The First 100 Seconds of Sleep of rEEGs Can Be a Reliable Scoring Method for D/EE-SWAS.

PURPOSE: Developmental/epileptic encephalopathy with spike wave activation with sleep, formerly known as electrical status epilepticus in sleep, is an electrographic pattern in which the interictal epileptiform activity is augmented by transition to sleep. Recent studies demonstrate the utility of the first 100 seconds of sleep of long-term monitoring (LTM) as a scoring method for electrical status epilepticus in sleep. Our aim was to measure the reliability of the spike-wave index (SWI) of the first 100 seconds of sleep of routine EEG (rEEG) as a tool for diagnosis of developmental/epileptic encephalopathy with spike wave activation with sleep. METHODS: Approximately three hundred forty LTMs were reviewed, and 25 studies from 25 unique patients had comparable rEEGs. Two neurophysiologists calculated the SWI of the first 100 seconds of spontaneous stage II non-random eye movement sleep, the first 5-minute bin of sleep, and three separate 5-minute bins throughout sleep in LTM. This was compared to the SWI of the first 100 seconds of sleep in rEEG. Agreement was analyzed using Lin's concordance correlation coefficient (CCC). RESULTS: Using 50% as a diagnostic cut-off, we observed moderate agreement between the SWI of the first 100 seconds of sleep of rEEG and three bin LTM (CCC = 0.94, 95% CI: 0.88-0.97). Agreement was slightly higher for the comparison to first bin LTM SWI (CCC = 0.96, 95% CI: 0.92-0.98) and first 100 seconds LTM SWI (CCC = 0.96, 95% CI: 0.92-0.98). CONCLUSIONS: This study demonstrates the first 100 seconds of sleep of rEEG technique as a time efficient diagnostic tool for patients with concern for developmental/epileptic encephalopathy with spike wave activation with sleep.

The Spike-Wave Index of the First 100 Seconds of Sleep Can Be a Reliable Scoring Method for Electrographic Status Epilepticus in Sleep.

INTRODUCTION: Electrical status epilepticus in sleep (ESES) is an electrographic pattern in which interictal epileptiform activity is augmented by the transition to sleep, with non-rapid eye movement sleep state characterized by near-continuous lateralized or bilateral epileptiform discharges. The aim of this study was to measure the reliability of the spike-wave index (SWI) of the first 100 seconds of sleep as a tool for the diagnosis of ESES. METHODS: One hundred forty studies from 60 unique patients met the inclusion. Two neurophysiologists calculated the SWI of the first 100 seconds of spontaneous stage II non-rapid eye movement sleep. This was compared with the SWI of the first 5 minutes of non-rapid eye movement sleep and the cumulative SWI of three 5-minute bins of sleep. Agreement between the three SWI methods were analyzed using several statistical tools and methods. RESULTS: Using an SWI of 50% as a diagnostic cutoff, 57% of records had a diagnosis of ESES based on the first 100 seconds of sleep. Fifty-four percent of records had a diagnosis of ESES based on the method of using the SWI of three bins. This resulted in a diagnostic accuracy of 92%, sensitivity of 96%, and specificity of 88%. Positive predictive values of children diagnosed with ESES using the first 100 seconds of sleep, compared with 3 combined bins, was determined to be 90% and a negative predictive value was determined to be 95%. CONCLUSIONS: This analysis confirmed the diagnostic accuracy of using the SWI of the first 100 seconds of sleep and the cumulative total of three 5-minute bins.

Prioritizing Hormone Therapy Over Vigabatrin as the First Treatment for Infantile Spasms: A Quality Improvement Initiative.

