title: Cognitive outcomes in febrile infection-related epilepsy syndrome treated with the ketogenic diet.
abstract: Febrile infection-related epilepsy syndrome (FIRES) is a newly recognized epileptic encephalopathy in which previously healthy school-aged children present with prolonged treatment-resistant status epilepticus (SE). Survivors are typically left with pharmacoresistant epilepsy and severe cognitive impairment. Various treatment regimens have been reported, all with limited success. The ketogenic diet (KD) is an alternative treatment of epilepsy and may be an appropriate choice for children with refractory SE. We report 2 previously healthy children who presented with FIRES and were placed on the KD during the acute phase of their illness. Both children experienced resolution of SE and were maintained on the KD, along with other anticonvulsant medications, for several months. Both were able to return to school, with some academic accommodations. These cases highlight the potential value of the KD as a preferred treatment in FIRES, not only in the acute setting but also for long-term management. Early KD treatment might optimize both seizure control and cognitive outcome after FIRES.
authors.affiliation: Division of Pediatric Neurology, University of Alabama, Birmingham, Alabama; rsingh@peds.uab.edu.
authors.forename: Rani K
authors.initials: RK
authors.lastname: Singh
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics and Communicable Diseases, C.S. Mott Children's Hospital; and.
authors.forename: Sucheta M
authors.initials: SM
authors.lastname: Joshi
authors.affiliation: Patient Food and Nutrition Services, University of Michigan Hospitals, University of Michigan, Ann Arbor, Michigan.
authors.forename: Denise M
authors.initials: DM
authors.lastname: Potter
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics and Communicable Diseases, C.S. Mott Children's Hospital; and.
authors.forename: Steve M
authors.initials: SM
authors.lastname: Leber
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics and Communicable Diseases, C.S. Mott Children's Hospital; and.
authors.forename: Martha D
authors.initials: MD
authors.lastname: Carlson
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics and Communicable Diseases, C.S. Mott Children's Hospital; and.
authors.forename: Renée A
authors.initials: RA
authors.lastname: Shellhaas
mesh_terms: D002648:Child; D003071:Cognition / Q000502:physiology*; D055423:Diet, Ketogenic / Q000379:methods; D005260:Female; D006801:Humans; D008297:Male; D003294:Seizures, Febrile / Q000175:diagnosis / Q000178:diet therapy* / Q000209:etiology; D013290:Streptococcal Infections / Q000150:complications / Q000175:diagnosis / Q000178:diet therapy*; D013577:Syndrome; D016896:Treatment Outcome
keywords: IQ; epilepsy; ketogenic diet