MRI evolution in FIRES

Marianna S. Rivas-Coppola, , Namrata Shah, Asim F. Choudhri, Robin Morgan, James W. Wheless.  Chronological evolution of Magnetic Resonance Imaging (MRI) Findings in children with Febrile-Infection Related Epilepsy Syndrome (FIRES).  Pediatric Neurology.  Published Online: September 22, 2015.

Abstract

Objective

To describe and analyze the chronological evolution of the radiological findings in seven children with Febrile – Infection Related Epilepsy Syndrome.

Methods

This is a retrospective study describing the radiological findings and evolution in seven children with Febrile-Infection Related Epilepsy Syndrome who presented from 2009 to 2013. The children all fit the defined clinical criteria for Febrile-Infection Related Epilepsy Syndrome; all had a history of normal psychomotor development who presented with acute onset catastrophic partial status epilepticus associated with a febrile illness or unspecific infectious process. The children were identified from the author’s weekly review of the pediatric inpatient service, and then the data was collected and analyzed retrospectively.

Results

Six males and one female age range 3 months to 9 years presented with status epilepticus preceded by a febrile illness. Extensive investigations for infectious, autoimmune and metabolic etiologies were negative. Multiple anti-epileptic medications were tried including drug-induced coma in all of them with poor response. Immunotherapy with intravenous steroids or IVIG (three patients had both) was tried in six of seven patients with a poor response. Ketogenic diet was initiated in four of seven patients with limited response. Serial magnetic resonance imaging (MRI) studies were done from initial presentation up to 18 months of follow up showing evolution from normal imaging to severe cerebral atrophy. Progressive cytotoxic edema involving mostly bilateral hippocampi and temporal lobes was appreciated in one to three weeks. At 1 month from seizure onset, mild to moderate cerebral atrophy and hippocampal sclerosis was appreciated that continued to progress over the next year. After 6- 12 months most of the patients showed moderate to severe cerebral atrophy and by one year cerebellar atrophy was also appreciated.

Conclusion

FIRES is a devastating epilepsy syndrome of childhood without a diagnostic biologic marker. The MRI findings appear to be progressive and typical, thus combined with the clinical course they can help confirm the diagnosis (Until a biologic marker is found.). This hopefully will allow multi-centered treatment protocols in the future.