Abstract

Febrile infection-related epilepsy syndrome (FIRES) is an exceedingly rare disorder that affects 1 in 1.000.000 children. However, we believe that FIRES is more common, since many cases remain undiagnosed. The exact pathogenesis of this clinical syndrome is still poorly understood. There are several theories of its development, including immune, genetic, and inflammatory-mediated ones. FIRES is known to have dismal outcomes with a death rate of up to 30 % in the acute phase and subsequent development (often immediately after the acute phase) of refractory epilepsy and mental retardation in 66–100 % of survivors. Despite the increasing number of publications, the problems of timely diagnosis and treatment of such patients have not yet been addressed. We describe 6 patients presumed to have had FIRES. The most common outcomes included drug-resistant epilepsy, as well as cognitive and behavioral disorders. Continuing seizures and epileptiform activity on the electroencephalogram trigger cognitive and behavioral disorders. The analysis of treatment outcomes indicates that combinations of carbamazepine / oxcarbazepine with other antiepileptic drugs are most effective.

Highlights

  • Cиндром эпилепсии, связанный с фебрильной инфекцией (Febrile Infection-Related Epilepsy Syndrome, Febrile infection-related epilepsy syndrome (FIRES)), встречается очень редко – с частотой примерно 1 случай на 1 млн детского населения

  • We believe that FIRES is more common, since many cases remain undiagnosed

  • We describe 6 patients presumed to have had FIRES

Read more

Summary

JOURNAL of NEUROLOGY

Связанный с фебрильной инфекцией: отдаленные последствия (собственный опыт наблюдения). Связанный с фебрильной инфекцией (Febrile Infection-Related Epilepsy Syndrome, FIRES), встречается очень редко – с частотой примерно 1 случай на 1 млн детского населения. Связанный с фебрильной инфекцией (Febrile Infection-Related Epilepsy Syndrome, FIRES), представляет собой форму эпилептической энцефалопатии, выделенную Международной противоэпилептической лигой в самостоятельную нозологическую единицу, диагностические критерии которой несколько различаются у авторов, опубликовавших собственные клинические наблюдения [6, 8, 12]. Что данные магнитно-резонансной терапии (МРТ) в острую фазу заболевания оказываются отрицательными у подавляющего большинства пациентов, лишь в некоторых случаях выявляется гиперинтенсивность сигнала преимущественно височных областей, а также базальных ганглиев и островка [6, 9, 12], вероятно, вторичного характера вследствие длительной эпилептиформной активности. Возраст дебюта Age at onset ность «светлого промежутка» Duration of symptomfree period

Нет None
No pathological activity is currently registered

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call

Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.