Essay Feldman Barrett Emotion Perception in Semantic Dementia

8/10/2019 Essay Feldman Barrett Emotion Perception in Semantic Dementia

1/59

Emotion perception in semantic dementia

EMOTION PERCEPTION IN SEMANTIC DEMENTIA

Emotion perception, but not afect perception, is impaired withsemantic memory loss

Kristen A. Lindquist,!

Maria "endron,#!

Lisa $e%dman &arrett,#,',( )

*&rad+ord C. Dicerson',(,-)

Department o+ Psc/o%o0, 1ni2ersit o+ Nort/ Caro%ina, C/ape% 3i%%,NC #4-55

# A6ecti2e Science Institute and Department o+ Psc/o%o0,Nort/eastern 1ni2ersit, &oston, MA 7#-

'Department o+ Psc/iatr, Massac/usetts "enera% 3ospita% and3ar2ard Medica% Sc/oo%, &oston, MA 7#((

(Martinos Center +or &iomedica% Ima0in0, Massac/usetts "enera%3ospita%, C/ar%esto8n, MA 7##5

-

$rontotempora% Disorders 1nit, Department o+ Neuro%o0,Massac/usetts "enera% 3ospita%, &oston, MA 7#((

* authors contributed equallyauthors contributed equally

Corresponding author:Kristen A. LindquistDepartment o+ Psc/o%o01ni2ersit o+ Nort/ Caro%ina, C/ape% 3i%%Da2ie '#risten.%indquist9unc.edu


2/59


Abstract

$or decades, psc/o%o0ists and neuroscientists /a2e

/pot/esi:ed t/at t/e a;i%it to percei2e emotions on ot/ers< +aces is

in;orn, pre=%in0uistic, and uni2ersa%. Concept no8%ed0e a;out emotion

/as ;een assumed to ;e epip/enomena% to emotion perception. In t/is

paper, 8e report >ndin0s +rom t/ree patients 8it/ semantic dementia

t/at cannot ;e e?p%ained ; t/is @;asic emotion 2ie8. T/ese patients,

8/o /a2e su;stantia% de>cits in semantic processin0 a;i%ities,

spontaneous% percei2ed p%easant and unp%easant e?pressions on

+aces, ;ut not discrete emotions suc/ as an0er, dis0ust, +ear, or

sadness, e2en in a tas t/at did not require t/e use o+ emotion 8ords.

Our >ndin0s support t/e /pot/esis t/at discrete emotion concept

no8%ed0e /e%ps trans+orm perceptions o+ a6ect Bpositi2e% or

ne0ati2e% 2a%enced +acia% e?pressions into perceptions o+ discrete

emotions suc/ as an0er, dis0ust, +ear and sadness. T/ese >ndin0s

/a2e important consequences +or understandin0 t/e processes

supportin0 emotion perception.

#


3/59


T/e a;i%it to percei2e discrete emotions suc/ as an0er, dis0ust,

+ear, sadness, etc. in ot/er peop%e is a +undamenta% part o+ socia% %i+e.

it/out t/is a;i%it, peop%e %ac empat/ +or %o2ed ones, mae poor

socia% ud0ments in t/e ;oardroom and c%assroom, and /a2e diFcu%t

a2oidin0 t/ose 8/o mean t/em /arm. T/e dominant paradi0m in

emotion researc/ +or t/e past (7 ears, ca%%ed t/e @;asic emotion

approac/, assumes t/at /umans e?press and detect in ot/ers discrete

emotions suc/ as an0er Bi.e., a sco8%, sadness Bi.e., a pout, +ear Bi.e.,

8ide ees, dis0ust Bi.e., a 8rin%ed nose, or /appiness Bi.e., a smi%e .

Scientists %ar0e% assume t/at t/is detection a;i%it is in;orn, uni2ersa%

across a%% cu%tures, and psc/o%o0ica%% primiti2e Bi.e., it cannot ;e

;roen do8n into more ;asic psc/o%o0ica% processes. Concept

no8%ed0e a;out discrete emotion t/at is represented in %an0ua0e is

assumed to ;e irre%e2ant to t/e a;i%it to percei2e discrete emotion in

+aces . T/is @;asic emotion 2ie8 is a standard part o+ t/e psc/o%o0

curricu%um tau0/t at uni2ersities in t/e estern 8or%d, and dri2es

researc/ in a ran0e o+ discip%ines inc%udin0 co0niti2e neuroscience ,

interpersona% communication and conGict ne0otiation and

psc/opat/o%o0 . T/e 1S 0o2ernment a%so re%ies on t/is +rame8or to

train securit personne% to identi+ t/e co2ert intentions o+ peop%e 8/o

pose a t/reat to its citi:ens .

Emotion concepts shape discrete emotion perception

'


4/59


Despite t/e pre2a%ence o+ t/e ;asic emotion 2ie8, 0ro8in0

e2idence su00ests t/at discrete emotion perception is not

psc/o%o0ica%% ;asic, and in +act depends on more @e%ementa%

psc/o%o0ica% processes suc/ as perceptions o+ ;asic a6ecti2e

2a%ence in +aces Bi.e., detectin0 +acia% ;e/a2iors t/at correspond to

positi2e 2. ne0ati2e 2. neutra% +ee%in0s, and # t/e a;i%it to mae

meanin0 o+ t/ose a6ecti2e +acia% ;e/a2iors usin0 concept no8%ed0e

a;out discrete emotion .

A 0ro8in0 %iterature demonstrates t/at accessi;%e emotion

concept no8%ed0e s/apes /o8 indi2idua%s mae meanin0 o+ a6ecti2e

+acia% e?pressions as instances o+ discrete emotions suc/ as @an0er,

@dis0ust, @+ear, etc. $or instance, t8o=ear o%d c/i%dren, 8/o possess

on% t/e rudimentar concepts o+ @sad and @/app, can

correspondin0% on% percei2e +aces in terms o+ a6ecti2e 2a%ence Be.0.,

t/e cate0ori:e a%% unp%easant +aces, inc%udin0 sco8%in0, poutin0, 8ide

eed and 8rin%e nosed +aces as @sad and a%% smi%in0 +aces as

@/app. Het as c/i%dren 0radua%% acquire additiona% discrete emotion

concepts o2er t/e course o+ de2e%opment Be.0., @an0er, @dis0ust,

@+ear, t/e are su;sequent% a;%e to percei2e unp%easant +aces

Bpouts, sco8%s, 8ide ees, 8rin%ed noses as instances o+ distinct

discrete emotion cate0ories. $or instance, ; a0e se2en, 8/en c/i%dren

In t/is paper, as in our prior 8or, 8e use t/e terms @a6ect or @a6ecti2e 2a%enceto re+er to /edonic tone Bi.e., positi2it, ne0ati2it, and neutra%it. e use t/e term@discrete emotion to re+er to instances o+ speci>c emotion cate0ories suc/ as an0er,dis0ust, +ear, etc.

(


5/59


/a2e %earned t/e meanin0s o+ @sad, @an0er, @+ear and @dis0ust

t/e re%ia;% percei2e poutin0 +aces as @sad, sco8%in0 +aces as

@an0er, 8ide eed +aces as @+ear, and 8rin%e nosed +aces as

@dis0ust .

T/e ro%e o+ concept no8%ed0e in emotion perception is not

%imited to ear% de2e%opment, /o8e2er. E2idence su00ests t/at e2en

/ea%t/ adu%ts re% on concept no8%ed0e durin0 emotion perception

tass, re0ard%ess o+ 8/et/er t/at tas e?p%icit% in2o%2es %a;e%in0 o+

+aces or not. $or instance, /ea%t/ adu%ts +rom most cu%tures can easi%

se%ect t/e 8ord t/at ;est matc/es t/e e?pression 8it/ a re%ati2e% /i0/

de0ree o+ a0reement 8/en emotion 8ord %a;e%s are e?p%icit% 0i2en in

an e?perimenta% tas Be.0., a posed +acia% e?pression is presented 8it/

a set o+ emotion adecti2es. Het it is possi;%e to dramatica%% impair

discrete emotion perceptionand t/us reduce accurac on a tas suc/

as t/is; mere% manipu%atin0 t/ose adu%ts< a;i%it to access t/e

meanin0 o+ discrete emotion 8ords. T/is e2en occurs in tass t/at do

not e?p%icit% require %a;e%in0 o+ +aces. $or instance, disruptin0 access

to t/e meanin0 o+ discrete emotion concepts suc/ as @an0er,

@dis0ust, or @+ear, ; /a2in0 participants repeat ot/er 8ords durin0

t/e discrete emotion perception tas Bca%%ed @2er;a% o2ers/ado8in0,

impairs participants< a;i%it to distin0uis/ ;et8een +acia% portraa%s o+

an0er and +ear as cate0orica%% di6erent emotiona% e?pressions . T/e

simp%e remo2a% o+ discrete emotion 8ords +rom t/e e?perimenta% tas

-


6/59


produces a simi%ar e6ect . An e?perimenta% manipu%ation t/at

temporari% renders t/e meanin0 o+ an emotion 8ord inaccessi;%e

ca%%ed semantic satiationa%so reduces t/e speed and accurac o+

discrete emotion perception . $or instance, a+ter repeatin0 a re%e2ant

discrete emotion 8ord Be.0., @an0er out %oud '7 times unti% t/e

meanin0 o+ t/e 8ord ;ecomes temporari% inaccessi;%e, participants

are s%o8er and %ess accurate to ud0e t/at t8o sco8%in0 +aces matc/

one anot/er in emotiona% content BLindquist et a%. #77J. Since t/e

emotion ud0ment tas used in Lindquist et a%. B#77J mi0/t imp%icit%

require use o+ discrete emotion 8ords, 8e rep%icated and e?tended

t/ese >ndin0s more recent% usin0 a perceptua% primin0 tas t/at does

not require t/e use o+ emotion 8ords. e +ound t/at +o%%o8in0 semantic

satiation o+ a re%e2ant discrete emotion 8ord Be.0., @an0er, a +ace

posin0 discrete emotion Be.0., a sco8%in0 +ace is %itera%% seen

di6erent% ; participants. $or instance, t/e emotiona% +ace percei2ed

8/en t/e meanin0 o+ an emotion 8ord is inaccessi;%e Be.0., a sco8%in0

+ace percei2ed a+ter semantic satiation o+ t/e 8ord @an0er does not

perceptua%% prime itse%+ on a %ater presentation . A%t/ou0/ t/ese

care+u% e?perimenta% manipu%ations produced data consistent 8it/ t/e

/pot/esis t/at concept no8%ed0e a;out emotion s/apes instances o+

positi2e and ne0ati2e +acia% musc%e mo2ements into perceptions o+

discrete emotions, a po8er+u% test o+ t/is /pot/esis is to e?amine

discrete emotion perception in peop%e 8/o /a2e natura%% occurrin0

J


7/59


and permanent% impaired concept no8%ed0e.

