Review Article Functional Status in Behavioral Variant ...downloads.hindawi.com/journals/bmri/2013/837120.pdfReview Article Functional Status in Behavioral Variant Frontotemporal Dementia:

Hindawi Publishing CorporationBioMed Research InternationalVolume 2013, Article ID 837120, 7 pageshttp://dx.doi.org/10.1155/2013/837120

Review ArticleFunctional Status in Behavioral VariantFrontotemporal Dementia: A Systematic Review

Thais Bento Lima-Silva,1 Valéria Santoro Bahia,1

Ricardo Nitrini,1 and Mônica Sanches Yassuda1,2

1 Neurology Department, University of Sao Paulo School of Medicine, Brazil2 Gerontology Department, School of Arts, Sciences and Humanities of the University of Sao Paulo,Avenida Dr. Eneas de Carvalho Aguiar 255, 05403-900 Sao Paulo, SP, Brazil

Correspondence should be addressed to Monica Sanches Yassuda; [email protected]

Received 14 April 2013; Accepted 20 September 2013

Academic Editor: Cristiano Capurso

Copyright © 2013 Thais Bento Lima-Silva et al. This is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properlycited.

The aim was to conduct a systematic review of studies that described the functional profile of patients with behavioral variantfrontotemporal dementia (bvFTD), published between 2000 and 2013. The bibliographic search was conducted using the terms“frontotemporal dementia” and “frontotemporal lobar degeneration” in combination with “independence,” “functionality,” “basicactivities of daily living,” “disability,” and scales that measure functional performance: “Disability Assessment for Dementia-DAD,”“Functional Activities Questionnaire (FAQ),” “Direct Assessment of Functional Status (DAFS).” To be included in the review, thestudy had to mention the characterization of the functional status of patients with bvFTD in the objectives of the study, using apreviously validated instrument of functional assessment. Fourteen studies met this criterion. The reviewed studies suggested thatindividuals with bvFTD have greater functional impairment when compared to those with other subtypes of frontotemporal lobardegeneration or Alzheimer’s disease.The studies documented a significant association between cognitive impairment andmeasuresof functionality in these patients. The cognitive profile of patients may predict faster functional decline.

1. Introduction

bvFTD is a clinical syndrome characterized by progressiveimpairment in behavior and personality as well as social,cognitive, and functional abilities, which predominantlyaffects middle-aged adults [1, 2]. These changes stem fromfrontotemporal lobar degeneration (FTLD) secondary to aspectrum of heterogeneous pathologies [3, 4]. Despite recentadvances in characterizing bvFTD, diagnosing this syndromeremains challenging. Patients may be erroneously consideredto be cognitively unimpaired, while others are diagnosedwithpsychiatric disorders or Alzheimer’s disease (AD) [5].

In 2011, a set of revised diagnostic criteria was proposedfor the bvFTD based on recent publications and a consortiumof international research groups [6]. The revised criteriawere validated through the retrospective review of clinicalrecords and the comparison of the accuracy of original and

revised criteria in a sample of patients from 16 brain bankswith autopsy-confirmed FTLD. With the revised criteria,a diagnosis of “possible” bvFTD requires three of the sixclinically discriminated characteristics (loss of inhibition,apathy/inertia, loss of empathy, perseveration/compulsivebehaviors, hyperorality, and dysexecutive neuropsychologicalprofile). “Probable” bvFTD requires the additional featuresof functional disability and characteristic neuroimaging,whereas bvFTD “with definitive frontotemporal lobar degen-eration” requires histopathological conformation or confir-mation of pathogenicmutation.Thenew criteria are expectedto contribute by allowing earlier diagnosis of the syndromeand differential diagnosis with psychiatric disorders.

There is a consensus among researchers of bvFTD that, inthe absence of specific biomarkers, investigating functionalstatus is essential for the diagnosis and treatment of the syn-drome, given that the impact on the activities of daily living

2 BioMed Research International

can be used as a diagnostic parameter [6]. Yet, studies inves-tigating the functional performance of patients with FTLD(bvFTD and other subtypes such as nonfluent progressiveaphasia-NFPA and semantic dementia-SD) are scarce [2].

