Rationalitéetvaleurcommunicativedusourireetdu

riredanslesyndromed'Angelman

FabienMathy,NatachaNaudet&SandrineVieillard

RencontresNationalesAFSA19&20janvier2018,CISL,LYON

De nombreuses descriptions scientifiques ou vulgarisées dusyndromed'Angelmansuggèrentquelesriressontnonseulementexcessifs,mais aussi immotivés (sans raison précise). Néanmoins,l'observation de nombreux parents est que cette expressionjoyeuseestàlafoisrationnelleetcommunicative.Nousprésentonsles résultats d'une étude expérimentale comparant des enfantsatteintsdusyndromed’Angelmanàungroupetémoinquivisaitàtester l'hypothèse que les réactions joyeuses des enfantsAngelman, bien que plus exubérantes, sont rationnelles etadaptéesàdescontextesappropriés.

Résumé

Ø IntroductionØ ProblématiqueØ HypothèsesØ MéthodologieØ RésultatsØ Conclusionetlimitesdel’étudeØ Perspectives

Plan

Ø Introduction*  DescriptionduSA

Plan

-  Étiologie non disputable (Williams, Driscoll, & Dagli,2010)

-  Descriptions cliniques claires (Angelman, 1961; Dan,2008),àl’exceptionparfoisdelacaractérisationdeladispositionjoyeuse:

-  « A happy dispositionwith frequent laughter » (WIKI, English), ouencore«withexcessiflaugther»(Williansetal.2010)

-  «Lesenfantssonttrèsjoyeux,riantdefaçoninappropriée»(WIKIFr.)

LeSyndromeAngelman

Syndrome d’Angelman (1/~20000)

CouverturedeDan(2008)

DispositionjoyeusedansleSA

•  L’occurrence accrue du rire dans le SA pourrait être liée à un déficitsocialgénéralisécaractérisantcesyndrome(Joleff&Ryan,1993).

•  LeriredansleSAprésenteunfaibleseuild'occurrenceet lescrisesderire tendent à être relativement fréquentes et dissociées desévénementscontextuels(Bower&Jeavons,1967;Elan,1975).

•  Le rire dans le SA est décrit comme « incessant », et arrivant sans

réponseàdesstimulationsenvironnantes(Nirenberg,1991).

² Endébat(Pelc,Cheron,&Dan,2008)² Caractèrepathognomonique(Summersetal.,1995)² Descriptionsplusdépréciatives:

Rirepathologique

²  Reliéàdesépilepsiesgélastiques(criseconsistantenunaccèsderirepréférentiellementliéeàunfoyerlocalisé)

²  Causéparuntraumatismecrânien(pathologicallaughingandcrying;cf.Tateno,Jorge,&Robinson,2004).

²  Causéparunaccidentvasculairecérébral (Wildetal., 2003).Cesauteursmontrentqu’ilexistedeuxvoiesneuronalessous-jacentesàl’expressiondurire:unevoieinvolontairesous-corticaleetunevoieplusvolontairecorticale(double-dissociation: impossiblepourcertainspatientscapablesdesourirespontanémentdesourirevolontairement;d’autrespatientsincapablesd’émotionspeuventbougersurcommandeleszygomatiques)

²  Fourireprodromique(Féré,1903)

Pourallerplusloin:Cf.ClassificationdesrirespathologiquesparPoeck(1969,1985)

La disposition joyeuse dans le syndrome d’Angelman ne correspondenrienauxcasrépertoriésderirepathologique…

Question

Lespersonnesporteusesd’unSyndromed’Angelmanrient-ellesdefaçonimmotivéecommelesuggèrentcertainesdescriptionsdusyndrome?Oudefaçonplusinternecommecelapeutêtreobservédanslaschizophrénieoulestroublesduspectreautistique(Huddenko,Stone,&Bachorowski,2009).

Questionsous-jacente(Adams&Oliver,2011)

Lespersonnesprésentantunhandicapmentalexpriment-elleslamêmepaletteémotionnellequelespersonnessanshandicap?Réponsepeuévidenteenraisondelapaucitédelalittérature:Lesauteursnotentquesurplusde2500articlesscientifiquessur30ans,seulement18concernentlehandicapmentalsévère,etparmiceux-ci,seulement3concernentlajoie.

Contre-exemplesdutableaudépréciatif

…

Résumé

Résumé

Stimulationsocialeet/ouvisuelle

Ø Introduction*  Développementdurire

Plan

*  Lespremierssouriresapparaissentpendantlesommeilparadoxal(Emde&Koening,1969)*  Unpeuplustard,lorsquel’enfantestsurlepointdes’endormir(Wolff,1987)*  Al’éveil,lesourireapparaîtle1ermois(Kraemeretal.,1999),lerireversle4emois(Ruch&

Ekman,1997)*  Vers2mois,lebébécommenceàréagiràdesévénementsexternes(Sroufe&Waters,1976),

telsquelavued’unvisage(Anisfeld,1982;Spitz,1946).*  Laquantitédesourireaugmentedurantles6premiersmois(Gewirtz,1965;Messinger,Fogel,

&Dickson,1999)etlesourireintervientprogressivementdansunerelationmutuelle(Kaye&Fogel,1980).*  Vers9mois,lesourireestutilisépourpartagerl’attentionenverslesobjetsd’intérêt(Morales

etal.2000).