A case study o discrete emotion perception in semantic

dementia

In t/e present report, 8e assessed discreteemotion perception

in indi2idua%s 8it/ a neurode0enerati2e disease t/at impairs access to

and use o+ concept no8%ed0e. Semantic dementia is a pro0ressi2e

neurode0enerati2e diseaseone +orm o+ primar pro0ressi2e ap/asia

t/at resu%ts in nota;%e impairments in concept no8%ed0e a2ai%a;i%it

and use . Researc/ on semantic dementia /as traditiona%%

documented impairments to conceptua% no8%ed0e +or o;ects, p%ants,

and anima%s . Suc/ impairments are associated 8it/ re%ati2e% +oca%

Btpica%% %e+t=%atera%i:ed neurode0eneration in t/e anterior tempora%

%o;es, 8/ic/ are /pot/esi:ed to ;e /u;s in a distri;uted net8or

su;ser2in0 semantic memor and conceptua% no8%ed0e . Ear% in t/e

course o+ t/e disease, semantic dementia is associated 8it/ t/e 2er

speci>c ina;i%it to understand t/e meanin0 o+ 8ords, amidst norma%

2isua% processin0, e?ecuti2e contro%, comportment and ;e/a2ior.

Sometimes termed t/e @tempora% %o;e 2ariant o+ +rontotempora%

dementia B$TD, semantic dementia is a su;=c%ass o+ t/e ;roader

dia0nosis o+ +rontotempora% dementia. As suc/, some patients can

de2e%op a ;roader set o+ %esions in +ronta% or ot/er tempora% %o;e

re0ions a6ectin0 ;rain areas in2o%2ed in ot/er psc/o%o0ica% processes

suc/ as e?ecuti2e contro% Be.0., dorso%atera% pre+ronta% corte? or 2isuo=

4


8/59


spatia% processin0 Be.0., /ippocampus, perir/ina% corte?. Due t/e

/etero0eneit in/erent in +rontotempora% dementia, 8e care+u%%

se%ected t/ree patients 8/o /ad speci>c anatomica% and ;e/a2iora%

pro>%es o+ semantic dementia t/is a%%o8ed us to per+orm a 2er

precise test o+ our /pot/esis a;out emotion perception. $irst, 8e

se%ected patients 8it/ a speci>c neurode0eneration pattern 8e

se%ected on% t/ose patients 8it/ re%ati2e% +oca% %esions to t/e anterior

tempora% %o;es. Second, 8e se%ected patients disp%ain0 speci>c

;e/a2iora% patterns 8e per+ormed a /ost o+ neuropsc/o%o0ica% tests

and contro% e?perimenta% tass to ensure t/at t/e patients in our

samp%e /ad semantic de>cits ;ut re%ati2e% preser2ed e?ecuti2e

+unction and 2isuospatia% processin0. To ru%e out t/at patients /ad

more 0enera% a6ecti2e a;norma%ities t/at mi0/t resu%t in impaired

discrete emotion perception, 8e a%so re%ied on c%inica% assessments to

ensure t/at patients /ad norma% comportment and ;e/a2iora%

approac/ and a2oidance. To0et/er, t/ese ri0orous inc%usion criteria

a%%o8ed us to per+orm a stron0 test o+ t/e /pot/esis t/at impaired

conceptua% no8%ed0e resu%ts in impaired discrete emotion perception.

"ro8in0 e2idence documents de>cits in emotion perception in

semantic dementia , ;ut no researc/ to date /as speci>ca%% addressed

t/e /pot/esis t/at impairments in concept no8%ed0e contri;ute to

impairments in discrete emotion perception. Patients 8it/ semantic

dementia /a2e diFcu%ties %a;e%in0 +acia% e?pressions o+ emotion , ;ut


9/59


suc/ >ndin0s are tpica%% interpreted as e2idence t/at patients can

understand t/e meanin0 o+ emotiona% +aces ;ut per+orm poor% on

e?perimenta% tass due to an ina;i%it to manipu%ate %a;e%s . I+ t/is

8ere t/e case, %an0ua0e 8ou%d /a2e an impact on t/e communication

o+ discrete emotion perception, ;ut not t/e understandin0 o+ emotiona%

+acia% ;e/a2iors. Het t/e possi;i%it remains t/at concept no8%ed0e

p%as a muc/ more inte0ra% ro%e in discrete emotion perception ;

/e%pin0 to trans+orm perceptions o+ a6ecti2e +acia% e?pressions into

perceptions o+ discrete emotions. In t/is 2ie8, conceptua% no8%ed0e

+or emotion is necessar +or discrete emotion perception to proceed. To

test t/is /pot/esis, 8e use a case stud approac/ to test emotion

perception a;i%ities in t/ree patients 8it/ semantic dementia. &ecause

t/ese t/ree patients /a2e re%ati2e% iso%ated impairments in semantic

memor 8it/out impairments in e?ecuti2e +unction, 2isuospatia%

a;i%ities, comportment, or ;e/a2iora% approac/ or a2oidance, t/e

pro2ide an opportunit to per+orm a tar0eted test o+ t/e /pot/esis

t/at concept no8%ed0e is necessar +or discrete emotion perception.

The Present Study

To assess 8/et/er concept no8%ed0e is important to norma%

discrete emotion perception, 8e desi0ned a tas in 8/ic/ patients 8it/

semantic dementia B8/o /a2e diFcu%t usin0 semantic %a;e%s cou%d

demonstrate t/eir discrete emotion perception a;i%ities 8it/out re%in0

on %in0uistic emotion concepts. e t/us desi0ned a sortin0 tas t/at

5


10/59


8ou%d assess spontaneous emotion perception and not emotion

%a;e%in0 per se. Patients 8ere presented 8it/ pictures o+ sco8%in0,

poutin0, 8ide eed, 8rin%e nosed, and smi%in0 +aces, as 8e%% as posed

neutra% +aces and 8ere ased to +ree% sort t/e pictures into pi%es

representin0 as man cate0ories as 8ere meanin0+u%. e used posed

depictions o+ discrete emotions Bt/e standard in most scienti>c studies

o+ emotion perception ;ecause t/e are ;e%ie2ed to ;e t/e c%earest

and most uni2ersa%% reco0ni:a;%e si0na%s o+ discrete emotions ; ;asic

emotion accounts .

To ru%e out t/at patients /ad ot/er de>cits t/at 8ou%d impair t/eir

per+ormance on t/e discrete emotion sort tas, 8e a%so ased t/em to

a%so per+orm a num;er o+ contro% sort tass. In t/ese contro% sort tass,

patients 8ere ased to sort t/e +aces into si? pi%es anc/ored 8it/ si?

num;ers Ba num;er anc/ored sort, to sort t/e +aces into si? pi%es

anc/ored 8it/ si? posed discrete emotiona% +acia% e?pressions Ba +ace

anc/ored sort, to sort t/e +aces into si? pi%es anc/ored 8it/ si?

emotion cate0or 8ords Ba 8ord anc/ored sort, or to +ree sort t/e

+aces ; identit Ban identit sort. /ereas t/e num;er and 8ord

anc/ored sorts ru%ed out t/e a%ternate /pot/esis t/at patients cou%d

per+orm a discrete sort 8/en cued to t/e correct num;er or names o+

cate0ories, t/e +ace anc/ored and identit sorts ru%ed out t/e more

0enera% a%ternati2e interpretations t/at patients did not understand

/o8 to sort pictures, # t/at t/e /ad 2isua% impairments t/at

7


11/59


pre2ented t/em +rom percei2in0 di6erences ;et8een +aces Bi.e.,

prosopa0nosia or more 0enera% 2isuospatia% de>cits, ' or t/at t/e

/ad co0niti2e impairments in e?ecuti2e +unction t/at 8ou%d cause

t/em to per+orm poor% on an sortin0 tas. Patients< per+ormance on

t/ese sorts,a%on0 8it/ our neuropsc/o%o0ica% >ndin0s Bsee

supp%ementar materia%s, ru%es out a%ternate e?p%anations o+ patientscits 8ou%d ;e associated 8it/ diFcu%t

percei2in0 same=2a%ence discrete emotiona% +acia% e?pressions Be.0.,

an0er 2s. +ear 2s. dis0ust 2s. sadness. Het mirrorin0 t/e de2e%opmenta%

>ndin0s t/at in+ants and oun0 c/i%dren, 8/o /a2e %imited conceptua%

no8%ed0e o+ emotion, can detect positi2e and ne0ati2e a6ect on +aces

, 8e predicted t/at patients 8it/ semantic dementia 8ou%d /a2e

re%ati2e% preser2ed perception o+ positi2e 2s. ne0ati2e 2s. neutra%

e?pressions Bi.e., a6ecti2e 2a%ence. T/is /pot/esis 8ou%d ;e

supported in our stud i+ /ea%t/ contro% adu%ts, 8/o /a2e access to

conceptua% no8%ed0e o+ emotion, spontaneous% produced si? pi%es +or

an0er, dis0ust, +ear, sadness, /appiness and neutra% e?pressions and

rare% con+used mu%tip%e ne0ati2e +aces +or one anot/er Be.0., 8ou%d

not treat poutin0, sco8%in0, 8ide=eed and 8rin%ed nose +aces as

mem;ers o+ t/e same cate0or ; p%acin0 t/em in t/e same pi%e.