New studies characterizing the functional profile ofpatients with bvFTD are warranted as they can contribute tothe diagnostic process and rehabilitation of patients. More-over, functional dependence in bvFTD creates burden oncaregivers and family members and generates additionalsocial costs. Functional rehabilitation and compensationmayalleviate some of these problems. Therefore, the aim of thepresent investigation was to carry out a systematic review ofstudies describing the functional status of patients diagnosedwith bvFTD, published between 2000 and 2013.

2. Methods

This review is based on a literature search conducted inSeptember 2013. The bibliographic search was carried outin Medline, PsycInfo, PubMed, LILAC’s, SciELO, and Sci-enceDirect databases using the search term “frontotem-poral dementia” and “frontotemporal lobar degeneration”in combination with “functionality”, “independence”, “basicactivities of daily living”, “disability”, and names of scalesthat measure functional performance: “Disability Assess-ment for Dementia-DAD”, “Functional Activities Question-naire (FAQ)”, and “Direct Assessment of Functional Status(DAFS)”, for retrieval of studies published between 2000 and2013.

A total of 119 studies were retrieved, including articles,theses, dissertations, and book chapters. For inclusion in thereview, studies had to cite in the objectives the characteriza-tion of the functional status of patients with bvFTD, usinga previously validated functional assessment instrument. Atotal of 14 clinical studies met this criterion.

3. Results

This review identified a limited number of studies whichaddressed the issue of functionality in patients with variantsof FTLD using validated instruments. Among these studies, asmall number included in their sample a significant numberof patients with bvFTD. We found two research studies thataddressed the correlation between functional performanceand executive dysfunction in patients with bvFTD, whencompared to other dementia cases [7, 8]. Two studies investi-gated functional performance compared to overall cognitiveperformance in patients with different dementia subtypes[9, 10]. Five studies compared functional performance amongpatients with various subtypes of FTLD [11–15] one studylinked functional performance to emotional aspects [16],another correlated functional decline in bvFTD patients withcaregiver burden [17], and finally, some studies comparedthe performance of patients with dementia in direct versusindirect assessments of functionality [8, 18, 19]. Two studiesinvestigated rate of functional change [2, 10].

The reviewed studies suggested that individuals withbvFTD have greater functional impairment when compared

to those with other subtypes of frontotemporal lobar degen-eration or Alzheimer’s disease. The studies documented asignificant association between cognitive impairment andmeasures of functionality in these patients. In addition, it hasbeen suggested that the cognitive profile of patients may pre-dict faster functional decline. The selected studies are listedin Table 1, with their respective objectives, samples, cognitiveand functional measures, and results.

4. Discussion

The aim of the present study was to conduct a systematicreview of studies investigating the functional profile ofpatients with bvFTD. A total of 14 clinical studies were iden-tified whose aims were predominantly to compare the cog-nitive and functional performances of patients with bvFTDagainst other dementia types; provide a description of thefunctional profile of FTLD subtypes, comparing them witheach other and noting which types presented greatest func-tional and cognitive decline; investigate the predictors offunctional decline in bvFTD; compare indirect and directfunctional performance of patients for different demen-tia subtypes; and assess scales for staging and measuringneuropsychiatric, dysexecutive, and functional symptoms inbvFTD.

The findings of the studies included in this review are inline with earlier reports indicating that patients with bvFTDare younger than patients with other dementias given thatearly onset and rapid progression are frequent, particularlywith regard to the bvFTD subtype [10]. Among the studiesreviewed (see Table 1), patient age ranged from 50 to 60 years.

Relations between Functional Impairment and Cognition.Some studies included in this review suggested a significantassociation between cognitive and functional performancein patients with bvFTD. Razani et al. [8], for instance,reported that impaired verbal fluency was a predictor offunctional incapacity, assessed directly by the DAFS-R andthat performance on the WCST was moderately correlatedwith IADLs as reported by caregivers. Piquard et al. [7] foundthat worse performance on the MMSE was associated withfunctional incapacity, and that higher score on the CDRwas apredictor of functional decline in patients with bvFTD. Addi-tionally, Josephs et al. [2] reported that poor performanceon executive, visuospatial, and language functions wereindicative of more rapid progression in functional activities.These findings point to a relationship between cognitive andfunctional performance among patients with bvFTD that isnot restricted to changes in executive functions. In otherwords, decline in other functions also appears to contribute tothe functional deficits in this condition. In disagreement withthe results outlined above, Mioshi et al. [9] failed to find anysignificant association between performance on the ACE-Rand the DAD.

Studies Comparing Functional Impairment across DementiaTypes. The studies reviewed suggested that individuals withbvFTD present greater functional impairment compared topatients with other FTLD subtypes or AD. Using the DAD,

BioMed Research International 3

Table1:Summaryof

clinicalstudies

exam

iningthefun

ctionalprofileo

fbvFTD

patie

nts.

Author/year

Objectiv

esSample

Cognitiv

e/functio

nalitymeasures

Results

Piqu

ardetal.,

2004

[7]

Stud

yrelationshipbetweenfunctio

nal

impairm

entand

perfo

rmance

inplanning

activ

ities

inFT

LDandAD

𝑁=40patie

nts(11FT

LDand29

AD)

Dem

entia

ratin

gscale(DRS

)Mini-m

entalstateexam

ination(M

MSE

)Cognitiv

ediffi

culties

scale(CD

S)Ph

ysicalself-maintenance

scale(ADL)

Instrumentalactivities

ofdaily

living(IA

DL)

Socialactiv

ities

scale(SA

DL)

Tower

ofLo

ndon

Dysexecutiveq

uestion

naire

(DEX

),thek

eysearch,the

zoomap,and

thes

ixelem

entsfro

mtheb

ehaviouralassessmento

fthe

dysexecutiv

esynd

rome(BA

DS)

Nosig

nificantd

ifferencesb

etweenbvFT

Dand

ADon

ADLs.N

orelationshipbetweenplanning

andexecutivefun

ctiontests

with

functio

nalityin

either

patie

ntgrou

pAnassociationwas

foun

dbetweenCD

Rand

MMSE

andfunctio

nalp

erform

ance

inthetwo

grou

ps

Marra

etal.,

2007

[11]

Com

pare

perfo

rmance

ofdemented

elderly

with

different

levelsof

severityin

questio

nnaireso

nbasic

activ

ities

ofdaily

living(BADL)

andinstr

umentalactivities

ofdaily

living(IA

DL).D

etermine

correlationbetweentheA

DL

questio

nnairesa

pplied

𝑁=90patie

nts

(AD=74,vascular=

7,mixed

=4,

frontotem

poral=

2,and

others∗

=3)

Clinicaldementia

ratin

gLawton-Brod

yindex

Katzindex

Functio

nalactivities

questio

nnaire

(PFA

Q)

IADLs

werem

oreimpaire

datearly

stageso

fdementia

andBA

DLs

atmorea

dvancedstages

Mioshietal.,

2007

[9]

AssessADLs

andcogn

itive

perfo

rmance

insubtypes

offro

ntotem

porald

ementia

,bvFT

D,n

onflu

entp

rogressiv

eaph

asia

(NFP

A),semantic

dementia

(SD)

Ascertainther

elationshipbetween

functio

nald

eficitsandcogn

itive

dysfu

nctio

nof

patie

ntsa

ndcompare

againstp

atientsw

ithAD

𝑁=59patie

ntsw

ithdementia

(bvFTD

=15,

NFP

A=10,SD15,and

AD

=19)

Disa

bilityAs

sessmentfor

dementia

(DAD)

Addenb

rooke’s

cogn

itive

exam

ination-revised

(ACE

-R)

Clinicaldementia

ratin

gscale(CD

R)