BACKGROUND AND OBJECTIVES: Infantile spasms (IS) are early childhood seizures with potentially devastating consequences. Standard therapies (adrenocorticotropic hormone [ACTH], high-dose prednisolone, and vigabatrin) are strongly recommended as the first treatment for IS. Although this recommendation comes without preference for one standard therapy over another, early remission rates are higher with hormone therapy (ACTH and high-dose prednisolone) when compared with vigabatrin. Using quality improvement (QI) methodology that included hormone therapy as the first treatment, we sought to increase the percentage of children with new-onset nontuberous sclerosis complex (TSC)-associated IS achieving 3-month electroclinical remission from a mean of 53.8% to ≥70%. METHODS: This was an observational consecutive sample cohort study at a single academic tertiary care hospital that compared a prospective intervention cohort (May 2019-January 2022, N = 57) with a retrospective baseline cohort (November 2015-April 2019, N = 67). Our initiative addressed key drivers such as the routine use of vigabatrin over hormone therapy as first treatment and the common initiation of a second treatment after 14 days for initial nonresponders. We included consecutive children without TSC presenting with new-onset IS diagnosed and treated between ages 2 and 24 months. We displayed our primary outcome and process measures as control charts in which the centerline is the quarterly (previous 3 months) mean based on statistical process control methodology. RESULTS: QI interventions that included the standardization of hormone therapy as the first treatment resulted in higher rates of 3-month remission, rising from 53.8% (baseline cohort) to 75.9% (intervention cohort). Process measure results included an increased rate of children receiving hormone therapy as first treatment (mean, 44.6%-100%) and a decreased number of days to both clinical follow-up after first treatment (mean, of 16.3-12.6 days) and starting a second treatment within 14 days for initial nonresponders (mean, 36.3-17.2 days). DISCUSSION: For children with IS, improved rates of 3-month electroclinical remission can be achieved with QI methodology. Implementation of similar QI initiatives at other centers may likewise improve local remission rates.

Electroencephalography Technologist Inter-rater Agreement and Interpretation of Pediatric Critical Care Electroencephalography.

OBJECTIVES: Electroencephalography (EEG) technologists commonly screen continuous EEG. Until now, the inter-rater agreement or sensitivity for important EEG findings has been unknown in this group. METHODS: Twenty-nine EEG technologists and three clinical neurophysiologists interpreted 90 five-minute samples of pediatric critical care EEG. Inter-rater agreement was examined with Cohen's kappa and Fleiss' kappa for EEG findings. A gold-standard consensus agreement was developed for examining sensitivity and specificity for seizures or discontinuity. Kruskal-Wallis tests with Benjamani-Hochberg corrections for multiple comparisons were utilized to examine associations between correct scoring and certification status and years of experience. RESULTS: Aggregate agreement was moderate for seizures and fair for EEG background continuity among EEG technologists. Individual agreement for seizures and continuity varied from slight to substantial. For individual EEG technologists, sensitivity for seizures ranged from 44 to 93% and sensitivity for continuity ranged from 81 to 100%. Raters with Certified Long Term Monitoring credentials were more likely to identify seizures correctly. SIGNIFICANCE: This is the first study to evaluate inter-rater agreement and interpretation correctness among EEG technologists interpreting pediatric critical care EEG. EEG technologists demonstrated better aggregate agreement for seizure detection than other EEG findings, yet individual results and internal consistency varied widely. These data provide important insight into the common practice of utilizing EEG technologists for screening critical care EEG.

Compliance With Standard Therapies and Remission Rates After Implementation of an Infantile Spasms Management Guideline.

BACKGROUND: We implemented an infantile spasms management guideline recommending standard therapies and, early start of next treatment. After six years, we determined (1) our compliance with standard therapies, (2) time to next treatment, and (3) rate of initial and three-month electroclinical remission with first, second, and third treatments. METHODS: This is a retrospective record review of newly diagnosed spasms from September 2012 to September 2018, with the onset age of two months to two years. RESULTS: Standard therapies (hormone or vigabatrin) were the first treatments in 114 of 115 consecutive patients. The second and third treatments were started within 14 days of failed treatment in only 21% and 24%, respectively. Remission with the first and second treatments was similar (41% and 40%). Remission was lower for the third treatment (15%), although higher if standard therapy was used (36%). Initial and three-month remission by the first treatment was significantly higher for adrenocorticotropic hormone (ACTH, 66% and 79%, respectively) and prednisolone (53% and 83%, respectively) than for vigabatrin (19% and 40%, respectively). There were no significant differences in patient characteristics or rates of remission between ACTH and prednisolone. CONCLUSIONS: Although we achieved excellent compliance with standard therapies as initial treatment, a next treatment often started after two weeks. Given the superiority of hormone therapies over vigabatrin and standard therapies over nonstandard therapies, as well as the potentially negative impact of delays in effective treatment, future interventions need to focus on increasing the use of hormone over vigabatrin (for patients without tuberous sclerosis complex), use of standard therapies as second and third treatments, and reducing delays to next treatment.