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ARTHRITIS & RHEUMATOLOGY Vol. 66, No. S3, March 2014, pp S51–S52 DOI 10.1002/art.38449 © 2014, American College of Rheumatology A33: Cognitive Performance Scores for the Pediatric Automated Neuropsychological Assessment Metrics in Childhood-Onset Systemic Lupus Erythematosus Patricia Vega-Fernandez, 1 Shana Vanderburgh, 2 Natasha M. Ruth, 3 Deborah M. Levy, 4 Frank A. Zelko, 5 Eyal Muscal, 6 Marisa S. Klein-Gitelman, 5 Adam Huber, 7 Kasha Wiley, 8 Erin C. Thomas, 9 Lori B. Tucker, 10 Tresa Roebuck-Spencer, 11 Jun Ying, 2 and Hermine Brunner 8 Background/Purpose: Children with SLE (cSLE) can experience neuropsychiatric SLE (NPSLE), com- monly manifesting as neurocognitive dysfunction which can interfere with normal development. Formal neurocognitive testing (FNCT) is the most accepted test for diagnosing neurocognitive deficits (NCD). Access to it is limited and costly, and it is time-consuming. The Pediatric Automated Neurophysi- ological Assessments Metrics (PedANAM) is a comput- erized battery of 10 subtests measuring various aspects of cognitive ability. Concurrent validity of PedANAM test scores with FNCT has been demonstrated. How- ever, the PedANAM generates several measures of accuracy (% of correct responses), processing speed and efficiency, and it is unclear how they can be used in a clinical setting. The usefulness of the PedANAM as a screen for NCD would be enhanced by the devel- opment of a PedANAM Cognitive Performance Score (PedANAM-CPS) to represent aspects of PedANAM task performance that are sensitive to NCD in cSLE. The purpose of this study was to develop a PedANAM-CPS for use in cSLE, using statistical methods. Methods: cSLE patients (pts) and age plus sex- matched healthy controls enrolled in a study of cognitive functioning and neuroimaging were studied. At the time of enrollment (visit 1—V1) and 18 months later (visit 2—V2), subjects completed the PedANAM and FNCT. Three candidate PedANAM-CPS measurement ap- proaches were explored via 3 statistical methods: 1) Sim- ple mean—of all subtest accuracy scores; 2) Logit scorevia a logistic regression model; 3) PCA score—using a Principal Component Analysis (PCA) method. The lat- ter 2 methods assigned in a different way a statistical weight to each subtest accuracy score. Fixed effect models were used to compare performance scores be- tween groups. Receiver operating characteristic (ROC) curves were used to assess the accuracy of the CPS as predictors of NCD as determined by FNCT. Results: 77 children (female 68%) were evaluated at V1; Nine cSLE pts with NCD, 31 with cSLE and no NCD, and 37 control with no NCD as per FNCT. At V1, age (values are mean standard devia- tion) of children was 13.6 2.4 years. For cSLE pts, disease activity (SLEDAI) was 4.9 4.4, and 77.5% were on oral prednisone (19.8 17.4 mg). Table 1 summarizes the PedANAM-CPS for all methods at V1. The Logit score best discriminated the groups, especially contrasting the NCD group against the other groups. The Logit and PCA scores showed 82% area under the ROC curve during the validation stage using V2 data. Table 1. Summary of PedANAM Cognitive Performance Score (PedANAM- CPS) Visit Score Mean SD p-value (1) Control (2) cSLE No NCD (3) cSLE w. NCD (1) vs. 2) (1) vs. (3) (2) vs. (3) 1 Simple mean 88.79 0.97 88.91 1.06 85.18 1.96 0.931 0.103 0.098 Logit score 0.04 0.13 0.04 0.15 0.84 0.27 0.992 0.005 0.006 PCA score 438.80 4.22 441.30 4.61 421.69 8.55 0.690 0.077 0.057 1 Cincinanti Children’s Hospital Medical Center, Cincinnati, OH, 2 University of Cincinnati, Cincinnati, OH, 3 Medical University of South Carolina, Charleston, SC, 4 The Hospital for Sick Children, Toronto, ON, 5 Northwestern University Feinberg School of Medicine, Chicago, IL, 6 Texas Children’s Hospital, Houston, TX, 7 IWK Health Centre, Halifax, NS, 8 Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, 9 Anne & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, 10 BC Children’s Hospital and University of British Columbia, Vancouver, BC, 11 University of Oklahoma, Nor- man, OK. S51

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Page 1: A33: Cognitive Performance Scores for the Pediatric Automated Neuropsychological Assessment Metrics in Childhood-Onset Systemic Lupus Erythematosus

ARTHRITIS & RHEUMATOLOGYVol. 66, No. S3, March 2014, pp S51–S52DOI 10.1002/art.38449© 2014, American College of Rheumatology

A33: Cognitive Performance Scores for the PediatricAutomated Neuropsychological Assessment Metrics in

Childhood-Onset Systemic Lupus Erythematosus

Patricia Vega-Fernandez,1 Shana Vanderburgh,2 Natasha M. Ruth,3 Deborah M. Levy,4

Frank A. Zelko,5 Eyal Muscal,6 Marisa S. Klein-Gitelman,5 Adam Huber,7 Kasha Wiley,8

Erin C. Thomas,9 Lori B. Tucker,10 Tresa Roebuck-Spencer,11

Jun Ying,2 and Hermine Brunner8

Background/Purpose: Children with SLE (cSLE)can experience neuropsychiatric SLE (NPSLE), com-monly manifesting as neurocognitive dysfunction whichcan interfere with normal development.

