Objective To evaluate the reproducibility and validity of the Pediatric Automated Neuropsychological Assessment Metrics (Ped-ANAM) when used in childhood-onset systemic lupus erythematosus (cSLE). Methods Forty children with cSLE and 40 matched controls were followed for up to 18 months. Formal neuropsychological testing at baseline was repeated after 18 months of follow-up; overall cognitive performance and domain-specific cognition (attention, working memory, processing speed and visuoconstructional ability) were measured and categorized as having normal cognition, mild/moderate or moderate/severe impairment. The 10 Ped-ANAM subtests were completed every 6 months and twice at baseline. Ped-ANAM performance was based on accuracy (AC), mean time to correct response (MNc), throughput, and coefficient of variation of the time required for a correct response (CVc) as a measure of response consistency. Results Particularly MNc scores demonstrated moderate to substantial reproducibility (intraclass correlation coefficients: 0.47-0.80). Means of select Ped-ANAM scores (MNc, AC, CVc) differed significantly between children with different levels of cognitive performance and allowed for the detection of moderate or severe cognitive impairment with 100% sensitivity and 86% specificity. Six Ped-ANAM subtests significantly correlated with the change in overall cognitive function in cSLE (baseline vs. 18 month; Spearman correlation coefficient > ±0.4; p<0.05, n=24). Conclusions The Ped-ANAM has moderate to substantial reproducibility, criterion and construct validity and may be responsive to change in cSLE. Additional research is required to confirm the Ped-ANAM's outstanding accuracy in identifying cognitive impairment and its usefulness in detecting clinically relevant changes in cognition over time.

Objective. To investigate the utility of questionnaire-based assessment of cognitive function and behavioral/emotional symptoms to screen for neurocognitive dysfunction in childhood-onset systemic lupus erythematosus (cSLE). Methods. Forty children with cSLE and 24 healthy controls ages 10 -16 years were enrolled. Formal neurocognitive testing (FNCT) was done to determine cognitive performance in 4 key areas that appear to be sensitive to the adverse effects of cSLE: attention, working memory, psychomotor speed, and visuoconstructional ability. Paper and pencil questionnaires sampling cognitive functioning and behavioral/emotional symptoms were also completed: the Subjective Awareness of Neuropsychological Deficits for Children (SAND-C) questionnaire by patients, and the Child Behavioral Checklist and the Behavior Rating Inventory of Executive Function (BRIEF) by parents.Results. Domain and summary scores of the BRIEF and SAND-C correlated modestly with participants' performance on FNCT. Questionnaire ratings did not discriminate subjects with different levels of cognitive ability as measured by FNCT. Conclusion. Contrary to some reports in adults with SLE, self-administered questionnaires of cognitive functioning and parent ratings of executive functioning do not appear well suited to replace FNCT in screening for neurocognitive impairment of children and adolescents with cSLE. However, they may provide information that is complementary to FNCT and therefore play a useful role in clinical followup.

BackgroundOf 37 pediatric rheumatology fellowship training programs in the United States, many have three or fewer fellows at a given time, making large-scale assessment of fellow performance difficult. An objective structured clinical examination (OSCE) is a scenario-based simulation method that assesses individual performance, thus indirectly measuring training program effectiveness. This study describes the development and implementation of two national pediatric rheumatology OSCEs and methods used for programmatic improvement.MethodsOSCEs for pediatric rheumatology fellows were held in 2009 and 2011 during national rheumatology meetings using scenarios and assessment forms originally developed by a fellowship program director. The seven scenarios tested medical knowledge, physical exam and interpersonal skills. Pediatric rheumatologist evaluators assessed fellows’ performance using checklists and gave immediate feedback. Program directors were sent summaries of their fellows’ performances. Fellows evaluated the OSCE, providing organizational and scenario improvement suggestions. Programmatic changes to the 2011 OSCE were based on 2009 performance data and program evaluation feedback.ResultsTwenty-two fellows participated in 2009 and 19 in 2011. Performance scores in similar scenarios did not change considerably over the two iterations. In 2009, 85.7% of participants reported desire to change clinical behavior. Assessors’ 2009 program evaluation data prompted changes in rating scales and removal of invalid or unreliable assessments. Negative evaluation data about individual stations decreased from 60% in 2009 to 15.4% in 2011. Fellows’ ratings of the experience’s overall value were similar in 2009 and 2011. The average experience ratings were lower among fellows who proposed scenario-specific improvements and higher among those who recommended organizational improvements.ConclusionsThe 2011 examination exhibited programmatic improvement via reduction in fellows’ scenario-specific negative feedback. Fellows’ overall satisfaction did not change. Further work in scenario selection, assessment validation and inter-rater reliability will improve future pediatric rheumatology OSCEs.

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