Abstracts

Rationale: Febrile Infection Related Epilepsy Syndrome (FIRES) is characterized by severe epileptic encephalopathy with intractable status epilepticus in young children, occurring few days after an idiopathic febrile illness. These seizures are refractory to common Anti-Epileptic drugs. Anesthetics have to be used to induce burst suppression in these patients, however, the patients may continue to have frequent seizures. Ketogenic diet, Anakinra, and Cannabidiol have been individually reported, in few case studies, to be useful in refractory status epilepticus in the setting of FIRES. However, there has been no reported case, where they have been used concurrently to treat the refractory seizures. Methods: A 7-year-old boy with no significant past history presented to the emergency department with status epilepticus and was treated with Midazolam and emergently intubated. This episode was preceded by 4 days of febrile illness. Over the next few days the patient had multiple episodes of seizures daily and he was successively placed on multiple antiepileptic medications including Keppra, Phenytoin, Valproate, Lacosamide and Clobazam, without significant improvement.  His EEG was characterized by a background of delta-theta slowing with frequent electrographic seizures and periods of subclinical status epilepticus. Later, he was started on Pentobarbital for inducing burst suppression.Due to intractable status epilepticus, he was placed on Ketogenic Diet, followed by Anakinra and Cannabidiol, which resulted in near-resolution of the seizures.During the hospitalization, the patient remained afebrile. His CSF studies showed mild lymphocytic pleocytosis, but CSF viral studies were negative. His serum infectious and inflammatory tests, autoimmune antibody panel was negative. MRI of the brain was notable for diffuse cerebral volume loss, with mild ventriculomegaly and bilateral medial temporal sclerosis. The patient received high dose IV Solumedrol followed by Plasmapheresis and was later placed on weekly Intravenous Immunoglobulin. Results: Patient’s seizures improved significantly after being placed on Ketogenic diet, Anakinra, and Cannabidiol.  He was weaned off Midazolam and Pentobarbital and later extubated. He was tapered off Anakinra, Cannabidiol and was discharged on the Ketogenic diet, Clobazam, Keppra, and Phenobarbital. He was given an outpatient plan to taper off the Phenobarbital, Ketogenic diet and in the 3 months follow up, parents reported only 1 clinical seizure. He continued to have cognitive dysfunction with impaired concentration and hyperactivity. Conclusions: FIRES has been associated with devastating outcomes with high mortality rates and a third of patients result in a vegetative or minimally conscious state. This case report supports the use of Ketogenic diet, Anakinra and cannabidiol as options to treat refractory seizures and provide a better outcome. It also reinforces requirement of further studies for use of Ketogenic diet, Anakinra and Cannabidiol, individually or concurrently, for treatment of FIRES. Funding: None