General Conceptual Ability Scores Research Articles

Association of Mild Iodine Insufficiency during Pregnancy with Child Neurodevelopment in Patients with Subclinical Hypothyroidism or Hypothyroxinemia.

Our objective was to evaluate whether iodine status in pregnant patients with either subclinical hypothyroidism or hypothyroxinemia in the first half of pregnancy is associated with measures of behavior and neurodevelopment in children through the age of 5 years. This is a secondary analysis of a multicenter study consisting of two randomized, double-masked, placebo-controlled treatment trials conducted in parallel. Patients with a singleton gestation before 20 weeks' gestation underwent thyroid screening using serum thyrotropin and free thyroxine. Participants with subclinical hypothyroidism or hypothyroxinemia were randomized to levothyroxine replacement or an identical placebo. At randomization, maternal urine was collected and stored for subsequent urinary iodine excretion analysis. Urinary iodine concentrations greater than 150 μg/L were considered iodine sufficient, and concentrations of 150 μg/L or less were considered iodine insufficient. The primary outcome was a full-scale intelligence quotient (IQ) score at the age of 5 years, the general conceptual ability score from the Differential Ability Scales-II at the age of 3 if IQ was not available, or death before 3 years. A total of 677 pregnant participants with subclinical hypothyroidism and 526 with hypothyroxinemia were randomized. The primary outcome was available in 1,133 (94%) of children. Overall, 684 (60%) of mothers were found to have urinary iodine concentrations >150 μg/L. Children of iodine-sufficient participants with subclinical hypothyroidism had similar primary outcome scores when compared to children of iodine-insufficient participants (95 [84-105] vs. 96 [87-109], P adj = 0.73). After adjustment, there was also no difference in IQ scores among children of participants with hypothyroxinemia at 5 to 7 years of age (94 [85 - 102] and 91 [81 - 100], Padj 1/4 0.11). Treatment with levothyroxine was not associated with neurodevelopmental or behavioral outcomes regardless of maternal iodine status (p > 0.05). Maternal urinary iodine concentrations ≤150 μg/L were not associated with abnormal cognitive or behavioral outcomes in offspring of participants with either subclinical hypothyroidism or hypothyroxinemia. · Most pregnant patients with subclinical thyroid disease are iodine sufficient.. · Mild maternal iodine insufficiency is not associated with lower offspring IQ at 5 years.. · Iodine supplementation in subclinical thyroid disease is unlikely to improve IQ..

Neurodevelopmental status and adaptive behavior of pediatric patients with mucopolysaccharidosis II: a longitudinal observational study

BackgroundMucopolysaccharidosis (MPS) II is a rare, X-linked lysosomal storage disease. Approximately two-thirds of patients have central nervous system involvement with some demonstrating progressive cognitive impairment (neuronopathic disease). The natural history of cognitive and adaptive function in patients with MPS II is not well-defined. This 2-year, prospective, observational study evaluated the neurodevelopmental trajectories of boys with MPS II aged ≥ 2 years and < 18 years.ResultsOverall, 55 patients were enrolled. At baseline, mean (standard deviation [SD]) age was 5.60 (3.32) years; all patients were receiving intravenous idursulfase. Cognitive and adaptive function were assessed using the Differential Ability Scales, Second Edition (DAS-II) General Conceptual Ability (GCA) and the Vineland Adaptive Behavior Scales, Second Edition (VABS-II) Adaptive Behavior Composite (ABC) scores, respectively. Baseline mean (SD) DAS-II GCA and VABS-II ABC scores were 78.4 (19.11) and 83.7 (14.22), respectively, indicating low cognitive function and moderately low adaptive behavior. Over 24 months, modest deteriorations in mean (SD) scores were observed for DAS-II GCA (−3.8 [12.7]) and VABS-II ABC (−2.0 [8.07]). Changes in DAS-II GCA scores varied considerably, and data suggested the existence of four potential patient subgroups: (1) patients with marked early impairment and rapid subsequent decline, (2) patients with marked early impairment then stabilization, (3) patients with mild early impairment then stabilization, and (4) patients without impairment who remained stable. Subgroup analyses revealed numerically greater DAS-II GCA score reductions from baseline in patients aged < 7 years at baseline (vs. those aged ≥ 7 years) and in patients with DAS-II GCA scores ≤ 70 at baseline (vs. those with scores > 70); between-group differences were nonsignificant. No clear subgroups or patterns were identified for individual changes in VABS-II ABC scores. In total, 49 patients (89.1%) reported ≥ 1 adverse event (AE) and nine patients (16.4%) reported serious AEs.ConclusionsSome patients with MPS II had rapid declines in cognitive ability, whereas others remained relatively stable after an initial decline. These insights provide a basis for more detailed analyses of different patient subgroups, which may enhance the definition and understanding of factors that influence cognitive and adaptive function in MPS II.Trial registrationClinicalTrials.gov, NCT01822184. Registered retrospectively: April 2, 2013.

