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A Novel EEG Evaluation Method for Children With Infantile Spasms

A Novel EEG Evaluation Method for Children With Infantile Spasms 1024 677 Lauren Dembeck

Clinician-researchers have developed a rapid training program to learn the BASED score, a novel EEG evaluation method. The easily accessible training program allows clinicians to reliably determine the response to treatment.

Infantile epileptic spasms syndrome is a severe form of early childhood epilepsy. Onset typically occurs in infants 4 to 8 months old and is characterized by clusters of many, sometimes dozens, of brief seizures per day. The syndrome is associated with developmental regression and can have significant negative consequences if not treated early.

“To achieve the best possible developmental and epilepsy outcomes for children with infantile epileptic spasms syndrome, we must provide early and effective treatment that leads to confirmed electroclinical remission,” explains John Mytinger, MD, attending child neurologist at Nationwide Children’s Hospital. “This includes both the clinical remission of infantile spasms and adequate EEG improvement.”

Dr. Mytinger and colleagues previously developed the Burden of AmplitudeS and Epileptiform Discharges (BASED) score, a grading scale to help determine if a child has an epileptic encephalopathy and whether remission has been achieved. The BASED score showed excellent inter-rater reliability (IRR) among electroencephalogram (EEG) reviewers, justifying its further development for clinical and research purposes.

“The BASED score is a rapid and convenient method to help determine electrographic remission,” says Dr. Mytinger, who is also an associate professor of Clinical Pediatrics and Neurology at The Ohio State University College of Medicine. “EEG readers can assign the score simply by visually examining the EEG recordings.”

Now, the researchers have developed a BASED training program and assessed the reliability among clinician learners of varying skill levels. This study was completed with financial support from the Pediatric Epilepsy Research Foundation (PERF) and the Children’s Hospital of Orange County (CHOC), and with non-financial support from the Pediatric Epilepsy Research Consortium (PERC). Their results were recently reported in the Journal of Clinical Neurophysiology.

In the study, 31 learners used the BASED training application to assess 12 unmarked EEGs, ranging in length from 1 to 6 hours, from children with infantile epileptic spasms syndrome. The learners included 2 child neurology residents, 3 clinical neurophysiology fellows, and 26 faculty physicians from 18 different medical centers.

Among all learners, the IRR for the final BASED score was good (intraclass correlation coefficient, 0.86), and the IRR for all individual BASED score elements was fair to good. The IRR for the presence or absence of epileptic encephalopathy was also good (Marginal Multirater Kappa, 0.63).

The researchers noted an IRR gradient for the assessment of background amplitude — poor for trainees, fair to good for faculty physicians, and excellent for expert reviewers — suggesting that the IRR of these elements may improve with experience.

“Our findings support the use of our training program for readers to quickly learn the BASED scoring method, providing a more standardized approach for determining electrographic remission in children with infantile epileptic spasms syndrome,” says Dr. Mytinger. “This provides a tool that clinicians can use to support treatment decisions; however, clinical acumen remains paramount.”

Future learners can follow the learning pathway, which includes studying the previous and current open access articles (including the associated videos and supplements).

“With its high inter-rater reliability, BASED score utilization in research and clinical care is expanding. However, we have not yet conducted a validation study of the score. The next step is to formally validate the BASED score as a predictive biomarker. We hypothesize that a post-treatment BASED score can strongly predict later intractable epilepsy. We are currently planning a study to test this hypothesis. Formal validation of the BASED score will make this the gold standard test for children with infantile epileptic spasms syndrome.”

 

References:

  1. Mytinger JR, Albert DVF, Aylward SC, Beatty CW, Bhalla S, Bhatia S, Brock GN, Ciliberto MA, Choudhari PR, Clark DJ, Cohen JM, Czech TM, Fredwall MM, Gonzalez-Giraldo E, Harini C, Hunter SE, Sandoval Karamian AG, Katyayan A, Kistler I, Kulkarni N, Liu VB, McCabe C, Murray T, Neville K, Patel SH, Pavuluri S, Phillips DJ, Samanta D, Sirsi D, Spelbrink EM, Stafstrom CE, Steenari M, Takacs DS, Terrill T, Tran L, Vidaurre J, Shrey DW. A Multicenter Training and Interrater Reliability Study of the BASED Score for Infantile Epileptic Spasms Syndrome. J Clin Neurophysiol. 2024 Jul 11. doi: 10.1097/WNP.0000000000001101. Epub ahead of print. PMID: 38995949.
  2. Mytinger JR, Vidaurre J, Moore-Clingenpeel M, Stanek JR, Albert DVF. A reliable interictal EEG grading scale for children with infantile spasms – The 2021 BASED score. Epilepsy Res. 2021 Jul;173:106631. doi: 10.1016/j.eplepsyres.2021.106631. Epub 2021 Apr 2. PMID: 33839516.

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About the author

Lauren Dembeck, PhD, is a freelance science and medical writer based in New York City. She completed her BS in biology and BA in foreign languages at West Virginia University. Dr. Dembeck studied the genetic basis of natural variation in complex traits for her doctorate in genetics at North Carolina State University. She then conducted postdoctoral research on the formation and regulation of neuronal circuits at the Okinawa Institute of Science and Technology in Japan.

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