Forfun

ctionalp

erform

ance,bvFTD

patie

ntsw

ere

mostimpaire

d(56%

with

functio

nality

preserved),w

hereas

NFP

AandSD

patie

ntsw

ere

lessim

paire

d(83%

and85%werefun

ctionally

preserved).A

Dsubjectshadinterm

ediate

perfo

rmance

(76%

show

edpreserved

functio

nality).O

nAC

E-R,

theN

FPAandSD

grou

pshadworse

perfo

rmance

than

bvFT

Dand

ADpatie

nts.DADperfo

rmance

didno

tcorrelate

with

cogn

itive

measures,CD

R,or

timed

iagn

osed

with

thed

isease

Razani

etal.,

2007a[

8]

Correlatep

erform

ance

onexecutive

functio

ntasksw

ithfunctio

nal

perfo

rmance

𝑁=33patie

ntsw

ithdementia

(3FT

LD,21A

D,

and10

VD)and

35healthy

controls

Wisc

onsin

card

sortingtest(W

CST)

Verbalflu

ency

(FAS)

Mod

ified

versionof

theL

awton-Brod

ymeasure

(IADL)

Dire

ctassessmento

ffun

ctionalstatus(DAFS)

Sign

ificant

correlationbetweenverbalflu

ency

andDAFS

.Mod

eratec

orrelationbetweenWCS

Tandperfo

rmance

onsubjectiv

efun

ctionalityscale

Razani

etal.,

2007b[17]

Assessther

elationshipbetween

perfo

rmance

onADLs

inpatie

ntsw

ithmild

dementia

andther

elatio

nshipwith

emotionalburdenof

caregivers

𝑁=34patie

ntsw

ithdementia

(5FT

LD,23A

D,

5VD,and

1with

undiagno

sedform

ofdementia

)

Dire

ctassessmento

ffun

ctionalstatus(DAFS)

Lawton-Brod

ymeasure

(IADL)

Caregiverb

urdeninventory(C

BI)

Briefsym

ptom

inventory(BSI)

Sign

ificant

correlations

betweenDAFS

,caregiver

burden

(CBI),andpsycho

logicalproblem

s(BS

I)OnDAFS

,impaire

dorientation,

commun

ication,

finances,andtransportw

erea

ssociatedwith

greaterb

urdenandho

stilityin

caregivers

Impaire

dfin

ancialabilitywas

strongestp

redictor

oftim

edependent

oncaregivers,w

hereas

impairm

ento

ntransportd

omainpredictedtim

esin

ceclinicald

iagn

osis

4 BioMed Research InternationalTa

ble1:Con

tinued.

Author/year

Objectiv

esSample

Cognitiv

e/functio

nalitymeasures

Results

Wicklun

det

al.,2007

[12]

Com

pare

functio

nalstatusinFT

LDand

AD

𝑁=100with

AD,57with

bvFT

D,and

61with

PPA

Instrumentalactivities

ofdaily

living(IA

DL)

Functio

nalabilitywas

mod

erately

impaire

din

ADandFT

Dandmild

lyim

paire

din

PPAFo

rall

grou

ps,m

orec

omplex

ADLs

wereimpaire

dearly

on,w

ithrelativ

epreservationof

self-care

activ

ities.Th

ecom

mun

icationscorew

astheleast

impaire

dnext

toself-care

forF

TDandADand

them

ostimpaire

dforP

PApatie

nts

Bressanetal.,

2007

[18]

Com

pare

inform

ationfro

mcaregiver

againstd

irectassessmento

fpatient

functio

nalp

erform

ance

𝑁=25patie

ntsw

ithdementia

,com

prising

1FT

LD,10AD,5

VD,3

alcoho

licdementia

,3D.

with

LewyBo

dies,1

corticob

asal,and

2dementia

sdue

tosevere

hypo

xia

Pfeffer

functio

nalactivities

questio

nnaire

(PFA

Q)