Cognitive and adaptive behaviors associated with disease severity and genotype in patients with mucopolysaccharidosis II

BackgroundMucopolysaccharidosis II (MPS II) is a rare, X-linked lysosomal storage disease caused by pathogenic variants of the iduronate-2-sulfatase gene (IDS) and is characterized by a highly variable disease spectrum. MPS II severity is difficult to predict based on IDS variants alone; while some genotypes are associated with specific phenotypes, the disease course of most genotypes remains unknown. This study aims to refine the genotype–phenotype categorization by combining information from the scientific literature with data from two clinical studies in MPS II. MethodsGenotype, cognitive, and behavioral data from 88 patients in two clinical studies (NCT01822184, NCT02055118) in MPS II were analyzed post hoc in combination with published information on IDS variants from the biomedical literature through a semi-automated multi-stage review process. The Differential Ability Scales, second edition (DAS-II) and the Vineland Adaptive Behavior Scales™, second edition (VABS-II) were used to measure cognitive function and adaptive behavior. ResultsThe most common category of IDS variant was missense (47/88, 53.4% of total variants). The mean (standard deviation [SD]) baseline DAS-II General Conceptual Ability (GCA) and VABS-II Adaptive Behavior Composite (ABC) scores were 74.0 (16.4) and 82.6 (14.7), respectively. All identified IDS complete deletions/large rearrangements (n = 7) and large deletions (n = 1) were associated with a published ‘severe’ or ‘predicted severe’ progressive neuronopathic phenotype, characterized by central nervous system involvement. In categories comprising more than one participant, mean baseline DAS-II GCA scores (SD) were lowest among individuals with complete deletions/large rearrangements 64.0 (9.1, n = 4) and highest among those with splice site variants 83.8 (14.2, n = 4). Mean baseline VABS-II ABC scores (SD) were lowest among patients with unclassifiable variants 79.3 (4.9, n = 3) and highest among those with a splice site variant 87.2 (16.1, n = 5), in variant categories with more than one participant. ConclusionsMost patients in the studies had an MPS II phenotype categorized as ‘severe’ or ‘predicted severe’ according to classifications, as reported in the literature. Patients with IDS complete deletion/large rearrangement variants had lower mean DAS-II GCA scores than those with other variants, as well as low VABS-II ABC, confirming an association with the early progressive ‘severe’ (neuronopathic) disease. These data provide a starting point to improve the classification of MPS II phenotypes and the characterization of the genotype–phenotype relationship.

