Abstract
An efficient and direct measure of social interactions and autism symptoms is needed for fragile X syndrome (FXS) research and clinical care. The Autism Screening Instrument for Educational Planning-Third Edition (ASIEP-3) Interaction assessment is a brief standardized measure that quantifies social responses under different conditions. The feasibility and validity of the ASIEP-3 was evaluated in 26 males and 13 females with FXS, along with cognitive testing and behavior questionnaires. The videos were scored at 10-second intervals, and the observed behaviors were scored as an interaction, independent play, no response, or aggression. In total, 39/41 participants successfully completed the ASIEP-3 (age M = 14.4 ± 10.2), with a range of cognitive abilities (abbreviated IQ (ABIQ) M = 58.9 ± 17.3, median = 50), behaviors (Aberrant Behavior Checklist (ABC) Total M = 37.00 ± 27.3), and autism diagnoses (N = 22/39). Reliable administration was demonstrated by all team members. The mean coded behaviors included interaction (40.6%), independent play (36.8%), no response (21.1%), and aggressive behavior (<10%). The interaction score was negatively correlated with the Social Communication Questionnaire (SCQ) score (p = 0.037), and the profiles differed by autism spectrum disorder (ASD) diagnosis. The intraclass correlation coefficients (ICCs) ranged from 0.79 to 0.93 for master’s level and above. Administration of the ASIEP-3 was feasible for FXS across sex, age, ability, and behavior ratings by a trained research team. Reliable scoring required advanced training in the assessment of social development and FXS experience. The scores correlated to ratings and diagnoses of ASD. The ASIEP-3 shows promise to reliably index social interactions in FXS.
Highlights
Fragile X syndrome (FXS) is the most common inherited form of intellectual disability (ID) and the most commonly known genetic cause of autism
A feasibility assessment was conducted to determine the percentage of individuals who participated in the ASIEP-3 and yielded useable data
As the ASIEP-3 was originally developed for use up to age thirteen, we examined the ASIEP-3 scores by age group to determine if the participants outside of the original standardization range had reasonable score profiles
Summary
Fragile X syndrome (FXS) is the most common inherited form of intellectual disability (ID) and the most commonly known genetic cause of autism It is caused by a trinucleotide expansion (CGG). The spectrum of phenotypic expression of FXS in males includes early developmental delays and intellectual disabilities ranging from mild to severe (with approximately 10% exhibiting a borderline IQ) due to decreased methylation of the CGG expansion or mosaicism [4,5]. Social difficulties are common as a result of these cognitive and speech deficits and anxiety, but other symptoms, including gaze aversion, social communication deficits, abnormal sensory reactivity, motor stereotypies, and ritualistic behaviors, may lead to a co-occurring diagnosis of autism spectrum disorder (ASD). Females with FXS can show higher rates of anxiety, social withdrawal, avoidance, and an increased risk for depression compared to typical females [13,14,15]
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