Abstracts

Rationale: Refractory status epilepticus (RSE) is defined as electrographic/electroclinical seizures refractory to two first-line anti-seizure medications (ASMs) including one Benzodiazepine. Super refractory status epilepticus (SRSE) is defined as status epilepticus (SE) that occurs continuously or reoccurs within 24 hours despite three ASMs, including at least one intravenous anesthetic medication. In cases associated with a clear radiological lesion, emergency neurosurgery is considered as a “last resort” treatment. Surgical options range from lesionectomy to hemispherectomy with variable outcomes. Furthermore, there are no clear guidelines as to when to attempt emergency surgery. Surgical resection for RSE/SRSE is rare and there are only a handful of cases in the literature, primarily in the pediatric population. We describe a case of a 48-year-old male with lesional epilepsy who presented with SRSE. Cessation of status epilepticus was achieved after a dedicated intracranial EEG (iEEG) exploration, electrocorticography, and finally focal surgical resection of the epileptogenic zone.


Methods: 48-year-old male with focal epilepsy secondary to a FCD (presumed type 2) treated with Oxcarbazepine presented as a transfer for worsening seizure burden. On presentation, continuous EEG presented time-locked right lower face clonic activity associated with lateralized periodic discharges in the left central region, consistent with SE (Figure 1A). Despite first and second line treatments with levetiracetam and IV lorazepam, he continued to have electroclinical seizures. The patient was intubated, started on propofol and transferred to neuroscience ICU. Initial serological work up was unremarkable. MRI brain revealed a small focus of hyperintensity in the left frontal lobe concerning for focal cortical dysplasia (FCD) (Figure 2A). On day 2-5 of admission, the patient continued to seizure on Valproic acid and Lacosamide. Propofol was switched to a combination of Midazolam and Ketamine infusion to achieve burst suppression (BS). On day 6, a lumbar puncture was performed, and CSF studies were unrevealing. After 48 hours of BS, anesthetic agents were weaned which resulted in seizure reemergence. Clobazam, Topiramate and Clonazepam were added without improvement. Neurosurgery was consulted on day 10, for surgical resection given the focality on EEG and MRI. Patient was discussed in refractory epilepsy conference on day 12. On day 16, the patient underwent implantation of intracranial grid and five depth electrodes. Intracranial EEG revealed continuous electrographic SE arising from left posterior frontal lobe. (Figure 1B). On day 19, the patient underwent resection of FCD which finally led to a cessation of SE. Pathology revealed FCD type IIB.


Results:
FCD lesionectomy led to the cessation of SRSE. The patient was seizure-free during the remainder of his hospital course. He was eventually weaned off all ASMs except for Oxcarbazepine, at his home dose.




Conclusions: We describe a rare adult case of SRSE resolution after emergent surgical resection. This case highlights the use of urgent surgical planning with iEEG and electrocorticography for emergent FCD resection leading to resolution of SRSE.


Funding: None