12/59


Patients, on t/e ot/er /and, 8ou%d spontaneous% sort +aces into pi%es

correspondin0 to positi2e, ne0ati2e, and neutra% a6ect and 8ou%d

additiona%% mae errors in 8/ic/ t/e con+used mu%tip%e ne0ati2e

+aces +or one anot/er Be.0., 8ou%d treat poutin0, sco8%in0, 8ide=eed

and 8rin%ed nose +aces as mem;ers o+ t/e same cate0or ; p%acin0

t/em in t/e same pi%e#. In contrast, a ;asic emotion /pot/esis 8ou%d

predict t/at emotion perception is a psc/o%o0ica%% primiti2e process

e2o%2ed +rom ancestra% primate communicati2e disp%as t/at does not

re% on concept no8%ed0e . I+ t/is /pot/esis 8ere supported, 8e

8ou%d not o;ser2e a di6erence ;et8een contro% participants< and

patients< sorts. At t/e 2er %east, i+ patients 8ere impaired in discrete

emotion perception as compared to contro%s, a ;asic emotion 2ie8

8ou%d not predict t/at patients 8ou%d s/o8 maintained perception o+

a6ect Bpositi2e, ne0ati2e and neutra% 2a%ence. Accordin0 to t/e ;asic

emotion 2ie8, t/e perception o+ discrete emotion is more

psc/o%o0ica%% +undamenta% t/an t/e perception o+ a6ect, meanin0

t/at discrete emotion perception s/ou%d precede a6ect perception .

Methods

Participants

e studied t/ree patients BE", a 47=ear o%d ri0/t=/anded ma%e

$, a J(=ear o%d ri0/t=/anded ma%e and CP, a -'=ear o%d ri0/t=

#1n+ortunate%, since t/ere 8ere not mu%tip%e ;asic=%e2e% cate0ories 8it/in t/e

superordinate cate0or o+ @positi2e, 8e cou%d not conduct a compara;%e ana%sis +orpositi2e +aces. T/is is ;ecause most ;asic emotion accounts consider /appiness to ;et/e on% positi2e ;asic emotion.

#


13/59


/anded +ema%e 8it/ a re%ati2e% rare +orm o+ neurode0enerati2e

disorder no8n as semantic dementia. Eac/ patient 8as dia0nosed

8it/ semantic dementia ; a team o+ neuro%o0ists ;ased on t/eir

;e/a2iora% smptoms, neuroanatom, and per+ormance on

neuropsc/o%o0ica% assessments. Eac/ patient presented to t/e c%inic

8it/ 0radua%% pro0ressi2e pro;%ems reca%%in0 t/e meanin0 o+ 8ords

Bi.e., anomia and 8as +ound to /a2e a semantic memor de>cit on

neuropsc/o%o0ica% testin0. Neuropsc/o%o0ica% tests 8ere co%%ected in

a /ospita% c%inic 8e re% on t/e neuropsc/o%o0ica% data co%%ected in

t/at settin0. Neuropsc/o%o0ica% tests re2ea%ed t/at patients /ad

re%ati2e% speci>c semantic de>cits amidst norma% inte%%ectua% a;i%ities,

e?ecuti2e +unction, and 2isuospatia% per+ormance. No patient e?/i;ited

e2idence o+ impaired reco0nition o+ 2isua% o;ects Bi.e., 2isua% a0nosia

or +aces Bi.e., prosopa0nosia. See supp%ementar on%ine materia%s

BSOM +or case /istories and neuropsc/o%o0ica% test resu%ts con>rmin0

t/e speci>cit o+ eac/ patient


14/59


did not inc%ude a patient contro% samp%e ;ecause it 8as diFcu%t to >nd

a samp%e o+ patients 8it/ anot/er +orm o+ dementia 8/ose

per+ormance 8ou%d demonstrate a c%ear dou;%e dissociation on t/e sort

tass 8e emp%oed. $or instance, patients 8it/ ot/er tpes o+

neurode0enerati2e diseases Be.0., &e/a2iora% ariant $rontomtepora%

Dementia A%:/eimercits,

;e/a2iora% impu%si2it, or impairments in a6ecti2e 2a%uation. As a

resu%t, 8e compared t/e per+ormance o+ our semantic dementia

samp%e direct% to /ea%t/ contro%s, 8/i%e a%so institutin0 mu%tip%e

contro% tass 8it/in t/e semantic dementia samp%e to ru%e out

a%ternate e?p%anations +or our >ndin0s. T/is approac/ 8as 8arranted

+or se2era% reasons. $irst, t/e aim o+ our stud 8as not to un2ei% ne8

de>nin0 c/aracteristics o+ semantic dementia, ;ut rat/er to iso%ate

t/ose c/aracteristics a%read no8n to e?ist in order to test a speci>c

/pot/esis a;out emotion perception. Second, 8e instituted a num;er

o+ 8it/in=su;ects contro% tass 8it/ our patient samp%e to ru%e out

ot/er e?p%anations o+ our >ndin0s. Nota;%, ;ot/ o+ t/ese approac/es

are taen in ot/er pu;%is/ed case studies o+ patients 8it/ semantic

dementia BLam;on=Ra%p/ et a%. #77.

Materials

Face sort stimuli.T/e set contained posed, caricatured,

pictures o+ +acia% e?pressions correspondin0 to J di6erent emotion

(


15/59


cate0ories Ban0ersco8%, sadnesspout, dis0ust8rin%ed nose,

+ear8ide ees, /appinesssmi%e, neutra%re%a?ed +acia% musc%es.

Ima0es 8ere se%ected +rom t/e IASLa; $ace Set B888.a6ecti2e=

science.or0 and t/e Researc/ Net8or on Ear% E?perience and &rain

De2e%opment $ace Set B#77J, NimStim $ace Stimu%us Set

/ttpQQ888.mac;rain.or0Qresources./tm . A %on0 2ersion o+ t/e tas

contained #7 identities and a s/ort 2ersion contained J. A%% identities

8ere European American and an equa% num;er o+ ma%e and +ema%e

identities 8ere used. Patients did not sort +aces sstematica%% ;

0ender on t/e emotion sort tass, so 8e do not discuss t/e 0ender o+

+aces +urt/er.

Number anchors. Si? pieces o+ .- ? paper containin0 t/e

num;ers =J ser2ed as pi%e anc/ors.

Face anchors. Si? pictures o+ a sin0%e 8oman +rom t/e NimStim

$ace Stimu%us set posin0 a sco8%, 8rin%ed nose, 8ide ees, pout,

smi%e and a posed neutra% +ace ser2ed as pi%e anc/ors.

ord anchors. Si? pieces o+ .- ? paper containin0 t/e

8ords @an0er, @dis0ust, @+ear, @/appiness, @sadness and @neutra%

ser2ed as pi%e anc/ors.

Procedure

T/e discrete emotion perception tass 8ere desi0ned to pro;e

c/an0es in a6ecti2e processin0 andQor discrete emotion perception.

-
http://www.affective-science.org/http://www.affective-science.org/http://www.macbrain.org/resources.htmhttp://www.affective-science.org/http://www.affective-science.org/http://www.macbrain.org/resources.htm


16/59


T/e +ree sort procedure 8as desi0ned to identi+ t/e cate0ories t/at

patients spontaneous% percei2ed in posed +acia% e?pressions 8it/

minima% constraints and 8it/out asin0 patients to e?p%icit% use

emotion 8ords. As suc/, 8e ased participants to sort into t/e

cate0ories t/e +ound @meanin0+u%. T/is open=ended instruction

necessari% ensured t/at t/e emotion cate0ories /pot/esi:ed to 0uide

perception 8ere not e?p%icit% in2oed ; t/e e?perimenta% procedure

at t/e outset o+ t/e tas. $ortunate%, t/e per+ormance o+ contro%

participants ensured t/at a%ternate e?p%anations o+ t/e +ree sort

>ndin0s 8ere not possi;%e. A%% contro% participants immediate%

understood t/at discrete emotion cate0ories 8ere t/e most meanin0+u%

8a to sort t/e +aces Bdespite t/e +act t/at ot/er cate0ories suc/ as

identit and 0ender 8ere a%so possi;%e. $urt/ermore, testin0 patients

on su;sequent contro% tass ensured t/at patients did not /a2e

diFcu%t +o%%o8in0 instructions, as t/e 8ere a;%e to sort ; perceptua%

+eatures o+ t/e e?pressions 8/en cued. Important%, additiona% contro%

tass Bconstrainin0 t/e num;er o+ pi%es patients 8ere to%d to mae, and

cuein0 patients 8it/ t/e names o+ emotion cate0ories did not /e%p

t/eir per+ormance, 8/ic/ 8ou%d ;e predicted i+ impaired per+ormance

8as simp% due to t/e interpretation o+ open=ended instructions. T/ese

additiona% tass t/us /e%ped to c%ari+ t/e interpretation t/at patientscits %ed to impairments in discrete emotion perception.