Analysis of cognitive ability and adaptive behavior assessment tools used in an observational study of patients with mucopolysaccharidosis II

BackgroundMucopolysaccharidosis II (MPS II) is a rare lysosomal storage disease characterized by cognitive impairment in most patients. This post hoc analysis evaluated changes in cognitive function, adaptive behavior and functional outcomes in patients with neuronopathic MPS II over time. Fifty-five children with MPS II were enrolled in a 24-month observational study (NCT01822184). The Differential Ability Scales, second edition (DAS-II; early years battery for ages 2 years 6 months to 6 years 11 months, school age battery for ages 7 years to 17 years 11 months), Vineland Adaptive Behavior Scales, second edition (VABS-II) and the Hunter Syndrome-Functional Outcomes for Clinical Understanding Scale (HS-FOCUS) were performed at baseline and 3-month intervals over 2 years. A subgroup of 38 children with a DAS-II General Conceptual Ability (GCA) score of 55–85 (below average–very low abilities) at any time during the study were included in this analysis.ResultsMean (standard deviation [SD]) early years DAS-II GCA score decreased from 73.4 (15.7, n = 22) at baseline to 62.7 (34.9, n = 6) at month 24. For the six patients with early years GCA assessments at baseline and month 24, mean (SD) GCA scores decreased from 72.3 (21.3) at baseline to 62.7 (34.9) at month 24. School age GCA scores were stable over 2 years: mean (SD) 72.4 (11.8, n = 10) at baseline; 74.3 (12.3, n = 8) at month 24. Mean (SD) VABS-II Adaptive Behavior Composite (ABC) scores were stable throughout the study (baseline, 81.8 [11.8, n = 36]; month 24, 81.0 [10.2, n = 13]). Some associations between items and domains of HS-FOCUS (p < 0.05) and DAS-II GCA and VABS-II ABC scores were shown, but there was no clear pattern of changes in HS-FOCUS over 2 years.ConclusionsThe DAS-II measured changes in cognitive function over 2 years in younger patients with MPS II, whereas cognitive function in older patients remained stable. Further research is required to confirm the content validity of the DAS-II in different patient populations with MPS II. The VABS-II and HS-FOCUS were not sensitive tools for measuring behavioral and functional changes over 2 years. These findings may inform selection of appropriate cognitive and behavioral assessment tools for future studies.

Very Preterm Early Motor Repertoire and Neurodevelopmental Outcomes at 8 Years.

Children born very preterm (<32 weeks' gestation) have more neurodevelopmental problems compared with term-born peers. Aberrant fidgety movements (FMs) are associated with adverse motor outcomes in children born very preterm. However, associations of aberrant FMs combined with additional movements and postures to give a motor optimality score-revised (MOS-R) with school-aged cognitive and motor outcomes are unclear. Our aim with this study was to determine those associations. Of 118 infants born <30 weeks' gestation recruited into a randomized controlled trial of early intervention, 97 had a general movements assessment at 3 months' corrected age and were eligible for this study. Early motor repertoire including FMs and MOS-R were scored from videos of infant's spontaneous movement at 3 months' corrected age. At 8 years' corrected age, cognitive and motor performances were evaluated. Associations of early FMs and MOS-R with outcomes at 8 years were determined using linear regression. Seventy-eight (80%) infants with early motor repertoire data had neurodevelopmental assessments at 8 years. A higher MOS-R, and favorable components of the individual subscales of the MOS-R, including the presence of normal FMs, were associated with better performance for general cognition, attention, working memory, executive function and motor function at 8 years; eg, presence of normal FMs was associated with a 21.6 points higher general conceptual ability score (95% confidence interval: 12.8-30.5; P < .001) compared with absent FMs. Favorable early motor repertoire of infants born <30 weeks is strongly associated with improved cognitive and motor performance at 8 years.

Projected Retained Ability Score (PRAS): A New Methodology for Quantifying Absolute Change in Norm-Based Psychological Test Scores Over Time.

A limitation of norm-based ability test scores is that they can only be used to evaluate relative change (compared with change in the norm sample), as opposed to absolute (raw) change in performance from Time 1 to Time 2. To address this limitation, a novel method (Projected Retained Ability Score [PRAS]) was developed to characterize absolute change in norm-based ability test scores. The PRAS method was applied to Differential Ability Scales®–Second Edition (DAS-II) General Conceptual Ability (GCA) scores in three cases of children with the neurodegenerative condition mucopolysaccharidosis type II (MPS II) who were assessed at two visits, 16 to 23 months apart. Although all three cases showed declines in norm-based GCA scores, the PRAS method revealed differences in absolute change in performance. The PRAS method allows for differentiation of slower-than-average improvement or stabilization versus deterioration of cognitive ability when norm-based scores decline from Time 1 to Time 2.

