Effect of neonatal seizure burden and etiology on the long-term outcome: data from a randomized, controlled trial.

Background: Neonatal seizures are common, but the impact of neonatal seizures on long-term neurologic outcome remains unclear. We addressed this question by analyzing data from an early-phase controlled trial of bumetanide to treat neonatal seizures.

Methods: Neonatal seizure burden was calculated from continuous video-EEG data.

Comparing the environmental impact of reusable and disposable dental examination kits: a life cycle assessment approach.

Introduction The global climate crisis has increased the emphasis placed on the sustainability and environmental consequences of our actions. The dental examination accounts for a large portion of dentistry's carbon footprint, more specifically, the production, sterilisation, transport, use and disposal of the dental examination kit. An attributional life cycle assessment (LCA) was carried out to compare the impact of a reusable stainless-steel examination kit and that of a disposable plastic examination kit.

Infantile spasms: Assessing the diagnostic yield of an institutional guideline and the impact of etiology on long-term treatment response.

Objective: Neuroimaging and genetic testing have been proposed for diagnostic evaluation of infantile spasms (IS), establishing etiology in ~60% of multicenter IS cohorts. A retrospective analysis of the yield of diagnostic etiology following an institutionally established guideline for investigation/treatment of IS was conducted, and the association between etiological subgroups and sustained response to standard treatment was evaluated.

Methods: Etiology of IS, neuroimaging, and genetic results were extracted from clinical records.

Keto-Sleuths - An Unusual Cause of Loss of Ketosis?

Ketogenic diets provide a non-pharmaceutical alternative for treatment of refractory epilepsy. When successful in reducing or eliminating seizures, medication numbers or doses may be reduced. Unexpected loss of ketosis is a common problem in management of patients on ketogenic diets and, especially when the diet is an effective treatment, loss of ketosis may be associated with an exacerbation in seizures.

Hippocampal Involvement With Vigabatrin-Related MRI Signal Abnormalities in Patients With Infantile Spasms: A Novel Finding.

Background: In a subset of infants exhibiting typical vigabatrin-related magnetic resonance imaging (MRI) changes, the authors observed additional hippocampal signal abnormalities. The authors investigated occurrence and significance of additional signal abnormalities.

Methods: A retrospective review of infantile spasms patients with typical vigabatrin-related MRI abnormalities was performed.

Cost-effectiveness of adrenocorticotropic hormone versus oral steroids for infantile spasms.

Objective: To compare the effectiveness and cost-effectiveness of adrenocorticotropic hormone (ACTH) and oral steroids as first-line treatment for infantile spasm resolution, we performed a systematic review, meta-analysis, and cost-effectiveness study.

Methods: A decision analysis model was populated with effectiveness data from a systematic review and meta-analysis of existing literature and cost data from publicly available prices. Effectiveness was defined as the probability of clinical spasm resolution 14 days after treatment initiation.

A Pilot Randomized, Controlled, Double-Blind Trial of Bumetanide to Treat Neonatal Seizures.

Objective: In the absence of controlled trials, treatment of neonatal seizures has changed minimally despite poor drug efficacy. We tested bumetanide added to phenobarbital to treat neonatal seizures in the first trial to include a standard-therapy control group.

Methods: A randomized, double-blind, dose-escalation design was employed.

Pediatric and adult neurologist perspectives on the challenges of sustaining a transfer clinic.

We present a novel epilepsy fellow-driven transfer clinic model and discuss the challenges experienced in finding sustainability; this is timely as many pioneering transition clinics are dissolving across North America. The goal of this clinic was to improve patient care and satisfaction, as measured by a post-visit telephone survey. Unfortunately, our transfer clinic model proved unsustainable due to several factors, broadly categorized as (1) cultural-societal differences between the pediatric and adult health care environments, (2) staffing issues, (3) lack of an established standardized process for transfer of care, and (4) financial and administrative barriers.

Ketogenic diet in Zambia: Managing drug-resistant epilepsy in a low and middle income country.

Globally, drug-resistant epilepsy affects one third of people living with epilepsy. With limitations in treatment options for refractory epilepsy in resource-limited regions, ketogenic diet therapy is an important option to consider. Utilizing the 2015 International League Against Epilepsy recommended minimum requirements for ketogenic diet therapy, three male children with refractory epilepsy, aged 2.

Mortality in infantile spasms: A hospital-based study.

Objective: To determine risk factors and causes for mortality during childhood in patients with infantile spasms (IS). We describe the overall goals of care for those who died.

Methods: This is a retrospective chart review of IS patients born between 2000 and 2011.

The Evolution of Subclinical Seizures in Children With Tuberous Sclerosis Complex.

Background: Subclinical seizures are electrographic seizures that present without subjective or objective clinical symptoms. In tuberous sclerosis complex, it is not known whether subclinical seizures occur alone, forewarn, or coexist with clinical seizures. To address this knowledge gap, we studied the prevalence and evolution of subclinical seizures in tuberous sclerosis complex.

Cannabis for refractory epilepsy in children: A review focusing on CDKL5 Deficiency Disorder.

Severe paediatric epilepsies such as CDKL5 Deficiency Disorder (CDD) are extremely debilitating, largely due to the early-onset and refractory nature of the seizures. Existing treatment options are often ineffective and associated with a host of adverse effects, causing those that are affected to seek alternative treatments. Cannabis based products have attracted significant attention over recent years, primarily driven by reports of miraculous cures and a renewed public preference for 'natural' therapies, thus placing intense pressure on health professionals and the government for regulatory change.

Detailed Magnetic Resonance Imaging (MRI) Analysis in Infantile Spasms.

Purpose: To evaluate initial magnetic resonance imaging (MRI) abnormalities in infantile spasms, correlate them to clinical characteristics, and describe repeat imaging findings.

Methods: A retrospective review of infantile spasm patients was conducted, classifying abnormal MRI into developmental, acquired, and nonspecific subgroups.

Results: MRIs were abnormal in 52 of 71 infantile spasm patients (23 developmental, 23 acquired, and 6 nonspecific) with no correlation to the clinical infantile spasm characteristics.

Seizure clustering during presurgical electroencephalographic monitoring in children.

Background: Presurgical evaluation with antiseizure medication tapering in patients with refractory epilepsy places them at risk for seizure clustering or prolonged seizures. We looked at the occurrence of seizure clustering (3 or more seizures within 24h) and prolonged seizures and the factors that influence seizure clustering and affect length of stay (LOS) in pediatric patients during presurgical monitoring.

Methods: We retrospectively reviewed the medical records of all consecutive admissions to the epilepsy monitoring unit (EMU) and included patients undergoing noninvasive presurgical evaluation.

Electrographic Seizures in Preterm Neonates in the Neonatal Intensive Care Unit.

Objective: Characterize clinical and electroencephalography (EEG) characteristics of preterm neonates undergoing continuous EEG in the neonatal intensive care unit.

Methods: Retrospective study of preterm neonates born less than 37 weeks' gestational age undergoing continuous EEG in the neonatal intensive care unit at Boston Children's Hospital over a 2-year period.

Results: Fifty-two preterms (46% male) had a mean gestational age of 32.

Use of the ketogenic diet to manage refractory epilepsy in CDKL5 disorder: Experience of >100 patients.

Objective: Pathogenic variants involving the CDKL5 gene result in a severe epileptic encephalopathy, often later presenting with features similar to Rett syndrome. Cardinal features of epilepsy in the CDKL5 disorder include early onset at a median age of 6 weeks and poor response to antiepileptic drugs. The ketogenic diet (KD) was first introduced in the 1920s as a treatment option for refractory epilepsy in children.

Diagnostic and Therapeutic Management of a First Unprovoked Seizure in Children and Adolescents With a Focus on the Revised Diagnostic Criteria for Epilepsy.

By definition, unprovoked seizures are not precipitated by an identifiable factor, such as fever or trauma. A thorough history and physical examination are essential to caring for pediatric patients with a potential first unprovoked seizure. Differential diagnosis, EEG, neuroimaging, laboratory tests, and initiation of treatment will be reviewed.

Electroencephalographic discontinuity during sevoflurane anesthesia in infants and children.

Background: Deep anesthesia in adults may be associated with electroencephalographic (EEG) suppression and higher rates of postoperative complications. Little is known about the impact of anesthetic depth on short- or long-term outcomes in pediatrics. Brain activity monitoring may complement clinical signs of anesthetic depth.

Genetics and genotype-phenotype correlations in early onset epileptic encephalopathy with burst suppression.

Objective: We sought to identify genetic causes of early onset epileptic encephalopathies with burst suppression (Ohtahara syndrome and early myoclonic encephalopathy) and evaluate genotype-phenotype correlations.

Methods: We enrolled 33 patients with a referral diagnosis of Ohtahara syndrome or early myoclonic encephalopathy without malformations of cortical development. We performed detailed phenotypic assessment including seizure presentation, electroencephalography, and magnetic resonance imaging.

Utility of initial EEG in first complex febrile seizure.

Objective: The risk of developing epilepsy following febrile seizures (FS) varies between 2% and 10%, with complex febrile seizures (CFS) having a higher risk. We examined the utility of detected epileptiform abnormalities on the initial EEG following a first CFS in predicting subsequent epilepsy.

Methods: This was a retrospective study of consecutive patients (ages 6-60 months) who were neurologically healthy or mildly delayed, seen in the ED following a first CFS and had both an EEG and minimum of 2-year follow-up.

Clinical and Neuroimaging Profile of Children with Lesions in the Corpus Callosum.

Purpose: T2-hyperintense signal changes in corpus callosum (CC) have been described in epilepsy and encephalitis/encephalopathy. Little is known about their pathophysiology. The aim of this study was to examine the clinical presentation and evolution of CC lesions and relationship to seizures.

Surgery for intractable epilepsy due to unilateral brain disease: a retrospective study comparing hemispherectomy techniques.

Background: Hemispherectomy is a surgical procedure used to treat medically intractable epilepsy in children with severe unilateral cortical disease secondary to acquired brain or congenital lesions. The major surgical approaches for hemispherectomy are anatomic hemispherectomy, traditional functional hemispherectomy, and peri-insular hemispherotomy. We describe the epilepsy outcome, including the need for reoperation, after hemispherectomy in patients with brain malformations or acquired brain lesions who underwent hemispherectomy for refractory epilepsy.

Safety and retention rate of rufinamide in 300 patients: a single pediatric epilepsy center experience.

Objective: Reports of studies evaluating rufinamide as an add-on therapy in children and adolescents with refractory epilepsy are restricted to a few publications. Prospective multicenter studies including children and adults have yielded important information about several types of epilepsies and syndromes. We evaluated the use of rufinamide in a single pediatric center with a large cohort and long-term follow-up period.

Clobazam: effect on frequency of seizures and safety profile in different subgroups of children with epilepsy.

Background: Clobazam has been used in clinical practice as an adjunctive treatment for diverse seizure types and epilepsy syndromes. We evaluated the efficacy and safety of clobazam in a large sample of patients with refractory epilepsy at a tertiary pediatric center.

Methods: We retrospectively reviewed patients treated with clobazam between January 2001 and July 2013 who had a follow-up visit at least one month after starting clobazam.