Investigating Social Competence in a Pilot Randomized Clinical Trial of a Theatre-Based Intervention Enhanced for Adults with Autism Spectrum Disorder.

Autism spectrum disorder (ASD) is characterized by challenges in social competence that persist in adulthood, yet few treatment options exist. A pilot randomized clinical trial (RCT) of a peer-mediated, theatre-based intervention with established efficacy in youth with ASD was examined in autistic adults. The final sample consisted of forty-seven 18-to-40-year-old participants randomized to the experimental (EXP N = 23) or waitlist control (WLC N = 24) condition. A multimodal, social interdependent model was employed to examine social competence changes in brain (incidental face memory (IFM) using event-related potentials), cognition (Wechsler Memory Scale-III), behavior (Contextual Assessment of Social Skills) and function (Social Responsiveness Scale (SRS); Adaptive Behavior Assessment Scale (ABAS) Social Composite). Using analysis of covariance in which pretest was controlled in the model, posttest between-group differences were observed on IFM (p = 0.016, η2 = 0.139, d = 0.79) and several social and adaptive functional (SRS, ABAS) outcomes in social communication and interaction (SCI) (p = 0.019, η2 = 0.121, d = -00.45), communication (p = 0.044 η2 = 0.09, d = -00.31), and motivation (p = 0.001, η2 = 0.229, d = -0.79) domains. At two-month follow-up, gains in social motivation remained (p = 0.041, η2 = 0.100, d = -0.77). The results offer preliminary support for a unique theatre-based social skills intervention for autistic adults who have few treatment options to enhance social competence. The trial was pre-registered with ClinicalTrials.gov (Identifier: NCT04349644).

Typical and atypical neural mechanisms support spoken word processing in Angelman syndrome.

Angelman syndrome (AS) is known to affect expressive and receptive communication abilities. This study examined individual differences in neural mechanisms underlying speech processing in children with AS (n = 24, M age = 10.01 years) and typical development (n = 30, M age = 10.82 years) using auditory event-related potentials during passive listening to common English words and novel pseudowords. A group of adults with AS (n = 7, M = 31.78 years) provided data about the upper developmental range. The typically developing group demonstrated the expected more negative amplitudes in response to words than pseudowords within 250-500 ms after stimulus onset at the left temporal scalp region. Children and adults with AS exhibited a similar left-lateralized pattern of word-pseudoword differentiation at temporal and parietal regions, but not the midline parietal memory response for known words observed in the typically developing group, suggesting typical-like word-pseudoword differentiation along with possible alterations in the automatic recall of word meaning. These results have important implications for understanding receptive and expressive communication processes in AS and support the use of auditory neural responses for characterizing individual differences in neurodevelopmental disorders with limited speech.

Sex differences in automatic emotion regulation in adolescents with autism spectrum disorder.

Autism may be underdiagnosed in females because their social difficulties are often less noticeable. This study explored sex differences in automatic facial emotion processing in 45 adolescents with autism spectrum disorder (22 female, 23 male), age 10-16 years, performing active target detection task and Go/NoGo tasks where faces with positive and negative emotional expressions served as irrelevant distractors. The combined sample demonstrated more accurate performance on the target detection (response initiation) than the Go/NoGo task (response inhibition), replicating findings previously reported in typical participants. Females exhibited greater difficulty than males with response initiation in the target detection task, especially in the context of angry faces, while males found withholding a response in the Go/NoGo block with happy faces more challenging. Electrophysiological data revealed no sex differences or emotion discrimination effects during the early perceptual processing of faces indexed by the occipitotemporal N170. Autistic males demonstrated increased frontal N2 and parietal P3 amplitudes compared to females, suggesting greater neural resource allocation to automatic emotion regulation processes. The associations between standardized behavioral measures (autism severity, theory of mind skills) and brain responses also varied by sex: more adaptive social functioning was related to the speed of perceptual processing (N170 latency) in females and the extent of deliberate attention allocation (P3 amplitudes) in males. Together, these findings suggest that males and females with autism may rely on different strategies for social functioning and highlight the importance of considering sex differences in autism. LAY SUMMARY: Females with autism may exhibit less noticeable social difficulties than males. This study demonstrates that autistic females are more successful than males at inhibiting behavioral responses in emotional contexts, while males are more likely to initiate a response. At the neural level, social functioning in females is related to the speed of automatic perceptual processing of facial cues, and in males, to the extent of active attention allocation to the stimuli. These findings highlight the importance of considering sex differences in autism diagnosis and treatment selection.

Visual food cue processing in children with Prader-Willi Syndrome.

Hyperphagia and the associated interest in food is a characteristic feature of Prader-Willi syndrome (PWS) that emerges during childhood and remains a life-long concern. This study examined neural responses reflecting food cue salience in children with PWS and typical controls, age 3-12 years. Visual event-related potentials were recorded while participants in satiated state passively viewed photographs of high- and low-calorie foods, animals, and neutral objects. Contrary to the prediction, children with PWS did not demonstrate greater than typical neural responses to food, suggesting that it is not an exceptionally motivationally salient stimulus in PWS. Caregiver reports of greater hyperphagia were associated with neural responses to low-calorie foods suggesting accelerated and more fine-grained visual stimulus categorization in terms of edibility and caloric content. Overall, the findings align more closely with the altered satiety rather than increased food reward models of hyperphagia in PWS.