Cognitive outcome in childhood of birth weight discordant monochorionic twins: the long-term effects of fetal growth restriction

AimIntrauterine growth restriction (IUGR) is associated with poorer outcomes in later life. We used a monochorionic twin model with IUGR in one twin to determine its impact on growth and...

Cognitive deficit in preschoolers born late-preterm

Background Late-preterm (LPT) birth accounts for a majority of preterm deliveries and until recently was considered low risk for poor cognitive outcome. Previously, we reported deficits in complicated LPT (cLPT) preschoolers (neonatal intensive care unit [NICU]-admitted). Aim To extend our prior study by comparing cognitive outcome in cLPT and uncomplicated LPT (uLPT; NICU non-admitted) preschoolers. Study design Single center retrospective cohort study of 118 LPT children born in 2004–2006 at 35–36 weeks of gestation; 90 cLPT and 28 uLPT, compared with 100 term-born (≥ 37 weeks of gestation and ≥ 2500 g) participants. Outcome measure A well-standardized measure of general conceptual ability (GCA), the Differential Ability Scales, Second Edition. Results cLPT participants had average mean performances but significantly poorer GCA, Nonverbal Reasoning, and Spatial scores than term-born children, and higher rates of Nonverbal Reasoning and Spatial impairment; uLPT did not differ from TERM. Combined LPT males were at eightfold greater risk than term-born males for nonverbal deficit, and at sevenfold greater risk for GCA impairment than LPT females. Conclusions Finding greater risk of cognitive deficit in those NICU-admitted due to clinical instability or birth weight < 2 kg compared with non-admitted preschoolers indicates that neonatal morbidities contribute to subtle cognitive deficits detectable at young age, with male gender an additive risk factor. LPT gestational age alone is an insufficient predictor of long-term neurocognitive outcome. Further study should elucidate salient etiologies for early emerging cognitive weaknesses and suggest appropriate interventions to prepare at-risk LPT preschoolers for elementary school entry.

Validity of the General Conceptual Ability Score From the Differential Ability Scales as a Function of Significant and Rare Interfactor Variability

.Some researchers have argued that discrepant broad index scores invalidate IQs, but others have questioned the fundamental logic of that argument. To resolve this debate, the present study used a nationally representative sample of children (N = 1,200) who were matched individually for IQ. Children with significantly uneven broad index score profiles and those with even broad index score profiles had equivalent reading and math skills. Discrepant broad index scores found in only 15%, 10%, 5%, and 1% of the population, respectively, also failed to differentially predict academic achievement. In addition, significantly higher Verbal broad index scores were not differentially predictive of reading achievement, nor were significantly higher Nonverbal/Spatial broad index scores differentially predictive of math achievement. It was concluded that the global ability score is the most parsimonious predictor of academic achievement, despite the presence of significant and rare variability among broad index scores.

Randomised trial of parental support for families with very preterm children: outcome at 5 years

Aims: To test the effectiveness of a home based developmental education intervention in improving outcome at 5 years for very preterm infants. Methods: The Avon Premature Infant Project (APIP) is...

The Utility of the Differential Abilities Scales with Specific Language-Impaired Preschoolers