Observatio

nof

patie

ntsd

uringsim

ulated

activ

ities

inclu

dedin

theP

FAQ

Sign

ificant

differences

foun

dbetweencaregiver

respon

sesa

nddirectob

servationof

patie

ntperfo

rmance.C

aregiversu

nderestim

ated

functio

nalcapacity

ofpatie

nts

Carvalho

etal.,2008

[13]

Com

pare

functio

nalcom

mun

ication

abilitie

sinFT

LDandADpatie

nts

𝑁=6patie

ntsw

ithAD

and8with

FTLD

Functio

nalassessm

ento

fcom

mun

icationskills

(ASH

A-FA

CS)

Functio

nalcom

mun

icationabilitie

sweres

imilar

forp

atientsw

ithADandFT

LD.FTL

Dpatie

nts

hadworse

perfo

rmance

oninference

comprehensio

nwhileADpatie

ntsh

adworse

perfo

rmance

onbasic

transactions

with

mon

ey

Bahiae

tal.,

2008

[14]

Verifyutilityof

afun

ctionaland

behavioralinventoryin

differential

diagno

sisbetweenFT

LDandAD

𝑁=12patie

ntsw

ithAD

and12

with

FTLD

Fron

talbehavioralinventory

(FBI)

Disa

bilityassessmentfor

dementia

(DAD)

FIBproved

accurateford

ifferentia

ldiagn

osis

betweenFT

LDandAD

DADfailedto

differentiategrou

psbu

thelp

edassessseverityof

thec

onditio

n

Mioshiand

Hod

ges,

2009a[

15]

Exam

inec

hanges

inactiv

ities

ofdaily

living(A

DL),including

subcom

ponents

ofinitiation,

planning

,ore

xecutio

nof

DAD

𝑁=10patie

ntsw

ithbvFT

Dph

en,6

with

bvFT

Dpath,11w

ithSemDem

,and

9PN

FA

Addenb

rooke’s

cogn

itive

exam

ination-revised

(ACE

-R)

Disa

bilityassessmentfor

dementia

(DAD)

PathologicalbvFT

D,Sem

Dem

,and

PNFA

grou

psshow

edsig

nificantd

eclin

einADLs

after

12mon

ths,whilethep

heno

copy

subgroup

didno

tEa

chvaria

ntshow

eddifferent

profi

leso

fdeclin

ein

subcom

ponentso

fADLs

Mioshietal.,

2009b[19

]

Describea

ctivities

ofdaily

livingin

behavioralvaria

ntfro

ntotem

poral

dementia

,and

correlateincaregivera

ndperfo

rmance-based

assessments

𝑁=18patie

ntsw

ithbvFD

T

Addenb

rooke’s

cogn

itive

exam

ination-revised

(ACE

-R)

Disa

bilityassessmentfor

dementia

(DAD)

Fron

talsystemsb

ehaviorscale(fr

ontal

dysfu

nctio

n)As

sessmento

fmotor

andprocessskills

Amod

elcombining

theg

lobalcognitio

nand

frontaldysfu

nctio

nexplainedthev

arianceo

nADLperfo

rmance.A

qualitativ

erating

distinguish

edbetweenpathologicandph

enocop

ypatie

ntsb

etterthanthep

erform

ance-based

assessment

Kipp

setal.,

2009

[16]

Com

pare

emotionrecogn

ition

behavior

andsocialfunctio

ning

inADandbvFT

Dpatie

nts

𝑁=28,14patie

ntsw

ithbvFT

Dand14

with

AD

Emotionhexagon

Disa

bilityassessmentfor

dementia

(DAD)

TheC

ambridge

behaviou

ralinventory

(CBI)

bvFT

Dgrou

phadworse

functio

nald

eficit,high

erscores

ontheC

BI,and

poorer

emotion

recogn

ition

comparedwith

theA

Dgrou

pRe

cogn

ition

ofem

otions

was

notcorrelatedwith

DADbu

tinstead

with

apathy

ontheC

BI

Mioshietal.,

2010

[10]

