Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status

Baxter, Peter; Clarke, Antonia; Cross, Helen; Harding, Brian; Hicks, Elaine; Livingston, John H.; Surtees, Robert

doi:10.1016/s1059-1311(02)00340-0

Cited by 89 publications

(102 citation statements)

References 7 publications

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“…A similar pattern had previously been reported under various names (2)(3)(4)(5)(6), and because the condition is triggered by fever, the eponym FIRES (feverinduced refractory epileptic encephalopathy in school-age children) is currently proposed (7). It occurs in previously healthy children, after a brief febrile episode, with an acute phase characterized by convulsive status epilepticus without any feature of central nervous system infection or inflammation.…”

supporting

confidence: 60%

¹⁸F-FDG PET Reveals Frontotemporal Dysfunction in Children with Fever-Induced Refractory Epileptic Encephalopathy

Mazzuca¹,

Jambaqué²,

Hertz-Pannier³

et al. 2010

J Nucl Med

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Fever-induced refractory epileptic encephalopathy in schoolage children (FIRES) is a recently described epileptic entity whose etiology remains unknown. Brain abnormalities shown by MRI are usually limited to mesial-temporal structures and do not account for the catastrophic neuropsychologic findings. Methods: We conducted FIRES studies in 8 patients, aged 6-13 y, using 18 F-FDG PET to disclose eventual neocortical dysfunction. Voxel-based analyses of cerebral glucose metabolism were performed using statistical parametric mapping and an age-matched control group. Results: Group analysis revealed a widespread interictal hypometabolic network including the temporoparietal and orbitofrontal cortices bilaterally. The individual analyses in patients identified hypometabolic areas corresponding to the predominant electroencephalograph foci and neuropsychologic deficits involving language, behavior, and memory. Conclusion: Despite clinical heterogeneity, 18 F-FDG PET reveals a common network dysfunction in patients with sequelae due to fever-induced refractory epileptic encephalopathy.

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supporting

confidence: 60%

¹⁸F-FDG PET Reveals Frontotemporal Dysfunction in Children with Fever-Induced Refractory Epileptic Encephalopathy

Mazzuca¹,

Jambaqué²,

Hertz-Pannier³

et al. 2010

J Nucl Med

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“…[1][2][3][4][5][6][7] The condition is characterized by a refractory SE and followed by drug-resistant epilepsy, with often severe neuropsychiatric sequelae or death. These entities have been identified by different acronyms, but febrile infection-related epilepsy syndrome (FIRES) is the one that best underscores the main features of the disorder.…”

mentioning

confidence: 99%

Claustrum damage and refractory status epilepticus following febrile illness

et al. 2015

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Objective: 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.Methods: A retrospective study (2010)(2011)(2012)(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.Results: 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.Conclusions: 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. Neurology ® 2015;85:1224-1232 GLOSSARY ADC 5 apparent diffusion coefficient; AED 5 antiepileptic drug; FIRES 5 febrile infection-related epilepsy syndrome; ICU 5 intensive care unit; IVIg 5 IV immunoglobulin; NORSE 5 new-onset refractory status epilepticus; PEX 5 plasma exchange; SE 5 status epilepticus.In the last 2 decades, several authors described a series of syndromes characterized by the development of a difficult to treat status epilepticus (SE) in previously healthy children after a febrile illness.1-7 The condition is characterized by a refractory SE and followed by drug-resistant epilepsy, with often severe neuropsychiatric sequelae or death. These entities have been identified by different acronyms, but febrile infection-related epilepsy syndrome (FIRES) is the one that best underscores the main features of the disorder.8 Cases with a similar clinical picture have been described also in adults and in these case series the most frequently used definition is new-onset refractory SE (NORSE).9-13 Recently, it was pointed out that different terms probably have been used to describe the same condition.14,15 However, there is no consensus among investigators. Adult cases are more heterogeneous, some with clear similarities with FIRES cases (with only the age at onset as the main difference), other...

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“…[1][2][3] At presentation, SE lasts for weeks and may result in death 4 or evolve into treatment-resistant epilepsy with associated severe intellectual disabilities. 1 The underlying pathophysiology may be immune-mediated, but adjunctive therapies to anticonvulsant medications, including immunotherapy with intravenous immunoglobulin, steroids, and plasmapheresis, have been used with limited success.…”

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confidence: 99%

Cognitive Outcomes in Febrile Infection-Related Epilepsy Syndrome Treated With the Ketogenic Diet

et al. 2014

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Febrile infection-related epilepsy syndrome (FIRES) is a newly recognized epileptic encephalopathy in which previously healthy school-aged children present with prolonged treatment-resistant status epilepticus (SE). Survivors are typically left with pharmacoresistant epilepsy and severe cognitive impairment. Various treatment regimens have been reported, all with limited success. The ketogenic diet (KD) is an alternative treatment of epilepsy and may be an appropriate choice for children with refractory SE. We report 2 previously healthy children who presented with FIRES and were placed on the KD during the acute phase of their illness. Both children experienced resolution of SE and were maintained on the KD, along with other anticonvulsant medications, for several months. Both were able to return to school, with some academic accommodations. These cases highlight the potential value of the KD as a preferred treatment in FIRES, not only in the acute setting but also for long-term management. Early KD treatment might optimize both seizure control and cognitive outcome after FIRES.

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Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status

Abstract: The similarity of the clinical features suggests that this is a consistent clinical syndrome.

Cited by 89 publications

References 7 publications

¹⁸F-FDG PET Reveals Frontotemporal Dysfunction in Children with Fever-Induced Refractory Epileptic Encephalopathy

¹⁸F-FDG PET Reveals Frontotemporal Dysfunction in Children with Fever-Induced Refractory Epileptic Encephalopathy

Claustrum damage and refractory status epilepticus following febrile illness

Cognitive Outcomes in Febrile Infection-Related Epilepsy Syndrome Treated With the Ketogenic Diet

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Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status

Abstract: The similarity of the clinical features suggests that this is a consistent clinical syndrome.

Cited by 89 publications

References 7 publications

18F-FDG PET Reveals Frontotemporal Dysfunction in Children with Fever-Induced Refractory Epileptic Encephalopathy

18F-FDG PET Reveals Frontotemporal Dysfunction in Children with Fever-Induced Refractory Epileptic Encephalopathy

Claustrum damage and refractory status epilepticus following febrile illness

Cognitive Outcomes in Febrile Infection-Related Epilepsy Syndrome Treated With the Ketogenic Diet

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¹⁸F-FDG PET Reveals Frontotemporal Dysfunction in Children with Fever-Induced Refractory Epileptic Encephalopathy

¹⁸F-FDG PET Reveals Frontotemporal Dysfunction in Children with Fever-Induced Refractory Epileptic Encephalopathy