Autism Spectrum Symptomatology in Children with Williams Syndrome Who Have Phrase Speech or Fluent Language

Abstract

To characterize autism spectrum-related symptomatology in children with Williams syndrome (WS) with phrase speech or fluent language, the Autism Diagnostic Observation Schedule Module 2 or 3 was administered. The cutoff for autism spectrum was met by 35% (14/40) who completed Module 2 and 30% (18/60) who completed Module 3. Similarities and differences in socio-communicative strengths and weaknesses as a function of language ability were identified. Symptom severity was negatively associated with IQ for participants with phrase speech but not for those with fluent language. The findings suggest an elevated risk of ASD for individuals with WS relative to the general population and contribute to a more nuanced sense of the socio-communicative functioning of children with WS.

Keywords: Autism spectrum disorders; Intellectual disability; Social communication; Williams syndrome.

Figure 1

Distribution of ADOS Classifications and Comparison Scores by Module

Figure 2. Module 2 Items with Infrequent Abnormality: Item Ratings

Note: Shared Enjoyment = Shared Enjoyment in Interaction; Initiation of JA = Spontaneous Initiation of Joint Attention; Response to JA = Response to Joint Attention; Self-Injurious Bx = Self-Injurious Behavior. The numbers on each bar indicate how many children (out of 40) received that score for that item.

Figure 3. Module 3 Items with Infrequent Abnormality: Item Ratings

Note: Unusual EC = Unusual Eye Contact; Shared Enjoyment = Shared Enjoyment in Interaction; Amt RSI = Amount of Reciprocal Social Interaction; Sensory Int = Unusual Sensory Interests in Play Material/Person; Mannerisms = Hand, Finger, and Other Complex Mannerisms; Rep Bx = Excessive Interest in or References to Unusual or Highly Specific Topics or Objects or Repetitive Behavior; Tant, Agg, Neg or Disruptive Bx = Tantrums, Aggression, Negative or Disruptive Behavior. The numbers on each bar indicate how many children (out of 60) received that score for that item.

Figure 4. Module 2 Items with Frequent Abnormality: Item Ratings

Note: Speech Abnormalities = Speech Abnormalities Associated with Autism; Directed Facial Exp = Directed Facial Expressions. The numbers on each bar indicate how many children (out of 40) received that score for that item.

Figure 5. Module 3 Items with Frequent Abnormality: Item Ratings

Note: Speech Abnormalities = Speech Abnormalities associated with Autism; Directed Facial Exp: Facial Expressions Directed toward Others; Empathy/Others’ Emotions = Empathy/Comments on Others’ Emotions. The numbers on each bar indicate how many children (out of 60) received that score for that item.