Individuals with Down syndrome (DS) are often described as socially engaged; however, challenges with social cognition, expressive language, and social interaction are also common in DS and are prospective outcomes of interest for clinical trials. The current study evaluates the psychometric properties of standardized measurements of social cognition and social behavior for potential use as outcome measures for children and adolescents with DS. Seventy-three youth ages 6 to 17 years old (M = 12.67, SD = 3.16) with DS were assessed on social cognition subtests of a neuropsychological assessment at two time points. Caregivers also completed a parent-report measure of social behavior. Measures were evaluated for feasibility, test-retest reliability, practice effects, convergent validity, and associations with broader developmental domains (i.e., age, cognition, and language). All social cognition and behavior measures met criteria for a portion of the psychometric indices evaluated, yet feasibility limitations were identified for the Developmental Neuropsychological Assessment, Second Edition (NEPSY-II) Affect Recognition subtest, and the NEPSY-II Theory of Mind subtest had problematic floor effects for percentile ranks. The Social Responsiveness Scale, Second Edition (SRS-2; T-scores) had high feasibility, moderate to excellent test-retest reliability, and no practice effects, suggesting this measure could be appropriate for use in clinical trials involving youth with DS.
Background
There is a critical need for the psychometric evaluation of outcome measures to be used in clinical trials targeting cognition in Down syndrome (DS). This study examines a specific cognitive skill that is of particular importance in DS, working memory, and the psychometric properties of a set of standardised measurements to assess working memory in individuals with DS.
Methods
Ninety children and adolescents ages 6 to 18 years old with DS were assessed on a selection of verbal and visuospatial working memory subtests of standardised clinical assessments at two time points to examine feasibility, distributional qualities, test–retest reliability and convergent validity against a priori criteria. Caregivers also completed an adaptive behaviour questionnaire to address working memory subtests' associations with broader developmental functioning.
Results
The Stanford Binet‐5 Verbal Working Memory, Differential Ability Scales‐2 Recognition of Pictures, Stanford Binet‐5 Nonverbal Working Memory and Wechsler Intelligence Scale for Children‐5 Picture Span measures met the most psychometric criteria overall across the full age and IQ range of the study. Although Differential Ability Scales‐2 Recall of Sequential Order and Differential Ability Scales‐2 Recall of Digits Backward met the fewest a priori criteria, follow‐up analyses suggested greater feasibility in specific age and IQ ranges.
Conclusions
Several working memory measures appear to be psychometrically sound and appropriate for use in clinical trials for children with DS, especially when focusing on raw scores. However, floor effects on standard scores and feasibility of some measures were problematic. Guidelines for use of the working memory subtests with this population are provided.
Background
While delays in cognitive development are detectable during early development in Down syndrome, the neuropsychological and biomedical underpinnings of cognitive skill acquisition in this population remain poorly understood.
Method
To explore this issue, 38 infants with Down syndrome [mean chronological age = 9.65 months; SD = 3.64] completed the Bayley Scales of Infant Development‐III and a set of laboratory tasks that measured sustained attention (duration of visual attention during a 1‐min object exploration task), attention shifting (mean latency to shift attention on an alternating object presentation task) and visual short‐term memory (dishabituation to a novel object on a change preference task).
Results
Latency to shift attention was negatively associated with Bayley Cognitive Scale raw scores, even when controlling for the effects of chronological age, r (33) = −.41, P = .02. In addition, prematurity status was associated with latency to shift attention.
Conclusions
Early attention shifting may be an important factor that facilitates overall cognitive skill acquisition in infants with Down syndrome, and premature birth may be a risk factor for difficulties on this dimension.
BackgroundDown syndrome (DS) is a neurogenetic disorder associated with risk for executive dysfunction, or difficulties with the cognitive processes required for planning volitional, goal‐directed behaviour. This study examines the developmental origins of difficulties with goal‐directed action planning in infants with DS to inform our understanding of the cognitive phenotype associated with DS and its implications for intervention.MethodFirst, the study compared the performance of infants with DS (n = 44, mean chronological age = 7.5 months, SD = 2.3) and typically developing infants (n = 31, mean chronological age = 7.5 months, SD = 2.9) on plan production and planning efficiency during an early planning task. Next, potential sources of variability in planning behaviour (motor performance and sensory processing) within the DS sample were examined. All infants completed an early planning laboratory task and the Bayley Scales of Infant Development‐III Cognitive Scale. The motor and sensory skills of infants with DS were assessed by the motor scales of the Bayley Scales of Infant Development‐III and the Infant Sensory Profile‐2. DS‐related biomedical history information was provided by caregivers for the infants with DS.ResultsBetween‐group differences in planning were observed on the dimensions of strategy production and completion, such that infants with DS were less efficient in their strategy execution than typically developing infants. In the DS group, motor skills and sensory processing were associated with planning efficiency on all components of the early planning task.ConclusionsLess efficient action planning in infants with DS may disrupt the shaping of goal‐directed behaviour, and the identification of early risk factors associated with planning efficiency has important implications for early intervention.
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