Social Smile Research Articles

Lissencephaly and facial dysmorphism: is it Miller–Dieker syndrome?

An 8-month-old girl presented with developmental delay and flexor jerks for the past 2 months. Examination showed unusual facial characteristics, such as bitemporal hollowing, prominent upper lip, high arched palate and retrognathia (Fig. 1a), microcephaly, spasticity, brisk muscle stretch reflexes and flexor-infantile spasms. She had no neck-control, visual following or social smile. Magnetic resonance imaging showed lissencephaly (Fig. 1b). Genetic analysis confirmed deletion of the 17p13.3 chromosome. Electroencephalograph showed hypsarrhythmia. Adrenocoticotropic hormone or Corticotropin therapy, rehabilitation and antenatal diagnosis for future pregnancies was offered to the parents. Lissencephaly can occur either as a result of a contiguous gene deletion disorder known as Miller–Dieker syndrome or as an isolated form known as isolated lissencephaly sequence (i.e. without facial dysmorphism or other abnormalities, such as abnormal genitalia or cerebellar hypoplasia) as a result of a single gene mutation in the LIS1 or DCX gene. Miller–Dieker syndrome, as a result of chromosome-band 17p13.3 deletions, is a multiple malformation syndrome characterized by severe lissencephaly (Dobyns’ grade 2; diffuse agyria with a few shallow sulci); characteristic facial features such as high, prominent forehead, bitemporal hollowing, short nose with upturned nares, protuberant upper lip and small jaw; and severe psychomotor impairment. Early-infantile seizures, especially infantile spasms with evolution into Lennox–Gastaut syndrome occur in the majority of patients. Genetic confirmation is crucial for prognosis and prenatal diagnosis.

A case of severe progressive early-onset epileptic encephalopathy: Unique GABAergic interneuron distribution and imaging

Early-onset epileptic encephalopathies include various diseases such as early-infantile epileptic encephalopathy with suppression burst. We experimentally investigated the unique clinicopathological features of a 28-month-old girl with early-onset epileptic encephalopathy. Her initial symptom was intractable epilepsy with a suppression–burst pattern of electroencephalography (EEG) from 7days of age. The suppression–burst pattern was novel, appearing during sleep, but disappearing upon waking and after becoming 2months old. The EEG showed multifocal spikes and altered with age. Her seizures demonstrated various clinical features and continued until death. She did not show any developmental features, including no social smiling or head control. Head MRI revealed progressive atrophy of the cerebral cortex and white matter after 1month of age. 123IMZ-SPECT demonstrated hypo-perfusion of the cerebral cortex, but normo-perfusion of the diencephalon and cerebellum. Such imaging information indicated GABA-A receptor dysfunction of the cerebral cortex.The genetic analyses of major neonatal epilepsies showed no mutation. The neuropathology revealed atrophy and severe edema of the cerebral cortex and white matter. GAD-immunohistochemistry exhibited imbalanced distribution of GABAergic interneurons between the striatum and cerebral cortex. The results were similar to those of focal cortical dysplasia with transmantle sign and X-linked lissencephaly with ARX mutation. We performed various metabolic examinations, detailed pathological investigations and genetic analyses, but could not identify the cause. To our knowledge, her clinical and pathological courses have never been described in the literature.

Parental Ethnotheories, Social Practice and the Culture-Specific Development of Social Smiling in Infants

In this article we argue that current theories on socioemotional development during infancy need to be reconceptualized in order to account for cross-cultural variation in caregiver–infant interaction. In line with the cultural-historical internalization theory of emotional development (Holodynski & Friedlmeier, 2006) and the ecocultural model of development (Keller & Kärtner, 2013), we argue that socioemotional development can be understood only in the context of social practice and underlying ethnotheories that give significance to infants' emotional expressions. Thus, culture-specific interpretations of and expectations concerning infants' expressive cues lead to culture-specific interactional routines. These, in turn, lead to culture-specific usage of these expressions by the developing child. To develop our argument, we focus on a specific aspect of early socioemotional development, namely, the emergence of social smiling during infancy. Interactional dynamics in autonomous cultural milieus are based on specific ethnotheories, most prominently that positive emotional exchange during face-to-face interaction is one of the most desirable ways of interacting with infants. However, the dominant ethnotheories concerning emotional development and their associated behavioral routines vary systematically across cultural milieus and are markedly different in prototypically relational cultural milieus, in which they center on infants' contentment. This has implications for infants' emotional expressivity and, possibly, experience.