Développementnormaldurire

4

ClmekRms lg Joy-Happhmss Memis

8

No Cheek Raising

6

5

4

Vel3, Sh'ong Smile,Mouth Open

8

7

6

4

Minimal Smile.Mouth Open

8

7t6

4

Strong Smile,Mouth Closed

81tong Smile,Mouth Closed

7

6

5

Figure 1. Positive emotion perceived in different types of infant smiles. Meanjoy-happiness ratings (on a scale from 0-8) and their standard errors for each pairof smiles. The smiles on the left are cheek-raise (Duchenne) smiles, and thesmiles on the right are not. The smiles in each horizontal pair were created withan identical smiling mouth. In each column, the top two smiles are open-mouthsmiles and the bottom two are not. Pairs of smiles also differ in the strength of thesmile portrayed. Adapted with permission from "Positive and negative: Infantfacial expressions and emotions," by D. Messinger (in press).

4

ClmekRms lg Joy-Happhmss Memis

8

No Cheek Raising

6

5

4

Vel3, Sh'ong Smile,Mouth Open

8

7

6

4

Minimal Smile.Mouth Open

8

7t6

4

Strong Smile,Mouth Closed

81tong Smile,Mouth Closed

7

6

5

Figure 1. Positive emotion perceived in different types of infant smiles. Meanjoy-happiness ratings (on a scale from 0-8) and their standard errors for each pairof smiles. The smiles on the left are cheek-raise (Duchenne) smiles, and thesmiles on the right are not. The smiles in each horizontal pair were created withan identical smiling mouth. In each column, the top two smiles are open-mouthsmiles and the bottom two are not. Pairs of smiles also differ in the strength of thesmile portrayed. Adapted with permission from "Positive and negative: Infantfacial expressions and emotions," by D. Messinger (in press).

Grandszygomatiques

MuscleorbiculaireDel’oeil

Sourirequidénotel’expressiond’unejoieinterne(Fox&Davidson,1988)

Musclesimpliqués:

EtudedeCicchetti&Sroufe(1976)

026 Child Developmentports the generality of the view that developmentis integrated and organized. At the same time, thedetailed longitudinal data provide considerable in-formation about Down's syndrome babies. Whilethe nature of the developmental process is similar,the picture of development with these childrendiffers substantially from that of normal infants.

Affectioe and cognítíoe deoelopmenú.-TheDown's syndrome inånts showed a progression inthe expression of positive affect similar to that oftheir normal counterparts, though the rate of de-velopment was, of course, much slower. Down'ssyndrome infants smiled and laughed ßrst at theintrusive, physically vigorous tactile and auditoryitems. Only much later, while afiective expressionto the auditory and tactile items was declining,did they smile and laugh a great deal to the itemswith a more explicit element of cognitive incon-gruity (see fig. 2).

This is the same observation that previouslysuggested a tie between cognitive developmentand laughter in normal infants. The progression inboth retarded and normal infants is from affectiveresponse to intrusive stimulation, to stimulationmediated by active attention, then to stimuluscontent or meaning, toward an ever more activeinvolvement in producing the stimulus itself(Sroufe & Waters 1976). Increasingly, it is notstimulation but the infant's effort in processingstimulus content or the infant's participation in theevent that produces the tension necessary for smil-ing and laughter.

With the Down's syndrome infants, it was notuntil the first half of the second year (vs. the sec-ond half of the first year with normal infants) thatitems in which the infant participated becameespecially salient. The "tug" item (no. 16) exem-

¡l08to12t¡¡ier0

Age in Months

Ftc. 2.-Month-by-month smiling of Down's syn-d¡ome i¡;,fants to the four categories of items.

plites the role of active participation at this time aswell as the afiective-cþgDitive interchange. It wasonly when the baby was actively able to participatein the game by reciprocally tugging the yarn thatafiective expression resulted. Simílarly, smilingand laughing resulted only when the infant spon-taneously grabbed the cloth from its own face (no.l9), or reached for the mother's tongue (no. 20),not when, for example, the mother placed the in-fant's hand on her tongue. During this sameperiod, efforts to get the mother to repeat theitem, for example, stufffng the cloth back in hermouth, and anticipatory smiling (i.e., smiling inadvance of item completion, ê.g., before themother's return in peek-a-boo), were also seen.This progressively more active involvement hasparallels in various realms of development duringinhncy, such as persistence in the pursuit of anobject or other goal (Brazelton 1969; Buhler 1930).Our data clearly indicate that this general de-velopmental principle applies also to Down's syn-drome infants.

The most compelling data with respect to theafiect-cognition tie arê from the cognitive test re-sults. Cognitive developmental level, as measuredby performance at 13 months on the Uzgiris-Huntscales and at 16 months on the Bayley MentalScale, paralleled the level of affectíÇe develop-ment as assessed by the 30laughter items. [n fact,the integrated nature of development-the closetie between affect and cognition-was more read-ily demonstrated with this group of retarded in-fants than with normal infants. With their retardedrate of development, changes in performancewere spread out over time, and at a given agethere was a great deal of variation between sub-jects. Infants' rankings on onset of smiling andlaughter, developmental level of smiling andlaughter'assessed by various criteria, onset ofnegative reactions in response to our items, andmotor and mental development were remarkablyconcordant (see table 3).

