Authors :
Presenting Author: Amerta Bai, MD, MS – University of Minnesota Medical Center
Felipe Rodridgues Marques Ferreira, MD – University of Minnesota Medical Center
Mengxuan Tang, MD – University of Minnesota Medical Center
Benjamin Miller, MD – University of Minnesota Medical Center
Oladi Bentho, MD – University of Minnesota Medical Center
Thomas Henry, MD – University of Minnesota Medical Center
Ilo Leppik, MD – University of Minnsota Medical Center
Mino Shams, MD – University of Minneosta Medical Center
Zhiyi Sha, MD, PhD – University of Minnesota Medical Center
Thaddeus Walczak, MD – University of Minnesota Medical Center
Zachary Sanger, MS – University of Minnesota Medical Center
Anant Naik, MD – University of Minnesota Medical Center
Sandipan Pati, MD – UT Health
Robert McGovern III, MD – University of Minnesota Medical Center
Sima Patel, MD – University Of Minnesota Medica Center
Rationale:
New-onset refractory Status Epilepticus (NORSE) presents as status epilepticus with no identifiable cause or prior epilepsy history. NORSE is associated with a 16-27% mortality rate and significant long-term neurological impairment. Traditional management strategies include anti-seizure medications (ASMs), anesthetics, and immunomodulation. When these approaches fail, neuromodulation, such as deep brain stimulation (DBS) targeting the anterior and centromedian nuclei of the thalamus (ANT & CM), has been explored. We report a case of cryptogenic NORSE with seizures localized to the bilateral posterior quadrants. This localization led us to target the bilateral pulvinar thalamus, a node with known interconnections to the bilateral parieto-occipital and temporal networks.
Methods:
We present the case of a 30-year-old male with autism and developmental delay, who was admitted for NORSE unresponsive to multiple ASMs, anesthetics, and a 48-day pentobarbital coma, as well as immunosuppressive therapy (see Table 1). An autoimmune etiology was suspected, and lab tests revealed oligoclonal bands in the cerebrospinal fluid (CSF), though no specific antibody was identified. Treatment included IVIG, Anakinra, methylprednisolone, rituximab, allopregnanolone, and intrathecal dexamethasone, but there was no clear response. As the patient continued to experience seizures and epileptiform activity localized to the bilateral posterior quadrants, we decided to proceed with sensing-enabled deep brain stimulation (Percept), targeting the pulvinar thalami using intraoperative MRI guidance.
Results:
We examined broadband pulvinar thalamic activity at a variety of different stimulation settings using a Bayesian optimization approach. Broadband activity was minimized with the following setting parameters. Deep pulvinar contacts were selected for continuous stimulation at 145 Hz frequency, 90 microsec pulse width, and amplitude was gradually increased to 5.9 mA over four days. Seizures stopped 8 days later after DBS implantation(see Fig 1). The patient remained on five anti-seizure medications, including clobazam, levetiracetam, lacosamide, perampanel, and phenobarbital. Clinically, the patient showed clinical improvement with some vocalization and voluntary movements.
Conclusions:
Managing NORSE requires a complex regimen of multiple ASMs, anesthetics, and immunomodulatory treatments. Cessation of status epilepticus was achieved through the innovative use of continuous stimulation targeting the pulvinar thalami with ASMs. This case underscores the critical importance of selecting thalamic neuromodulatory targets based on the specific localization of the patient’s epileptogenic network.
Funding: None