Evidence of a Distinct Behavioral Phenotype in Young Boys With Fragile X Syndrome and Autism

ObjectiveHow does the behavioral expression of autism in fragile X syndrome (FXS + Aut) compare with idiopathic autism (iAut)? Although social impairments and restricted, repetitive behaviors are common to these variants of autism, closer examination of these symptom domains may reveal meaningful similarities and differences. To this end, the specific behaviors comprising the social and repetitive behavioral domains in young children with FXS + Aut and iAut were profiled. MethodTwenty-three male subjects 3 to 5 years old with FXS + Aut were matched by age to a group of 38 boys with iAut. Repetitive behavior was assessed using the Repetitive Behavior Scales-Revised. Social behavior was evaluated using Autism Diagnostic Observation Schedule social item severity scores. ResultsRates of stereotypy, self-injury, and sameness behaviors did not differ between groups, whereas compulsive and ritual behavior scores were significantly lower for subjects with FXS + Aut compared with those with iAut. Those with FXS + Aut scored significantly lower (less severe) than the iAut group on five Autism Diagnostic Observation Schedule measurements of social behavior: gaze integration, quality of social overtures, social smile, facial expressions, and response to joint attention. ConclusionsThe behavioral phenotype of FXS + Aut and iAut are most similar with respect to lower-order (motoric) restricted, repetitive behaviors and social approach, but differ in more complex forms of restricted, repetitive behaviors and some social response behaviors. These findings highlight the phenotypic heterogeneity of autism overall and its unique presentation in an etiologically distinct condition.

A cross-cultural comparison of the development of the social smile: A longitudinal study of maternal and infant imitation in 6- and 12-week-old infants

Social smiling is universally regarded as being an infant's first facial expression of pleasure. Underlying co-constructivist emotion theories are the assumptions that the emergence of social smiling is bound to experiences of face-to-face interactions with caregivers and the impact of two developmental mechanisms – maternal and infant imitation. We analyzed mother–infant interactions from two different socio-cultural contexts and hypothesized that cross-cultural differences in face-to-face interactions determine the occurrence of both of these mechanisms and of the frequency of social smiling by 12-week-old infants. Twenty mother–infant dyads from a socio-cultural community with many face-to-face interactions (German families, Münster) were compared with 24 mother–infant dyads from a socio-cultural community with few such interactions (rural Nso families, Cameroon) when the infants were aged 6 and 12 weeks. When infants were 6 weeks old, mothers and their infants from both cultural communities smiled at each other for similar (albeit very short) amounts of time and used imitated each other's smiling similarly rarely. In contrast, when infants were 12 weeks old, mothers and their infants from Münster smiled at and imitated each other more often than did Nso mothers and their infants.

Eye contact with neutral and smiling faces: effects on autonomic responses and frontal EEG asymmetry

In our previous studies we have shown that seeing another person “live” with a direct vs. averted gaze results in enhanced skin conductance responses (SCRs) indicating autonomic arousal and in greater relative left-sided frontal activity in the electroencephalography (asymmetry in the alpha-band power), associated with approach motivation. In our studies, however, the stimulus persons had a neutral expression. In real-life social interaction, eye contact is often associated with a smile, which is another signal of the sender's approach-related motivation. A smile could, therefore, enhance the affective-motivational responses to eye contact. In the present study, we investigated whether the facial expression (neutral vs. social smile) would modulate autonomic arousal and frontal EEG alpha-band asymmetry to seeing a direct vs. an averted gaze in faces presented “live” through a liquid crystal (LC) shutter. The results showed that the SCRs were greater for the direct than the averted gaze and that the effect of gaze direction was more pronounced for a smiling than a neutral face. However, in this study, gaze direction and facial expression did not affect the frontal EEG asymmetry, although, for gaze direction, we found a marginally significant correlation between the degree of an overall bias for asymmetric frontal activity and the degree to which direct gaze elicited stronger left-sided frontal activity than did averted gaze.

