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Multifocal multilobar focal cortical dysplasia type IIa in Febrile Infection Related Epilepsy Syndrome (FIRES). | LitMetric

Multifocal multilobar focal cortical dysplasia type IIa in Febrile Infection Related Epilepsy Syndrome (FIRES).

Clin Neurol Neurosurg

Department of Neurology, Rhode Island Hospital/Warren Alpert Medical School of Brown University, Providence, RI, United States; Division of Child Neurology, Department of Pediatrics, Hasbro Children's Hospital, Providence, RI, United States.

Published: June 2022

AI Article Synopsis

  • A seven-year-old girl with type I diabetes experienced altered mental status and convulsions following a febrile illness, leading to a diagnosis related to FIRES ( febrile infection-related epilepsy syndrome).
  • Laboratory tests were mostly normal, but EEG indicated seizures from the right occipital lobe, prompting a decision to withdraw life support.
  • Post-mortem analysis revealed brain abnormalities consistent with focal cortical dysplasia type IIa, suggesting a potential link to the seizures and recommending early consideration of this condition in similar FIRES cases.

Article Abstract

A seven-year-old girl with history of type I diabetes and no history of seizures presented for altered mental status with convulsions nearly one week after a febrile illness. Serum and laboratory studies were normal with EEG showing biparietal fast activity and seizures originating from right occipital lobe consistent with FIRES. A collaborative decision was ultimately made to withdraw care. Post-mortem whole brain histopathological examination revealed diffuse abnormalities in multiple areas including both parietal lobes and the right parieto-occipital junction consistent with focal cortical dysplasia type IIa. We believe this to be the first report that describes focal cortical dysplasia type IIa co-localizing with epileptogenic areas on EEG in a case of FIRES, and recommend that focal cortical dysplasia be considered as an etiology early in the course of FIRES.

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Source
http://dx.doi.org/10.1016/j.clineuro.2022.107247DOI Listing

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