Deep brain stimulation of the centromedian thalamic nucleus for the treatment of FIRES.

Febrile infection-related epilepsy syndrome (FIRES) is a rare, life-threatening complication of febrile illness in previously healthy individuals followed by super-refractory status epilepticus. Deep brain stimulation (DBS) has been demonstrated to be a promising therapy for the treatment of intractable epilepsy. Here we present a pediatric patient with FIRES whose seizures were mitigated by acute DBS of the bilateral centromedian thalamic nucleus (CMTN). This is a previously healthy 11-year-old female who presented emergently with altered mental status, fever, and malaise after 1-week of lethargy, anorexia, fever, and abdominal pain. The patient began having seizures shortly after admission. After thorough work-up for encephalitis and other potential etiologies, this patient was diagnosed with FIRES due to super-refractory status epilepticus. Status epilepticus persisted despite pharmacologic management, immunotherapy, and vagus nerve stimulation. DBS of the bilateral CMTN (CM-DBS) was pursued after 56 days of hospitalization, and she demonstrated considerable improvement in baseline mental status 30-days after DBS insertion. This report highlights application of CM-DBS for super-refractory status epilepticus in FIRES. This region is a diffusely connected brain region and has been shown to modulate neural networks contributing to seizure propagation and consciousness, therefore neurostimulation is a potential therapeutic intervention for patients with super-refractory status epilepticus.