Background/Purpose: The Childhood-onset Systemic Lupus Erythematosus (cSLE) Neuropsychological Battery (AC&R 2010; 62: 1029-33) was introduced to standardize neuropsychological testing (NPT), specifically probing the domains Attention, Working Memory, Psychomotor Speed, Visuoconstructural Ability (VCA). The Pediatric Automated Neuropsychological Assessment Metrics (PedANAM) software was developed to measure cognition in children ³ 10 years. Performance on the PedANAM subtests is assessed by accuracy (AC = % of correct answers), mean reaction time for correct responses (MNc), & throughput (TP), the number of correct responses per minute. The purpose of this study was to (1) assess the reproducibility & (2) investigate the criterion validity of the PedANAM, using the cSLE Neuropsychological Battery as external standard.

Methods: cSLE patients and best-friend controls completed the PedANAM twice at baseline and then every 6 months. NPT was done at baseline and repeated after 18 month; Performance on Formal Neurocognitive Testing was standardized to age/gender/race-specific z-scores (mean 0, standard deviation 1) and then used to measure overall and domain-specific cognition (attention, processing-speed, working memory, VCA). Neurocognitive dysfunction (NCD) was graded as: no-NCD: all z-scores > -1; mild/moderate NCD: at most two z-scores < -1 but > -2 or one z-score < -2; moderate/severe: at least three z-scores < -1 or two z-scores < -2]. Intraclass and Spearman correlation coefficients were done to assess reproducibility and responsiveness to change.

Results: Among 40 cSLE & 40 controls (85% females), 13 (16.25%) had NCD at baseline and 18-month follow-up data were available for 24 cSLE and 16 control patients.  Most subtests had good reproducibility in mean reaction time (MNc) and fair-good reproducibility in accuracy (AC) and consistency (CVc) (Table 1). Changes of AC scores were found positively related to changes of NCD domain scores. In particular, improved (worsening) NCD attention was found positively related to improved (or worsening) AC scores in Matching to Sample, Procedural Reaction Time, and Spatial Processing (r’s >0.3, p’s<0.05) over 18 months. Positive and negative relationships were also found between NCD domain scores and PedANAM MNc and CVc scores respectively (Table2).

Conclusion: The PedANAM has good reproducibility especially for MNc measures. Changes cognitive domain scores were found significantly related to changes of performance scores of several PedANAM subtests over 18 months of follow up. These results are in line with our earlier research.