In light of this evidence for the close tie be-tween affective and cognitive development, andgiven the relative ease of assessing affective ex-pression i¡r the "sensory-affective" infant (Steehler& Carpeùter 1967), more emphasis should begiven to affect in studies of general development.The most r¡sefi.rl strrdies will be those that examineaffect and cognition concurrently,

The role of tension in affect expression.-Thefindings with this retarded sarnple are also rele-vant to a hypothesis concerning the role of tensionin afnective expression (Sroufe & Waters 1976).Down's syndrome infants as a group arehypotonic, so a lag in affect expression would be

Cl 80-IJ.t to

€rto3Ê 50otr-=õ 'oO30IJbzoÀ t0

- Soclal

..'...'Visual

- Tactilo

- Auditory

Aucoursdelapremièreannée,onobservechezl’enfantnormaluneaugmentationdessouriresetdesrirespourdesstimulicomplexesdenaturesocialeouvisuelle.Cesréactionssupplantentdesréactionsplusprimairesauxstimuliintensestactilesetauditifs(chatouilles,variationsdelavoix).Ici,lemêmepatternapparaîtavecunretardd’environ6moischezdesenfantsatteintsdusyndromedeDown.

EtudedeCicchetti&Sroufe(1976)

026 Child Developmentports the generality of the view that developmentis integrated and organized. At the same time, thedetailed longitudinal data provide considerable in-formation about Down's syndrome babies. Whilethe nature of the developmental process is similar,the picture of development with these childrendiffers substantially from that of normal infants.

Affectioe and cognítíoe deoelopmenú.-TheDown's syndrome inånts showed a progression inthe expression of positive affect similar to that oftheir normal counterparts, though the rate of de-velopment was, of course, much slower. Down'ssyndrome infants smiled and laughed ßrst at theintrusive, physically vigorous tactile and auditoryitems. Only much later, while afiective expressionto the auditory and tactile items was declining,did they smile and laugh a great deal to the itemswith a more explicit element of cognitive incon-gruity (see fig. 2).

This is the same observation that previouslysuggested a tie between cognitive developmentand laughter in normal infants. The progression inboth retarded and normal infants is from affectiveresponse to intrusive stimulation, to stimulationmediated by active attention, then to stimuluscontent or meaning, toward an ever more activeinvolvement in producing the stimulus itself(Sroufe & Waters 1976). Increasingly, it is notstimulation but the infant's effort in processingstimulus content or the infant's participation in theevent that produces the tension necessary for smil-ing and laughter.

With the Down's syndrome infants, it was notuntil the first half of the second year (vs. the sec-ond half of the first year with normal infants) thatitems in which the infant participated becameespecially salient. The "tug" item (no. 16) exem-

¡l08to12t¡¡ier0

Age in Months

Ftc. 2.-Month-by-month smiling of Down's syn-d¡ome i¡;,fants to the four categories of items.

plites the role of active participation at this time aswell as the afiective-cþgDitive interchange. It wasonly when the baby was actively able to participatein the game by reciprocally tugging the yarn thatafiective expression resulted. Simílarly, smilingand laughing resulted only when the infant spon-taneously grabbed the cloth from its own face (no.l9), or reached for the mother's tongue (no. 20),not when, for example, the mother placed the in-fant's hand on her tongue. During this sameperiod, efforts to get the mother to repeat theitem, for example, stufffng the cloth back in hermouth, and anticipatory smiling (i.e., smiling inadvance of item completion, ê.g., before themother's return in peek-a-boo), were also seen.This progressively more active involvement hasparallels in various realms of development duringinhncy, such as persistence in the pursuit of anobject or other goal (Brazelton 1969; Buhler 1930).Our data clearly indicate that this general de-velopmental principle applies also to Down's syn-drome infants.

The most compelling data with respect to theafiect-cognition tie arê from the cognitive test re-sults. Cognitive developmental level, as measuredby performance at 13 months on the Uzgiris-Huntscales and at 16 months on the Bayley MentalScale, paralleled the level of affectíÇe develop-ment as assessed by the 30laughter items. [n fact,the integrated nature of development-the closetie between affect and cognition-was more read-ily demonstrated with this group of retarded in-fants than with normal infants. With their retardedrate of development, changes in performancewere spread out over time, and at a given agethere was a great deal of variation between sub-jects. Infants' rankings on onset of smiling andlaughter, developmental level of smiling andlaughter'assessed by various criteria, onset ofnegative reactions in response to our items, andmotor and mental development were remarkablyconcordant (see table 3).

In light of this evidence for the close tie be-tween affective and cognitive development, andgiven the relative ease of assessing affective ex-pression i¡r the "sensory-affective" infant (Steehler& Carpeùter 1967), more emphasis should begiven to affect in studies of general development.The most r¡sefi.rl strrdies will be those that examineaffect and cognition concurrently,

The role of tension in affect expression.-Thefindings with this retarded sarnple are also rele-vant to a hypothesis concerning the role of tensionin afnective expression (Sroufe & Waters 1976).Down's syndrome infants as a group arehypotonic, so a lag in affect expression would be

Cl 80-IJ.t to

€rto3Ê 50otr-=õ 'oO30IJbzoÀ t0

- Soclal

..'...'Visual

- Tactilo

- Auditory

L’idéeestquel’enfantdevientdeplusenplusactifetattentifpourextrairelesensetl’incongruitédesstimulations,aucontrairedelaréceptionpluspassivedesstimuliauditifsettactilesquisontdenatureintrusiveetquidemandentunestimulationvigoureuse.Cedéveloppementdemandedesressourcescognitivesquisedéveloppentaveclamaturation.

