Differences in Cerebellar Volume as a Diagnostic and Prognostic Biomarker in Children and Adolescents With Epilepsy of Unknown Etiology.

Introduction And Objective: Epilepsy is one of the most common brain diseases during childhood and adolescence. Atrophy in different brain areas is possible during epilepsy. This study aimed to verify whether cerebellar volume differences could be detected by volume analysis using magnetic resonance imaging (MRI) in children with epilepsy.

[Transition to adult care for patients with epilepsy].

The transition of children and adolescents with epilepsy to the adult healthcare system presents many challenges. The disease is frequently accompanied by cognitive and developmental impairments that make it difficult to achieve self-management of the disease. Seizures are often associated with a loss of consciousness; therefore, conversations regarding medical history often take place only between the physician and the parents.

Expanded phenotype of AARS1-related white matter disease.

Purpose: Recent reports of individuals with cytoplasmic transfer RNA (tRNA) synthetase-related disorders have identified cases with phenotypic variability from the index presentations. We sought to assess phenotypic variability in individuals with AARS1-related disease.

Methods: A cross-sectional survey was performed on individuals with biallelic variants in AARS1.

Bi-allelic HPDL Variants Cause a Neurodegenerative Disease Ranging from Neonatal Encephalopathy to Adolescent-Onset Spastic Paraplegia.

We report bi-allelic pathogenic HPDL variants as a cause of a progressive, pediatric-onset spastic movement disorder with variable clinical presentation. The single-exon gene HPDL encodes a protein of unknown function with sequence similarity to 4-hydroxyphenylpyruvate dioxygenase. Exome sequencing studies in 13 families revealed bi-allelic HPDL variants in each of the 17 individuals affected with this clinically heterogeneous autosomal-recessive neurological disorder.

Epilepsy and cannabidiol: a guide to treatment.

The growing interest in cannabidiol (CBD), specifically a pure form of CBD, as a treatment for epilepsy, among other conditions, is reflected in recent changes in legislation in some countries. Although there has been much speculation about the therapeutic value of cannabis-based products as an anti-seizure treatment for some time, it is only within the last two years that Class I evidence has been available for a pure form of CBD, based on placebo-controlled RCTs for patients with Lennox-Gastaut syndrome and Dravet syndrome. However, just as we are beginning to understand the significance of CBD as a treatment for epilepsy, in recent years, a broad spectrum of products advertised to contain CBD has emerged on the market.

Pharmacology and drug interactions of cannabinoids.

Cannabinoids include a variety of substances, of which cannabidiol (CBD) is the main substance investigated for the treatment of epilepsy, and this will be the focus in the present review. CBD preparations exist in various forms. There are significant differences in quality control regarding content and reproducibility for an approved drug versus herbal preparations.

Strain Ultrasound Elastography of Muscles in Healthy Children and Healthy Adults.

Purpose:  Standardized application and evaluation of the strain ultrasound elastography method (USE) by means of a strain color scale (SCS) and a strain ratio analysis. To determine differences in muscle elasticity in healthy children and adults.

Materials And Methods:  Initially Mm.

Seizure-alerting behavior in dogs owned by people experiencing seizures.

Introduction: The unpredictability of epileptic seizures is considered an important threat to the quality of life of a person with epilepsy. Currently, however, there are no tools for seizure prediction that can be applied to the domestic setting. Although the information about seizure-alert dogs - dogs that display changes in behavior before a seizure that are interpreted by the owner as an alert - is mostly anecdotal; living with an alerting dog (AD) has been reported to improve quality of life of the owner by reducing the stress originating from the unpredictability of epileptic seizures and, sometimes, diminishing the seizure frequency.

Effects of Levetiracetam and Sulthiame on EEG in benign epilepsy with centrotemporal spikes: A randomized controlled trial.

Purpose: BECTS (benign childhood epilepsy with centrotemporal spikes) is associated with characteristic EEG findings. This study examines the influence of anti-convulsive treatment on the EEG.

