The impact of hypsarrhythmia on infantile spasms treatment response: Observational cohort study from the National Infantile Spasms Consortium.

Objective: The multicenter National Infantile Spasms Consortium prospective cohort was used to compare outcomes and phenotypic features of patients with infantile spasms with and without hypsarrhythmia.

Methods: Patients aged 2 months to 2 years were enrolled prospectively with new-onset infantile spasms. Treatment choice and categorization of hypsarrhythmia were determined clinically at each site.

Imaging of Glutamate Concentration in Sturge-Weber Syndrome.

Investigators from Wayne State University studied a cohort of children with Sturge-Weber syndrome (SWS) and epilepsy using both glucose-based positron emission tomography (FDG-PET) to evaluate metabolic activity and proton magnetic resonance spectroscopic imaging (MRSI) to evaluate glutamate turnover.

Spectral Electroencephalogram Analysis for the Evaluation of Encephalopathy Grade in Children With Acute Liver Failure.

Objective: Spectral electroencephalogram analysis is a method for automated analysis of electroencephalogram patterns, which can be performed at the bedside. We sought to determine the utility of spectral electroencephalogram for grading hepatic encephalopathy in children with acute liver failure.

Design: Retrospective cohort study.

Prognosis with Incidental Rolandic Spikes.

Investigators from Johns Hopkins University reported a cohort of 27 patients with incidentally-noted rolandic spikes (RS) on EEG.

Epilepsy Following Neonatal Seizures Secondary to Hemorrhagic Stroke in Term Neonates.

Intracranial hemorrhage accounts for about 50% of all pediatric stroke. Studies of term infants with intracranial hemorrhage have shown favorable motor and cognitive outcome. The goal of this study was to examine the risk of developing epilepsy in full-term infants with intracranial hemorrhage.

Case Report: Intravenous and Oral Pyridoxine Trial for Diagnosis of Pyridoxine-Dependent Epilepsy.

Pyridoxine-dependent epilepsy is a rare, autosomal recessive, treatable cause of neonatal seizures. Genetic testing can confirm mutations in the ALDH7A1 gene, which encodes antiquitin. To avoid delays in initiating treatment while awaiting confirmatory genetic testing, it is recommended that all neonates with unexplained seizures should receive trial of intravenous (IV) pyridoxine to assess for responsiveness.

Utility of the electroencephalogram in attention deficit hyperactivity disorder.

An electroencephalogram (EEG) has not been routinely utilized in the evaluation of children with attention deficit hyperactivity disorder (ADHD). The utility of the EEG in ADHD is unclear. A recent study in our laboratory using sleep and sleep deprivation routinely found one in four non-epileptic children evaluated for attention deficit disorder has epileptiform discharges in the EEG, more than half focal.

Ictal asystole and anti-N-methyl-D-aspartate receptor antibody encephalitis.

Anti-N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis is a recently identified autoimmune disorder that is increasingly recognized in children. Most cases occur in girls and women and may be paraneoplastic with an associated ovarian teratoma. Characteristic clinical features include neuropsychiatric symptoms, dyskinesias, decreased consciousness, and autonomic instability.

Frequency of epileptiform discharges in the sleep-deprived electroencephalogram in children evaluated for attention-deficit disorders.

The authors determined the frequency of epileptiform discharges in the electroencephalogram (EEG) of a cohort of children and adolescents referred to a neurology specialty clinic for evaluation of attention-deficit disorders. Of 624 records, 461 (73.9%) were normal and 163 (26.

Neuroimaging findings in children with rare or novel de novo chromosomal anomalies.

Background: De novo constitutional chromosomal anomalies provide important insights into the genetic loci responsible for congenital neurological disorders. However, most phenotypic descriptions of patients with rare chromosomal abnormalities are published as individual case reports or small group studies, making genotype-phenotype correlations unclear. Moreover, many clinical genetic reports do not include neuroimaging.

T-cell lymphoma presenting with neurologic features in immunocompetent children.

Systemic T-cell lymphoma presenting with neurologic symptoms is infrequently reported in immunocompetent children. We investigated the presenting features in all 20 immunocompetent children diagnosed with T-cell lymphoma at our institution from 1992-2004. Four children presented with neurologic features.

Systemic lymphoma mimicking acute disseminated encephalomyelitis.

We describe a 10-year-old immunocompetent male whose initial presentation was consistent with the diagnosis of acute disseminated encephalomyelitis. He relapsed 3 months later, with new neurologic signs and lymphadenopathy. T-cell lymphoma was diagnosed by lymph node and stereotaxic brain biopsy.