Ketogenic Diet for a Young Adult Patient With Chronic-Phase Febrile Infection-Related Epilepsy Syndrome.

Koji Obara,Tomoko Ono

doi:10.7759/cureus.22099

Abstract

Febrile infection-related epilepsy syndrome (FIRES) is a rare disease, whereby refractory status epilepticus (a severe epileptic syndrome) occurs in previously healthy individuals following a febrile illness. Here, we report a patient with FIRES who received ketogenic diet (KD) therapy initiated in the chronic phase. A 21-year-old man presented with status epilepticus, following fever and headache. In the acute phase, his seizures were refractory to conventional antiepileptic drugs and were suppressed only by intravenous anesthetics. In the chronic phase, he showed frequent seizures with concurrent severe cognitive decline. Twenty-seven months after onset, the patient was started on KD. Consequently, his seizure frequency rapidly reduced while his cognitive function slowly improved, albeit incompletely. Recently, KD has been shown to both reduce seizures and improve cognitive prognoses in children with FIRES. Although early KD may help in both seizure control and cognitive prognosis, it is likely that KD can be applied to adult patients with chronic FIRES.

Highlights

Febrile infection-related epilepsy syndrome (FIRES) is an epileptic encephalopathy in which previously healthy individuals present with prolonged status epileptics (SE) following a febrile illness [1,2,3]
May help in both seizure control and cognitive prognosis, it is likely that ketogenic diet (KD) can be applied to adult patients with chronic FIRES
Seizure frequency gradually decreases in the chronic phase of FIRES, in many cases, these last throughout the patient’s lifetime and is accompanied by cognitive decline [2,3]

Summary

Introduction

Febrile infection-related epilepsy syndrome (FIRES) is an epileptic encephalopathy in which previously healthy individuals present with prolonged status epileptics (SE) following a febrile illness [1,2,3]. He was started on glucocorticoid pulse therapy, plasma exchange (PE), and high-dose intravenous immunoglobulin (IVIG) sequentially. Twenty-seven months after onset, brain magnetic resonance imaging (MRI) showed atrophy of the bilateral frontal lobes and mesial temporal lobes and dilatation of the lateral ventricles (Figure 2A). Twenty-seven (27) months after the onset: (A) MRI shows atrophy of the bilateral frontal lobes and mesial temporal lobes and dilatation of the lateral ventricles. IMP-SPECT showed improvement of hypoperfusion in the right-dominant frontal and parieto-temporal cortices (Figure 2D). Processing speed and verbal memory did not improve, and he required significant support in most activities of daily living

Discussion

Conclusions

Disclosures