such as grunting, arm rubbing or foot tapping are
common in FTD. Repetitive, stereotyped motor
behaviours may constitute complex routines as well
as simple motor mannerisms. These take a variety of
forms. For example, one patient, on entering a room
would tap out the same rhythm on each of the four
walls before being seated. Another sang Ave Maria
repetitively. Another counted every step made with
his left foot and multiplied this by two every 100
steps to announce how far he had walked. Another
wrote a continuous commentary of all conversations
and activities on any available scraps of paper.
Another would empty her wardrobe of clothes daily,
carry the clothes to another part of the room, where
she would lay them out on the floor, following which
she would return them into their correct place in the
wardrobe. The most commonly reported individual
behaviour was rhythm clapping, followed by tune
singing or humming. Although less common than
simple motor stereotypies these complex routines are
important diagnostically because of their rarity in
other dementias. Similarly, although wandering and
pacing occur in other dementias, it is specifically in
FTD that patients may pace a fixed rather than
random route, giving the pacing a more repetitive,
stereotyped quality than occurs in AD or CvD.
Stereotyped behaviours in FTD may have a super-
stitious, ritualistic quality, such as refusal to walk on
the cracks between paving stones. One man, for
example, always stepped on to flagstones with his
left foot.
A number of previous studies have highlighted the
association between repetitive and compulsive
behaviours and frontotemporal dementia (22,
27–29), providing support for the role of frontal–
striatal circuitry in compulsive behaviours (30). The
extensive range of behaviours that fall within this
rubric has been well highlighted (27). Although some
patients with FTD have been regarded as fulfilling
criteria for obsessive–compulsive disorder (OCD)
(27–29) the majority of patients in this study would
not do so. Compulsive acts do not appear to be
linked to intrusive thought processes as in OCD and
in most patients there is no indication of induced
374
stress or anxiety if routines are disrupted. Moreover,
despite the presence of verbal and motor stereotypies
and repetitive routines, patients typically show a
notable absence of concern for personal hygiene,
and do not show the repetitive checking behaviours
and excessive attention to detail in carrying out tasks
that is characteristic of OCD. It seems reasonable to
assume that the repetitive behaviours in these
patients arise as a consequence of the loss of the
supervisory role of the frontal lobes to implement
effective goal-directed, purposive and novel beha-
viours and to inhibit existing programs of behaviour.
Whether there are distinct mechanisms underlying
complex and simple forms of repetitive behaviour is
worthy of further exploration. It has been suggested
(27) that complex repetitive routines may result from
aberrant motor plans, and more elementary beha-
viours to disinhibited motor programs.
Subjects were consecutive referrals to a neuro-
logical clinic, the rationale being to include as rep-
resentative a sample as possible of each diagnostic
group. The groups were not therefore precisely
matched for gender and age. However, odds ratios
were age and gender adjusted, so that putative
effects of age and gender are controlled. Moreover,
examination of behavioural profiles across groups as
a function of age and gender suggests that they
contribute little to behavioural differences.
Traditionally, comparative studies of dementia
attempt to match for overall dementia severity.
However, measures of overall severity are necessa-
rily artificial, since patients with different dementing
illnesses break down along differing rather than
common dimensions of impairment. Nevertheless,
there is good evidence that the group differences in
this study cannot be accounted for by notional
differences in overall dementia severity. Patients
were well matched with respect to duration of
symptoms. Since the disorders have relatively
comparable time courses this suggests that patients
were at an equivalent stage of illness. Moreover, the
presence of dissociations in the findings rules out a
general explanation in terms of severity. Whereas
many behavioural symptoms were more common in
FTD, cognitively-based behaviours relating to
memory and spatial dysfunction were more
common in AD. They were more likely to mislay
objects, to be disoriented in the home and to have
difficulty in the spatial location of objects in the
immediate environment. In the realm of language,
echolalia and mutism were more common in FTD
and the use of wrong words (verbal and phonemic
paraphasias) more common in AD. Such dissocia-
tions suggest that differences are genuinely disease-
related and not artefacts of overall severity. The
dissociation between aphasic symptoms and mutism