Does Primary Narcissism Exist in Newborn Babies? Evidence from Sleep Science

Does Primary Narcissism Exist in Newborn Babies? Evidence from Sleep Science

Facial Behaviors and Emotional Reactions in Consumer Research

Gauging emotional reactions is a cornerstone of consumer research. The most common way emotions are assessed is self-report. But self-report is notoriously unreliable, and affected by many factors that confound their interpretation. Facial expressions are objective markers of emotional states, and are well grounded in decades of research. Yet, the research documenting the potential utility of facial expressions of emotion as a biometric marker in consumer research is limited. This study addresses this gap, presenting descriptive analyses of the facial expressions of emotion produced in typical consumer research. Surprisingly, the most prevalent expressions produced were disgust and social smiles; smile of true enjoyment were relatively rare. Additionally, expressions were generally of low intensity and very short durations. These findings demonstrate the potential utility for using facial expressions of emotion as markers in consumer research, and suggest that the emotional landscapes of consumers may be different than what is commonly thought

Smile Esthetics: Age Related Changes, and Objective Differences between Social and Spontaneous Smiles

To evaluate the importance of age in orthodontic treatment by studying the dependence of smile and resting parameters on age and to expose differences between social and spontaneous smiles. Subjects consisted of 67 individuals aged between 17 and 55. The video recordings were transferred to a computer 200 still frames were captured for each individual 50 were captured in resting position, 50 during speech, 50for social, and 50for spontaneous smiles. One picture was selectedfrom each group based on how pictures reflected the desired point ANOVA and Scheffe Post-hoc tests were performed on smile measurements. In all the resting parameters, statistically significant differences were observed among age groups. Also, the response of these parameters to age differs between men and women. Statistically significant diferences were found in some smile parameters among diferent age groups, for both smile types. We find significant differences between social and spontaneous smiles. Age related alterations should be taken into consideration during treatment planning, especially in women. Due to its high consistency, there are advantages with using a spontaneous smile in soft-hard tissue evaluations. We also emphasize the necessity to take dynamic registrations for a true functional evaluation.

Differential Prefrontal White Matter Development in Chimpanzees and Humans

A comparison of developmental patterns of white matter (WM) within the prefrontal region between humans and nonhuman primates is key to understanding human brain evolution. WM mediates complex cognitive processes and has reciprocal connections with posterior processing regions [1, 2]. Although the developmental pattern of prefrontal WM in macaques differs markedly from that in humans [3], this has not been explored in our closest evolutionary relative, the chimpanzee. The present longitudinal study of magnetic resonance imaging scans demonstrated that the prefrontal WM volume in chimpanzees was immature and had not reached the adult value during prepuberty, as observed in humans but not in macaques. However, the rate of prefrontal WM volume increase during infancy was slower in chimpanzees than in humans. These results suggest that a less mature and more protracted elaboration of neuronal connections in the prefrontal portion of the developing brain existed in the last common ancestor of chimpanzees and humans, and that this served to enhance the impact of postnatal experiences on neuronal connectivity. Furthermore, the rapid development of the human prefrontal WM during infancy may help the development of complex social interactions, as well as the acquisition of experience-dependent knowledge and skills to shape neuronal connectivity.

Child Neurology: Epilepsy of infancy with migrating focal seizures

Epilepsy of infancy with migrating focal seizures is a rare, infantile epileptic encephalopathy characterized by normal early development, refractory focal seizures arising independently from both hemispheres, and severe psychomotor retardation. In the revised terminology by the International League Against Epilepsy, it has been classified as an “electro-clinical syndrome” of “unknown cause” with onset in infancy.1 Affected infants have progressive psychomotor retardation and decline of head circumference percentile. We present a 6-month-old boy diagnosed with this entity and discuss the approach to an infant with unexplained refractory seizures. A 6-month-old boy presented with developmental delay and focal seizures. He was the second child born to a third-degree consanguineous couple. His perinatal period had been uneventful and he had attained social smile by the age of 6 weeks. He started having seizures at the age of 2 months. The seizures consisted of deviation of eyes and head along with tonic posturing of arm or leg of either side and associated flushing. The seizures usually lasted for 1–2 minutes. The initial frequency was 2–3 per day but gradually over the next 4 months, it increased to 50–60 per day. He had been treated with phenobarbitone, phenytoin, carbamazepine, topiramate, and levetiracetam without success. He had lost social smile, and not gained any new developmental milestones. Family history was not significant. Examination revealed a well-thriving baby with head circumference of 40 cm (<−2 SD). There were no neurocutaneous markers or dysmorphic features. The neurologic examination revealed reduced interaction and alertness, normal cranial nerves including fundus, and normal tone with brisk tendon reflexes and extensor …

Smiling in newborns during communicative wake and active sleep

Many studies have investigated infant smiling behaviour during wake and active sleep. In the majority of studies authors agreed that two month-old infants exhibit social smiles. However it is not clear if newborns exhibit different kinds of smiles during wake and active sleep to two month-old infants and if the communicative context can affect the quantity and quality of these smiles. The aim of this study was to test if different kinds of communication affects the amount and the quality of smiles during different behavioural states. Smiling behaviour was analyzed during interactive wake (IW) and active sleep (AS), in 40 newborns randomly assigned to 4 groups of 10 newborns each: continuous tactile communication group, discontinuous non periodic tactile communication group, discontinuous periodic tactile communication group, absent communication group. In the total sample the open/closed mouth (social/non social) smiles ratio was 38/5 during IW and 1/28 during AS ( p < .001). Moreover these ratios were different between the four groups. Different kinds of tactile communication affect amount and quality of the smiles of newborns during IW and AS. Moreover the morphological difference of the smiles during IW and AS suggested that for newborns the smiling behaviour during IW could have a social meaning, as it does for two month-old infants.

Laughter: notes on a passion

Uncovering an archive of laughter, from the forbidden giggle to the explosive guffaw. Most of our theories of laughter are not concerned with laughter. Rather, their focus is the laughable object, whether conceived of as the comic, the humorous, jokes, the grotesque, the ridiculous, or the ludicrous. In Laughter, Anca Parvulescu proposes a return to the materiality of the burst of laughter itself. She sets out to uncover an archive of laughter, inviting us to follow its rhythms and listen to its tones. Historically, laughter-especially the passionate burst of laughter-has often been a faux pas. Manuals for conduct, abetted by philosophical treatises and literary and visual texts, warned against it, offering special injunctions to ladies to avoid jollity that was too boisterous. Returning laughter to the history of the passions, Parvulescu anchors it at the point where the history of the grimacing face meets the history of noise. In the civilizing process that leads to laughter's falling into disrepute, as Nietzsche famously put it, we can see the formless, contorted face in laughter being slowly corrected into a calm, social smile. How did the twentieth century laugh? Parvulescu points to a gallery of twentieth-century laughers and friends of laughter, arguing that it is through Georges Bataille that the century laughed its most distinct laugh. In Bataille's wake, laughter becomes the passion at the heart of poststructuralism. Looking back at the century from this vantage point, Parvulescu revisits four of its most challenging projects: modernism, the philosophical avant-gardes, feminism, and cinema. The result is an overview of the twentieth century as seen through the laughs that burst at some of its most convoluted junctures.

A Prospective Study of the Emergence of Early Behavioral Signs of Autism

To examine prospectively the emergence of behavioral signs of autism in the first years of life in infants at low and high risk for autism. A prospective longitudinal design was used to compare 25 infants later diagnosed with an autism spectrum disorder (ASD) with 25 gender-matched low-risk children later determined to have typical development. Participants were evaluated at 6, 12, 18, 24, and 36 months of age. Frequencies of gaze to faces, social smiles, and directed vocalizations were coded from video and rated by examiners. The frequency of gaze to faces, shared smiles, and vocalizations to others were highly comparable between groups at 6 months of age, but significantly declining trajectories over time were apparent in the group later diagnosed with ASD. Group differences were significant by 12 months of age on most variables. Although repeated evaluation documented loss of skills in most infants with ASD, most parents did not report a regression in their child's development. These results suggest that behavioral signs of autism are not present at birth, as once suggested by Kanner, but emerge over time through a process of diminishment of key social communication behaviors. More children may present with a regressive course than previously thought, but parent report methods do not capture this phenomenon well. Implications for onset classification systems and clinical screening are also discussed.

Crime Scene Investigation: The Chief Inspectors’ Display Rules

Television as one factor in the “cycle of role division” (Eagly 1987; Shields 2002) can aid to perpetuate traditional role attitudes as well as set new norms. The aim of the study is to examine gender differences in facial expression of emotion on TV as well as changes of these differences over time. A content analysis of 12 episodes of a prominent German crime series from two time samples (1979–1981 / 2005) was done by employing the Emotional Facial Action Coding System (EmFACS, Friesen and Ekman 1983). Results show that there are gender differences in stereotypically male emotions: Male actors express these emotions significantly more frequently than female actors, while the latter show more “social smiles”. These differences are reduced over time.

