The spectrum of epilepsy in children with 15q13.3 microdeletion syndrome.

Purpose: To further define the epilepsy phenotype in a cohort of children with 15q13.3 microdeletion syndrome.

Methods: We retrospectively reviewed the phenotypic spectrum of all children aged < 18 years with epilepsy and 15q13.

Ketonuria and Seizure Control in the Medium Chain Triglyceride and Classic Ketogenic Diets.

We hypothesized that children receiving medium-chain triglyceride ketogenic diet (MCTKD) experience similar seizure reduction despite lower ketosis compared with classic ketogenic diet (CKD). Children initiating CKD or MCTKD were enrolled in a prospective observational study. Forty-five children completed 6 months of KD (n = 17 MCTKD, n = 28 CKD).

Why child neurologists talk about SUDEP: Results from two cross-sectional surveys.

Objective: To characterize SUDEP discussion practices of child neurologists approximately 6 and 12 months after publication of the American Academy of Neurology SUDEP Clinical Practice Guideline and explore factors associated with discussion practice.

Methods: Child Neurology Society members (~2450) were electronically surveyed in November 2017 and May 2018 regarding their practice of discussing SUDEP with patients with epilepsy or their caregivers. Multivariable proportional odds ordinal logistic regression evaluated factors associated with discussing SUDEP with a greater proportion of epilepsy patients/caregivers.

Autopsy-reported cause of death in a population-based cohort of sudden unexpected death in epilepsy.

Objective: Sudden unexpected death in epilepsy (SUDEP) is a diagnosis of exclusion; the definition includes individuals with epilepsy who die suddenly without an identifiable toxicological or anatomical cause of death. Limited data suggest underidentification of SUDEP as the cause of death on death certificates. Here, we evaluate the autopsy-reported cause of death in a population-based cohort of SUDEP cases.

Neutropenia in Children Treated With Ketogenic Diet Therapy.

Objectives: The objectives were to investigate the relationship between ketogenic diet therapy and neutropenia in children with epilepsy.

Methods: A retrospective chart review of children who initiated ketogenic diet at the Hospital for Sick Children between January 1, 2000, and May 1, 2018 was performed. Factors associated with the development of neutropenia during ketogenic diet therapy were evaluated and the relationship between development of a significant or suspected infection and neutrophil count was analyzed.

The influence of the number of part-set cues on order retention.

Across seven experiments, the present article examined the influence of the number of part-set cues on order retention, as assessed by both reconstruction of order and serial recall tests. Historically, part-set cueing facilitation occurs when half of the items are provided as valid part-set cues on tests of order memory. Using a variety of list lengths (10 or 16), numbers of cues (0-14), and types of cues (full or partial), the present experiments showed disparate effects of the number of part-set cues on reconstruction and serial recall tasks.

Temporal trends and autopsy findings of SUDEP based on medico-legal investigations in the United States.

Objective: To determine time trends and distinguishing autopsy findings of sudden unexpected death in epilepsy (SUDEP) in the United States.

Methods: We identified decedents where epilepsy/seizure was listed as cause/contributor to death or comorbid condition on the death certificate among all decedents who underwent medico-legal investigation at 3 medical examiner (ME) offices across the country: New York City (2009-2016), San Diego County (2008-2016), and Maryland (2000-2016). After reviewing all available reports, deaths classified as definite/probable/near SUDEP or SUDEP plus were included for analysis.

Magnetoencephalographic (MEG) brain activity during a mental flexibility task suggests some shared neurobiology in children with neurodevelopmental disorders.

Background: Children with neurodevelopmental disorders (NDDs) exhibit a shared phenotype that involves executive dysfunctions including impairments in mental flexibility (MF). It is of interest to understand if this phenotype stems from some shared neurobiology.

Methods: To investigate this possibility, we used magnetoencephalography (MEG) neuroimaging to compare brain activity in children (n = 88; 8-15 years) with autism spectrum disorders (ASD), attention deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD), as they completed a set-shifting/mental flexibility task.

Addressing the Language Binding Problem With Dynamic Functional Connectivity During Meaningful Spoken Language Comprehension.

During speech, how does the brain integrate information processed on different timescales and in separate brain areas so we can understand what is said? This is the language binding problem. Dynamic functional connectivity (brief periods of synchronization in the phase of EEG oscillations) may provide some answers. Here we investigate time and frequency characteristics of oscillatory power and phase synchrony (dynamic functional connectivity) during speech comprehension.

Effectiveness of antiepileptic drug tapering in the pediatric epilepsy monitoring unit.

Objective: Limited evidence on the relationship between antiepileptic drug (AED) tapering and the likelihood of a seizure during an Epilepsy Monitoring Unit (EMU) admission is available, and no evidence specific to the pediatric population has been published. Our study sought to determine whether AED tapering leads to increased seizure likelihood in a pediatric EMU setting.

Methods: We performed a retrospective chart review of children admitted to the pediatric EMU at the Hospital for Sick Children in Toronto between June 1, 2014 and June 1, 2016.

Drowning in epilepsy: A population-based case series.

Objectives: The risk of drowning is reported to be 15-19 times greater in people with epilepsy compared to the general population. Despite this disproportionate burden, there is limited data about the circumstances surrounding drowning deaths in people with epilepsy. This population-based case series characterizes drowning deaths in people with epilepsy.

Incidence of sudden unexpected death in epilepsy in children is similar to adults.

Objective: To determine the incidence of sudden unexpected death in epilepsy (SUDEP) in children in Ontario, Canada.

Methods: Cases of suspected pediatric SUDEP occurring between January 1, 2014, and December 31, 2015, in Ontario, Canada, were eligible for inclusion. Potential cases were identified through 3 sources: a national pediatrician surveillance program, child neurologist report, and screening of provincial forensic autopsies.

Inhibition in the face of emotion: Characterization of the spatial-temporal dynamics that facilitate automatic emotion regulation.

Emotion regulation mediates socio-cognitive functions and is essential for interactions with others. The capacity to automatically inhibit responses to emotional stimuli is an important aspect of emotion regulation; the underlying neural mechanisms of this ability have been rarely investigated. Forty adults completed a Go/No-go task during magnetoencephalographic (MEG) recordings, where they responded rapidly to either a blue or purple frame which contained angry or happy faces.

Mental flexibility: An MEG investigation in typically developing children.

Mental flexibility is a core property of cognitive executive functions, relying on an extended frontoparietal network in the brain. fMRI research comparing typically developing children and adults has found that children from an early age recruit the same "classic" brain areas associated with mental flexibility as adults; however, there is evidence that the timing of activation may be different. To investigate the temporal dynamics of brain activity associated with mental flexibility in children, we recruited 22 typically developing children (8-15 years) to complete a set-shifting task in the MEG.

Water quality guidance for protection of freshwater mussels (Unionidae) from ammonia exposure.

Ammonia toxicity data for freshwater mussels (Unionidae), a significantly imperiled taxa, were used to derive estimates of concentrations that would not likely be harmful in acute and chronic exposures and to assess the protectiveness of current U.S. Environmental Protection Agency (U.