reinforces the view that mutism in FTD reflects the
patients’ adynamia and loss of generational cap-
abilities secondary to frontal lobe dysfunction rather
than a primary aphasia.
It is generally accepted that psychotic symptoms
are relatively rare in FTD, and some studies have
demonstrated a lower incidence compared to AD
(16, 17, 19). In the present study psychiatric
symptoms of hallucinations and delusions were
numerically less common in FTD than in other
groups although, because of the relatively low
incidence across all groups, they did not alter
significantly diagnostic odds.
The largest differences in symptom frequency
occurred, as expected, between FTD and AD. It had
been anticipated that CvD might share some
behavioural similarities with FTD on the basis of
disruption to subcortical-frontal circuitry. Never-
theless, an important finding of the study is that
behavioural features that strongly differentiate FTD
from AD also typically distinguish FTD from CvD,
albeit with reduced magnitude. By contrast, with few
exceptions, those behaviours do not distinguish AD
from CvD. Such findings indicate the importance of
those behavioural symptoms both in the character-
ization of FTD and its differentiation from other
dementias.
The features that emerged from the logistic
regression analysis that best discriminate FTD
from other dementias were loss of basic emotions,
food cramming, pacing a fixed route, an absence of
difficulty in locating objects and an absence of
insightfulness. The symptom of insightfulness, as
operationally defined in this study, referred not to
the capacity to report symptoms of illness explicitly
but rather to the implied awareness of difficulty,
indicated by patients’ demonstration of distress,
anxiety, loss of confidence or withdrawal when
confronted by tasks that they are unable to perform.
It was found in a previous study (15) that
insightfulness determined in this way strongly
discriminated between FTD and AD, whereas
conventional measures of insight, requiring explicit
report of symptoms, did not. The data from this
study support the earlier data in showing that
insightfulness as defined by patients’ emotional
response when confronted by difficulties signifi-
cantly differentiated FTD from other dementias:
AD and CvD patients showed a degree of insightful-
ness whereas FTD patients did not.
Previous comparative studies of neuropsychiatric
symptoms in FTD such as irritability, aggression,
depression, and anxiety, have not yielded entirely
uniform results. Some studies found significant
differences in the incidence of irritability between
FTD and AD patients (17, 19) whereas other studies
Behaviour in frontotemporal dementia
(16, 31) in keeping with our own findings, have not.
Some studies have reported verbal outbursts to be
more common in FTD (22) whereas the present
study suggests that aggressive behaviour is no more
common in FTD than in AD or CvD. Assessment
methodology may play a part in accounting for some
disparities. Depression, for example, has been
reported to be more common in FTD than AD
(17). However, it has been pointed out (32) that the
rating scale employed in that study incorporates
apathy as a component of depression, thereby falsely
elevating depression scores in FTD patients, in
whom apathy and loss of volition are prominent
features. It is unlikely that methodological factors
are a sufficient explanation for lack of consensus. It
would appear from the present study that con-
ventional neuropsychiatric symptoms, such as irrit-
ability, while they may have a place in the
characterization of FTD, are less reliable indicators
of diagnosis than are other more unusual beha-
viours, which are uniquely associated with FTD.
In this study, the initial selection of patients was
based on a combination of historical, neurological,
neuropsychological and neuroimaging data, to
avoid the potential for circularity inherent in a
purely behavioural diagnosis. Moreover, the beha-
vioural features explored in this study extend beyond
those outlined in current criteria for FTD, and are
not specified in AD and CvD criteria. The
demonstration of highly significant behavioural
differences, derived from ‘‘blind’’ interview, rein-
forces the view that behavioural symptoms are
critical to the diagnosis of FTD and may be more
reliable than cognitive test scores in differentiating
FTD from other dementias (21, 33).
The features that best discriminate FTD from
other dementias fall into three principle domains: i)
affect, ii) oral behaviours and iii) repetitive, stereo-
typed behaviours. Reduction in the capacity to
demonstrate primary emotions, in particular, was a
virtually ubiquitous feature of FTD, even in the
early stages of the disorder. An additional salient
feature identified by the present study was a loss of
appropriate response to painful stimuli. In FTD,
pathological change is primarily cortical in early
disease, although limbic structures such as the
hippocampus and amygdala are affected later in
the course (4). The precise mechanisms underlying
patients’ unusual behaviours and the particular role
of the frontal and temporal lobes and of limbic
structures in contributing to behavioural changes
remains to be explored.
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Appendix: Manchester Behavioural Questionnaire
The appendix lists behavioural symptoms and
provides a broad definition. For the sake of economy,
it does not specify the precise wording adopted in the
interview. Although the semi-structured nature of the
test instrument allowed for re-phrasing and provision
of examples, to ensure that behavioural symptoms
were appropriately understood, wording of questions
was standardized as far as possible to optimize
consistency of administration.
Affect and social behaviour behaviour
Loss of basic Loss of the capacity to show a)
emotions happiness, b) sadness, c) fear, d)
anger, e) surprise and f) disgust
Exaggerated Inappropriate or exaggerated
emotional displays of a) happiness, b)
display sadness, c) fear, d) anger, e)
surprise and f) disgust
 Behaviour in frontotemporal dementia