Formal neurocognitive testing (FNCT) is themost accepted test for diagnosing neurocognitive deficits(NCD). Access to it is limited and costly, and it istime-consuming. The Pediatric Automated Neurophysi-ological Assessments Metrics (PedANAM) is a comput-erized battery of 10 subtests measuring various aspectsof cognitive ability. Concurrent validity of PedANAMtest scores with FNCT has been demonstrated. How-ever, the PedANAM generates several measures ofaccuracy (% of correct responses), processing speedand efficiency, and it is unclear how they can be usedin a clinical setting. The usefulness of the PedANAMas a screen for NCD would be enhanced by the devel-opment of a PedANAM Cognitive Performance Score(PedANAM-CPS) to represent aspects of PedANAMtask performance that are sensitive to NCD in cSLE.

The purpose of this study was to develop aPedANAM-CPS for use in cSLE, using statisticalmethods.

Methods: cSLE patients (pts) and age plus sex-matched healthy controls enrolled in a study of cognitivefunctioning and neuroimaging were studied. At the time

of enrollment (visit 1—V1) and 18 months later (visit2—V2), subjects completed the PedANAM and FNCT.Three candidate PedANAM-CPS measurement ap-proaches were explored via 3 statistical methods: 1) Sim-ple mean—of all subtest accuracy scores; 2) Logit score—via a logistic regression model; 3) PCA score—using aPrincipal Component Analysis (PCA) method. The lat-ter 2 methods assigned in a different way a statisticalweight to each subtest accuracy score. Fixed effectmodels were used to compare performance scores be-tween groups. Receiver operating characteristic (ROC)curves were used to assess the accuracy of the CPS aspredictors of NCD as determined by FNCT.

Results: 77 children (female � 68%) wereevaluated at V1; Nine cSLE pts with NCD, 31 withcSLE and no NCD, and 37 control with no NCD as perFNCT. At V1, age (values are mean � standard devia-tion) of children was 13.6 � 2.4 years. For cSLE pts,disease activity (SLEDAI) was 4.9 � 4.4, and 77.5%were on oral prednisone (19.8 � 17.4 mg). Table 1summarizes the PedANAM-CPS for all methods at V1.The Logit score best discriminated the groups, especiallycontrasting the NCD group against the other groups.The Logit and PCA scores showed � 82% area underthe ROC curve during the validation stage using V2data.

Table 1. Summary of PedANAM Cognitive Performance Score (PedANAM-CPS)

Visit Score

Mean � SD p-value

(1) Control(2) cSLE No

NCD(3) cSLE w.

NCD(1) vs.

2)(1) vs.

(3)(2) vs.

(3)

1 Simple mean 88.79 � 0.97 88.91 � 1.06 85.18 � 1.96 0.931 0.103 0.098Logit score �0.04 � 0.13 �0.04 � 0.15 0.84 � 0.27 0.992 0.005 0.006PCA score 438.80 � 4.22 441.30 � 4.61 421.69 � 8.55 0.690 0.077 0.057

1Cincinanti Children’s Hospital Medical Center, Cincinnati,OH, 2University of Cincinnati, Cincinnati, OH, 3Medical University ofSouth Carolina, Charleston, SC, 4The Hospital for Sick Children,Toronto, ON, 5Northwestern University Feinberg School of Medicine,Chicago, IL, 6Texas Children’s Hospital, Houston, TX, 7IWK HealthCentre, Halifax, NS, 8Cincinnati Children’s Hospital Medical Center,Cincinnati, OH, 9Anne & Robert H. Lurie Children’s Hospital ofChicago, Chicago, IL, 10BC Children’s Hospital and University ofBritish Columbia, Vancouver, BC, 11University of Oklahoma, Nor-man, OK.

S51

Page 2: A33: Cognitive Performance Scores for the Pediatric Automated Neuropsychological Assessment Metrics in Childhood-Onset Systemic Lupus Erythematosus

Conclusion: Candidate PedANAM-CPS de-rived from the Logit and PCA scores seem to performbetter than an index based on the simple means atdiscriminating cSLE pts with NCD from cSLE andcontrol children with normal cognition. Furtheranalysis in a larger sample is needed to better de-

termine accuracy performance scores with clinicalrelevance.

Disclosure: P. Vega-Fernandez, None; S. Vanderburgh, None; N. M. Ruth,None; D. M. Levy, None; F. A. Zelko, None; E. Muscal, None; M. S.Klein-Gitelman, None; A. Huber, None; K. Wiley, None; E. C. Thomas, None;L. B. Tucker, None; T. Roebuck-Spencer, None; J. Ying, None; H. Brunner,None.

S52 THURSDAY, APRIL 3, 2014; 8:30 AM–6:00 PM