Spoken word processing in Rett syndrome: Evidence from event-related potentials.

This study examined the feasibility of using auditory event-related potentials to evaluate spoken word processing during passive listening in girls with Rett syndrome (n = 11) and typical peers (n = 33), age 4-12 years. The typical group demonstrated the expected pattern of more negative amplitudes within 200-500 ms in response to words than nonwords at left temporal sites. In participants with Rett syndrome, word-nonword differentiation was observed at the right temporal sites. More negative left hemisphere amplitudes in response to words were associated (at trend level) with better receptive language skills and more adaptive behavior. The results indicate that girls with Rett syndrome differentiate known words from novel nonwords, but may do so using potentially atypical neural processes. Brain-behavior correlations support validity of the proposed neural markers of word processing, making passive listening paradigms a promising approach for assessing speech and language processing in participants with limited spoken language skills.

Feasibility of using auditory event-related potentials to investigate learning and memory in nonverbal individuals with Angelman syndrome.

The combination of intellectual, communicative, and motor deficits limit the use of standardized behavioral assessments of cognition in individuals with Angelman syndrome (AS). The current study is the first to objectively evaluate learning and memory in AS using auditory event-related potentials (ERP) during passive exposure to spoken stimuli. Fifteen nonverbal individuals with the deletion subtype of AS (age 4-45 years) completed the auditory incidental memory paradigm. Auditory ERPs were recorded in response to a sequence of unfamiliar nonwords, in which one randomly selected stimulus was repeated multiple times and the rest were presented once. Larger parietal responses within 200-500 ms for the repeated nonword compared to novel distracters were associated with caregiver reports of more adaptive communication skills. These findings demonstrate good tolerability of ERP procedures (94% success rate) and indicate that persons with AS can acquire new information following repeated auditory exposure, even in the absence of explicit memorization instructions. Strong associations between the caregiver reports of adaptive functioning and neural indices of auditory learning and memory support the utility of brain-based measures for objectively evaluating higher-order information processing in nonverbal persons with neurodevelopmental disorders.

Distinguishing response to names in Rett and MECP2 Duplication syndrome: An ERP study of auditory social information processing.

Despite significant advances at the level of basic research, the characterization of higher-level processes in Rett and MECP2 Duplication syndrome remains understudied. In this pilot study, we assessed social-emotional information processing by testing whether children (ages 4-12years) with Rett (n=9) and MECP2 Duplication syndrome (n=7) distinguished their own spoken name from other names. We hypothesized that own and familiar names would elicit more positive parietal P300 responses than unknown names, and that the groups would have different neural responses to these stimuli. The MECP2 Duplication group partially mirrored the parietal responses to own name observed in typically developing participants, and better name discrimination correlated with more adaptive behaviors. Conversely, participants with RTT did not resemble the typical group, and showed greater responses to close other names at frontal/central regions. These results may reflect the different consequences of too much (MECP2 Duplication) vs. too little (RTT) MeCP2 protein.

Response to own name in children: ERP study of auditory social information processing.

Auditory processing is an important component of cognitive development, and names are among the most frequently occurring receptive language stimuli. Although own name processing has been examined in infants and adults, surprisingly little data exist on responses to own name in children. The present ERP study examined spoken name processing in 32 children (M=7.85years) using a passive listening paradigm. Our results demonstrated that children differentiate own and close other's names from unknown names, as reflected by the enhanced parietal P300 response. The responses to own and close other names did not differ between each other. Repeated presentations of an unknown name did not result in the same familiarity as the known names. These results suggest that auditory ERPs to known/unknown names are a feasible means to evaluate complex auditory processing without the need for overt behavioral responses.

Social and emotional processing in Prader-Willi syndrome: genetic subtype differences.

BACKGROUND: People with Prader-Willi syndrome (PWS) demonstrate social dysfunction and increased risk of autism spectrum disorder, especially those with the maternal uniparental disomy (mUPD) versus paternal deletion genetic subtype. This study compared the neural processing of social (faces) and nonsocial stimuli, varying in emotional valence, across genetic subtypes in 24 adolescents and adults with PWS. METHODS: Upright and inverted faces, and nonsocial objects with positive and negative emotional valence were presented to participants with PWS in an oddball paradigm with smiling faces serving as targets. Behavioral and event-related potential (ERP) data were recorded. RESULTS: There were no genetic subtype group differences in accuracy, and all participants performed above chance level. ERP responses revealed genetic subtype differences in face versus object processing. In those with deletions, the face-specific posterior N170 response varied in size for face stimuli versus inverted faces versus nonsocial objects. Persons with mUPD generated N170 of smaller amplitude and showed no stimulus differentiation. Brain responses to emotional content did not vary by subtype. All participants elicited larger posterior and anterior late positive potential responses to positive objects than to negative objects. Emotion-related differences in response to faces were limited to inverted faces only in the form of larger anterior late positive potential amplitudes to negative emotions over the right hemisphere. Detection of the target smiling faces was evident in the increased amplitude of the frontal and central P3 responses but only for inverted smiling faces. CONCLUSION: Persons with the mUPD subtype of PWS may show atypical face versus object processes, yet both subtypes demonstrated potentially altered processing, attention to and/or recognition of faces and their expressions.