Methods: In a randomized controlled trial including 43 children with BECTS, EEGs were performed prior to treatment with either Sulthiame or Levetiracetam as well as three times under treatment.

Intrauterine growth restriction improves cerebral O2 utilization during hypercapnic hypoxia in newborn piglets.

Data are scant regarding the capacity of cerebrovascular regulation during asphyxia for prevention of brain oxygen deficit in intrauterine growth-restricted (IUGR) newborns. We tested the hypothesis that IUGR improves the ability of neonates to withstand critical periods of severe asphyxia by optimizing brain oxygen supply. Studies were conducted to examine the effects of IUGR on cerebral blood flow (CBF) regulation and oxygen consumption (cerebral metabolic rate for oxygen, CMRO(2)) at different stages of asphyxia (hypercapnic hypoxaemia) in comparison to pure hypoxia (normocapnic hypoxaemia).

[Do subclinical epileptiform discharges really affect short-term memory in children?].

Objective: Deficits in concentration, specific developmental disorders, and behaviour problems often impair the educational abilities of children and adolescents with epilepsy, even if the subjects are seizure-free. The impact of subclinical epileptiform discharges that persist despite adequate antiepileptic treatment is not yet understood. Some studies suggest that these lead to simultaneous transitory cognitive impairment, thereby affecting short-term memory functions.

Levetiracetam in children with refractory epilepsy: a multicenter open label study in Germany.

Purpose: To evaluate the efficacy and tolerability of Levetiracetam (LEV) in a large pediatric cohort with drug-resistant epilepsy from a prospective multicenter observational study.

Methods: We report the results of a multicenter observational survey of a cohort of 285 pediatric patients (mean: 9.9 years, range: 0; 6-17; 11) with refractory generalized and focal epilepsy who received Levetiracetam as an add-on open label treatment trial.

Diffusion tensor imaging in children and adolescents with tuberous sclerosis.

Background: Tuberous sclerosis (TS) is characterised by benign hamartomatous lesions in many organs. Diffusion tensor imaging (DTI) can detect microstructural changes in pathological processes.

Objective: To determine apparent diffusion coefficient (ADC) and fractional anisotropy (FA) maps in children with TS and to investigate the diffusion properties in cortical tubers, white-matter lesions, perilesional white matter, and contralateral normal-appearing white matter, and to compare the results with ADC and FA maps of normal age- and sex-matched volunteers.

Neuroprotective effects of topiramate after hypoxia-ischemia in newborn piglets.

Background: Perinatal hypoxia-ischemia (HI) is associated with delayed cerebral damage, which involves receptor-mediated excitotoxicity. Until now, successful interventions to reduce excitotoxicity early after HI in experimental settings failed to transform into clinical applications owing to negative side effects. A promising new approach using the anticonvulsant Topiramate (TPM) has shown to be effective to reduce brain damage after early HI in a rodent model of combined TPM-hypothermia.

Early diagnosis of cerebral involvement in Sturge-Weber syndrome using high-resolution BOLD MR venography.

Sturge-Weber syndrome (SWS) is a congenital disorder characterized by a vascular birthmark and neurological abnormalities. Typical imaging findings using MRI or CT are superficial cerebral calcification, atrophy and leptomeningeal enhancement. We present a neonate diagnosed with SWS because of a port-wine stain.

Effect of routine MR imaging of the brain at 1.5 T on subsequent magnetoencephalography: results in nine volunteers.

Purpose: To investigate whether routine magnetic resonance (MR) imaging of the brain with a whole-body 1.5-T imager affects the results of subsequent magnetoencephalography (MEG).

Materials And Methods: Nine healthy volunteers (six women, mean age of 23 years, age range of 20-27 years; three men, mean age of 24 years, age range of 23-25 years) underwent one MEG session before and two MEG sessions after MR imaging of the brain.