A Multicenter Training and Interrater Reliability Study of the BASED Score for Infantile Epileptic Spasms Syndrome

Author:

Mytinger John R.1,Albert Dara V. F.1,Aylward Shawn C.1,Beatty Christopher W.1,Bhalla Sonam23,Bhatia Sonal4,Brock Guy N.56,Ciliberto Micheal A.7,Choudhari Purva R.8,Clark Daniel J.1,Cohen Jennifer Madan9,Czech Theresa M.7,Fredwall Megan M.10,Gonzalez-Giraldo Ernesto11,Harini Chellamani12,Hunter Senyene E.13,Sandoval Karamian Amanda G.14,Katyayan Akshat15,Kistler Isaac1617,Kulkarni Neil1,Liu Virginia B.18,McCabe Corinne10,Murray Thomas1,Neville Kerri19,Patel Shital H.20,Pavuluri Spriha21,Phillips Donald J.18,Samanta Debopam22,Sirsi Deepa23,Spelbrink Emily M.24,Stafstrom Carl E.25,Steenari Maija18,Takacs Danielle S.15,Terrill Tyler23,Tran Linh26,Vidaurre Jorge1,Shrey Daniel W.18

Affiliation:

1. Department of Pediatrics, Division of Pediatric Neurology, Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio, U.S.A.;

2. Division of Neurology, Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, U.S.A.;

3. Division of Pediatric Neurology, Children's Healthcare of Atlanta, Atlanta, Georgia, U.S.A.;

4. Department of Pediatrics, Division of Pediatric Neurology, Medical University of South Carolina and Shawn Jenkins Children's Hospital, Charleston, South Carolina, U.S.A.;

5. Department of Biomedical Informatics, College of Medicine;

6. Center for Biostatistics, Wexner Medical Center, Biostatistics Resource at NCH (BRANCH), Nationwide Children's Hospital and The Ohio State University, Columbus, OH, U.S.A.;

7. Stead Family Department of Pediatrics, University of Iowa, Iowa City, Iowa, U.S.A.;

8. Department of Pediatrics, Division of Pediatric Neurology, University of Texas Southwestern, Dallas, Texas, U.S.A.;

9. Department of Neurology, Connecticut Children's Hospital, University of Connecticut, Hartford, Connecticut, U.S.A.;

10. Department of Pediatrics, Division of Pediatric Neurology, Nationwide Children's Hospital, Columbus, Ohio, U.S.A.;

11. Department of Neurology, Division of Epilepsy, University of California, San Francisco, San Francisco, California, U.S.A.;

12. Department of Neurology, Division of Epilepsy, Harvard University, Boston, Massachusetts, U.S.A.;

13. Department of Neurology, Division of Child Neurology, University of North Carolina Hospitals, Chapel Hill, North Carolina, U.S.A.;

14. Division of Neurology, Department of Pediatrics, University of Utah, Salt Lake City, Utah, U.S.A.;

15. Department of Pediatric Neurology and Developmental Neuroscience, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, U.S.A.;

16. Center for Biostatistics, The Ohio State University Wexner Medical Center, Columbus, Ohio, U.S.A.;

17. Biostatistics Resource at Nationwide Children's Hospital, Abigail Wexner Research Institute at Nationwide Children's Hospital, Columbus, Ohio, U.S.A.;

18. Division of Neurology, Orange California & Department of Pediatrics, Children's Hospital of Orange County, UC Irvine, Orange, California, U.S.A.;

19. Department of Pediatrics, Division of Pediatric Neurology, University of Michigan, Ann Arbor, Michigan, U.S.A.;

20. Department of Pediatrics, Division of Pediatric Neurology, Duke University Medical Center, Durham, North Carolina, U.S.A.;

21. Children's Hospital and Medical Center, Omaha, Nebraska, U.S.A.;

22. Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas, U.S.A;

23. Department of Pediatrics, Division of Pediatric Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, U.S.A;

24. Department of Neurology, Division of Child Neurology, Stanford Children's Hospital, Stanford University, Palo Alto, California, U.S.A;

25. Division of Pediatric Neurology, Departments of Neurology and Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland, U.S.A;

26. Comprehensive Epilepsy Program, Jane and John Justin Neuroscience Center, Cook Children's Medical Center, Fort Worth, Texas, U.S.A.

Abstract

Purpose: The best possible outcomes in infantile epileptic spasms syndrome require electroclinical remission; however, determining electrographic remission is not straightforward. Although the determination of hypsarrhythmia has inadequate interrater reliability (IRR), the Burden of AmplitudeS and Epileptiform Discharges (BASED) score has shown promise for the reliable interictal assessment of infantile epileptic spasms syndrome. Our aim was to develop a BASED training program and assess the IRR among learners. We hypothesized moderate or better IRR for the final BASED score and the presence or absence of epileptic encephalopathy (+/−EE). Methods: Using a web-based application, 31 learners assessed 12 unmarked EEGs (length 1–6 hours) from children with infantile epileptic spasms syndrome. Results: For all readers, the IRR was good for the final BASED score (intraclass correlation coefficient 0.86) and +/−EE (Marginal Multirater Kappa 0.63). For all readers, the IRR was fair to good for all individual BASED score elements. Conclusions: These findings support the use of our training program to quickly learn the BASED scoring method. The BASED score may be a valuable clinical and research tool. Given that the IRR for the determination of epileptic encephalopathy is not perfect, clinical acumen remains paramount. Additional experience with the BASED scoring technique among learners and advances in collaborative EEG evaluation platforms may improve IRR.

Funder

Pediatric Epilepsy Research Foundation

Children’s Hospital of Orange County Chief Scientific Officer Award

Children’s Hospital of Orange County Neuroscience Institute

Publisher

Ovid Technologies (Wolters Kluwer Health)

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