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Table 2 Summary statistics for clinical measures

From: Prospective and detailed behavioral phenotyping in DDX3X syndrome

Domain Evaluation Variable n Range Mean SD % with atypical score
Intellectual and Adaptive Functioning Mullen Scales, Stanford-Binet-5, DAS-II1 Full scale DQ 15 9.9–97.8 34.1 22.5 93
Nonverbal DQ 15 14.1–78.4 34.1 16.8 93
Verbal DQ 15 5.8–117.3 35.1 28.7 93
Vineland-32 Communication 15 20–87 43.7 24.7 87
Daily Living Skills 15 20–73 48.6 17.5 100
Socialization 15 20–90 55.3 20.6 87
Adaptive Behavior Composite 15 20–76 49.5 18.8 100
Motor Functioning ADI-R, Psychiatric Evaluation2 Age of crawling (months) 15 5–24 15.2 6.0 87
Age of walking independently (months) 13 11–36 26 8.1 93
Vineland-32 Motor Skills 10 20–77 57.6 18.6 100
Fine Motor 10 1–10 6.0 3.7 100
Gross Motor 10 1–12 7.9 4.3 60
VMI-63 Standard score 13 < 45–87 68.3 10.0 85
DCDQ4 Total score 8 15–45 23.8 9.7 100
Expressive and Receptive Language ADI-R, Psychiatric Evaluation2 Age of first word (months) 10 15–60 28.7 14.0 87
Age of phrased speech (months) 7 36–60 48.6 9.8 100
Expressive Vocabulary Test2 Standard score 7 44–123 77.6 23.5 57
Peabody Picture Vocabulary Test2 Standard score 13 20–111 59.7 31.2 69
Vineland-32 Expressive Language 15 1–14 3.6 4.3 93
Receptive Language 15 1–14 5.7 4.8 80
MacArthur-Bates Communicative Development Inventory Words Understood 13 15–396 277.9 125.8 n/a
Words Produced 13 0–396 164.8 186.1 n/a
Early Gestures 13 6–18 12.7 4.9 n/a
Later Gestures 13 4–45 27.2 15.4 n/a
Total Gestures 13 10–63 39.9 19.5 n/a
ASD Symptomatology SRS-25 Total score 15 50–96 68.9 14.0 73
Social Communication 15 48–94 69.7 16.4 60
Social Awareness 15 56–90 70.4 10.6 80
Social Cognition 15 51–84 66.8 10.7 80
Repetitive Behavior 15 52–90 72.6 13.5 80
Social Motivation 15 38–86 58.2 14.1 47
RBS-R Insistence on Sameness 14 0–9 2.57 2.93 n/a
Stereotyped Behavior 14 0–6 2.64 2.02 n/a
Sensory Features SAND2 Sensory seeking 15 7–28 17.27 7.3 87
Hyporeactivity 15 0–21 10.40 6.7 80
Hyperreactivity 15 2–11 5.67 3.5 47
Tactile 15 2–19 13.67 4.5 93
Auditory 15 0–23 10.6 6.3 73
Visual 15 0–17 9.07 5.5 73
Behavioral Comorbidities CBCL6 Internalizing 14 33–69 53.50 9.51 14
Depressive 14 50–77 58.86 8.42 21
Anxiety 14 50–76 54.71 7.06 14
Externalizing 14 44–73 58.43 8.11 21
ADHD 14 54–72 64.29 6.60 50
Oppositional/Defiant 14 51–64 54.71 5.30 0
Aberrant Behavior Checklist5 Hyperactivity 15 < 40–65 53.60 7.50 20
Irritability 15 < 40–67 50.67 9.79 13
Lethargy/Social Withdrawal 15 < 40–60 48.11 7.64 7
Stereotypy 15 40–73 49.53 10.14 20
Inappropriate Speech 15 40–67 48.93 8.03 7
Vineland-32 Internalizing Behavior 15 15–22 18.5 2.2 33
Externalizing Behavior 15 11–21 17.5 2.6 20
  1. DAS-II: Differential Abilities Scales, Second Edition; ADI-R: Autism Diagnostic Interview-Revised; VMI-6: Beery-Buktenica Developmental Test of Visual-Motor Integration, Sixth Edition; DCDQ: Developmental Coordination Disorder Questionnaire; SRS-2: Social Responsiveness Scale, Second Edition; RBS-R: Repetitive Behavior Scales-Revised; SAND: Sensory Assessment for Neurodevelopmental Disorders; CBCL: Achenbach Child Behavior Checklist
  2. 1Atypical if < 77.5
  3. 2Atypical if standard score 1.5 SD above or below population mean
  4. 3Atypical if standard score < 50, per manual
  5. 4Atypical if score 15–46 (5 y–7 y 11 m), 15–55 (8 y–9 y 11 m), 15–57 (10 y–15 y)
  6. 5Atypical if T score > 60
  7. 6Atypical if T score > 65