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Table 3 Assessment of the autistic behavioral domains and subdomains based on the Autism Diagnostic Interview–Revised algorithm a

From: Presence of autism, hyperserotonemia, and severe expressive language impairment in Williams-Beuren syndrome

Assessment Patient 1 Patient 2
  Ages 4 to 5 years Age 17 years Ages 4 to 5 years Age 19 years
Total social interaction 2 1 2 1
 B1: Failure to use nonverbal behaviors to regulate social interaction 2 1 2 1
 B2: Failure to develop peer relationships 2 1 3 1
 B3: Lack of shared enjoyment 3 2 3 1
 B4: Lack of socioemotional reciprocity 2 1 2 1
Total nonverbal communication 3 2 3 1
 C1: Delayed spoken language and failure to compensate through gesture 2 2 2 1
 C4: Lack of varied spontaneous make-believe or social imitative play 3 1 3 0
Total repetitive behaviors and stereotyped patterns 3 2 2 2
 D1: Encompassing preoccupation or circumscribed pattern of interestb 3 2 2 2
 D2: Compulsive adherence to nonfunctional routines or rituals 1 1 2 0
 D3: Stereotyped and repetitive motor mannerismsc 3 2 3 3
 D4: Preoccupations with part of objects or nonfunctional elements 2 1 1 2
  1. aThe scoring has been transformed in median values as previously described [45] (0: autistic behavior is not present, 1: mild impairment, 2: moderate impairment, and 3: severe impairment). bPatient 1 showed unusual repetitive sensory interests, such as fascination for spinning objects and water in the shower, sniffing people, actively seeking “wind sensations” (manual or electric fan) and interest in musical objects with hyperresponsivity to auditory stimuli. Patient 2 showed unusual repetitive sensory interests, such as fascination with spinning objects and water in the bath, sniffing people and interest in musical objects with hyperacusis to loud sounds. (He covered his ears at screams, babies crying and sounds of an airplane). cPatient 1 showed finger mannerisms and head-rocking, and patient 2 showed hand-flapping and toe-walking.