Proposed Pathway for the Utilization of Pediatric Ambulatory EEG.

Purpose: The clinical utility of pediatric ambulatory-EEG (A-EEG) has been studied for decades, but limited information exists regarding which variables influence its utility. The authors aimed to evaluate clinical/EEG variables that may influence A-EEG yields and to develop a pathway for A-EEG utilization in children.

Methods: Single-center retrospective review of A-EEGs performed from July 2019 to January 2021 in a tertiary referral center.

Genotype-phenotype correlations in SCN8A-related disorders reveal prognostic and therapeutic implications.

We report detailed functional analyses and genotype-phenotype correlations in 392 individuals carrying disease-causing variants in SCN8A, encoding the voltage-gated Na+ channel Nav1.6, with the aim of describing clinical phenotypes related to functional effects. Six different clinical subgroups were identified: Group 1, benign familial infantile epilepsy (n = 15, normal cognition, treatable seizures); Group 2, intermediate epilepsy (n = 33, mild intellectual disability, partially pharmaco-responsive); Group 3, developmental and epileptic encephalopathy (n = 177, severe intellectual disability, majority pharmaco-resistant); Group 4, generalized epilepsy (n = 20, mild to moderate intellectual disability, frequently with absence seizures); Group 5, unclassifiable epilepsy (n = 127); and Group 6, neurodevelopmental disorder without epilepsy (n = 20, mild to moderate intellectual disability).

Creation and implementation of an electronic health record note for quality improvement in pediatric epilepsy: Practical considerations and lessons learned.

Objective: To describe the development of the Pediatric Epilepsy Outcome-Informatics Project (PEOIP) at Alberta Children's Hospital (ACH), which was created to provide standardized, point-of-care data entry; near-time data analysis; and availability of outcome dashboards as a baseline on which to pursue quality improvement.

Methods: Stakeholders involved in the PEOIP met weekly to determine the most important outcomes for patients diagnosed with epilepsy, create a standardized electronic note with defined fields (patient demographics, seizure and syndrome type and frequency and specific outcomes- seizure type and frequency, adverse effects, emergency department visits, hospitalization, and care pathways for clinical decision support. These were embedded in the electronic health record from which the fields were extracted into a data display platform that provided patient- and population-level dashboards updated every 36 hours.

Neck Circumference-Height Ratio as a Predictor of Sleep Related Breathing Disorder in Children and Adults.

Study Objectives: While neck circumference (NC) is a useful predictor of obstructive sleep apnea (OSA) in adults, childhood OSA is difficult to predict clinically. We utilized the neck circumference-height ratio (NHR) to normalize NC in growing children. Our study aimed to determine if (1) NC is a reproducible clinical measurement; (2) NHR predicts OSA in children; (3) this metric translates to adults.

Prevalence of Interictal Epileptiform Discharges in Children Less Than One Year of Age Referred for Polysomnography.

This study aimed to identify the prevalence of interictal epileptiform discharges (IEDs) in patients less than one year of age referred for polysomnogram (PSG), to quantify the number of IEDs and characterize sleep stage(s) during which IEDs occur, and to relate the reason for PSG referral and IED frequency. This was a retrospective cohort study of children less than 12 months of age referred for PSG studies. Fifty study participants had sufficient EEG data, and 49 were included in the study analysis.

Sulthiame therapy for continuous spike and wave in slow-wave sleep.

Corticosteroids are often considered as the first therapeutic choice in children with continuous spike and wave in slow-wave sleep on electroencephalogram; however, they are associated with significant adverse effects. "Idiopathic" forms of continuous spike and wave in slow-wave sleep may represent the severe end of the spectrum of benign rolandic epilepsy of childhood. This report describes a 5-year-old male with language delay who presented with a single focal-onset, nocturnal seizure and had continuous spike and wave in slow-wave sleep on electroencephalography.