$ina%%, t/e identit sort ru%ed out t/at patients did not understand /o8

J


17/59


to sort +aces, /ad a 2isua% impairment t/at pre2ented t/em +rom

percei2in0 di6erences ;et8een +aces Bi.e., prosopa0nosia or ot/er

2isuospatia% de>cits, or /ad co0niti2e impairments in e?ecuti2e

+unction t/at 8ou%d cause t/em to per+orm poor% on an sortin0 tas.

Emotional ree sort. Participants 8ere /anded t/e +ace sort

stimu%i and 8ere ased to +ree% mae pi%es to represent t/e num;er o+

meanin0+u% cate0ories t/e percei2ed in t/e set. Patients 8ere to%d, @I

am going to give you a pile of pictures. What I want you to do is

organize them into groups that are meaningful to you. ou can create

as many piles as you need to. !t the end" each pile should be sorted

so that only one type of picture is included. It is sometimes helpful to

loo# through the set of pictures $rst before you begin sorting. %his is

not timed" so feel free to ta#e as long as you need. ou can also

change the piles while you are sorting or at the end&it is up to you. 'o

you have any questions( Patients 8ere t/en ased to sort t/e pictures

into pi%es on t/e ta;%e. $o%%o8in0 comp%etion o+ t/e sort, t/e researc/er

ased t/e patient se2era% questions inc%udin0 @Can you tell me

about how you sorted the pictures() Since a%% patients indicated t/at

t/e /ad sorted ; +ee%in0, t/e researc/er ne?t ased +@,ow

con$dent are you that all of the people in each pile feel e-actly the

same way( ot con$dent" somewhat con$dent or very con$dent()

Ne?t, t/e researc/er 8ent t/rou0/ eac/ pi%e 8it/ t/e participant and

ased, @What is in this pile() I+ t/e patient responded 8it/ emotion or

4


18/59


a6ect 8ords Be.0., @/app, @dis0ust, @+ear or @0ood, @;ad, t/e

researc/er ased t/e patient to %a;e% t/e +acia% action on t/e ne?t

round ; asin0, /What e-pression is on these people0s faces() I+ t/e

patient ne2er used an emotion 8ord to descri;e t/e pi%e, t/e

researc/er prompted,/What emotion are the people in this pile

feeling() In t/e present report, a%% patients understood t/e instructions

8e%% and eac/ immediate% sorted t/e +aces into pi%es representin0 t/e

a6ecti2e meanin0 o+ t/e +ace Bpositi2e, ne0ati2e or neutra%. Once t/e

content o+ eac/ pi%e 8as recorded, t/e researc/er s/ued t/e +aces

and mo2ed on to t/e ne?t tas.

Number anchored sort.T/e researc/er indicated t/at t/e

patient s/ou%d mae si? pi%es ; %ain0 do8n t/e si? num;er anc/ors.

T/is contro% tas cued t/e patient to t/e +act t/at t/ere 8ere si?

perceptua% cate0ories in t/e +ace set and ru%ed out t/e a%ternate

interpretation t/at participants mere% did not understand t/at 8e

8anted t/em to sort into si? pi%es. Patients 8ere to%d, @ow I want you

to again sort these pictures based on feeling" but this time I am going

to as# you to ma#e 1 piles. I have 1 di2erent numbers that I will lay

out for you so that you can #eep trac# of how many piles you create.

!gain" I want you to sort based on feeling. In each pile" there should be

only people who feel the same way. !t the end" each pile you0ve made

should have pictures of only people who feel the same way. !re these

instructions clear()


19/59


Face anchored sort.T/e researc/er indicated t/at t/e patient

s/ou%d mae si? pi%es ; %ain0 do8n t/e si? +ace anc/ors. T/is contro%

tas cued t/e patient to t/e +act t/at 8e 8ere asin0 t/em to sort into

si? cate0ories ;ased on t/e perceptua% +eatures o+ t/e +aces in t/e set.

It ru%ed out t/e a%ternate interpretations t/at participants mere% did

not understand t/at 8e 8anted t/em to sort ;ased on t/e +acia%

e?pressions or t/at patients /ad diFcu%t 2isua%% detectin0 di6erences

in t/e e?pressions. Patients 8ere to%d, @ow I want you to again sort

these pictures based on feeling" but this time I am going to start the

piles for you. ,ere are si- di2erent pictures of the same woman. %he

woman feels di2erently in each of the pictures. !gain" I want you to

sort the pictures into these piles I have already started based on

feeling. In each pile" there should be only people who feel the same

way. !t the end of your sorting" each pile you0ve made should have

pictures of only people who feel the same. !re these instructions

clear() A+ter patients made pi%es, t/e tas proceeded as in t/e emotion

+ree sort.

ord anchored sort.T/e researc/er indicated t/at t/e patient

s/ou%d mae si? pi%es ; %ain0 do8n t/e si? 8ord anc/ors. T/is cued

t/e patient to t/e names o+ t/e si? emotion cate0ories in t/e +ace set.

Addin0 8ords to t/e e?perimenta% tas tpica%% /e%ps /ea%t/ adu%ts

;ecome more accurate at discrete emotion perception . T/e 8ord=

anc/ored sort tas t/ere+ore ru%ed out se2era% important a%ternati2e

5


20/59


e?p%anations o+ our data. $irst, it ru%ed out t/e possi;i%it t/at patients

8ere a;%e to percei2e and sort ; discrete emotion ;ut /ad not done so

in t/e +ree sort tas ;ecause t/e instructions 8ere too 2a0ue or

;ecause t/e +ound ot/er cate0ories to ;e more re%e2ant. Second, t/is

contro% tas ru%ed out t/e possi;i%it t/at participants /ad intact

concept no8%ed0e ;ut per+ormed poor% on t/e +ree sort ;ecause t/e

didn


21/59


accurate%, t/en t/is 8ou%d ;e e2idence t/at t/e /ad ot/er co0niti2e

or 2isua% de>cits t/at 8ou%d mae t/em unsuita;%e participants +or t/e

emotion perception tas. T/e researc/er stated, @In this pile there are

pictures of a bunch of people. %here are several pictures of each

person in the pile. What I would li#e you to do is to sort the pictures

into piles based on their identity. ou can create as many new piles as

you need to. !t the end" each pile you0ve made should have pictures of

only 3 person in it. !re these instructions clear() /en t/e patient

>nis/es, t/e researc/er ass, @,ow con$dent are you that all of the

people in each pile are the same e-act person( ot con$dent"

somewhat con$dent or very con$dent()

"ata Analysis

To assess patients< per+ormance, 8e computed t/e num;er o+

pi%es t/e created on t/e emotiona% +ree sort and t/e percenta0e o+

errors B8/ere patients con+used one tpe o+ +ace +or anot/er. Errors

8ere t/e percenta0e o+ +aces portrain0 a di6erent e?pression +rom

t/e predominant e?pression in t/e same pi%e Be.0., t/e num;er o+

sco8%in0 and 8ide eed +aces in a pi%e consistin0 predominant% o+

poutin0 +aces. Error tpes 8ere computed ; determinin0 t/e o2era%%

num;er o+ 8it/in=2a%ence Be.0., one ne0ati2e +ace con+used 8it/

anot/er or cross=2a%ence Be.0., one ne0ati2e +ace con+used 8it/ a

neutra% +ace errors. See Ta;%e +or a %ist o+ error tpes.

#


22/59


e used a modi>ed t=test to statistica%% compare patients< error

percenta0es to t/ose o+ t/e (( contro% participants. T/is met/od is

+requent% used in case studies to compare patient samp%es to contro%

samp%es.