Management of bronchiolitis without antibiotics: a multicentre randomized control trial in Bangladesh

To ascertain that antibiotics have no role in the management of bronchiolitis. Multicentre randomized control trial (RCT). Five purposively selected teaching hospitals in Bangladesh. Children under 24 months old with bronchiolitis. Children were randomized into three groups of therapeutic interventions: parenteral ampicillin (P-Ab), oral erythromycin (O-Ab) and no antibiotic (N-Ab) in adjunct to supportive measures. Clinical improvement was assessed using 18 symptoms/signs which were graded on a two-point recovery scale of 'rapid' and 'gradual', indicating improvement within 'four days' and 'beyond four days', respectively. Each intervention group consisted of 98 +/- 1 children having comparable clinico-epidemiological characteristics at the baseline. The trial revealed that most chesty features (features appearing to arise from chest, i.e. cough, breathing difficulty, wheeze, chest indrawing, tachypnoea, tachycardia, rhonchi and crepitation) demonstrated a gradual recovery, beyond 4th admission day and, not differing among the three intervention groups (p > 0.23, p < 0.62, p = 0.54, p < 0.27, p = 0.75, p = 0.76, p = 0.81, p > 0.98, respectively). Most non-chesty features (features appearing to arise away from chest, i.e. feeding/sleeping difficulties, social smile, restlessness, inconsolable crying, nasal flaring, fever and hypoxaemia) demonstrated a rapid recovery, within 4 days, remaining comparable among the three intervention groups (p < 0.07, p = 0.65, p = 0.24, p < 0.61, p = 0.22, p = 0.84, p = 0.29 and p = 0.96, respectively). However, nasal symptoms (runny nose and nasal blockage) also showed no difference among groups (p = 0.36 and p = 0.66, respectively). Thus, the dynamics of clinical outcome obviates that children not receiving antibiotics had similar clinical outcome than those who did. In hospital settings, managing bronchiolitis with only supportive measures but without antibiotics remains preferable.

Can we observe spontaneous smiles in 1-year-olds?

Five infants were observed longitudinally. In over 30 h of observations, seven spontaneous smiles and one spontaneous laugh were found. All smiles were observed in infants between the ages of 10 and 15 months. These data indicate that spontaneous smiles do not disappear at 2 months of age and they still exist at over 15 months. This disproves some emotional expression theories, where spontaneous smiles are considered to be precursor to and replaced by social smiles. Our data suggest that those theories must be revised and provide new perspectives on this field of studies.

Low bispectral index values in hydranencephaly

Editor, We read with great interest the article by Prabhakar et al. [1] regarding the use of bispectral index (BIS) monitoring for an elective frontal craniotomy and fenestration in a 2-year-old boy with bilateral frontal lobe porencephalic cysts. During the procedure, the patient presented low preanaesthetic BIS values (39) that showed no significant change (between 30 and 40), despite a reduction in the inspired concentration of sevoflurane from 1 to 0.2%. No burst suppression pattern was observed, and BIS values remained low after anaesthetic recovery. In the discussion, the authors suggest that intracranial pressure increments could be responsible for the low BIS values.

Evolução das manifestações pré-linguísticas em crianças normais no primeiro ano de vida

OBJETIVO: A presente pesquisa teve como objetivo determinar a frequência e a ocorrência de elementos pré-linguísticos em crianças normais durante o primeiro ano de vida. MÉTODOS: A amostra constituiu-se de 33 crianças nascidas a termo e normais, no Hospital de Clínicas de Porto Alegre, selecionadas aleatoriamente para um estudo de coorte. As informações, de acordo com as variáveis sorriso social, sons inarticulados, balbucio monossilábico, balbucio polissilábico, primeiras palavras/palavras-frase, jargão e frases, foram obtidas através do inquérito (informações dos pais), da observação espontânea do examinador e de estímulos facilitadores. RESULTADOS: A partir dos resultados obtidos, é possível estabelecer um padrão de desenvolvimento normal das manifestações pré-linguísticas. CONCLUSÕES: Os resultados indicaram a necessidade de revisar dados de avaliação de linguagem já estabelecidos por pesquisas realizadas no passado e as avaliações em uso atualmente.

The Social Orienting Continuum and Response Scale (SOC-RS): A Dimensional Measure for Preschool-aged Children

Children with autism show deficits in social referencing, joint attention, orienting to their names, and social smiling as early as the first year of life. The present study describes the development of the Social Orienting Continuum and Response Scale (SOC-RS), a quantitative scale assessing each of these behaviors during the course of video-recorded Autism Diagnostic Observation Schedule (ADOS) sessions. The SOC-RS was shown to be reliable and valid, and when applied to a longitudinal sample of children studied at 2 and 4 years of age, was shown to be sensitive to decreased levels of social referencing, joint attention, orienting to name, and social smiling in autism. The implications of these findings and potential applications of the SOC-RS are discussed.