Infantile spasms: guidance for pediatricians

In a cross-sectional study of 100 parents of children with infantile spasms, the median time from spasm onset to first visit with any health care provider was 5 days, but the median time from onset to first visit with an "effective provider" (one who provided both accurate diagnosis and prescription for appropriate first-line treatment) was 24 days,5 a delay attributed at least in part to poor awareness of the condition among providers.5 Given that worse outcomes may be associated with even a 1-week delay in treatment from onset, it is critical that pediatric health care providers are proficient in recognizing this condition.67 Clinical features Infantile spasms An infantile spasm is brief and abrupt, generally 1 to 3 seconds, with muscle contraction that can include the head, neck, trunk, and/or extremities. Home video recording, first advised by the Child Neurology Society to streamline IESS management at the onset of the COVID-19 pandemic, has since been endorsed as a continued recommendation toward timely intervention.4 In preparing to evaluate a patient with possible IESS, pediatricians should ask caregivers to record suspected events. The EEG pattern during the spasm itself is a high-amplitude sharp or slow wave followed by a relative electrodecrement. Because the interictal EEG is generally abnormal, it is not necessary to capture a spasm during the EEG recording to support the diagnosis. Workup for an underlying etiology if not known is important not only because some are associated with other health concerns requiring monitoring and intervention, but also because it can guide management, as some etiologies may respond better to different treatment approaches.9 Identification may also guide appropriate counseling of families, including prognostication and possible genetic counseling.

Diagnosing and managing childhood absence epilepsy by telemedicine.

The diagnosis of childhood absence epilepsy (CAE) is typically based on history and description of spells, supported by an office-based positive hyperventilation test and confirmed by routine electroencephalography (EEG). In the current coronavirus disease 2019 (COVID-19) pandemic, many pediatric neurologists have switched to telemedicine visits for nonemergent outpatient evaluations. We present a series of children diagnosed as having CAE on the basis of a positive hyperventilation test performed during remote televisits. Several of these children were begun on treatment for CAE prior to obtaining an EEG, with significant seizure reduction. Our series documents the feasibility of CAE diagnosis and management by telemedicine.

Ketogenic diet therapy provision in the COVID-19 pandemic: Dual-center experience and recommendations.

The current coronavirus-19 pandemic has changed dramatically how neurologists care for children and adults with epilepsy. Stay-at-home orders and resistance to hospitalizations by patients have led epileptologists to engage in telemedicine and reevaluate how to provide elective services. Ketogenic diet therapy is often started in the hospital, with families educated in hospital-based classes, but this is difficult to do in this current pandemic. At our two academic centers, both our pediatric and adult epilepsy diet centers have had to quickly consider alternative methods to both start and maintain ketogenic diet therapy. This paper provides several examples of how ketogenic diet therapy can be provided to patients in unique ways, along with recommendations from other experts and patients, learned over the past few months.