EtudedeCicchetti&Sroufe(1976)

026 Child Developmentports the generality of the view that developmentis integrated and organized. At the same time, thedetailed longitudinal data provide considerable in-formation about Down's syndrome babies. Whilethe nature of the developmental process is similar,the picture of development with these childrendiffers substantially from that of normal infants.

Affectioe and cognítíoe deoelopmenú.-TheDown's syndrome inånts showed a progression inthe expression of positive affect similar to that oftheir normal counterparts, though the rate of de-velopment was, of course, much slower. Down'ssyndrome infants smiled and laughed ßrst at theintrusive, physically vigorous tactile and auditoryitems. Only much later, while afiective expressionto the auditory and tactile items was declining,did they smile and laugh a great deal to the itemswith a more explicit element of cognitive incon-gruity (see fig. 2).

This is the same observation that previouslysuggested a tie between cognitive developmentand laughter in normal infants. The progression inboth retarded and normal infants is from affectiveresponse to intrusive stimulation, to stimulationmediated by active attention, then to stimuluscontent or meaning, toward an ever more activeinvolvement in producing the stimulus itself(Sroufe & Waters 1976). Increasingly, it is notstimulation but the infant's effort in processingstimulus content or the infant's participation in theevent that produces the tension necessary for smil-ing and laughter.

With the Down's syndrome infants, it was notuntil the first half of the second year (vs. the sec-ond half of the first year with normal infants) thatitems in which the infant participated becameespecially salient. The "tug" item (no. 16) exem-

¡l08to12t¡¡ier0

Age in Months

Ftc. 2.-Month-by-month smiling of Down's syn-d¡ome i¡;,fants to the four categories of items.

plites the role of active participation at this time aswell as the afiective-cþgDitive interchange. It wasonly when the baby was actively able to participatein the game by reciprocally tugging the yarn thatafiective expression resulted. Simílarly, smilingand laughing resulted only when the infant spon-taneously grabbed the cloth from its own face (no.l9), or reached for the mother's tongue (no. 20),not when, for example, the mother placed the in-fant's hand on her tongue. During this sameperiod, efforts to get the mother to repeat theitem, for example, stufffng the cloth back in hermouth, and anticipatory smiling (i.e., smiling inadvance of item completion, ê.g., before themother's return in peek-a-boo), were also seen.This progressively more active involvement hasparallels in various realms of development duringinhncy, such as persistence in the pursuit of anobject or other goal (Brazelton 1969; Buhler 1930).Our data clearly indicate that this general de-velopmental principle applies also to Down's syn-drome infants.

The most compelling data with respect to theafiect-cognition tie arê from the cognitive test re-sults. Cognitive developmental level, as measuredby performance at 13 months on the Uzgiris-Huntscales and at 16 months on the Bayley MentalScale, paralleled the level of affectíÇe develop-ment as assessed by the 30laughter items. [n fact,the integrated nature of development-the closetie between affect and cognition-was more read-ily demonstrated with this group of retarded in-fants than with normal infants. With their retardedrate of development, changes in performancewere spread out over time, and at a given agethere was a great deal of variation between sub-jects. Infants' rankings on onset of smiling andlaughter, developmental level of smiling andlaughter'assessed by various criteria, onset ofnegative reactions in response to our items, andmotor and mental development were remarkablyconcordant (see table 3).

In light of this evidence for the close tie be-tween affective and cognitive development, andgiven the relative ease of assessing affective ex-pression i¡r the "sensory-affective" infant (Steehler& Carpeùter 1967), more emphasis should begiven to affect in studies of general development.The most r¡sefi.rl strrdies will be those that examineaffect and cognition concurrently,

The role of tension in affect expression.-Thefindings with this retarded sarnple are also rele-vant to a hypothesis concerning the role of tensionin afnective expression (Sroufe & Waters 1976).Down's syndrome infants as a group arehypotonic, so a lag in affect expression would be

Cl 80-IJ.t to

€rto3Ê 50otr-=õ 'oO30IJbzoÀ t0

- Soclal

..'...'Visual

- Tactilo

- Auditory

L’étudedeCicchetti&Sroufemontrequeledéveloppementdurireestliéauquotientdedéveloppement(échelledeBayley).LesseulsenfantsatteintsdusyndromedeDownquisuiventundéveloppementàunrythmenormaldurireétaientdeuxenfantsporteurd’uneT21enmosaïque.Lesquatreenfantslesplushypotoniquessontceuxaccusantleplusderetard.

Ø Introduction*  Etudedesconditionsd’apparitionduriredansleSA

Plan

Objectif:examinerlesinfluencesenvironnementalessurlesriresetlessouriresdansleSAParticipants:3enfantsatteintsduSA(de7,11et17ans)Procédure:3typesdeconditions:conditionInteraction,conditionProximitéetconditionSeul.Blocsdetroissessionsparjour,etavecautotalseptàhuitblocsdesessionsRésultats:LesriresetsouriressurviennentpendantdesévénementscontextuelssociauxappropriésLimites:petitéchantillon;pasdepriseencompteduquotientdedéveloppement(QD)

ConditionInteraction:L’expérimentateurétaitassisàcôtédel'enfant,eninteractioncontinuedefaçonenthousiaste,toutenjouantavecl'undesjouetspréférésdel'enfant.ConditionProximité:L'expérimentateurétaitassisprèsdel'enfantmaisn'initiaitaucunesorted'interactionsocialeaveclui,etcecimêmelorsquel'enfantfaisaitdestentativespourattirerl'attentiondel'expérimentateur.ConditionSeul:L'enfantestlaisséseuletestautoriséàs'engagerdansdesactivitéssolitaires,commeparexemples'amuseravecunjouet,feuilleterlespagesd'unmagazine,ousimplementresterassis.