Loss of Fails be appropriately embar- Crams food Crams and stores food in mouth
embarrassment rassed for self or others without swallowing
Irritability More irritable than formerly Increased Drinks more alcohol than for-
Aggression Verbally or physically aggres- alcohol merly
sive or threatening consumption
Insightfulness Reacts to difficulties by becom- Increased Smokes a) more, b) repetitively.
ing upset, distressed, anxious, smoking c) Seeks out cigarettes
losing confidence or withdrawn Oral Puts inanimate objects in mouth
Excessive Worries excessively about little exploration
worrying things of objects
Selfishness More self-centred, shows lack of Wandering Wanders from home or needs
consideration for others supervision to stop wandering
Disinhibition Laughs inappropriately, makes Pacing Paces home
jokes in bad taste and inap- Hyposexuality Diminished interest in sexual
propriate comments. Acts in activity
disinhibited manner in public Hypersexuality Increased interest in sexual
Social avoidance Avoids social contact with activity, sexual preoccupations
people Hyposomnia Sleeps less than formerly
Seeks out Seeks other people, follows Hypersomnia Sleeps more than formerly
social contact people around
Neglect of Less concerned about cleanli-
hygiene ness. Needs to be prompted to Repetitive behaviours, compulsions and rituals
wash and change clothes Simple motor a) vocalizations (e.g. grunting,
Loss of interest Shows a general loss of interest stereotypies humming)
in hobbies and leisure activities b) facial movements (e.g. lip
smacking, sniffing)
c) upper limb (e.g. hand rub-
Sensory behaviours bing, scratching, picking)
Loss of Shows no awareness of painful d) lower limb (e.g. foot tapping)
awareness of sensations such as heat, sharp Complex a) oral (e.g. reciting same nur-
pain objects, knocks, falls motor routines sery rhyme, singing same song)
Loss of smell Sense of smell lessened b) upper limb (e.g. clapping
Exaggerated Inappropriate or exaggerated same rhythm)
sensory response response to pain, touch, noise, c) dancing complex dance rou-
odours and tastes tine
Exaggerated Inappropriate or exaggerated Paces fixed route Stereotyped pacing, rhythmical,
heat/cold response to heat and cold same direction
response Verbal Has favoured word or phrase
stereotypies that keeps repeating
Verbal Repeats words or phrases imme-
Eating and vegetative behaviours perseverations diately after uttering them
Preference Altered food preference in Repetitive themes Continually repeats or sticks to
for sweet foods favour of sweet foods the same theme in conversation
Preference for Altered food preference in Counts objects Constantly counts things
savoury foods favour of savoury foods Aligns objects Needs constantly to order/align
Food fads More selective and picky than things
formerly Adherence to Sticks to a strict timetable for
Loss of Less selective about what eats. daily routine ordinary daily activities
discrimination Eats indiscriminately Arranges Always arranges belongings in a
Overeats Eats more than formerly belongings in certain way
Eats continually Would carry on eating indefi- same way
if food present nitely if food present Overconcern More/overly concerned about
Steals food Actively takes food from the with cleanliness being neat and clean
from others’ plates of others Clockwatching Constantly watches and worries
plates about the time
Seeks out food Actively looks for extra food, Excessive Preoccupied with checking
e.g. raids the biscuit tin checking things such as lightswitches

377
Bathgate et al.

Excessive Unnecessary attention to detail Lost in Difficulty finding way in own

attention to when carrying out tasks familiar neighbourhood
detail surroundings
Upset if Upset if timetable is interfered Disoriented Difficulty finding way within the
routine disrupted with and activities prevented in home home
Needs to do If has an idea to do something, Fails to Sees objects but does not know
things has to do it immediately recognize objects what they are
immediately Difficulty Slow to find objects placed in
Unusual Eccentric toileting and groom- locating objects front of them
toileting routines ing habits Uses wrong Substitutes inappropriate word
Superstitious Behavioural rituals or supersti- words for correct one
rituals tions, such as avoiding standing Mutism Has stopped talking altogether
on cracks between paving stones

Environmental dependency
Hoarding Collects up, hides or hoards
Hallucinations and delusions
objects
Touches or Touches, picks up and examines Suspiciousness Has become more suspicious/
handles objects objects, puts in pocket less trusting
Echolalia Copies words or sentences Delusions of Has believed possessions to be
uttered by others theft stolen
Echopraxia Imitates actions carried out by False belief Believes other people to be
others that people living in the home
Utilization Uses or mimics use of objects in in home
phenomena environment Misidentification Assumed familiar people to be
Reads Reads aloud notices in street or phenomena someone else
notices aloud public buildings Visual Sees visions, such as people or
hallucinations/ animals
illusions
Cognitively-mediated behaviours Auditory Hears noises or voices not
Mislays objects Puts objects down and cannot hallucinations/ experienced by others
find them illusions

378
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