#esults and "iscussion

Consistent 8it/ our /pot/esis t/at concept no8%ed0e is

necessar +or norma% emotion perception, contro% participants 8/o /ad

intact concept no8%ed0e +or discrete emotion spontaneous%

percei2ed sco8%in0, poutin0, 8ide=eed, 8rin%e=nosed, smi%in0 +aces,

and neutra% +aces, as instances o+ an0er, sadness, +ear, dis0ust,

/appiness and neutra% emotion. T/is occurred despite t/e open=ended

nature o+ our instructions to sort t/e cate0ories into t/ose t/e +ound

most @meanin0+u%. Contro% participants on a2era0e produced si? Bor

more pi%es to represent t/e discrete emotion cate0ories in t/e set and

tended not to con+use ne0ati2e +aces 8it/ one anot/er in t/eir pi%es

Bi.e., /ad %o8 ne0=ne0 error rates see Ta;%e . T/e maorit o+ contro%

participants BJ spontaneous% produced eit/er si? or se2en pi%es

and 5J o+ contro% participants produced si? or more. On% t8o contro%

participants B( produced +e8er t/an si? pi%es on t/e sort tas one

participant produced +our pi%es and one produced >2e. Nota;%, no

contro% participants produced t/ree pi%es. T/at on% one indi2idua% +rom

t/e contro% samp%e B#.# spontaneous% produced +our pi%es on t/e

sort tas, and none produced t/ree, stands in star contrast to t/e +act

##


23/59


t/at 77 o+ our patient samp%e produced +our or +e8er pi%es on t/e

sort tas. See Ta;%e +or contro%s< and patients< mean error rates and

$i0ure ' +or an e?amp%e o+ a contro% participantndin0s is t/at patients 8ere a;%e to percei2e

discrete emotion on +aces, ;ut mere% t/ou0/t t/at a6ect 8as t/e more

@meanin0+u% cate0or. Het patients< per+ormance on t/e 2arious

contro% tass e6ecti2e% ru%es out t/is a%ternate interpretation. $or

instance, no patient 8as a;%e to sort ; discrete emotion 8/en ased

to sort t/e +aces into si? cate0ories, or 8/en e?p%icit% ased to sort

into pi%es +or @an0er, @dis0ust, @+ear, @sadness, @/appiness or

@neutra%. Anot/er interpretation o+ our >ndin0s is t/at a6ect

perception 8as mere% easier +or patients t/an discrete emotion

perception. A0ain, t/e per+ormance o+ t/e contro% participants, a%on0

8it/ t/e per+ormance o+ t/e patients on t/e 2arious contro% tass, ru%es

out t/is a%ternate interpretation. I+ a6ect perception 8as easier t/an

discrete emotion perception, t/en contro% participants cou%d a%so /a2e

taen t/e @eas route and sorted +aces ; a6ect as 8e%%, ;ut t/e did

#'


24/59


not. More to t/e point, patients continued to sort ; a6ect on t/e

contro% tass, e2en 8/en t/ese tass pro2ided e?tra structure and

remo2ed co0niti2e %oad ; cuin0 patients to t/e num;er, appearance,

and e2en names o+ t/e discrete emotion cate0ories. T/e most

parsimonious e?p%anation o+ our >ndin0s is t/us t/at participants /ad a

preser2ed a;i%it to percei2e a6ect ;ut 8ere una;%e to percei2e

discrete emotion on +aces.

e ;e0in ; discussin0 t/e >ndin0s +rom patient E", 8/o 8as

our >rst case, and as a resu%t, per+ormed +e8er contro% tass t/an

su;sequent cases. e ne?t discuss t/e >ndin0s +rom patients $ and

CP, 8/o per+ormed a%% t/e contro% tass in our ;atter.

Patient E$

Emotional ree sort

Consistent 8it/ our /pot/esis t/at concept no8%ed0e is

necessar +or discrete emotion perception, ;ut not a6ect perception,

E" +ree sorted emotiona% +acia% e?pressions into t/ree pi%es Bsee $i0ure

( t/at /e %ater %a;e%ed @/app, @not/in0 and @rou0/. Compared to

contro%s, E" made more errors in 8/ic/ /e con+used ne0ati2e

Bsco8%in0, poutin0, 8ide=eed and 8rin%e=nosed +aces +aces 8it/ eac/

ot/er tB('#.4, pU.7 see Ta;%e V, indicatin0 t/at /e cou%d not

percei2e t/e di6erences ;et8een e?pressions +or an0er, dis0ust, +ear,

and sadness.

Face%anchored sort

#(


25/59


E"


26/59


and $, CP made more errors in 8/ic/ s/e con+used ne0ati2e +aces

8it/ one anot/er Bsco8%in0, poutin0, 8ide=eed and 8rin%e=nosed

+aces t/an did contro%s tB('.J7, pU.7- one=tai%ed see Ta;%e V,

;ut s/e rare% con+used ne0ati2e +aces +or positi2e Bsmi%in0 +aces.

A%t/ou0/ CP produced one pi%e t/at contained predominant% sco8%in0

+aces on t/e emotion +ree sort, 8e do not t/in t/is is e2idence t/at

s/e understood t/e cate0or o+ an0er. $irst, CP did not produce t/is

pi%e spontaneous%. S/e ;e0an t/e tas ; sortin0 +aces into t/ree pi%es

representin0 positi2e, neutra% and ne0ati2e a6ect Bpi%e ' and ( 8ere a

sin0%e pi%e, ;ut s/e random% sp%it t/is ne0ati2e pi%e into t8o ne0ati2e

pi%es +o%%o8in0 a cue +rom researc/ers t/at s/e cou%d c/ec /er pi%es

;e+ore mo2in0 on. T/e +act t/at s/e sp%it /er pi%e into additiona% pi%es

+o%%o8in0 a cue +rom t/e researc/ers su00ests t/at s/e mi0/t /a2e

rea%i:ed t/at t/ere s/ou%d ;e more cate0ories in t/e set Be2en i+ s/e

cou%d not percei2e t/em. Second, t/e name t/at CP spontaneous%

used to %a;e% t/ese t8o pi%es imp%ies t/at s/e did not see t/e +aces in

pi%e ' as cate0orica%% di6erent +rom t/e +aces in pi%e (. Rat/er, t/e

%a;e%s @not up and @rea%% not up su00est t/at s/e e?perienced t/e

+aces in /er t8o ne0ati2e pi%es as di6erin0 in intensit o+

unp%easantness Ba%t/ou0/ t/e +aces s/e p%aced in t/is pi%e 8ere not

rated as more intense ; a separate 0roup o+ /ea%t/ indi2idua%s.

$ina%%, as 8e discuss ;e%o8, CP did not s/o8 a consistent pattern o+

#J


27/59


distin0uis/in0 sco8%in0 +aces +rom ot/er ne0ati2e +aces in t/e

su;sequent sort tass t/at s/e and $ per+ormed.

At >rst ;%us/, it mi0/t a%so appear t/at ;ot/ $ and CP 8ere a;%e

to speci>ca%% percei2e dis0ust ;ecause t/e p%aced a%% 8rin%ed=nose

+aces in a sin0%e pi%e in t/e +ree sort tas, ;ut it is un%ie% t/at t/e

8ere disp%ain0 discrete emotion perception. /en ased to per+orm

%ater sorts Be.0., t/e num;er and 8ord anc/ored sorts, neit/er $ nor

CP continued to p%ace 8rin%ed nose +aces into a sin0%e pi%e Be.0., see

$i0ure J +or a depiction o+ CP


28/59


;et8een +acia% e?pressions ;ut did not understand t/e psc/o%o0ica%

meanin0 ;eond ;asic a6ecti2e 2a%ence.

Number%anchored sort

$ and CP per+ormed t/e num;er=anc/ored sort to pro2ide

additiona% support +or t/e interpretation t/at t/e did not percei2e si?

meanin0+u% cate0ories in t/e test stimu%i Be2en 8/en t/e 8ere cued to

t/e correct num;er. $ made >2e pi%es correspondin0 to a6ect Bone

pi%e +or smi%in0 +aces, one +or neutra% +aces, and +our pi%es containin0

2arious mi?es o+ ne0ati2e +aces in 8/ic/ /e con+used sco8%in0,

poutin0, 8ide=eed and 8rin%e=nosed +aces see Ta;%e # +or errors. CP

made si? pi%es correspondin0 to a6ect Bone pi%e +or smi%in0 +aces, one

+or neutra% +aces, and +our pi%es containin0 2arious mi?es o+ ne0ati2e

+aces in 8/ic/ s/e con+used sco8%in0, poutin0, 8ide=eed and 8rin%e=

nosed +aces see Ta;%e # +or errors. Nota;%, neit/er patient seemed to

t/in t/at si? cate0ories 8ere appropriate +or descri;in0 t/e perceptua%

cate0ories present in t/e +ace set. $ c/ose not to use t/e si?t/ anc/or

durin0 /is sort and CP spontaneous% ased 8/ 8e /ad ased /er to

sort t/e stimu%i into so man pi%es. T/is num;er=anc/or sort a%so

a%%o8ed us to o;ser2e t/e insta;i%it in ;ot/ patients< ne0ati2e pi%es

across sort tass. $or instance, a%t/ou0/ CP produced one pi%e in t/e

+ree sort t/at contained more sco8%in0 +aces t/an t/e ot/er pi%e, t/ese

sco8%in0 +aces 8ere distri;uted across t/ree ne0ati2e pi%es in t/e

#


29/59


num;er=anc/ored sort, indicatin0 t/at s/e did not in +act percei2e t/em

as mem;ers o+ a sin0%e co/erent emotion cate0or Bsee $i0ure -.

