Febrile infection-related epilepsy syndrome: A study of 12 patients

Abstract

FIRES is a well-defined severe epileptic syndrome, probably in the group of epileptic encephalopathies, characterized by focal or multifocal seizures arising from the neocortical regions with an unknown etiology. Immunoglobulin and the ketogenic diet may be considered a potentially efficacious treatment.

Cognitive Outcomes in Febrile Infection-Related Epilepsy Syndrome Treated With the Ketogenic Diet

“…7 Another report described 2 children treated with the KD in the acute phase with a 50% to 70% seizure reduction. 16 Detailed cognitive outcomes were not reported in either study.…”

Recognizing Autoimmune-Mediated Encephalitis in the Differential Diagnosis of Limbic Disorders

“…63 Febrile infection-related epilepsy syndrome, or acute encephalitis with refractory repetitive partial seizures, is considered a severe epileptic encephalopathy with multifocal refractory status epilepticus, which occurs mostly in young children but also in adult patients. 64 The initial phase is characterized by a simple febrile infection, followed by an acute phase with recurrent focal seizures that evolve rapidly into refractory status epilepticus, generally without fever and additional neurologic features. The diagnosis is made after an exhaustive negative search for an active CNS infection and autoimmune or metabolic disorders.…”

“…Early MR imaging may be normal in approximately half of the cases; however, T2 abnormalities are detected in some patients, predominantly in the temporal regions but also in the insula and basal ganglia, which mimics LE. 64,65 In the chronic phase, MR imaging shows mesial temporal sclerosis in half of the patients, and bilateral hypometabolism of orbitofrontal and temporoparietal regions is often demonstrated on PET. 64 The etiology and mechanisms that underlie it are still unknown, and, even though an autoimmune mechanism could be considered and autoantibodies have previously been described in epilepsy, up to now there is no evidence to support that autoantibodies are the etiology of febrile infectionrelated epilepsy syndrome.…”

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

“…R. H. Caraballo и соавт. описали по-ложительный эффект применения кетогенной диеты в острой фазе FIRES у 2 пациентов: у одного из них частота приступов снизилась на 50-75 %, у второго -менее чем на 50 % [5]. 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.…”