Febrile infection-related epilepsy syndrome: long-term consequences (own observations)
https://doi.org/10.17650/2073-8803-2020-15-1-40-46
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.
About the Authors
A. V. Drobitova
Epineuro LLC
Russian Federation
151/8 Bolshaya Sadovaya St., Saratov 410071, Russia
Russian Federation
151/8 Bolshaya Sadovaya St., Saratov 410071, Russia
A. Yu. Karas
Epineuro LLC
Russian Federation
151/8 Bolshaya Sadovaya St., Saratov 410071, Russia
Russian Federation
151/8 Bolshaya Sadovaya St., Saratov 410071, Russia
N. V. Maslova
V. I. Razumovskiy Saratov State Medical University
Russian Federation
Department of Psychiatry, Narcology, Psychotherapy, and Clinical Psychology, 112 Bolshaya Kazachya St., Saratov 410012, Russia
Russian Federation
Department of Psychiatry, Narcology, Psychotherapy, and Clinical Psychology, 112 Bolshaya Kazachya St., Saratov 410012, Russia
K. Yu. Mukhin
Svt. Luka’s Institute of Child Neurology and Epilepsy
Russian Federation
5 Nagornaya St., Troitsk, Moscow 108840, Russia
Russian Federation
5 Nagornaya St., Troitsk, Moscow 108840, Russia
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Review
For citations:
Drobitova A.V., Karas A.Yu., Maslova N.V., Mukhin K.Yu. Febrile infection-related epilepsy syndrome: long-term consequences (own observations). Russian Journal of Child Neurology. 2020;15(1):40-46. (In Russ.) https://doi.org/10.17650/2073-8803-2020-15-1-40-46
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