Develo

pan

oveltoolto

characteriz

eFT

LDseveritybasedon

functio

nal

disabilityandbehavioralchanges.As

sess

rateof

change

over

timein3FT

LDvaria

nts(bvFT

D,SD,and

NFP

A)

𝑁=95patie

nts,28

bvFT

D,21N

FPA,26SD

,and20

controls

Addenb

rooke’s

cogn

itive

exam

ination-revised

(ACE

-R)

Clinicaldementia

ratin

g(C

DR)

Fron

totempo

rald

ementia

ratin

gscale(FR

S)

FTLD

progressiondifferedam

ongvaria

nts

bvFT

Dpatie

ntsp

rogressedrapidlywhereas

subjectswith

SDpresentedslo

were

volutio

nFD

RSproved

agoo

dscalefor

staging

and

determ

iningFT

LDprogression


Table1:Con

tinued.

Author/year

Objectiv

esSample

Cognitiv

e/functio

nalitymeasures

Results

Joseph

setal.,

2011[2]

Relateb

ehavioral,neurop

sychological,

andsociod

emograph

icfactorsto

determ

inep

redictivefactorsfor

functio

nald

eclin

einbvFT

D

𝑁=97patie

ntsw

ithbvFT

D

Clinicaldementia

ratin

g(C

DR)

Trailm

akingpartsA

andB

Bosto

nnamingtest

Category

fluency

(sum

ofanim

als,vegetables,

andfruits)

Mini-m

entalstatuse

xamination(M

MSE

)Neuropsychiatric

inventory(N

PI)

Wechslera

dults

intelligences

cale(W

AIS-R)

Magnetic

resonanceimaginganalysisgeneratin

gan

anatom

icalsubtype

Predom

inantly

frontotem

poraland

frontal

atroph

y,worse

perfo

rmance

onexecutive,

visuospatia

land

lang

uage

functio

ns,losso

finhibitio

n,agitatio

n/aggressio

nandnighttime

behaviors,andgreatera

geatdiseaseo

nsetwere

associated

with

faste

rrateo

ffun

ctionald

eclin

e

Notes.FTL

D:frontotem

porallob

ardegeneratio

n,bvFT

D:behavioralvariant

frontotem

porald

ementia

,NFP

A:non

fluentp

rogressiv

eaph

asia,SD:sem

antic

dementia

,VD:vasculard

ementia

,and

AD:dem

entia

oftheA

lzheimer

type.

∗

Dem

entia

scausedby

traumaticbraininjury

orby

norm

alpressure

hydrocephalus.

6 BioMed Research International

Mioshi et al. [9] showed that patients with bvFTD had poorerfunctional performance than patients with SD, NFPA or AD.This group had lower scores even on BADLs such as gettingdressed, feeding, and hygiene. For IADLs, the worst perfor-mances were seen in finances, correspondence, and leavingthe home. Poorer performances were also evident for the useof the telephone, domestic and leisure time activities, man-aging medications, and preparing meals, besides the impair-ments observed in AD patients. The authors highlightedthe devastating impact of functional changes on the everydayroutine of patients with bvFTD and the burden placed ontheir caregivers. These results were confirmed in later studiesby the same group [15, 16].

Mioshi and Hodges [15] aimed to examine changes inactivities of daily living (ADL), including subcomponents ofthe DAD and level of functional impairment in the FRS.The patients were subdivided into bvFTD pathological andphenocopy subgroups (patients who met diagnostic criteriabut the disease did not progress), SD, and PNFA. The resultsindicated that pathological bvFTD, SD and PNFA groupsshowed significant decline in ADLs after 12 months, whilethe phenocopy subgroup did not. According to the FRS, thevariants showed different levels of functional impairment.Virtually, all patients with bvFTD fell within the moderate,severe, very severe, or profound categories, whereas for thePNFA and SD groups, 25% fell within the mild category andthere were no patients regarded as very severe or profound.The proportion of patients with PNFA in the severe categorywas about the double in comparison to patients with SD. Ingeneral, results suggested patients with bvFTD were moredependent on BADLs and IADLs than the other variants. Inaddition, functional performance and cognitive scores weresignificantly correlated and FTD variants showed differentialannual rates of functional decline, with faster decline amongbvFTD patients.