T/e insta;i%it in sortin0 t/at $ and CP demonstrated +rom one

tas to t/e ne?t is simi%ar to t/e insta;i%it in sortin0 t/at 8as o;ser2ed

in t/e patient LE, 8/o ;ecame ap/asic a+ter a stroe . LE produced

di6erent pi%es 8/en ased to sort +aces across t/ree di6erent

instances. T/ese >ndin0s su00est t/at 8it/out access to t/e meanin0

o+ 8ords, patients cannot mae re%ia;%e psc/o%o0ica% interpretations o+

discrete emotiona% +acia% e?pressions across instances. In comparison

to t/e ear%ier 8or 8it/ LE, our >ndin0s are no2e% in t/at our patients

demonstrated sta;%e a6ect perception across sort tass, e2en as t/e

cou%d not re%ia;% distin0uis/ sadness, +ear, dis0ust, and an0er +rom

one anot/er across tass. Moreo2er, un%ie LE, 8/o /ad de>cits in

%e?ica% retrie2a% ;ut not semantic memor, our patients< %ac o+ discrete

emotion concept no8%ed0e a2ai%a;i%it pro2ides t/e ;est test o+ t/e

/pot/esis t/at discrete emotion concept no8%ed0e is necessar +or

discrete emotion perception B;ut not a6ect perception.

ord%anchored sort

$ and CP ne?t per+ormed t/e 8ord=anc/ored sort to address t/e

possi;i%ities t/at t/e did not >nd discrete emotion cate0ories to ;e

t/e most @meanin0+u% cate0ories in t/e set, ;ut cou%d sort ; t/ese

cate0ories 8/en prompted, # t/at t/e 8ere mere% una;%e to

spontaneous% retrie2e t/e 8ords to support discrete emotion

#5


30/59


perception, ;ut t/at t/e cou%d per+orm t/e tas i+ 8e pro2ided t/e

correct 8ords +or t/em. Addin0 emotion 8ords to a discrete emotion

perception tas a%most a%8as impro2es /ea%t/ adu%ts< per+ormance

t/e are muc/ more @accurate at detectin0 t/e discrete emotiona%

meanin0 o+ a +acia% action Be.0., a sco8% 8/en ased to se%ect t/e

meanin0 +rom a %ist o+ 8ords Be.0., @an0er, @dis0ust, @+ear,

@/appiness, @sadness t/an 8/en t/e are ased to spontaneous%

0enerate t/e %a;e% t/emse%2es . Adu%ts remem;er +acia% e?pressions as

;ein0 more intense e?emp%ars o+ a particu%ar discrete emotion Be.0.,

/appiness 8/en t/e /a2e pre2ious% paired t/at +acia% e?pression

8it/ a 8ord Be.0., @/app t/an a non=8ord Be.0., a nonsense 8ord .

Pro2idin0 %a;e%s +or +acia% e?pressions can a%so impose t/e perception

o+ cate0ories 8/ere it did not e?ist ;e+ore in /ea%t/ adu%ts. E2en

oun0 c/i%dren are more accurate 8/en ased to matc/ a +ace Be.0.,

sco8% to a 8ord Be.0., @an0er t/an 8/en ased to matc/ a +ace to

anot/er +ace depictin0 t/e same e?pression Be.0., anot/er sco8% . Het

anc/orin0 t/e pi%es 8it/ emotion 8ords did not impro2e $


31/59


somet/in0 ;ad to ou did not /e%p, and patients cou%d not use t/is

in+ormation to mae meanin0 o+ t/e +acia% e?pressions posed in t/e

p/oto0rap/s. T/ese >ndin0s con>rm t/at our patients did in +act /a2e

impaired concepts +or emotion.

!dentity sort

$ina%%, $ and CP per+ormed t/e identit sort to ru%e out

a%ternate interpretations t/at t/e did not understand t/e instructions

o+ a sort tas, /ad 2isua% de>cits t/at impaired per+ormance on t/e

emotiona% sort tass, or /ad 0enera% e?ecuti2e impairments t/at 8ou%d

inter+ere 8it/ an sortin0 tas. &ot/ $ and CP sorted t/e +aces

per+ect% ; identit Bproducin0 7 errors, ru%in0 out t/at t/eir

per+ormance on t/e pre2ious tass 8ere caused ; ot/er co0niti2e or

perceptua% de>cits unre%ated to emotion concept no8%ed0e.

'onclusion

Our >ndin0s are consistent 8it/ rapid% 0ro8in0 e2idence t/at

emotion concept no8%ed0e supports t/e norma% perception o+ discrete

emotion cate0ories suc/ as an0er, dis0ust, +ear, sadness, etc. .

Pre2ious >ndin0s +rom our %a;s indicate t/at temporari% impairin0

access to and use o+ emotion concept no8%ed0e in /ea%t/ oun0

indi2idua%s impairs discrete emotion cate0ori:ation , inGuencin0 e2en

t/e +ormation o+ emotion percepts +rom t/e structura% +eatures o+

posed +aces . & contrast, addin0 8ords to a tas /e%ps /ea%t/

participants percei2e cate0orica% ;oundaries ;et8een posed a6ecti2e

'


32/59


+acia% e?pressions 8/ere t/e ot/er8ise cou%d not B$u0ate et a%. #77.

C/i%dren Biden * Russe%%, #77' and adu%ts are more accurate at

pairin0 a sco8% 8it/ t/e 8ord @an0er t/an 8it/ anot/er sco8%in0 +ace,

su00estin0 t/at 8ords si0ni+in0 concept no8%ed0e mi0/t actua%%

add somet/in0 to t/e perception o+ a discrete emotion, trans+ormin0 a

percept o+ a ne0ati2e +ace into a discrete percept o+ an0er. E2en

neuroima0in0 e2idence is consistent 8it/ t/e idea t/at concept

no8%ed0e p%as a ro%e in constructin0 instances o+ discrete emotion

;rain areas in2o%2ed in t/e representation o+ semantic no8%ed0e suc/

as t/e media% pre+ronta% corte?, anterior tempora% %o;e, media%

tempora% %o;e, and 2entro%atera% pre+ronta% corte? are routine%

in2o%2ed in ;ot/ emotiona% perceptions and e?periences across t/e

neuroima0in0 %iterature . Our data t/us add a crucia% dimension to t/is

%iterature ; demonstratin0 t/at adu%ts 8it/ semantic impairment due

to anterior tempora% %o;e neurode0eneration cannot percei2e an0er,

sadness, +ear, or dis0ust as discrete emotions in peop%ecits o;ser2ed on discrete emotion perception tass are

t/ou0/t to stem +rom diFcu%ties %a;e%in0 stimu%i, not +rom diFcu%ties in

discrete emotion perception per se . Our >ndin0s s/o8 t/at patientsndin0 is consistent 8it/ e2idence s/o8in0

t/at in+ants, todd%ers, and non=/uman primates, 8/o %ac sop/isticated

%an0ua0e capacit, can percei2e a6ecti2e 2a%ence in +aces, 2oices, and

;odies e2en 8/en t/e do not re%ia;% distin0uis/ discrete emotiona%

e?pressions +rom one anot/er . On% as c/i%dren acquire t/e meanin0 o+

emotion 8ords suc/ as @an0er, @+ear, @sadness and @dis0ust 8it/

norma% de2e%opment do t/e acquire t/e a;i%it to re%ia;% distin0uis/

;et8een sco8%in0, 8ide eed, poutin0 and 8rin%ed nosed +aces as

e?pressions o+ t/ese cate0ories . T/ird, t/is >ndin0 is consistent 8it/

cross=cu%tura% e2idence t/at a%% cu%tures percei2e 2a%ence on +aces,

e2en amidst di6erences in t/e speci>c discrete emotions t/e percei2e

on +aces or e2en di6erences in 8/et/er discrete emotions are

percei2ed at a%% . $ina%%, simi%ar to ot/er @%ast in, >rst out t/eories o+

de2e%opment 2s. neurode0eneration, our >ndin0s are consistent 8it/

ot/er researc/ on semantic dementia documentin0 t/e pro0ressi2e

@prunin0 o+ concepts o2er t/e course o+ disease pro0ression +rom t/e

semantica%% su;ordinate %e2e% Be.0., %ion 2s. ti0er to t/e ;asic %e2e%

Be.0., cat 2s. do0 to t/e superordinate %e2e% Be.0., anima% 2s. p%ant . To

our no8%ed0e, our >ndin0s are t/e >rst to demonstrate a simi%ar

pattern o+ @prunin0 +or emotion concepts. A%t/ou0/ it is not

addressa;%e in t/e present stud, +uture researc/ mi0/t use

%on0itudina% met/ods to speci>ca%% in2esti0ate t/e @prunin0

'J


37/59


/pot/esis as it pertains to discrete emotion 2. 2a%ence perception. I+

2a%ence ud0ments re% on concept no8%ed0e, and 2a%ence concepts

are superordinate to discrete emotion concepts, t/en 8e mi0/t e?pect

2a%ence perception to diminis/ o2er t/e course o+ neurode0eneration,

+o%%o8in0 discrete emotion perception. Het i+ intact 2a%ence perception

re%ies on ot/er psc/o%o0ica% mec/anisms ;esides concept no8%ed0e

per se t/en it is possi;%e t/at 2a%ence perception 8i%% ;e maintained

o2er t/e course o+ t/e disease.

!mplications

T/e o;ser2ation t/at peop%e 8it/ semantic dementia /a2e

preser2ed a6ect perception ;ut impaired discrete emotion perception

/as important imp%ications +or ;ot/ ;asic and app%ied science. T/ese

>ndin0s cannot ;e accommodated ; @;asic emotion accounts 8/ic/

assume t/at emotiona% e?pressions are @psc/o%o0ica% uni2ersa%s and

constitute a set o+ ;asic, e2o%2ed +unctions, t/at are s/ared ; a%%

/umans and 8/ic/ /a2e e2o%2ed +rom ear% primate communication .

A%t/ou0/ our >ndin0s run contrar to ;asic emotion mode%s, our stud

8as not desi0ned to speci>ca%% test ot/er mode%s o+ emotion, suc/ as

appraisa% mode%s, t/at t/emse%2es mae no predictions a;out t/e ro%e

o+ %an0ua0e and conceptua% no8%ed0e in emotion perception.

