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.

Montelukast potentiates the anticonvulsant effect of phenobarbital in mice: An isobolographic analysis

“…Moreover, Lenz and colleagues (2014) have shown that montelukast not only has anticonvulsant effect but also protects against BBB disruption and leukocyte infiltration in a cannula-lesioned cortical area, suggesting that montelukast also decreases neuroinflammation and/or exposure to blood-borne elements [15]. It is well known that areas of focal hyperexcitability can develop in the brain after trauma, infection, ischemia, and excitotoxicity, probably due to neuroinflammation and/or exposure to blood-borne elements [43][44][45][46][47][48][49]. Furthermore, pranlukast treatment protects against global cerebral ischemia in rats and inhibits the expression of NMDA-NR2A receptor [50,51].…”

Febrile infection‐related epilepsy syndrome treated with anakinra

“…With the exception of anaesthetic coma, which is not sustainable, and the ketogenic diet in some patients 2, 3 , treatment that effectively controls seizures is lacking in FIRES, and outcomes are generally poor 1 .…”

Febrile Infection-Related Epilepsy Syndrome (Fires) in Schoolchildren: Literature Review and Own Observations

“…Также в литературе встречает-ся описание применения кетогенной диеты наряду с АЭП у 2 пациентов с FIRES как в остром периоде, так и в хроническую фазу сроком от нескольких ме-сяцев до 1 года. Авторы отмечают, что на фоне прово-димой терапии парциальные приступы были редкими, а когнитивные нарушения -легкой степени выражен-ности [14].…”