Abstracts

Rationale: Febrile infection-related epilepsy syndrome (FIRES) is a rare and devastating encephalopathic condition initially reported in the pediatric population. Recently, FIRES has also been observed in adults, characterized by the acute onset of refractory seizures or status epilepticus following a febrile illness. Adult FIRES requires prompt and aggressive treatment to manage refractory seizures and minimize long-term neurological damage. There is limited but growing evidence from case reports suggesting that deep brain stimulation (DBS) can be effective in reducing seizure frequency and improving the quality of life in pediatric FIRES patients. Here, we report two adult patients with FIRES who received centromedian thalamic nucleus (CM) DBS implantation and demonstrated notable improvement in seizure management.


Methods: Clinical, laboratory, brain imaging, and EEG data, along with DBS program data and treatment outcomes, were analyzed.


Results: The study included two patients: a 28-year-old female and a 26-year-old male. Both patients presented with a febrile illness 1-3 days prior to experiencing confusion and new-onset seizures. Cerebrospinal fluid (CSF) profiles showed pleocytosis and elevated protein levels. The seizures began as focal aware seizures and focal impaired awareness seizures, evolving into generalized tonic-clonic seizures, and became refractory to multiple anti-seizure medications. Given the concern for a seronegative autoimmune etiology, both patients received immunotherapy without impact on seizure control. Both scalp and invasive stereo-EEG (sEEG) indicated that seizures were multifocal with a widespread network. Bilateral CM nucleus DBS implantations were performed, and the patients were followed for six months. The female patient became free of convulsions and focal impaired awareness seizures, with a 60% reduction in focal aware seizures. The male patient experienced over a 50% reduction in seizures and maintained a two-month seizure-free interval.


Conclusions: Our report highlights the application of CM-DBS in adult FIRES. The CM region is considered to have diffuse network connections, particularly involving both temporal and frontal regions. CM-DBS can be a viable option for symptomatic treatment, significantly reducing generalized debilitating seizures, as observed in pediatric FIRES patients.


Funding: No