Appraisa% mode%s mae speci>c predictions a;out t/e ro%e o+ appraisa%

@c/ecs or @action tendencies +or t/e production o+ +acia%

e?pressions. Aside +rom e2idence su00estin0 t/at /ea%t/ adu%ts mae

'4


38/59


appraisa%=consistent persona%it in+erences a;out sco8%in0, poutin0

and smi%in0 +aces , t/ere is %itt%e e2idence speci>ca%% assessin0 t/e

ro%e o+ appraisa% c/ecs in emotion perception. Re%ati2e% more studies

/a2e addressed t/e ro%e o+ action tendencies durin0 emotion

perception, 8it/ most studies +ocusin0 on t/e percei2ernds t/e

situation certain or action tendencies Be.0., 8/et/er t/e person

e?pressin0 emotion is %ie% to Gee t/e situation or 8ou%d mae t/e

percei2er 8ant to Gee t/e situation. Nor did an o+ t/e patients in our

samp%e mae comments re%ated to appraisa%s or action tendencies

8/en sortin0 +aces Be.0., none said @/e %oos uncertain or @/e is 0oin0

to run a8a. One possi;i%it is t/at t/e content o+ appraisa%s

concernin0 t/e situation Be.0., no8in0 8/et/er t/e situation in 8/ic/

emotion occurs is certain, contro%%a;%e, etc. and no8%ed0e a;out

8/ic/ action tendencies accompan certain emotions are part o+ t/e

discrete emotion concept no8%ed0e t/at ;ecomes impaired in

'


39/59


40/59


Ac(nowled)ements

K.A.L. and M.". contri;uted equa%% to manuscript preparation and

s/ou%d ;e considered oint >rst aut/ors. L.$.&. and &.C.D. contri;uted

equa%% and s/ou%d ;e considered oint senior aut/ors. e t/an Dere

Issaco8it: and Xenn Stan%e +or t/eir assistance in co%%ectin0 contro%

data. T/is researc/ 8as supported ; a Nationa% Institutes o+ 3ea%t/

Director


41/59


(


42/59


Fi)ure *. M#! scans o patients E$, F& and 'P.T=8ei0/ted MRI

scans o+ Ba patient E" B; patient $ Bc and patient CP s/o8in0 %e+t=

%atera%i:ed anterior tempora% %o;e atrop/ ima0es are s/o8n in

radio%o0ica% orientation Bt/e %e+t /emisp/ere appears on t/e ri0/t /and

side o+ t/e ima0e.

(#


43/59


Fi)ure +.Eample o ace stimuli. E?amp%es o+ +ace stimu%i +rom

t/e IASLa; set used in t/e +ace sort tass.

('


44/59


Fi)ure -. Eamples o a control participants perormance on

the ree sort tas(. A J5=ear o%d man made si? pi%es to represent t/e

si? cate0ories.

((


45/59


Fi)ure /.Patients perormance on the emotion ree sort tas(.

In E"rst pi%e contained predominant% /app +aces,

(-


46/59


t/e second pi%e contained predominant% neutra% +aces and t/e t/ird

pi%e contained predominant% ne0ati2e +aces Bsco8%in0, poutin0, 8ide=

eed and 8rin%e=nosed +aces. In $rst pi%e contained

a%% /app +aces, t/e second pi%e contained a%% neutra% +aces, and t/e

t/ird and +ourt/ pi%es contained a%% ne0ati2e +aces. In CPrst pi%e contained predominant% /app +aces, t/e second pi%e

contained a%% neutra% +aces, and t/e t/ird and +ourt/ pi%es contained a%%

ne0ati2e +aces.

(J


47/59


Fi)ure 0. /en ased to sort +aces into si? pi%es anc/ored 8it/ t/e

num;ers =J, CP created one pi%e +or positi2e +aces, one +or neutra%

+aces and +our +or ne0ati2e +aces. T/is tas indicated t/e insta;i%it in

/er sortin0 +rom one instance to t/e ne?t.

(4


48/59


Table 1. Emotion Free Sort Performance

#

piles

NEG-NEUT POS-NEUT NEG-NEG NEUT-NEG POS-NEG NEG-POS NEUT-POS

%

Total

Errors

EGa 3 1.66% 0% 46.67%* 5.83% 0% 1.66% 5.83%* 61.66%

FZb 4 0% 0% 44.44%* 0% 0% 0% 0% 44.44%

CPb 4 0% 0% 36.11%* 0% 0% 2.77% 0% 38.89%

O!a

7.82

(SD=

2.99)

2.88% 0.13% 21.72% 2.80% 0.27% 0.55% 1.69% 30.04%

a long variant of free sort

s!ort variant of free sort

* in"i#ates statisti#all$ "ifferent fro #ontrols (&'.05)

!bbre"iations

S$ stan"ar" "eviation

O! ol"er a"lts

NEG-NEUT errors in !i#! negative fa#es ere &t in a &ile of &re"oinantl$ netral fa#es

POS-NEUT refers to errors in !i#! &ositive fa#es ere &t in a &ile of &re"oinantl$ netral fa#es

NEG-NEG errors in !i#! one t$&e of negative fa#e as &t in a &ile #onsisting &re"oinantl$ of anot!er negative fa#

NEUT-NEG errors in !i#! netral fa#es ere &t in a &ile of &re"oinantl$ negative fa#es

POS-NEGerrors in !i#! &ositive fa#es ere &t in a &ile of &re"oinantl$ negative fa#es

NEG-POS errors in !i#! negative fa#es ere &t in a &ile of &re"oinantl$ &ositive fa#es

NEUT-POS errors in !i#! netral fa#es ere &t in a &ile of &re"oinantl$ &ositive fa#es


49/59

Emotion perception in semantic dementia (5


50/59


Emotion perception, but not afect perception, is impaired with

semantic memory loss

Supplementary 1nline Materials

Additional Patient Data

Patients 8ere tested in t/e neuro%o0 c%inic at a %oca% &oston

/ospita%. e report t/e neuropsc/o%o0ica% tests t/at 8ere

administered as part o+ a ;atter per+ormed ; a trained

neuropsc/o%o0ist.

'ase *

'ase history and presentation

E" is a 47=ear=o%d ma%e /i0/ sc/oo% 0raduate 8/o 8ored +or

most o+ /is adu%t %i+e as an en0ineer at a construction compan. 3e

le . Performance on !ll Emotion Sort Tas&s

# piles NEG-

NEUT

POS-

NEUT

NEG-NEG NEUT-NEG POS-NEG NEG-POS NEUT-POS

e sort 4 0% 0% 44.44% 0% 0% 0% 0%

er an#!or 5 0% 0% 33.33% 0% 0% 2.78% 2.78%

e an#!or 8 0% 0% 19.44% 0% 0% 0% 0%

r" an#!or 6 0% 0% 27.78% 0% 0% 2.78% 0%

e sort 4 0% 0% 36.11% 0% 0% 2.77% 0%

er an#!or 6 2.86% 0% 34.29% 0% 0% 0% 2.86%

e an#!or 6 5.71% 0% 17.14% 0% 0% 0% 0%

r" an#!or 6 5.71% 0% 31.43% 0% 0% 0% 0%


51/59


presented in #77J 8it/ 0radua%% pro0ressi2e smptoms o+ diFcu%t

8it/ @memor and 8ord >ndin0 in speec/. At t/e time o+ presentation,

/is +ami% reported t/at /e 8as /a2in0 diFcu%t remem;erin0 8ords

and /ad stopped readin0 ;oos. 3e 8as sti%% doin0 t/e +ami% >nances

8it/out diFcu%t. 3is +ami% did not report c/an0es in mood or

persona%it. On /is >rst e2a%uation in #77J, /e demonstrated 8ord=

>ndin0 diFcu%ties and anomia ;ut norma% a6ect and comportment.

Structura% MRI demonstrated +oca% atrop/ o+ t/e %e+t tempora% po%e

and /e 8as dia0nosed 8it/ Primar Pro0ressi2e Ap/asia=Semantic

2ariant o+ semantic dementia. $indin0s +rom a +u%% neuropsc/o%o0ica%

;atter per+ormed in #77J are as +o%%o8s.

!ntellectual abilities.T/is patient /ad norma% inte%%ectua%

a;i%ities.3e /ad an a2era0e estimated inte%%i0ence score on t/e

esc/%er Test o+ Adu%t Readin0 and per+ormed superior% on t/e

Ra2en


52/59


Memory.T/is patient /ad norma% reco0nition memor, ;ut

consistent 8it/ t/e semantic dementia dia0nosis, /ad poor

per+ormance on t/e 8ord reca%% portion o+ t/e Consortium to Esta;%is/

a Re0istr +or A%:/eimer


53/59


one minute eac/ a%t/ou0/ /e 8as sti%% a;%e to repeat %en0t/

sentences. 3e /ad se2ere impairments in ;ot/ immediate and de%aed

2er;a% memor and 8as no %on0er a;%e to use cues to increase /is

memor per+ormance Bno 8ords reca%%ed a+ter a ;rie+ de%a. 3is Mini

Menta% State E?am score o+ 5Q'7 8as impaired on% as a resu%t o+ /is

8ord retrie2a% impairment.

Amidst t/ese %an0ua0e de>cits, E" demonstrated norma%

memor +or 2isua% materia% and norma% 2isua% construction Be.0., /e

8as a;%e to dra8 a c%oc and p%ace t/e /ands correct% to 7 minutes

a+ter . 3is ca%cu%ationa% a;i%ities remained norma% /e 8as a;%e to

per+orm simp%e addition and su;traction. 3is mood 8as ud0ed

c%inica%% to ;e norma% and /e demonstrated appropriate a6ect durin0

c%inica% and researc/ 2isits. 3is son reported no si0ni>cant ;e/a2iora%

pro;%ems at /ome.

