Baumer, Fiona M.

Developmental/epileptic encephalopathy with spike-wave activation in sleep (DEE-SWAS) is newly proposed nomenclature put forth by the International League Against Epilepsy (ILAE) to replace the problematic electrographic and/or clinical phenotypes of electrical status epilepticus of sleep (ESES) and continuous spike-wave in sleep (CSWS). The nomenclature update represents a noble effort to minimize the confusion of how to define and appropriately utilize this alphabet soup of acronyms, thereby aiding in future clinical and research efforts. The name change fails to capture greater challenges within the field, which still plague diagnosis and treatment and stagnates substantive research advancements. Through a directed literature review of DEE-SWAS with emphasis on the new ILAE nomenclature and the RESCUE-ESES trial, we will highlight major persistent quandaries in the field. These include inadequate or insufficient diagnostic biomarkers (ie, the spike wave index), the highly variable clinical manifestations, ranging from dubious associations to profound developmental regression, presumptively caused by spike-wave activation in sleep, and variable and often ineffective treatment paradigms. We will also review the broader diagnostic evaluation of DEE-SWAS. By doing so, we aim to shed light on crucial research and clinical questions that could advance our understanding of diagnosing and treating children with DEE-SWAS, as well as addressing the uncertainty surrounding the neurological effects of sleep-activated epileptiform discharges.

Purpose: These American Clinical Neurophysiology Society technical standards suggest best practices for electrical stimulation mapping (ESM) with subdural and stereotactic depth electrodes for seizure induction and mapping of brain function. Methods: A working group was convened from American Clinical Neurophysiology Society membership with expertise in ESM. PubMed searches were performed to identify pertinent peer-reviewed literature. Recurrent meetings reviewed progress, built consensus by discussion, and developed evidence-based recommendations to the extent possible. Recommendations: Stimulators used for ESM should have sufficient dynamic range, ability to interrupt a stimulus train, and ictal disrupt mechanism(s). Charge density should be calculated for the specific electrodes and ESM settings, the maximum safe limits being 52 to 57 μC/cm 2 /phase for subdural electrodes and not established for stereotactic depth electrodes. Subdural ESM for functional mapping is typically performed at 50-Hz pulse frequency, 200- to 300-µs pulse width, 2- to 8-s train duration, and 1- to 20-mA current strength. Stereo ESM is commonly performed using 2 different pulse frequencies: 1 Hz (300–500 µs pulse width, train duration up to 30 s, and often a constant current of 3–5 mA), and 50 Hz (100–500 µs pulse width, train duration 2-8 s, and 0.5–10 mA current intensity). Conclusions: This guideline provides technical standards for the performance of ESM, which will likely evolve over time with advances in technology and additional evidence (also see Graphical Abstract).

ObjectiveThe aim of this study was to determine whether selection of treatment for children with infantile spasms (IS) varies by race/ethnicity.MethodsThe prospective US National Infantile Spasms Consortium database includes children with IS treated from 2012 to 2018. We examined the relationship between race/ethnicity and receipt of standard IS therapy (prednisolone, adrenocorticotropic hormone, vigabatrin), adjusting for demographic and clinical variables using logistic regression. Our primary outcome was treatment course, which considered therapy prescribed for the first and, when needed, the second IS treatment together.ResultsOf 555 children, 324 (58%) were non‐Hispanic white, 55 (10%) non‐Hispanic Black, 24 (4%) non‐Hispanic Asian, 80 (14%) Hispanic, and 72 (13%) other/unknown. Most (398, 72%) received a standard treatment course. Insurance type, geographic location, history of prematurity, prior seizures, developmental delay or regression, abnormal head circumference, hypsarrhythmia, and IS etiologies were associated with standard therapy. In adjusted models, non‐Hispanic Black children had lower odds of receiving a standard treatment course compared with non‐Hispanic white children (odds ratio [OR], 0.42; 95% confidence interval [CI], 0.20–0.89; p = 0.02). Adjusted models also showed that children with public (vs. private) insurance had lower odds of receiving standard therapy for treatment 1 (OR, 0.42; CI, 0.21–0.84; p = 0.01).InterpretationNon‐Hispanic Black children were more often treated with non‐standard IS therapies than non‐Hispanic white children. Likewise, children with public (vs. private) insurance were less likely to receive standard therapies. Investigating drivers of inequities, and understanding the impact of racism on treatment decisions, are critical next steps to improve care for patients with IS. ANN NEUROL 2022;92:32–44

Circumstances of the COVID-19 pandemic have mandated a change to standard management of infantile spasms. On April 6, 2020, the Child Neurology Society issued an online statement of immediate recommendations to streamline diagnosis and treatment of infantile spasms with utilization of telemedicine, outpatient studies, and selection of first-line oral therapies as initial treatment. The rationale for the recommendations and specific guidance including follow-up assessment are provided in this manuscript. These recommendations are indicated as enduring if intended to outlast the pandemic, and limited if intended only for the pandemic health care crisis but may be applicable to future disruptions of health care delivery.