Cognitive Performance Scores for the Pediatric Automated Neuropsychological Assessment Metrics in Childhood‐Onset Systemic Lupus Erythematosus

Abstract

Objective To develop and initially validate a global cognitive performance score for the Pediatric Automated Neuropsychological Assessment Metrics (PedANAM-CPS) to serve as a screening tool of cognition in childhood lupus. Methods Patients (n=166) completed the nine subtests of the PedANAM battery, each of which provides three principal performance parameters (accuracy, mean reaction time for correct responses, throughput). Cognitive ability was measured by formal neurocognitive testing or estimated by the Pediatric Perceived Cognitive Function Questionnaire-43 to determine the presence or absence of neurocognitive dysfunction (NCD). A subset of the data was used to develop four candidate PedANAM-CPS indices with supervised or unsupervised statistical approaches: PedANAM-CPSUWA i.e. unweighted averages of the accuracy scores of all PedANAM-subtests; PedANAM-CPSPCA, i.e. accuracy scores of all PedANAM-subtests weighted through principal components analysis; PedANAM-CPSlogit i.e. algorithm derived from logistic models to estimate NCD-status based on the accuracy scores of all of the PedANAM-subtests; and PedANAM-CPSmultiscore i.e. algorithm derived from logistic models to estimate NCD-status based on select PedANAM performance parameters. Using the remaining data PedANAM-CPS candidates were validated. Results PedANAM-CPS indices were moderately correlated with each other (|r|>0.65). All of the PedANAM-CPS’s discriminated children by NCD-status across datasets (p<0.036). The PedANAM-CPSmultiscore had the highest area under the receiver operating characteristic curve (AUC) across all datasets for identifying NCD-status (AUC >0.74), followed by the PedANAM-CPSlogit, the PedANAM-CPSPCA and the PedANAM-CPSUWA respectively. Conclusions Based on preliminary validation and considering ease of use, the PedANAM-CPSmultiscore and the PedANAM-CPSPCA appear to be best suited as global measures of PedANAM performance.

Challenges of Diagnosing Cognitive Dysfunction With Neuropsychiatric Systemic Lupus Erythematosus in Childhood

“…Our earlier studies suggest that the Ped‐ANAM is a sensitive screening tool, yielding accuracy of 97% for identifying children with cSLE and clinically relevant cognitive impairment . Indeed, a Ped‐ANAM‐CPS multiscore of >4.63 or a Ped‐ANAM‐CPS PCA score of <0.25 are over 80% sensitive to capture children with lower than expected cognitive ability . Future studies with larger numbers of normal controls will be needed to confirm the above cutoff values.…”

“…However, obtaining the caregiver's perception of the child's cognitive function by completing the Pediatric Perceived Cognitive Function–43 questionnaire may provide additional cues to a child's cognitive ability . This questionnaire has been validated for use in cSLE and probes select cognitive domains such as attention, memory retrieval, and working memory .…”

“…These performance metrics have been combined in various ways to produce meaningful summary statistics (e.g., using regression models or averaged ANAM performance scores) . Recently, several cognitive performance scores (Ped‐ANAM‐CPS) have been developed to summarize various Ped‐ANAM test scores into a single score . However, based on preliminary validation and considering its ease of use, 2 scores, the Ped‐ANAM‐CPS multiscore and the Ped‐ANAM‐CPS primary component analysis (PCA) , appear to be best suited as global measures of Ped‐ANAM performance .…”

“…Recently, several cognitive performance scores (Ped‐ANAM‐CPS) have been developed to summarize various Ped‐ANAM test scores into a single score . However, based on preliminary validation and considering its ease of use, 2 scores, the Ped‐ANAM‐CPS multiscore and the Ped‐ANAM‐CPS primary component analysis (PCA) , appear to be best suited as global measures of Ped‐ANAM performance . The higher the Ped‐ANAM‐CPS PCA, the better the cognitive ability.…”

Biomarkers for CNS Involvement in Pediatric Lupus

“…Recently, this test has been validated in a larger cohort and composite scores for facilitating clinical use developed [72]. Further testing is needed to determine how the Pediatric Automated Neuropsychologic Assessment Metrics performs longitudinally as a measure of disease progression or remission in pediatric cNPSLE.…”