'ase +


$ is a J(=ear o%d ma%e 8it/ a Masters o+ &usiness

Administration and Certi>ed Pu;%ic Accountant de0ree, 8/o 8ored as

an accountant. 3e presented in #775 8it/ 0radua%% pro0ressi2e 8ord

>ndin0 diFcu%ties. In particu%ar, /e +or0ot names o+ peop%e /e /ad

no8n +or man ears. Once an a2id reader, /e ;e0an /a2in0

diFcu%ties +o%%o8in0 ne8spaper artic%es. 3e reported mi%d depression

and an?iet as a resu%t o+ /is smptoms, ;ut 8as not dia0nosa;%e 8it/

-'


54/59


a +orma% mood disorder. 3e /ad no ;e/a2iora% smptoms or persona%it

c/an0e. On /is >rst e2a%uation, /e demonstrated si0ni>cant 8ord

>ndin0 diFcu%ties and semantic parap/asic errors durin0 casua%

con2ersation. Structura% MRI demonstrated ;i%atera% tempora% po%e

atrop/ 8it/ more prominent atrop/ in t/e %e+t tempora% po%e. 3e 8as

dia0nosed 8it/ PPA=S Bsemantic dementia. $indin0s +rom a +u%%

neuropsc/o%o0ica% ;atter per+ormed in #775 are as +o%%o8s.


a;i%ities. 3e /ad a2era0e Perceptua% Reasonin0 and orin0 Memor

on t/e esc/%er Adu%t Inte%%i0ence Sca%e=I. 3e per+ormed in t/e /i0/

ran0e on Matri? Reasonin0 B5stpercenti%e.

Attention and Eecuti2e unction.T/is patient /ad norma%

attention and e?ecuti2e +unction. 3is di0it span 8as /i0/ a2era0e B4

+or8ard and - in re2erse, as 8as /is 2isua% attention B+or8ard span J,

;ac8ard span (=-. 3e /ad no diFcu%t on a 0rap/omotor

sequencin0 tas Ba%ternatin0 M and N. Per+ormance on t/e DKE$S Trai%

Main0 Test 8as ;order%ine BTrai%s A, (' seconds, (t/ percenti%e Trai%s

&, 7 seconds, #nd percenti%e. 3e demonstrated no diFcu%t on a

0oQno=0o tas. 3is 8as a;%e to quic% %earn a seria% /and mo2ement

sequence 8it/ /is ri0/t /and and 8as a;%e to trans+er %earnin0 to /is

%e+t /and. Concept +ormation and co0niti2e Ge?i;i%it 8as preser2ed

BCST, JQJ cate0ories.

-(


55/59


56/59


tar0et 8ordYs meanin0 Be.0., does naZ2e mean innocent or stupidW +e%%

in t/e impaired ran0e. In contrast, /is a;i%it to access ot/er aspects

spoen %an0ua0e, inc%udin0 p/ono%o0ica%, morp/o%o0ica% and sntactic

in+ormation 8as intact. 3e 8as a;%e to distin0uis/ 8ords +rom non=

8ords, to distin0uis/ 8ord meanin0 ;ased on morp/o%o0 Be.0.,

emp%oer 2s. emp%oee, ;us 2s. ;usses, and 8as a;%e to appreciate

t/e sntactic +orm o+ sentences as assessed on a sentence=picture

matc/in0 tas, /o8e2er, and a%% scores +e%% in t/e a2era0e ran0e. &asic

compre/ension usin0 t/e toen test 8as preser2ed.

Pro5le at time o emotion testin)

& #7#, 8/en $ comp%eted t/e present e?perimenta% tass, /e

/ad under0one pro0ressi2e impairment in t/e domain o+ %an0ua0e and

semantic memor. 3e 8as e?/i;itin0 anomia and semantic memor

impairment 8it/ Guent and articu%ate speec/. Per+ormance 2er;a%

Guenc tass 8as impaired Ber;a% Guenc +or anima%s 2e0eta;%es

#. 3is Mini Menta% State E?am 8as #-Q'7. er;a% memor encodin0

8as impaired ;ut /e retained 8/at /e %earned a+ter a #7=minute de%a

Bec/s%er Lo0ica% Memor Immediate ( De%aed (.

Amidst t/ese %an0ua0e de>cits, $ disp%aed norma% 2isuospatia%

+unction B/e 8as a;%e to cop a picture o+ a penta0on on t/e Mini

Menta% State E?am and accurate% dra8 a c%oc 8it/ t/e instructed

time. A%t/ou0/ some aspects o+ e?ecuti2e +unction demonstrated

-J


57/59


pro0ressi2e impairment +rom #775 BTrai% Main0 Test A -7s Trai%

Main0 Test & #' s, ot/ers remained re%ati2e% intact Be.0., di0it span

+or8ard 4, ;ac8ard, J. 3is mood 8as ud0ed c%inica%% to ;e norma%

and /e demonstrated appropriate a6ect durin0 c%inica% and researc/

2isits. 3is 8i+e reported no ;e/a2iora% pro;%ems at /ome.

'ase -


CP is a -'=ear=o%d +ema%e 8it/ a Masterndin0 diFcu%ties.

At t/e time o+ presentation, /er +ami% reported t/at s/e /ad ;een

stru00%in0 to >nd 8ords Bmost% nouns and /ad e?perienced some

minor diFcu%ties 8it/ memor retrie2a% +or con2ersations. T/e denied

an c/an0es in mood or persona%it. On /er >rst e2a%uation, s/e

demonstrated 8ord=>ndin0 diFcu%t 8it/ anomia and pauses durin0

spontaneous speec/ ;ut norma% a6ect and comportment. Structura%

MRI demonstrated %e+t tempora% po%e atrop/. S/e 8as dia0nosed 8it/

PPA=S Bsemantic dementia. $indin0s +rom a +u%% neuropsc/o%o0ica%

;atter per+ormed in #77 are as +o%%o8s.


a;i%ities. S/e /ad a /i0/ a2era0e estimated inte%%i0ence score on t/e

esc/%er Test o+ Adu%t Readin0.

-4


58/59


Attention and Eecuti2e unction.T/is patient /ad norma%

attention and e?ecuti2e +unction. Per+ormance 8as in t/e superior

ran0e on an auditor attention span test and a2era0e on t/e Menta%

Contro% su;test o+ t/e esc/%er Memor Sca%e III. Di0it span 8as 4

+or8ard and J in re2erse. Per+ormance on a num;er=%etter sequencin0

tas 8as in t/e a2era0e ran0e. isconsin Card Sort 8as per+ormed at

an a2era0e %e2e% 8it/ no perse2erati2e errors and J cate0ories

o;tained. Per+ormance on DKE$S Trai% Main0 Test 8as in t/e /i0/

a2era0e to superior ran0e. Co%or 8ord inter+erence 8as in t/e a2era0e

to /i0/ a2era0e ran0e.

Memory.T/is patient /ad norma% memor. Per+ormance on t/e

esc/%er Memor Sca%e III Lo0ica% Memor 8as in t/e /i0/ a2era0e

ran0e +or immediate reca%% B4-t/percenti%e, 8it/ '7 minute de%aed

reca%% in t/e /i0/ a2era0e ran0e B(t/percenti%e. One /undred percent

o+ encoded materia% 8as retained a+ter t/e de%a. isua% memor 8as in

t/e /i0/ a2era0e ran0e B#7=minute de%aed reca%% o+ Re=O >0ure

Jt/percenti%e.

3isuospatial s(ills.T/is patient /ad norma% 2isuospatia% si%%s.

3er 2isuospatia% attention 8as /i0/ a2era0e on t/e esc/%er Adu%t

Inte%%i0ence Sca%e III. Re=O >0ure cop 8as 5-t/percenti%e.

4an)ua)e. Consistent 8it/ t/e semantic dementia dia0nosis,

t/is patient /ad impaired per+ormance on %an0ua0e tests. Per+ormance

on t/e &oston Namin0 Tas 8as impaired B4Q'# spontaneous%

-


59/59


additiona% correct response 8as o;tained 8it/ p/onemic cues

semantica%% correct descriptions 8ere o+ten o;tained Be.0., @ou t/ro8

it +or dart. Semantic access +rom pictures 8as re%ati2e% maintained

on t/e Pramids and Pa%m Trees test B(5Q-#. Le?ica% 2er;a% Guenc

8as a2era0e Btota% $AS #5 ;ut semantic 2er;a% Guenc 8as

;order%ine BDKE$S cate0or tota% #-.

Pro5le at time o emotion testin)

& #7, 8/en CP comp%eted t/e present e?perimenta% tass,

s/e /ad under0one pro0ressi2e impairment in t/e domain o+ %an0ua0e

and semantic memor. S/e 8as e?/i;itin0 se2ere anomia and semantic

memor impairment 8it/ Guent and articu%ate speec/. 3er Mini Menta%

State E?am 8as #4Q'7, 8it/ errors due to %an0ua0e de>cits. Amidst

t/ese %an0ua0e de>cits, /er ;asic ca%cu%ationa% si%%s Baddition and

su;traction, 2isuospatia% si%%s Be.0., s/e 8as a;%e to cop a picture o+

a penta0on on t/e Mini Menta% State E?am and accurate% dra8 a c%oc

8it/ t/e instructed time and attention remained intact. 3er mood 8as

ud0ed c%inica%% to ;e norma% and s/e demonstrated appropriate a6ect

and comportment durin0 c%inica% and researc/ 2isits. 3er /us;and

reported no ;e/a2iora% pro;%ems at /ome.

-5

Documents

Essay Feldman Barrett Emotion Perception in Semantic Dementia