The expanding spectrum of febrile infection-related epilepsy syndrome (FIRES)

Neurology and Neurophysiology Unit, Complejo Hospitalario Torrecárdenas, Almeria, Spain. Electronic address: .
Seizure (Impact Factor: 1.82). 12/2012; 22(2). DOI: 10.1016/j.seizure.2012.11.006
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Available from: PJ Serrano Castro
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    ABSTRACT: Febrile infection-related epilepsy syndrome (FIRES) is a rare but devastating condition of the children and only a few adults with similar clinical conditions have been reported so far. Two female patients in their thirties presented with explosive onset of intractable epilepsy induced by fever followed by cognitive problems and they were diagnosed as “limbic encephalitis” in the beginning. However, neither paraneoplastic nor infectious etiology was detected despite detailed investigations. Their video-EEG monitoring showed multifocal origin of epileptic activity consistent with childhood series of FIRES. PET studies showed bilateral mesial temporal and frontal lobe hypometabolism, respectively. Many antiepileptic drugs and relevant immunotherapeutics were not able to control their seizures. Furthermore, ketogenic diet did not provide prominent benefits after this delay in the diagnosis of FIRES in adult patients. Clinical pictures compatible with FIRES could also be seen in adults and may be misdiagnosed as “limbic encephalitis” without any evidence of paraneoplastic etiology. Inclusion of adult cases for pathogenetic and therapeutic investigations for FIRES may shorten the time to disclose the etiology for this terrible neurological condition.
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    ABSTRACT: To characterize the clinical, EEG, and brain imaging findings in an adult case series of patients with de novo refractory status epilepticus (SE) occurring after a febrile illness. A retrospective study (2010-2013) was undertaken with the following inclusion criteria: (1) previously healthy adults with refractory SE; (2) seizure onset 0-21 days after a febrile illness; (3) lacking evidence of infectious agents in CSF; (4) no history of seizures (febrile or afebrile) or previous or concomitant neurologic disorder. Among 155 refractory SE cases observed in the study period, 6 patients (17-35 years old) fulfilled the inclusion criteria. Confusion and stupor were the most common symptoms at disease onset, followed after a few days by acute repeated seizures that were uncountable in all but one. Seizures consisted of focal motor/myoclonic phenomena with subsequent generalization. Antiepileptic drugs failed in every patient to control seizures, with all participants requiring intensive care unit admission. Barbiturate coma with burst-suppression pattern was applied in 4 out of 6 patients for 5-14 days. One participant died in the acute phase. In each patient, we observed a reversible bilateral claustrum MRI hyperintensity on T2-weighted sequences, without restricted diffusion, time-related with SE. All patients had negative multiple neural antibodies testing. Four out of 5 surviving patients developed chronic epilepsy. This is a hypothesis-generating study of a preliminary nature supporting the role of the claustrum in postfebrile de novo SE; future prospective studies are needed to delineate the specificity of this condition, its pathogenesis, and the etiology. © 2015 American Academy of Neurology.
    No preview · Article · Sep 2015 · Neurology