The burden of pediatric status epilepticus: Epidemiology, morbidity, mortality, and costs.

PURPOSE: To summarize the epidemiology, morbidity, mortality, and costs of status epilepticus (SE) in the pediatric population. METHOD: Review of the medical literature. RESULTS: The overall incidence of pediatric SE is roughly 20 per 100,000 children per year, with overall mortality of 3%. Underlying etiology is the biggest risk factor for SE, with symptomatic (acute > remote) etiologies associated with worse outcomes. The most common cause of SE in children is febrile SE, though this entity occurs primarily in early childhood. After a first episode, the risk of recurrence is similar to the risk after a first unprovoked seizure (25-40%). SE is expensive, regularly costing more than $10,000 per episode and often more than $100,000 for refractory cases. CONCLUSION: SE is not an uncommon neurologic emergency and depending on the associated etiology can carry significant morbidity, mortality, and cost especially if treatment is not performed in a timely manner.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Rasmussen-like Syndrome: An Association?

BACKGROUND: N-methyl-D-aspartate (NMDA) receptor encephalitis is an immune-mediated condition that has a broad spectrum of manifestations, including seizures, coma, psychosis, and focal neurological deficits. Although usually a diffuse process, unihemispheric involvement mimicking early stages of Rasmussen encephalitis can occur. Rasmussen's encephalitis is a unique syndrome characterized by progressive hemiplegia, drug-resistant focal epilepsy, cognitive decline, and hemispheric brain atrophy contralateral to the hemiplegia. PATIENT DESCRIPTION: We describe a two-year-old girl with progressive right weakness and epilepsia partialis continua, concerning for early Rasmussen's encephalitis, who tested positive for anti-NMDA receptor antibodies. She experienced complete clinical recovery after immunotherapy. Anti-NMDA receptor antibodies were absent at three weeks and again at one year after the first treatment of intravenous immunoglobulin. CONCLUSIONS: There are few reports of Rasmussen-like encephalitis in individuals with anti-NMDA receptor antibody positivity. Thus the clinical significance of this association is yet to be determined. In addition, several other antibodies have been documented in individuals with Rasmussen encephalitis. The lack of a consistently reported antibody in Rasmussen encephalitis patients and the temporary nature of the anti-NMDA receptor antibody in our patient raise the following question: Is the presence of anti-NMDA receptor antibodies the cause of the symptoms or secondary to the pathogenic process?