Abstract Details

Title Pediatric Case of Febrile Illness-Related Epilepsy Syndrome with Subsequent Diagnosis of Central Nervous System Vasculitis

Topic Autoimmune Neurology

Presentation(s) P6 - Poster Session 6 (11:45 AM-12:45 PM)

Poster/Presentation
Number 8-013

Objective Discuss a pediatric patient presenting with FIRES, later found to have CNS vasculitis.

Background Febrile Infection-Related Epilepsy Syndrome (FIRES) is a subset of New-Onset Refractory Status Epilepticus (NORSE) in which the individual has a febrile illness 24 hours to 2 weeks prior to onset of refractory status epilepticus. While the acute management of FIRES involves primarily seizure control, there is increasing literature suggesting immune dysfunction may play a role.

Design/Methods N/a

Results

A 17-year-old previously healthy female developed generalized tonic-clonic seizures refractory to first line treatments. She was quickly escalated to pentobarbital. MRI showed increased T2 signal and restricted diffusion in the left hippocampus. CSF studies revealed mild pleocytosis and elevated protein. High dose steroids and anakinra were initiated on day 2 of hospitalization (DOH 2) and plasma exchange on DOH 3. Cytokine panel revealed elevated IL-6 in CSF and tocilizumab was administered on DOH 15.

She was weaned off Phenobarbital on DOH 31 after treatment with seven anti-seizure medications, ketogenic diet, and VNS placement on DOH 26. She was finally discharged on DOH 114 able to abulate with assistance and had somme cognitive deficits. Steroids and anakinra were discontinued 1-2 months after discharge.

One month later, she presented with lethargy. CSF indices were similar to prior and IgG index was elevated. EEG showed no electrographic seizure activity. MRI brain revealed foci of nodular and linear leptomeningeal enhancement within both cerebral hemispheres. Brain biopsy demonstrated vasculitis. She was started on cyclophosphamide and began to improve clinically.

Conclusions The pathoetiology of FIRES and strategies for its management are not well understood. We present a young woman with FIRES who was treated with a combination of anti-seizure and immune therapies. With cessation of immunotherapy, she had worsening mental status without return of seizures, and was found to have CNS vasculitis which impacted her long-term management.

Authors/Disclosures
Shivani Misra, MD, MPH (UT Southwestern and Children's Health Center for Autism Care) PRESENTER	Dr. Misra has nothing to disclose.
Cynthia X. Wang, MD (Children's Dallas Neurology)	Dr. Wang has nothing to disclose.

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