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 workup 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.