Sampling the insulo-opercular region with invasive recordings is crucial given the importance of this region in epileptic networks and a variety of electroclinical presentations. However, implantation of the insulo-opercular region via stereoelectroencephalography (sEEG) is considered technically challenging given complex vascular and gray matter relationships in this region. We investigated the safety of insulo-opercular sEEG exploration in children and young adults using standard sEEG approaches: (1) orthogonal insulo-opercular (including the pseudo-orthogonal insulo-opercular approach) and (2) medial-lateral insular oblique approach.

Low-grade epilepsy-associated tumors (LEATs) are a common cause of drug-resistant epilepsy in children. Herein, we demonstrate the feasibility of using tumor tissue derived from stereoelectroencephalography (sEEG) electrodes upon removal to molecularly characterize tumors and aid in diagnosis. An 18-year-old male with focal epilepsy and MRI suggestive of a dysembryoplastic neuroepithelial tumor (DNET) in the left posterior temporal lobe underwent implantation of seven peri-tumoral sEEG electrodes for peri-operative language mapping and demarcation of the peri-tumoral ictal zone prior to DNET resection. Using electrodes that passed through tumor tissue, we show successful isolation of tumor DNA and subsequent analysis using standard methods for tumor classification by DNA, including Glioseq targeted sequencing and DNA methylation array analysis. This study provides preliminary evidence for the feasibility of molecular diagnosis of LEATs or other lesions using a minimally invasive method with microscopic tissue volumes. The implications of sEEG electrodes in tumor characterization are broad but would aid in diagnosis and subsequent targeted therapeutic strategies.

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.

Palliative epilepsy surgery via corpus callosotomy (CC) or vagus nerve stimulation (VNS) is commonly employed for drug-resistant seizures in Lennox-Gastaut Syndrome (LGS). VNS is less effective at reducing seizures but has fewer adverse events, CC is more effective for seizure control, particularly atonic seizures, but can be associated with serious adverse events, and yet their relative cost-effectiveness remains unknown.