New Case Report Shows Utility of Deep Brain Stimulation to Treat Febrile Infection-Related Epilepsy Syndrome (FIRES)

A team from the UPMC Children’s Hospital of Pittsburgh Divisions of Child Neurology and Pediatric Neurosurgery recently published a case report detailing one of the first times deep brain stimulation was used to treat a case of febrile infection-related epilepsy syndrome (FIRES) in an 11-year-old female patient. The study was published in December in the journal Epilepsia Open.

The report was authored by Taylor J. Abel, MD, chief of Pediatric Epilepsy Surgery and surgical director of the UPMC Children’s Epilepsy Center; Luis D. Fernandez MD, from the Division of Child Neurology and UPMC Children’s Epilepsy Center; William P. Welch, MD, director of Epilepsy Neurostimulation at UPMC Children’s; and Jasmine L. Hecht, BS, an MD/PhD student at the University of Pittsburgh School of Medicine.

FIRES is a rare, yet challenging condition to treat in pediatric and adult patients with high rates of both mortality and morbidity. FIRES most often presents as a complication in individuals with no prior history of epilepsy who experience a febrile illness. Status epilepticus of a refractory and extended duration are hallmarks of the condition manifesting within a short period from the onset of the febrile illness.

Many aspects of the disorder remain to be fully explained. Causative pathways have been proposed, however concrete understandings of its origins and mechanisms of development remain elusive.

In the case diagnosed and managed by the UPMC Children’s team, an 11-year-old girl who was otherwise healthy prior to the episode was admitted to the hospital with symptoms including fever, headache, altered mental status, and non-responsiveness. Seizure activity was first detected after the patient was admitted to the hospital and continued to increase in frequency and duration.

Extensive testing and evaluations ruled out viral or bacterial infections or autoimmune conditions as the cause of the underlying illness, and genetic testing was unremarkable.

Ultimately, seizure activity and areas of onset were identified using EEG and stereo EEG. Surgical interventions were discussed as medications failed to mitigate the patient's seizures. The first intervention using vagus nerve stimulation produced no appreciable changes or reduction in seizure activity.

Subsequently, deep brain stimulation applied to the centromedian nucleus of the thalamus in an attempt to control or eradicate the patient’s seizures.

Learn more about the diagnostic and clinical challenges presented by this case, patient follow-up and outcomes, and how the UPMC Children’s team ultimately was able to successfully use DBS to combat an emergent and challenging presentation of FIRES in a pediatric patient.

Reference

Hecht JL, Fernandez LD, Welch WP, Abel TJ. Deep Brain Stimulation of the Centromedian Thalamic Nucleus for the Treatment of FIRES. Epilepsia Open. 2021 Dec 4. Doi: 10.1002/epi4.12568. Online ahead of print.