Abstracts

Rationale: Febrile infection-related epilepsy syndrome (FIRES) is characterized by a severe epileptic encephalopathy with refractory status epilepticus associated with fever. The etiology and patho-physiology are still an enigma. However, immunologic and inflammatory process involving CNS has been hypothesized but the treatment guideline have yet to be developed. We reviewed and analyzed our patients with FIRES with an attempt to identify the treatment protocol associated with the good outcome.Methods: This is a retrospective case series of 8 patients of FIRES seen over a period of 15 years from 2000 to 2015.Results: There were total of 8 patients in our database that met the criteria for the diagnosis of FIRES. Out of 8 patients, 5 (60%) were male with mean age at onset 9.8 ± 4.4 years (range 6 years - 18 years 11 mo). Five (60%) patients presented with focal status epilepticus and 3 (40%) presented with generalized status epilepticus. CSF showed pleocytosis in 4 patients but in others it was normal. One patient was positive for enterovirus in CSF. One patient was positive for Voltage Gated Potassium Channel (VGKC) antibody in serum but CSF was not checked, NMDA antibody was negative in this patient. NMDA and other antibodies were negative both in serum and CSF in 4 patients and in 3 other patients it was not checked. One patient had low IgA and IgE level in the serum. MRI brain were normal in 4 patients (50%). All patients were treated initially with multiple anti-epileptic medications without any response, 6 were also treated with either phenobarb or pentobarb coma or both. Patient who was positive for enterovirus was treated with IVIG and Preconaril (experimental medication) with some temporary response. Patient with VGKC antibody+ did well after IVIG therapy. 4 patients were treated with high dose Methylprednisolone (MP), IVIG and PLEX and 2 with only MP and IVIG with only temporary response. In view of no good response with above therapies, 5 patients were started on Ketogenic diet, out of which 2 patients had good seizure control once ketosis was achieved but in other 3 patients persistent ketosis could not be achieved. Four patients died during acute phase, one patient did well with better seizure control but died after 6 years because of acute respiratory issue. Two patients develop refractory epilepsy, one requiring VNS and monthly IVIG therapy and another one epilepsy surgery but none of the patients who survived were completely at baseline.Conclusions: FIRES is a devastating epileptic encephalopathy characterized by refractory focal or generalized status epilepticus unresponsive to traditional anti-epileptic medications. In our cohort of patients, immunotherapy with MP, IVIG and PLEX were only temporarily effective except in autoimmune antibody positive (VGKC Ab +) case. The patients on ketogenic diet had relatively good response although it was started late. Therefore, we hypothesized that if ketogenic diet could be initiated early, then it may help with acute phase as well as may change the long-term outcome of this condition.