A longitudinal cohort study of mediators of health-related quality of life after pediatric epilepsy surgery or medical treatment.

Objectives: The purpose of this longitudinal cohort study was to examine the variables that influence health-related quality of life (HRQOL) after epilepsy surgery in children. We examined whether treatment type (surgical vs medical therapy) and seizure control are related to other variables that have been shown to influence HRQOL, namely depressive symptoms in children with epilepsy or their parents, and the availability of family resources.

Methods: In total, 265 children with drug-resistant epilepsy were recruited from eight epilepsy centers across Canada at the time of their evaluation for candidacy for epilepsy surgery and were assessed at baseline, 6-month, 1-year, and 2-year follow-up.

Trajectory of Health-Related Quality of Life After Pediatric Epilepsy Surgery.

Importance: Health-related quality of life (HRQOL) is regarded as a key outcome for evaluating treatment efficacy. However, it is uncertain how HRQOL evolves after epilepsy surgery compared with medical therapy, such as whether it continues to improve over time, improves and then remains stable, or deteriorates after a period of time.

Objective: To assess trajectory of HRQOL over 2 years in children with drug-resistant epilepsy (DRE) treated with surgery compared with medical therapy.

Longitudinal changes in emotional functioning following pediatric resective epilepsy surgery: 2-Year follow-up.

Objective: To examine longitudinal changes and predictors of depression and anxiety 2 years following resective epilepsy surgery, compared to no surgery, in children with drug-resistant epilepsy (DRE).

Method: This multicenter cohort study involved 128 children and adolescents with DRE (48 surgical, 80 nonsurgical; 8-18 years) who completed self-report measures of depression and anxiety at baseline and follow-up (6-month, 1-year, 2-year). Child demographic (age, sex, IQ) and seizure (age at onset, duration, frequency, site and side) variables were collected.

A National Spinal Muscular Atrophy Registry for Real-World Evidence.

Background: Spinal muscular atrophy (SMA) is a devastating rare disease that affects individuals regardless of ethnicity, gender, and age. The first-approved disease-modifying therapy for SMA, nusinursen, was approved by Health Canada, as well as by American and European regulatory agencies following positive clinical trial outcomes. The trials were conducted in a narrow pediatric population defined by age, severity, and genotype.

Hashimoto Encephalopathy Presenting With Stroke-Like Episodes in an Adolescent Female: A Case Report and Literature Review.

Background: Hashimoto encephalopathy is a rare form of encephalopathy thought to be of autoimmune etiology. Cognitive changes and seizures are the most commonly reported presenting manifestation. Stroke-like episodes have also been documented in these individuals.

The differential diagnosis of spastic diplegia.

Spastic diplegia is the most common form of cerebral palsy worldwide. Many disorders mimic spastic diplegia, which can result in misdiagnosis for the child with resultant negative treatment and family counselling implications. In this paper, the authors provide a brief review of spastic diplegia and the various disorders in the differential diagnosis.

Basal Ganglia Injury With Extrapyramidal Presentation: A Complication of Meningococcal Meningitis.

Basal ganglia injury, accompanied by extrapyramidal signs, has been described in the setting of chronic tuberculous meningitis; however, such injury rarely occurs in acute bacterial meningitis and has never been reported with meningococcal meningitis. We report the case of a boy who developed tongue bradykinesia and dysarthria 1 week following presentation with meningococcal meningitis. Magnetic resonance imaging revealed bilateral basal ganglia lesions, suspected to result from cytotoxic edema secondary to infection.

Fetal exposure to alcohol, developmental brain anomaly, and vitamin a deficiency: a case report.

Prenatal alcohol exposure is a cause of congenital brain malformations such as hydrocephalus; however, a complete mechanism accounting for this phenomenon has yet to be discovered. We report a case of a newborn who was exposed to alcohol throughout pregnancy and presented with low serum vitamin A and hydrocephalus. To our knowledge, the connection between prenatal ethanol exposure, vitamin A deficiency, and a developmental brain anomaly has never been described in humans before.

Pontocerebellar hypoplasia type 3 with severe vitamin A deficiency.

Pontocerebellar hypoplasia consists of a rare heterogeneous group of congenital neurodevelopmental disorders. It is characterized by hypoplasia and atrophy of the cerebellar cortex, dentate nuclei, pontine nuclei, and inferior olives. We present an 18-month-old infant with pontocerebellar hypoplasia type 3 and severe vitamin A deficiency.