EtudedeOliveretal.(2002)

� American Association on Mental Retardation 197

VOLUME 107, NUMBER 3: 194–200 � MAY 2002 AMERICAN JOURNAL ON MENTAL RETARDATION

Angelman syndrome C. Oliver, L. Demetriades, and S. Hall

Figure 1. Percentage duration of laughing and smiling observed in each session in each condition foreach child.

Table 1. Mean Percentage Duration (and SD) ofSmiling and Laughing Behavior Observed byChild and Condition

Child/Condition

Laughing

Mean SD

Smiling

Mean SD

RebeccaAloneInteractionProximity

.0829.271.17

.2316.631.25

.8653.3920.20

.8916.528.10

MariaAloneInteractionProximity

.7449.638.80

1.088.026.59

2.0928.8116.39

2.397.407.42

ChristopherInstructionsInteractionProximity

1.6313.24

.05

1.0010.35

.12

32.7754.196.50

12.5113.451.76

during which the children were engaged in smil-ing and laughing varied systematically across con-ditions. For Rebecca and Maria, high levels ofsmiling occurred in the interaction condition(53.39% and 28.81%, respectively), intermediatelevels occurred in the proximity condition(20.20% and 16.39%), and low levels in the alone

condition (.86% and 2.09%). For Christopher,high levels of smiling occurred in the interactionand instructions conditions (54.19% and 37.77%respectively), whereas low levels of smiling oc-curred in the proximity condition (6.50%).

As with smiling behavior, the degree to whichthe children engaged in laughing also variedacross conditions. For Rebecca, high levels oflaughing occurred in the interaction condition(29.27%), whereas low levels occurred in the prox-imity and alone conditions (1.17% and .08%, re-spectively). For Maria, high levels occurred in theinteraction condition (49.63%), intermediate lev-els occurred in the proximity condition (8.80%),whereas low levels occurred in the alone condi-tion (.74%). For Christopher, high levels of laugh-ing were observed in the interaction condition(13.24%), and low levels were observed in the in-structions and proximity conditions (1.63% and.05%, respectively).

For Rebecca and Maria, then, low levels oflaughing and smiling occurred in the alone con-dition and intermediate levels, in the proximitycondition. High levels of smiling and laughing oc-curred in the interaction condition. These datasuggest that laughing and smiling occurred duringcontexts that were appropriate (i.e., those that in-volved social interaction). High levels of laughingand smiling did not occur in the alone and prox-

EtudedeOliveretal.(2002)

Sessions------->

Sessions------->

Objectif:AnalyserlesinfluencessocialessurlesriresetlessourireschezlesenfantsatteintsduSAetdistinguerlescomposantesdel'interactionsocialequiprovoquentlesplushautsniveauxd'expressivité.Participants:11enfantsatteintsduSAâgésde4à11ansProcédure:3typesdeconditions:conditiond''interactionélevée,conditiond''interactionfaible,etconditionSeul.Résultats:L’expressiondurireetdusouriredansleSAestrenforcéeparl'interactionsocialeélevéeimpliquantl'attentiondel'adulteetplusprécisémentlaparole,letoucher,lesourire,lerireetlecontactparleregard.

Conditiond'interactionélevée:L’expérimentateurétaitassisàcôtédel'enfant,eninteractioncontinue,defaçonenthousiasteavecl’enfant,toutenjouantavecl'undesjouetspréférésdel'enfant.Conditiond'interactionfaible:L'expérimentateurétaitassisprèsdel'enfant,luiparle,neregardepasl’enfant,gardeuneexpressionfacialeneutre,etcecimêmelorsquel'enfantfaisaitdestentativespourattirerl'attentiondel'expérimentateur.ConditionSeul:L'enfantestlaisséseuletautoriséàs'engagerdansdesactivitéssolitaires,commeparexemples'amuseravecunjouet,feuilleterlespagesd'unmagazine,ousimplementresterassissurlachaise.

EtudedeHorsler&Oliver(2006)

*  Pargénéralisation:LesréactionsdejoiedesenfantsporteursduSA sont rationnelles et motivées par leur contexte, y comprispourdescontextesdiversifiésautresquesociaux.

*  Lesrireset lessouriressontprovoquéspar lesmêmesfacteursetapparaissentdanslesmêmesconditionspourdesenfantsSAetdesenfantscontrôles(non-SA)présentantunmêmequotientdedéveloppement.

*  LesriresetlessouriressontplusintensesdansleSA,maissontdéclenchésparlesmêmesstimuli.

Hypothèses

Objectif méthodologique à Détecter les éléments déclencheurs du rirechez lesenfantsSAetappliquerunemesure laplusobjectivepossibledesréactionsémotionnelles.

