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

John R Mytinger¹, Dara V F Albert¹, Shawn C Aylward¹, Christopher W Beatty¹, Sonam Bhalla^{2

3}, Sonal Bhatia⁴, Guy N Brock^{5

6}, Micheal A Ciliberto⁷, Purva R Choudhari⁸, Daniel J Clark¹, Jennifer Madan Cohen⁹, Theresa M Czech⁷, Megan M Fredwall¹⁰, Ernesto Gonzalez-Giraldo¹¹, Chellamani Harini¹², Senyene E Hunter¹³, Amanda G Sandoval Karamian¹⁴, Akshat Katyayan¹⁵, Isaac Kistler^{16

17}, Neil Kulkarni¹, Virginia B Liu¹⁸, Corinne McCabe¹⁰, Thomas Murray¹, Kerri Neville¹⁹, Shital H Patel²⁰, Spriha Pavuluri²¹, Donald J Phillips¹⁸, Debopam Samanta²², Deepa Sirsi²³, Emily M Spelbrink²⁴, Carl E Stafstrom²⁵, Maija Steenari¹⁸, Danielle S Takacs¹⁵, Tyler Terrill²³, Linh Tran²⁶, Jorge Vidaurre¹, Daniel W Shrey¹⁸

Affiliations

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 .

PMID: 40009427
PMCID: PMC11864053
DOI: 10.1097/WNP.0000000000001101

Multicenter Study

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

John R Mytinger et al. J Clin Neurophysiol. 2025.

. 2025 Mar 1;42(3):230-234.

doi: 10.1097/WNP.0000000000001101. Epub 2024 Jul 11.

Authors

Affiliations

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 .

PMID: 40009427
PMCID: PMC11864053
DOI: 10.1097/WNP.0000000000001101

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.

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Conflict of interest statement

The authors have no funding or conflicts of interest to disclose.

References

1. Shields WD. Infantile spasms: little seizures, BIG consequences. Epilepsy Curr 2006;6:63–69. - PMC - PubMed
1. O'Callaghan FJK, Edwards SW, Alber FD, et al. . Vigabatrin with hormonal treatment versus hormonal treatment alone (ICISS) for infantile spasms: 18-month outcomes of an open-label, randomised controlled trial. Lancet Child Adolesc Health 2018;2:715–725. - PubMed
1. Hussain SA, Kwong G, Millichap JJ, et al. . Hypsarrhythmia assessment exhibits poor interrater reliability: a threat to clinical trial validity. Epilepsia 2015;56:77–81. - PubMed
1. Mytinger JR, Hussain SA, Islam MP, et al. . Improving the inter-rater agreement of hypsarrhythmia using a simplified EEG grading scale for children with infantile spasms. Epilepsy Res 2015;116093–98. - PubMed
1. Mytinger JR. Definitions and diagnostic criteria for infantile spasms and west syndrome - historical perspectives and practical considerations. Semin Pediatr Neurol 2021;38:100893. - PubMed

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88-06042021, 2021/Pediatric Epilepsy Research Foundation, Childrenâ€™s Hospital of Orange County Chief Scientific Officer Award, Childrenâ€™s Hospital of Orange County Neuroscience Institute

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

Affiliations

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

Authors

Affiliations

Abstract

Conflict of interest statement

References

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources