-Related Epilepsy: From Self-Limited Infantile Epilepsy to Atypical Epilepsy Phenotypes.

Background And Objectives: Pathogenic variants in the gene cause self-limited infantile epilepsy (SeLIE). Recently, atypical epilepsy phenotypes have been described. We explore the phenotypic spectrum of -related epilepsy through international collaboration.

Pediatric Neurology Workforce in Canada: A 5-Year Update.

Background: In 2013, a task force was developed to discuss the future of the Canadian pediatric neurology workforce. The consensus was that there was no indication to reduce the number of training positions, but that the issue required continued surveillance. The current study provides a 5-year update on Canadian pediatric neurology workforce data.

Lethal Neonatal Rigidity and Multifocal Seizure Syndrome--A Misnamed Disorder?

Objective: Lethal neonatal rigidity and multifocal seizure syndrome is a newly recognized genetic disorder associated with early onset of rigidity, multifocal epilepsy, developmental arrest, and early death. It is an autosomal recessive condition resulting from a mutation in the BRAT1 (BRCA1 [breast cancer-1]-associated ataxia telangiectasia mutated activator 1) gene. There are few cases in the literature, and all patients have died before age 2 years, most within the first 6 months of life.

The McMaster Pediatric Migraine Questionnaire: a prospective validation study.

Objective: Migraine affects approximately 4%-11% of elementary school children; yet reaching a diagnosis in this age group can be challenging. The goal of this study was to develop a screening migraine questionnaire that could be easily implemented by a general pediatrician and validate its use in diagnosing migraine in children 5-12 years old.

Methods: A questionnaire, the McMaster Migraine Tool, was developed using the International Classification of Headache Disorders-II criteria for migraine.

Micturition-induced seizures: a rare form of reflex epilepsy.

Background: Reflex seizures are referred to as epileptic seizures that are consistently precipitated by a specific sensory stimulus. Reflex seizures induced by micturition are very rare, and few cases have been described.

Methods: This study reports a case of an 11-year-old girl with seizures provoked by micturition with reference made to previously reported cases in the literature.

The magnetic resonance imaging appearance of monophasic acute disseminated encephalomyelitis: an update post application of the 2007 consensus criteria.

Acute disseminated encephalomyelitis (ADEM) is an immunologically mediated inflammatory disease of the central nervous system that typically occurs after a viral infection or recent vaccination, and is most commonly seen in the pediatric population. In 2007 the International Pediatric Multiple Sclerosis Study Group proposed a consensus definition for ADEM for application in research and clinical settings. This article gives an overview of ADEM in children, focusing on differences that have emerged since the consensus definition was established.

Neonatal cerebral sinovenous thrombosis: sifting the evidence for a diagnostic plan and treatment strategy.

Although cerebral sinovenous thrombosis (CSVT) is an uncommon disorder in neonates, the incumbent morbidity, mortality, and adverse neurodevelopmental sequelae highlight the importance of establishing an early diagnosis with an appropriate therapeutic plan. The clinical signs and symptoms of the condition are subtle and invariably masquerade under the umbrella of a broad spectrum of neonatal illnesses. A high index of diagnostic suspicion is essential for investigating and initiating treatment in a timely fashion before major complications ensue.

The influence of sex on limbic volume and perfusion in AD.

The goal of the current study was to determine whether Alzheimer's disease (AD) pathology affects the limbic system of men and women differently as measured by in vivo neuroimaging. Magnetic resonance imaging (MRI) and coregistered single photon emission computed tomography (SPECT) were used to examine the limbic system in 20 men and 20 women with probable AD compared to 40 age- and education-matched normal controls (20 men, 20 women). Limbic volumes and relative perfusion values were obtained from the MR images and coregistered SPECT scans, respectively.

Limbic system perfusion in Alzheimer's disease measured by MRI-coregistered HMPAO SPET.

The goal of this study was to perform a systematic, semi-quantitative analysis of limbic perfusion in patients with Alzheimer's disease (AD) using coregistered single-photon emission tomography (SPET) images aligned to magnetic resonance (MR) images. Limbic perfusion in 40 patients with mild to moderate AD was compared with that of 17 age-, sex-, and education-matched normal controls (NC). HMPAO SPET scans and 3D T1-weighted MR images were acquired for each subject.