1)Participantsrépartisen3groupes:-  20enfantsSA(dont11filleset9garçons;Mage=7ans2mois)-  21enfantsContrôle(dont9garçons;Mage=1ans6mois)-  7enfantsTSAbasniveau(dont4garçons;Mage=9ans11mois)2)Procédure:-  Evaluationduniveaudedéveloppementpar laBECS(Batteried’Évaluation

CognitiveetSocio-Emotionnelle,Jean-LouisAdrien,2008)-  Condition d’interaction (comportant 16 stimulations sélectionnées de

l’étudedeCicchetti et Sroufe, 1976) et Conditionde jeu seul (onmet a ladispositiondel’enfantlesobjetsdelaBECS)

3)Méthode : enregistrement vidéo pour le codage intuitif d’excitabilité etd’expressivité(évaluéespar3jugesindépendants)+CodageFACS

Méthodologie

Ø Méthodologie*  DescriptiondelaFACS

Plan

à UtilisationdelaFACS(FacialActingCodingSystem),fondéesurladécompositiondespluspetitsmouvementsperceptiblesdesmusclesduvisage(Ekman&Friesen,1978;Ekman,Friesen&Hager,2002)

FACS

à LaFACSaétéélaboréeafind’améliorerlecodagedesémotions.Auparavant,lesémotionsétaientdirectementcodées(e.g.,Est-cequelevisagemontreuneexpressiondejoie?),cequiposaitunproblèmedesubjectivitéducodage.Eneffet,iln’yapasdethéorieconsensuellepourdécrypterlesémotions.

à Codageexhaustifetexclusif;fiable(mesuresinter-jugesfiables).à Exemple:surleplanmorphologique,possiblededistinguer18typesde

sourires.à Manuelde527pages!Certificationrequise.

à  Sérieinspiréedecetteméthodestandardisée:

FACS

Lietome–RéalisationSamBaum

FACS

Fig.5(ManuelFACS).Musclesquisous-tendentlesunitésd'action(UA)chargésdemodifierl'aspectduhautduvisage:sourcils,front,ouverturedel’oeil,etpaupièressupérieuresetinférieures.LesnumérossurlevisagereprésententlesnumérosdesAU:1et2,relèvementdusourcilàdifférentsendroits;4,froncement;6,relèvementdelajoue;7,plissementdesyeux;,etc.

DifférentstypesdesourireaveclaFACS

Visageneutre Souriresocial SourireDuchenne Sourire/rireBoucheouverte

AucuneAU AUs12+25Étirementducoindeslèvres(12),séparées(25)

AUs6+12+25+26Relèvementdesjoues(6),étirementducoin

deslèvres(25),séparées(25),et

mâchoireabaissée(26)

AUs6+12+25+27MêmesAUs(6+12+25),

avecuneintensitémaximale,etmâchoire

étirée(27)

*  Sourire Duchenne : implique une combinaison des AUs 6(relèvementdes joues)et12(relèvementsymétriqueetobliquedu coin des levres). L'AU 5 élargit l'ouverture des yeux exposant plus desclère(danslecasoùparexdeuxémotionssontcombinées,lajoieetlasurprisepar exemple) alors que l'AU 6 la rétrécit avec le soulèvement des joues quicreuselesilloninfra-orbitaletabaisseégalementlessourcils.

*  LerireimpliquelesAUs6,25(quidéterminelaséparationentreleslèvres)et26(abaissementdelamâchoirelorsquelesmusclesqui maintiennent celle-ci fermée se relâchent). Le rire n’implique pasforcément27quireprésenteuneouvertureextrêmedelamâchoire(parexemplelorsd'unbâillement)etdoncopposéà26.

Exemples

NB.Duchenne(1806-1875)adécritenpremierceriresincèreetutilisedefaçonplusgénéralel’électricitépourdéclencherlesmusclesfaciaux(Lemécanismedel’expressionfacialehumaine,1862)

BabyFACS

*  Oster(2000,2004)proposeuncodageplusadaptéàl’anatomiedesbébés,malgrélefaitquelesUnitésd’ActiondelaFACSsontparfaitementadaptablesauxmusclesfaciauxdesbébés(entièrementfonctionnelsàlanaissance)

4

ClmekRms lg Joy-Happhmss Memis

8

No Cheek Raising

6

5

4

Vel3, Sh'ong Smile,Mouth Open

8

7

6

4

Minimal Smile.Mouth Open

8

7t6

4

Strong Smile,Mouth Closed

81tong Smile,Mouth Closed

7

6

5

Figure 1. Positive emotion perceived in different types of infant smiles. Meanjoy-happiness ratings (on a scale from 0-8) and their standard errors for each pairof smiles. The smiles on the left are cheek-raise (Duchenne) smiles, and thesmiles on the right are not. The smiles in each horizontal pair were created withan identical smiling mouth. In each column, the top two smiles are open-mouthsmiles and the bottom two are not. Pairs of smiles also differ in the strength of thesmile portrayed. Adapted with permission from "Positive and negative: Infantfacial expressions and emotions," by D. Messinger (in press).

Tiréde:Bolzanietal.(2002)

RelationFACSetémotions

Nijmegen participated in the validation study,with a mean age of 21.2 years (SD 4.0). Allhad normal or corrected-to-normal vision andreceived t10 or course credits for participation.The validation study was programmed usingPsychoPy (Peirce, 2007), a python library forconducting psychological experiments.