The study byWicklund et al. [12] examined the functionalprofile of patients diagnosed with AD, bvFTD, and primaryprogressive aphasia (PPA). Results showed that functionalability was moderately impaired in AD and bvFTD andmildly impaired in PPA. Self-care activities were the leastimpaired in all groups, whereas more complex ADLs, such asshopping and management of finances, were impaired earlyon. Communication ability was the least impaired, next toself-care for bvFTD and AD and the most impaired for PPApatients.

Two studies failed to find differences among patientsdiagnosed with FTLD and AD on assessments of function-ality. Carvalho et al. [13] found no significant difference infunctional communication between AD and FTLD patients.Similarly, Bahia et al. [14] found no significant differencesbetween patients with AD and FTLD on DAD scores. Intheir sample, DAD did not prove accurate for differentialdiagnosis of these dementia types.These negative results maybe ascribed to the small sample size or to the fact that samplecould not be stratified into FTLD subtypes.

Two of the reviewed studies examined functional perfor-mance of FTLD patients with direct assessment, involvingthe observation of the patient during functional activities.Razani et al. [17] found significant correlations between

poor performance on the DAFS and increased burden andpsychological distress of caregivers. Impaired performanceon orientation, communication, finances and transport wereassociated with greater burden and hostility in caregivers.Comparing direct and indirect assessments of functionalityin a mixed cohort of dementia patients, only one of whichhad FTLD, Bressan et al. [18] noted discordance between theopinion of caregivers/family members and actual observedperformance of the patient, whereby informants often under-estimated patients’ potential to perform ADLs.

The reviewed studies highlighted the importance of car-rying out functional assessment of patients with suspectedbvFTD given the relevance of these changes for the diagnosisand clinical management of this dementia subtype. Someauthors, such as Razani et al. [8], highlighted the dysex-ecutive profile of these patients and the significant impacton performing ADLs. Patients in the cited study exhibiteddifficulties starting tasks, estimating time, switching betweentasks, and dealing with tasks of differing priorities. Also, sub-jects had problems controlling their impulses and exhibiteddeficits in chronological ordering, impatience, and emotionallability, directly impacting the performance of ADL. It iswidely accepted that cognitive changes explain the functionaldeficits seen in patients with bvFTD. However, authors suchas Mioshi et al. [15] suggested that apathy and impulsiveness,typical among this patient group, may also influence thefunctional deficits observed.

Notably, some studies suggested that patients with bvFTDhad lower survival rate compared to patients with AD,possibly associated with greater functional impairment [10].Studies involving scales of severity and staging of bvFTD,such as the investigations by Marra et al. [11], Mioshi et al.[10], and Josephs et al. [2], support the hypothesis that theseverity of bvFTD, together with its cognitive and neuropsy-chiatric symptoms, may modulate functional performance.

The present work has limitations including the paucityof studies identified investigating the functional profile ofpatients with bvFTD. Additionally, some of the reviewedstudies included a very small number of patients diagnosedwith bvFTD, and this fact may render themmore informativeabout dementia in general than bvFTD. It is noteworthy thatthe vast majority of studies in the current literature haveinvestigated the functional profile of AD patients, healthycontrols, and elderly with mild cognitive impairment [19–24]; therefore, the functional status of patients with FTLDrequires further investigation. Future clinical and epidemio-logic studies on FTLD should include instruments assessingfunctional performance in order to better characterize thepatient profile. Such studies should have an impact onrehabilitation strategies geared towards patients with FTLD.Suchfindings should be relevant for the planning anddeliveryof support for families andmay reinforce the need for tailoredcaregiver support that should take into account the diseasestage and the functional limitations of the patient.

Acknowledgment

This research was funded by Fundacao de Amparo a Pesquisado Estado de Sao Paulo, Grant no. 11/04804-1.


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