Document Type
Clinical Case Reports
Date of Poster
2021
Date of Submission
June 2021
Abstract
Objective: Discuss a case of new onset refractory status epilepticus (NORSE) in an otherwise healthy adult that had a positive outcome and suggests considering immunomodulators as first line treatment.
Background: NORSE is diagnosed when refractory status patients have no known history of seizures and an unremarkable workup. Other than antiepileptics and anesthetic coma, there are currently no established guidelines for management. Typically, IV steroids, plasma exchange, and IVIG are first line treatments. When these fail, second line immunomodulators are considered. Deciding between these agents is often arbitrary, and no particular agent has demonstrated consistently positive results. Prognosis is poor with a 27% mortality rate. Survivors spend an average of 15 days in the ICU and most sustain cognitive impairment and drug-resistant epilepsy.
Methods/Design: A 35-year-old woman presented after a two-week febrile illness with new onset seizures that rapidly progressed to NORSE. She was treated with Levetiracetam and Fosphenytoin, followed by Lacosamide, but quickly required intubation and a Midazolam drip. EEG continued to show bilateral independent temporal seizures. She continued to seize through a pentobarbital coma. She underwent MRI, autoimmune and infectious workup, and extensive CSF studies that were all unremarkable. She did not respond to antibiotics, IV methylprednisolone, or IVIG. After nearly 3 weeks of hospitalization, Rituximab was initiated.
Results: Within 2 weeks of starting Rituximab, the patient’s EEG normalized and her mental status returned to baseline. She was continued on polytherapy and discharged to rehabilitation. She remained seizure free until efforts were made to wean her off phenobarbital, at which time she had a single breakthrough convulsion with rapid return to baseline. She continues to live with her husband and perform her ADLs independently.
Conclusions: This case suggests that early initiation of Rituximab or other immunomodulatory therapy in NORSE may shorten hospital stays, improve cognitive outcomes, and boost long-term response to AEDs.
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VCU School of Medicine GME Resident and Fellow Research Day Posters