Ketamine Versus Midazolam as the First-Line Continuous Infusion for Status Epilepticus in Children with Cardiac Disease.

Background: The treatment of status epilepticus (SE) in children with cardiac disease is challenging given their often-tenuous hemodynamic state. We aim to determine whether ketamine is safe and effective in children with cardiac disease as the first-line continuous infusion for the treatment of refractory SE (RSE) and to compare ketamine to midazolam for the treatment of RSE in this population.

Methods: This is a single-center retrospective cohort study of pediatric patients with cardiac disease and RSE admitted to the cardiac intensive care unit at a tertiary children's hospital between January 1, 2017 and June 30, 2023.

Prenatally Diagnosed Absent Septum Pellucidum and Septo-Optic Dysplasia: A Narrative Review and Practical Recommendations for Pediatric Neurologists.

Evaluation of the cavum septum pellucidum is required in standard second-trimester screening fetal anatomy ultrasound scans. The absence of septum pellucidum triggers further evaluation and referral for subspecialty counseling. Absence of septum pellucidum is linked to other midline anomalies including septo-optic dysplasia.

Progressive Loss of Cerebral Structures in ALG11-Related Congenital Disorder Glycosylation.

Background: Congenital disorders of glycosylation (CDG) are a group of metabolic disorders related to dysfunctional glycoprotein and glycolipid biosynthesis. ALG11-related CDG is a rare member of this group, characterized by severe neurodevelopmental impairment, progressive microcephaly, sensorineural hearing loss, and epilepsy. The objective of this report is to provide an update on the phenotype and brain magnetic resonance imaging (MRI) at age seven years for a patient initially described in early infancy with fetal brain disruption sequence.

Examining Infant and Child Neurodevelopmental Outcomes After Lyme Disease During Pregnancy.

Lyme disease is the most common vector-borne disease in the United States. Recent environmental and socioecological changes have led to an increased incidence of Lyme and other tick-borne diseases, which enhances the urgency of identifying and mitigating adverse outcomes of Lyme disease exposure. Lyme disease during pregnancy, especially when untreated, may lead to adverse pregnancy and neonatal outcomes; however, long-term child outcomes following utero exposure to Lyme disease have not yet been systematically assessed.

Building a growing genomic repository for maternal and fetal health through the PING Consortium.

Background: Prenatally transmitted viruses can cause severe damage to the developing brain. There is unexplained variability in prenatal brain injury and postnatal neurodevelopmental outcomes, suggesting disease modifiers. Of note, prenatal Zika infection can cause a spectrum of neurodevelopmental disorders, including congenital Zika syndrome.

Preoperative autonomic failure in neonates with critical congenital heart disease.

Background: Neonates with critical congenital heart disease (cCHD) undergo a complicated transition to ex-utero life. However, continuous monitoring of autonomic tone using heart rate variability is currently lacking.

Materials And Methods: We retrieved continuous electrocardiograms from the time of admission or from 10 days prior to surgery for neonates with dextro-transposition of the great arteries (d-TGA) and hypoplastic left heart syndrome (HLHS).

Prenatally Diagnosed Holoprosencephaly: Review of the Literature and Practical Recommendations for Pediatric Neurologists.

Holoprosencephaly (HPE) is one of the most common malformations in embryonic development. HPE represents a continuum spectrum that involves the midline cleavage of forebrain structures. Facial malformations of varying degrees of severity are also observed.

Asymmetry of Directed Brain Connectivity at Birth in Low-Risk Full-Term Newborns.

Purpose: Functional connectivity hubs were previously identified at the source level in low-risk full-term newborns by high-density electroencephalography (HD-EEG). However, the directionality of information flow among hubs remains unclear. The aim of this study was to study the directionality of information flow among source level hubs in low-risk full-term newborns using HD-EEG.

Frequency Domain Template Subtraction Approach to Attenuate Maternal Electrocardiogram in Fetal Electrocardiogram.

We develop a frequency domain template subtraction approach to attenuate the maternal ECG in the abdominal ECG measured from pregnant women. The proposed approach was tested on five public fetal ECG datasets simultaneously measured with ECG from the fetal scalp. The method's performance was compared with the template subtraction approach in the time domain using the accuracy and association metrics.

Bedside monitoring tools and advanced signal processing approaches to monitor critically-ill infants.

There is a substantial body of literature that supports neonatal monitoring and signal analysis of the collected data to provide valuable insights for improving patient clinical care and to inform new research studies. This comprehensive monitoring approach extends beyond the collection of conventional vital signs to include the acquisition of continuous waveform data from patient monitors and other bedside medical devices. This paper discusses the necessary infrastructure for waveform retrieval from bedside monitors, and explores options provided by leading healthcare companies, third-party vendors or academic research teams to implement scalable monitoring systems across entire critical care units.

Mindfulness Exercises Reduce Acute Physiologic Stress Among Female Clinicians.

Importance: Approximately 50% of clinicians experience excessive emotional, physical, and mental stress, with repercussions across the entire medical system. Mindfulness exercises may mitigate this excessive stress. Heart rate variability (HRV) is an objective stress measure that can quantify which mindfulness exercises provide the greatest stress reduction.

Multiple Pediatric Extracorporeal Membrane Oxygenation Runs and Futility. What Are the Limits?