Procedure. Only the frontal view images (908-camera) were validated. Participants were pre-sented with pictures from only one of the databasesubsets, either adults or children. For each model,gaze direction, and facial expression, originallytwo images were present in the validation stimulusset. From these two only the one with the highestinter-rater agreement concerning the intendedexpression was retained for inclusion in the finaldatabase.

The validation started with an attractivenessrating. Participants scored the neutral, straight-gaze images of all subset models on a 5-point scale,ranging from unattractive to attractive. Image

order was randomised. This task familiarised theparticipants with all models in the subset.

Next, participants rated the images of 9 subsetmodels on several dimensions. For each model,participants viewed images with all three gazedirections combined with eight emotional expres-sions, summing up to 216 images for eachparticipant. This way, participants saw equalnumbers of emotions from each model. Whichmodels were presented was chosen randomlyacross participants, with the constraint that everyimage was rated by at least 20 participants.

In each trial, a randomly chosen image fromthe 9 subset models presented to the participantwas shown in the centre of the screen. For eachimage, participants successively judged: (a) thedepicted expression; (b) the intensity of theexpression; (c) the clarity of the expression; (d)the genuineness of the expression; and (e) thevalence of the image, in this order. Before thetask, participants got instructions for each ratingdimension. For each judgement, the current

Figure 2. Targeted action units (AU) for all emotional expressions. [To view this figure in colour, please visit the online version of thepaper.]

VALIDATION OF RAFD

COGNITION AND EMOTION, 2010, 24 (8) 1381

Langneretal.(2010)

*  Darwin(1872)s’estintéresséaucoursdesespérégrinationsauxémotionsetexpressionsfaciales (dérivant d’après lui de comportements plus primitifs de cris ou de pleurs). Cesexpressions semblent avoir une valeur adaptative à l’environnement et un caractèreuniversel/innéenfaveurd’unecontinuitéévolutiveentreespèces.

*  Leplaisir (associéaugoûtdu sucreparexemple)provoqueuneprotrusionde la languechezdenombreuxmammifèresquipourraitêtrelafondationdusourire.

Expressionduvisageetémotions:Trèsvieillequestion!

Résultatsillustrésparvidéo

Ø Méthodologie*  DescriptiondelaBECS

Plan

Batteried'EvaluationCognitiveetSocio-Emotionnelle

LaBECSdonne:-  unindicededéveloppementcognitif

(7domaines;e.g.,encastrementdeformesgéométriques)

-  unindicesocio-émotionnel(9domaines;imiterfairesemblantdetéléphoner,avecunebanane)

-  unindiceglobal

https://www.ecpa.fr/psychologie-clinique/test.asp?id=1386

16stimulationsdelaphased’interactioninspiréesdel’étudedeCicchetietSroufe(1979)

!

Stimuli'auditifs' Stimuli'sociaux'Avec%une%voix%mécanique,%varier%le%son%de%la%voix%de%bas%en%haut%en%disant%«%Boo9Boo9Boo9Baa9Baa9Baa9Boo9Boo9Boo%».%

Permettre%à%l’enfant%de%saisir%un%fil,%puis%tirer%3%fois%en%essayant%de%ne%pas%s’éloigner%de%l’enfant.%Pause%puis%reprendre.%

En%utilisant%une%voix%forte%et%profonde,%prononcer%«%BOOM%BOOM%BOOM%»%à%1%seconde%d’intervalle.%

Envoyer%une%balle%à%l’enfant%:%3%fois.%

Faire%tourner%une%boîte%à%musique.% Mettre%un%tissu%dans%la%bouche%et%se%pencher%suffisamment%proche%de%l’enfant%pour%qu’il%le%saisisse.%Permettre%à%l’enfant%de%tirer%le%chiffon.%

Chuchoter,%à%proximité%de%l’enfant%:%«%Coucou%bébé,%comment%vas9tu%?%».%

Faire%une%grimace%à%l’enfant.%

Stimuli'tactiles' Stimuli'visuels'Doux%tapotements%de%la%joue%avec%un%objet%mou%:%3%fois.%%

Courir%sur%place%:%3%fois%

Attirer%l’attention%de%l’enfant%sur%les%doigts%vers%l’enfant,%puis%faire%un%chatouillis%sur%le%ventre%de%l’enfant.%Si%le%rire%est%réussi,%faire%d’autres%essais%non%suivis%de%chatouilles.%

Faire%le%mouvement%de%voler%:%3%fois.%

Souffler%doucement%sur%les%cheveux%de%l’enfant%pendant%3%secondes%(souffler%à%partir%du%côté,%sur%le%dessus%de%la%tête%de%l’enfant).%

Faire%des%bulles%devant%l’enfant%:%3%fois%

Doux%chatouillis%avec%les%doigts%sous%le%menton%de%l’enfant%pendant%3%secondes.%

Secouer%la%tête%vigoureusement%à%une%distance%de%1m%du%visage%de%l’enfant.%

Ø Résultats

Plan

Batteried'EvaluationCognitiveetSocio-Emotionnelle

GRP

BECSCog

BECSSocio-émo

BECSGlobal

SA N 20 20Mean 2.9 2.5 2.7SD .5 .5 .5

CTRL N 21 21Mean 3.4 3.1 3.2SD .3 .6 .4

TSA N 7 7Mean 3.4 3.3 3.3SD .5 .5 .5

GroupeSAsignifica=vementplusfaiblequelegroupeCTRL(différenced’âgepassuffisante)

Comparaisondesnotesobtenuesauxdeuxéchellescontinuesparlestrois

groupes

SA

CTRL

TSA

Comparaisondesnotesobtenuesauxdeuxéchellescontinuesparlestrois

groupes

r=.71;p=.002;N=16

r=.64;p=.007;N=16

r=.44;p=.09;N=16

SA

CTRL

TSA

Comparaisondesmoyennesdesnotesd’excitabilitéetd’expressivitéentreleGroupeSAetleGroupe

Contrôle

SA

r=.55;p=.03BF10=5.6ModérémentenfaveurDel’hypothèse.

