Seizure Rescue Medication Use among US Pediatric Epilepsy Providers: A Survey of the Pediatric Epilepsy Research Consortium.

OBJECTIVE: To assess how pediatric neurologists prescribe home seizure rescue medications to treat acute prolonged seizures and clusters of seizures in children. STUDY DESIGN: A brief, email survey was sent to the members of the Pediatric Epilepsy Research Consortium assessing seizure rescue medication prescribing practices for patients of different age groups, cognitive abilities, and seizure type. Survey responses were anonymous. RESULTS: Thirty-six respondents (of 76 surveyed; 47% response rate) completed the survey. Rectal diazepam was the most commonly chosen rescue medication for a prolonged convulsive seizure in a severely developmentally delayed 16-year-old (44%) and typical and delayed 7-year-old (44% and 61%, respectively), 3-year-old (78% and 86%, respectively), and 9-month-old (83%) patients. Most responders (69%) indicated that developmentally typical 16-year-olds would be prescribed intranasal midazolam. For clusters of seizures, clonazepam orally disintegrating tablets were the most frequent first-line option in all age groups, except developmentally delayed 3-year-old and 9-month-old children, for whom rectal diazepam was chosen more commonly. Medication dosing generally followed standard dosing guidelines with very few exceptions. CONCLUSIONS: Rectal diazepam remains the most frequently used rescue medication for prolonged seizures for nearly all age groups, except in developmentally typical teenagers, for whom intranasal midazolam is used more often. Clonazepam orally disintegrating tablets are the most frequently used medication for treatment of clusters of seizures, except in younger patients. Further work is necessary to establish best practices for type and administration route of seizure rescue medications.

The Janus-faced nature of Rasmussen's encephalitis.

Rasmussen encephalitis (RE) is an inflammatory unilateral progressive medically refractory epilepsy associated with hemiparesis, cognitive dysfunction, and hemispheric atrophy. Here, we present 2 cases from our institution that demonstrate the dual nature of RE in 2 similarly aged children. Overall, 2 types of RE have been described: type 1 has a short prodromal phase and more explosive onset and type 2 has a longer prodromal of partial seizures followed by hemiparesis and atrophy. Younger patients are more likely to fit into the type 1 presentation and have been described as more likely to have dual pathology. Perhaps the patients with a more acute onset are more likely to have a dual pathology as is found in our 2 cases. We review the typical findings in RE and discuss current treatment options, highlighting new experimental treatments.