The purpose of this study was to examine the utility of the Differential Ability Scales (DAS) in distinguishing preschoolers with specific language impairment (SLI) and specific subtypes of language delays. Three hundred forty-eight language-impaired preschoolers' response patterns across DAS General Conceptual Ability (GCA), Ability cluster, and subtest scores were compared to response profiles of 57 preschoolers without language impairment as well as the standardized mean. Participants' specific type of language impairment (Articulation, Expressive, Receptive, Pragmatic, Expressive-Receptive, Expressive-Articulation, Receptive-Articulation, and Articulation-Pragmatic) was then compared to their response patterns. Results indicated that, in general, SLI preschoolers had significantly lower GCA, Verbal Ability, Nonverbal Ability, and subtest scores on the DAS compared to preschoolers without language impairment. However, different response patterns across the GCA, Ability clusters, and subtests emerged depending on the SLI subtype. SLI children with Articulation-only impairments tended to score higher on Ability Cluster and subtest scores, whereas children with Receptive and Expressive-Receptive impairment scored lowest. Discriminant analysis revealed that subtype profiles could predict SLI subtype in 26% to 38% of the cases. Finally, factor analysis indicated a new factor structure for both younger and older preschool batteries, suggesting that practitioners working with the SLI-referred population should carefully consider the utility of the original DAS factors.

Concurrent validity of the early screening profiles and the differential ability scales with an at-risk preschool sample

The relationship between the Early Screening Profiles (ESP; Harrison, 1990) and the Differential Ability Scales (DAS; Elliott, 1990a) was studied. The participants were 94 (57 males, 37 females) at-risk preschoolers ranging in age from 3 years 6 months to 5 years 11 months (M= 4 years 7 months; SD= 6.3 months). The sample included 72 Caucasians, 21 African Americans, and one Hispanic. This study found that the DAS GCA (General Conceptual Ability) mean score was significantly lower than the ESP Total Screening mean score. However, a strong relationship (r= .78) was found between the ESP Total Screening and DAS GCA scores, indicating that the ESP is a good predictor of cognitive ability among at-risk preschoolers. © 2000 John Wiley & Sons, Inc.

Identifying at-risk preschoolers: The discriminant validity of the differential ability scales

The discriminant validity of the Upper Preschool Level of the Differential Ability Scales (DAS) was studied using 32 at-risk preschoolers (females = 14, males = 18) with a mean age of 57.28 months (SD = 4.43) and 30 normal preschoolers (females = 13, males = 17) with a mean age of 56.40 months (SD = 6.46). The results indicated that the DAS was an excellent measure to use when trying to differentiate between at-risk and normal preschoolers. The DAS could reliably identify, better than chance, whether a child was at-risk or normal. Specifically, the General Conceptual Ability score was found to be approximately 77% accurate in correctly classifying at-risk from normal preschoolers. In addition, the use of the DAS in an abbreviated format by selecting specific core and/or diagnostic subtests during the preschool screening process was strongly supported. © 1999 John Wiley & Sons, Inc.

The relationship between the Differential Ability Scales (DAS) and the Wechsler Intelligence Scale for Children?Third Edition (WISC-III) for students with learning disabilities

As part of a mandated three-year reevaluation, the Differential Ability Scales (DAS) was administered to 53 children identified as having a learning disability approximately 3 years after each had been administered the Wechsler Intelligence Scale for Children—Third Edition (WISC-III). The DAS's General Conceptual Ability score (GCA), and Verbal and Spatial Cluster scores were nonsignificantly different from the WISC-III's Full Scale, Verbal, and Performance scores. The Nonverbal Reasoning score of the DAS (a measure of Fluid intelligence not assessed by the WISC-III) was significantly different from both the Verbal and Performance scores. High correlations were found between the similar constructs on the two tests. Approximately 96% of the students obtaining a certain classification from the WISC-III obtained a comparable classification from the DAS. © 1996 John Wiley & Sons, Inc.

Relationship between the Bracken Basic Concept Scale and the Differential Ability Scales with an at-risk sample of preschoolers.

This study investigated the relationship between the Bracken Basic Concept Scale and the Differential Ability Scales with 35 at-risk preschoolers between the ages 3-6 and 5-11. A Pearson product-moment correlation coefficient of .70 was obtained between the Bracken Basic Concept Scale Total Test scores and the General Conceptual Ability scores of the Differential Ability Scales. This association supports the use of the Bracken scale to predict intelligence if replicated with a larger sample and specific handicaps. In addition, support for the administration of the more brief Bracken School Readiness Composite compared to using the Bracken Total Test score in the prediction of the Differential Ability Scales General Conceptual Ability score was found.