Abstracts

Rationale: Fever-induced refractory epileptic encephalopathy in school-aged children (FIRES) is an acute encephalopathy in previously healthy school-aged children which is triggered by fever. It presents as seizures evolving to refractory status epilepticus (SE) and this is followed shortly after by severe cognitive impairment and intractable epilepsy. Deaths have been reported. There have been a few recent reports of the efficacy of the ketogenic diet in these children with cessation of their SE. Methods: We report a case of a developmentally normal 8 year old girl who presented following a brief history of fever and headache with a few generalised seizures which progressed quickly to an intractable SE. Investigations directed towards infection, metabolic causes, autoimmune disorders, and antibody-mediated encephalopathy did not reveal a cause. Initial electroencephalogram (EEG) showed almost continuous high voltage epileptic activity involving both hemispheres, particularly the left. Magnetic resonance imaging (MRI) was normal. Treatment of the SE in intensive care consisted of boluses and infusions of midazolam as well as sodium thiopental which induced burst suppression but clinical seizures returned when the infusions were weaned. The patient also received phenobarbital, phenytoin, levetiracetam, valproate and intravenous/oral steroids without any immediate effects seen. Ketogenic diet (KD), in the form of Ketocal at 1:4 ratio of combined protein and carbohydrate to fat, was introduced via nasogastric tube after 22 days of illness following which the patient was extubated.Results: Initially, the patient had intermittent seizures, some of which were prolonged. Blood ketone levels were low whilst remaining on steroids and during the early phase. Two weeks following institution of KD, the patient only had isolated brief seizures occurring once to three times a week. The patient progressed to an oral ketogenic diet without any adverse effects. The patient has made progress in motor and speech functions although her cognition is significantly impaired.Conclusions: This case report illustrates the preliminary evidence available regarding the potential effectiveness of the ketogenic diet in a patient with FIRES in an intensive care setting.