Comparaisondesmoyennesdesnotesd’excitabilitéetd’expressivitéentreleGroupeSAetleGroupe

Contrôle

SA

Grimace

Bulles

ItemplusplaisantauxSA

VFFFFFFF

ItemplusplaisantauxCTRL

Comparaisondesmoyennesdesnotesd’excitabilitéetd’expressivitéentreleGroupeSAetleGroupe

Contrôle

SAVFFFFFFF

AnalysesFACSGroupeANGELMAN

AU#

Intensité

AnalysesFACSGroupeCONTRÔLE

AU#

Intensité

DifférenceGlobaleFACSEntreGroupeCONTRÔLEetSA

- =6jouesrelevées7,plissementdesyeux

12,relèvementcoinlèvres

25,lèvresséparées26,ouverturebouche

AUcomplémentairesdiverses(partiesduvisagenonvisible,enfantquimâche,etc.)

Ctrl SA

53,54,têteenavantouabaissée

Différence Unités d’action FACS Groupe SA et CONTRÔLE

CtrlSA

Corrélation(sanslesvaleursdouble-zéro):r=.97,p<.001 BF10>100(Extrêmement enfaveurdel’hypothèse)

EffectifmoyenparparticipantparUnitéd’Actionpour16items

Différence Unités d’action FACS Groupe SA et CONTRÔLE

CtrlSA

Signification:Lesdeuxgroupesprésententlemêmetypederéactionauxstimuli.

EffectifmoyenparparticipantparUnitéd’Actionpour19items

DifférenceIntensitésFACSGroupeSAetCONTRÔLE

CtrlSA

Effectifmoyenparparticipantparniveaud’intensitépour19items

Différence

(intensitésmesuréespourchaqueitemdans

chaquegroupe)Voirdiaposuivante

(Intensitépourtouslesitemspourchaque

groupe)

DifférenceIntensitésFACSGroupeSAetCONTRÔLE

DifférencesignificativeentreSAetCtrl

ValeurmoyennedesintensitésdesAU

t(15)=4.9,p<.001,Cohen'sd=1.219;BF10=154(Extrême),95% CI: [0.7, 2.4]

DifférenceIntensitésFACSGroupeSAetCONTRÔLE

ValeurmoyennedesintensitésdesAU

Signification:LegroupeSAréagitdefaçonplusintenseauxstimuli.

DifférenceIntensitésFACSGroupeSAetCONTRÔLE

Valeurmoyennedesintensités

desAU(+/-1SE)

AuditifsTactiles

SociauxVisuels

Letestsstatistique(ANOVA)montreuniquementuneffetdugroupe.Pasd’effetdelacatégoriedesstimulations.Pasd’effetd’interaction.

Signification:LegroupeSAréagitdefaçonplusintenseauxstimuliquellequesoitlacatégoriedesstimuli.Cerésultattendàindiqueruncaractèrerationnelauxréactionsdejoie.

Récaprésultatsprincipaux

*  Réaction de même nature aux mêmes stimuli, maisréactionsplusintenses

*  Conclusion : Les enfants SA ont des comportementsadaptés à la situation et similaire à des enfants dequotientdedéveloppementproche.

*  Limites:-RésultatsBECSnonéquivalents(SA<groupecontrôle)- Analyses à conduire en condition seule (cf. étudesd’Oliver)

*  Mesuredeladuréedesexpressionsémotionnellesdejoie(durationmarkers?réactionsprolongéesdesenfantsSA?)*  Analyseacoustiquedel’amplitudeetdeladuréedesrires*  Analyseautomatiséedesexpressionsfaciales(Noldus,Face

Reader;AFA4,cf.Messingeretal.,2004).Voirdiaposuivante.*  Connectercesrecherchesauxthéoriesduplaisir(cf,

KringelbachetBerridge,TiCS,2009).

Approfondissementsàenvisager

(Analysesautomatisées)

Figure 1. Active Appearance Models (AAM)a) The AAM is a mesh that tracks and separately models rigid head motion (e.g., yaw, pitch,and roll, visible in the upper left hand portion of the images) and non-rigid facial movementover time (left to right). b) In this output display, each partner's face is outlined to illustratelip corner movement, mouth opening, the eyes, brows, and the edges of the lower face; theseoutlines are reproduced in iconic form to the right of each partner.

Messinger et al. Page 15

Infancy. Author manuscript; available in PMC 2009 November 1.

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anuscriptN

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uthor Manuscript

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anuscript

AFA4FaceReader

Merciàtous!Merciausoutiendel’AFSA!!

Merciauxparents!!!Merciauxenfants!!!!

RencontresNationalesAFSA19&20janvier2018,CISL,LYON