Chronological Evolution of Magnetic Resonance Imaging Findings in Children With Febrile Infection-Related Epilepsy Syndrome

Rivas-Coppola, Marianna; Shah, Namrata; Choudhri, Asim F.; Morgan, Robin; Wheless, James W.

doi:10.1016/j.pediatrneurol.2015.09.003

Cited by 31 publications

(34 citation statements)

References 26 publications

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“…Clinical, neurophysiologic, and MRI features of the patient were consistent with FIRES . In accordance, EEG frequently shows global slowing and multifocal seizures and brain MRI in the initial stages of FIRES is often normal or with minor abnormalities, whereas later evolution typically leads to brain atrophy . Whether these changes are due to prolonged intractable seizures and involve brain inflammation remains uncertain .…”

Section: Discussionmentioning

confidence: 65%

“…1 In accordance, EEG frequently shows global slowing and multifocal seizures and brain MRI in the initial stages of FIRES is often normal or with minor abnormalities, whereas later evolution typically leads to brain atrophy. 9 Whether these changes are due to prolonged intractable seizures and involve brain inflammation remains uncertain. 10 A series of brain MRI studies was collected in our patient and matched to continuous EEG monitoring, showing the topographic correspondence of the epileptic multiple focuses with the alterations on brain MRI.…”

Section: Discussionmentioning

confidence: 99%

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Therapeutic effect of Anakinra in the relapsing chronic phase of febrile infection–related epilepsy syndrome

et al. 2019

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Summary Febrile infection–related epilepsy syndrome (FIRES) is a severe epileptic encephalopathy with presumed inflammatory origin and lacking effective treatments. Anakinra is the human recombinant interleukin 1 receptor antagonist clinically used in autoinflammatory or autoimmune conditions. We report a case of FIRES for which the spatial and temporal match between electroencephalography (EEG) and magnetic resonance imaging (MRI) focal alterations provides support for the detrimental synergic interplay between seizures and inflammation that may evolve to permanent focal lesions and progressive brain atrophy in weeks to months. Brain biopsy showed aspects of chronic neuroinflammation with scarce parenchymal lymphocytes. We report the novel evidence that anakinra reduces the relapse of highly recurrent refractory seizures at 1.5 years after FIRES onset. Our evidence, together with previously reported therapeutic effects of anakinra administered since the first days of disease onset, support the hypothesis that interleukin 1β and inflammation‐related factors play a crucial role in seizure recurrence in both the acute and chronic stages of the disease.

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Section: Discussionmentioning

confidence: 65%

Section: Discussionmentioning

confidence: 99%

Therapeutic effect of Anakinra in the relapsing chronic phase of febrile infection–related epilepsy syndrome

et al. 2019

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“…Four patients had normal imaging and one had right hippocampal edema within 3 days of onset. Progressive cytotoxic edema was then demonstrated bilaterally in temporal regions including hippocampi between 1 and 3 weeks . At 1 month, there was varying severity of atrophy, which continued to progress to severe cerebral atrophy and cerebellar volume loss at 1 year .…”

Section: Discussionmentioning

confidence: 98%

“…Progressive cytotoxic edema was then demonstrated bilaterally in temporal regions including hippocampi between 1 and 3 weeks . At 1 month, there was varying severity of atrophy, which continued to progress to severe cerebral atrophy and cerebellar volume loss at 1 year . Whether changes are due to prolonged intractable seizures or the disease entity itself remains uncertain …”

Section: Discussionmentioning

confidence: 99%

The spectrum of neuroimaging findings in febrile infection‐related epilepsy syndrome (FIRES): A literature review

et al. 2019

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Summary Febrile infection‐related epilepsy syndrome (FIRES) is a rare severe epileptic syndrome occurring in previously healthy children and characterized by refractory status epilepticus (SE) following a febrile illness. Brain imaging findings in affected patients have been reported in few case series and some case reports. This article is a comprehensive review of the magnetic resonance imaging (MRI) characteristics in all reported patients with a diagnosis of FIRES, describing the findings in the acute and chronic phases of the disease, and discussing possible pathogenesis and radiologic differential diagnoses. Most of the patients had normal brain scans in the acute phase (61%) and about 25% of the patients reported in literature had abnormalities in the temporal lobes. Changes in the basal ganglia and rarely in thalami or brainstem have also been described, as well as diffuse cerebral edema in a minority of patients during the acute phase. The chronic phase of the disease was characterized by atrophic changes and evidence of mesiotemporal sclerosis. An understanding of these MRI abnormalities is necessary to support the diagnosis of FIRES and exclude mimics.

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“…[132–134] The brain MRI is initially normal but as the disease progresses, mesio-temporal T2/FLAIR hyperintensities become apparent and almost all patients eventually develop cerebral atrophy and MTS. [135] Due to the refractoriness of status epilepticus, patients frequently need pharmacologically-induced coma. Anesthetics are often inefficient and their prolonged use seems to negatively influence cognitive outcome.…”

Section: Introductionmentioning

confidence: 99%

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis

Spatola

Dalmau

2017

Current Opinion in Neurology

147

137

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Purpose of review To assess the seizure manifestations and risk of epilepsy in encephalitis associated to antibodies against neuronal cell-surface (AE) or myelin-associated antigens, and to review several chronic epileptic disorders including, Rasmussen’s encephalitis (RE), fever-induced refractory epileptic syndromes (FIRES), and new-onset refractory status epilepticus (NORSE). Recent findings Seizures are a frequent manifestation of AE. Some AE may associate with characteristic features: faciobrachial dystonic seizures (anti-LGI1 encephalitis), EEG extreme delta brush (anti-NMDAR), or multifocal FLAIR-MRI abnormalities (anti-GABAAR). In anti-LGI1 encephalitis, cortical, limbic, and basal ganglia dysfunction results in different types of seizures. AE or myelin-antibody associated syndromes are often immunotherapy-responsive and appear to have a low risk for chronic epilepsy. In contrast patients with seizures related to GAD65-antibodies (an intracellular antigen) frequently develop epilepsy and have suboptimal response to treatment (including surgery). RE or FIRES may occur with autoantibodies of unclear significance and rarely respond to immunotherapy. A study of patients with NORSE showed that 30% developed chronic epilepsy. Summary Although seizures are frequent in all types of AE, the risk for chronic epilepsy is dependent on the antigen: lower if located on the cell-surface, and higher if intracellular. For other disorders (RE, FIRES, NORSE) the prognosis remains poor.

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Chronological Evolution of Magnetic Resonance Imaging Findings in Children With Febrile Infection-Related Epilepsy Syndrome

Cited by 31 publications

References 26 publications

Therapeutic effect of Anakinra in the relapsing chronic phase of febrile infection–related epilepsy syndrome

Therapeutic effect of Anakinra in the relapsing chronic phase of febrile infection–related epilepsy syndrome

The spectrum of neuroimaging findings in febrile infection‐related epilepsy syndrome (FIRES): A literature review

Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis

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