Despite high mortality rates, pediatric extracorporeal membrane oxygenation (ECMO) redeployments are frequently discussed in everyday clinical care. We aim to investigate predictors of mortality in those patients. Clinical data from a single pediatric center were retrospectively analyzed.

Technical considerations for placental tissue processing and the subsequent impact on genome-wide DNA methylation analysis.

To assess the impact of postnatal processing on placental DNA methylation, array data from flash-frozen placental tissue was compared to perfluorocarbon-immersed and formalin-fixed paraffin-embedded placental tissue. We observed that tissue exposed to perfluorocarbon showed no significant DNA methylation differences when compared to unprocessed tissue, while formalin processing altered the quality and reliability of the data produced on the DNA methylation array platform. Placental DNA methylation allows for the study of gene-environment interactions that influence the fetal environment and development.

Circadian rhythm development in preterm infants. The role of postnatal versus postmenstrual age.

Background, Aims: Circadian rhythm maturation may be disturbed in premature infants undergoing neonatal intensive care. We used continuous heart rate recordings across the entire neonatal intensive care period to study circadian rhythm development in preterm infants and to evaluate the roles of postmenstrual (PMA) versus postnatal age (PNA).

Materials And Methods: The circadian rhythm was calculated using a cosine fit of heart rate.

Fetal Brain MRI Abnormalities in Pyruvate Dehydrogenase Complex Deficiency.

Background And Objectives: Pyruvate dehydrogenase complex deficiency (PDCD) is a disorder of mitochondrial metabolism that is caused by pathogenic variants in multiple genes, including . Typical neonatal brain imaging findings have been described, with a focus on malformative and encephaloclastic features. Fetal brain MRI in PDCD has not been comprehensively described.

Neurodevelopmental Outcomes of Preschoolers with Antenatal Zika Virus Exposure Born in the United States.

Neurodevelopmental outcomes for preschool-age children in the United States with in utero Zika virus (ZIKV) exposure have not yet been reported. We performed a case-control study to assess whether children exposed in utero to ZIKV have abnormal neurodevelopment at age 4-5 years compared to unexposed controls. Thirteen ZIKV-exposed cases that did not have microcephaly or other specific features of congenital Zika syndrome and 12 controls were evaluated between ages 4-5 years.

Feasibility and success of a non-sedated brain MRI training protocol in 7-year-old children from rural and semi-rural Colombia.

Background: Brain magnetic resonance imaging (MRI) is a crucial tool for clinical evaluation of the brain and neuroscience research. Obtaining successful non-sedated MRI in children who live in resource-limited settings may be an additional challenge.

Objective: To present a feasibility study of a novel, low-cost MRI training protocol used in a clinical research study in a rural/semi-rural region of Colombia and to examine neurodevelopmental factors associated with successful scans.

Advancing fetal diagnosis and prognostication using comprehensive prenatal phenotyping and genetic testing.

Prenatal diagnoses of congenital malformations have increased significantly in recent years with use of high-resolution prenatal imaging. Despite more precise radiological diagnoses, discussions with expectant parents remain challenging because congenital malformations are associated with a wide spectrum of outcomes. Comprehensive prenatal genetic testing has become an essential tool that improves the accuracy of prognostication.

Increasing Provider Utilization of a Seizure Action Plan in the Outpatient Setting.

Objectives: A seizure action plan (SAP) is a powerful tool that provides actionable information for caregivers during seizures. Guidelines have expressed the need for individualized SAPs. Our quality improvement team aimed to increase implementation of an SAP within a pediatric tertiary center, initially among epilepsy providers and expanded to all neurology providers.

Fetal Cerebral Ventriculomegaly: A Narrative Review and Practical Recommendations for Pediatric Neurologists.

Fetal cerebral ventriculomegaly is one of the most common fetal neurological disorders identified prenatally by neuroimaging. The challenges in the evolving landscape of conditions like fetal cerebral ventriculomegaly involve accurate diagnosis and how best to provide prenatal counseling regarding prognosis as well as postnatal management and care of the infant. The purpose of this narrative review is to discuss the literature on fetal ventriculomegaly, including postnatal management and neurodevelopmental outcome, and to provide practice recommendations for pediatric neurologists.

Enasidenib-induced hepatitis in an individual with Type II D2-hydroxyglutaric aciduria.

Type II D-2-Hydroxyglutaric aciduria (T2D2HGA) is caused by a gain-of-function pathogenic variant in Isocitrate Dehydrogenase 2 (IDH2). Patients with T2D2HGA commonly present with developmental delay, seizures, cardiomyopathy, and arrhythmias. The recently approved IDH2-inhibitor Enasidenib targets the p.

Congenital infectious encephalopathies from the intrapartum period to postnatal life.

Congenital infections are a common but often underrecognized cause of fetal brain abnormalities, as well as fetal-neonatal morbidity and mortality, that should be considered by all healthcare professionals providing neurological care to fetuses and newborns. Maternal infection with various pathogens (cytomegalovirus, Toxoplasmosis, Rubella virus, Parvovirus B19, lymphocytic choriomeningitis virus, syphilis, Zika virus, varicella zoster virus) during pregnancy can be transmitted to the developing fetus, which can cause multisystem dysfunction and destructive or malformative central nervous system lesions. These can be recognized on fetal and neonatal imaging, including ultrasound and MRI.