Seizures and Epilepsy: FIRES

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April 12, 2024
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Seizures and Epilepsy: FIRES

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FIRES is a severe epileptic encephalopathy after a febrile event occurring mainly in the pediatric population (7 and 10 years of age), affecting males > females, with a severe, usually striking neurological outcome. FIRES can begin at a variable age but is rare in adults. It is characterized by focal or multifocal seizures arising from the neocortical regions with unknown etiology.

The pathogenesis of FIRES is not completely understood. The hypotheses include autoimmune, inflammatory, and/or genetics.

Clinical manifestations of FIRES:

1st phase is characterized by a febrile episode with usually no high temperature, cough and/or diarrhea of short days duration.

2nd phase starts after a period of wellbeing (24 h and 2 weeks) then presenting with an acute, sudden, recurrent episodes of afebrile focal seizures (in some cases with secondary generalization) of short duration and interposed by confusional state and poor reactivity. Seizures barely respond to anticonvulsant treatment and tend to rapidly evolve into Refractory Status Epilepticus (RSE), that may last for a few days to weeks.

3rd phase, the chronic period is usually manifested by neurological sequelae with cognitive and language impairment, motor dysfunction and drug resistant seizures.

Which of the following statements is FALSE regarding ages of manifestation of the following epileptic encephalopathies?
EME and Ohtahara syndrome occur in neonates.
West and Dravet syndrome occur in infants.
Lennox-Gastaut and Landau-Kleffner syndrome occur in infants.
CSWS occurs in childhood and adolescence.
Correct Answer: 

C is false. Epileptic encephalopathies are an epileptic condition characterized by epileptiform abnormalities associated with progressive cerebral dysfunction. In the classification of the International League Against Epilepsy eight age-related epileptic encephalopathy syndromes are recognized. These syndromes include early myoclonic encephalopathy and Ohtahara syndrome in the neonatal period, West syndrome and Dravet syndrome in infancy, myoclonic status in nonprogressive encephalopathies, and Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spike waves during slow wave sleep in childhood and adolescences. Other epileptic syndromes such as migrating partial seizures in infancy and severe epilepsy with multiple independent spike foci may be reasonably added. In this paper, we provide an overview of epileptic encephalopathies including clinical neurophysiological features, cognitive deterioration, and management options especially that these conditions are generally refractory to standard antiepileptic drugs.

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