Effect of Rituximab on Seizure Burden in Patients with Rasmussen Encephalitis: A Case Series

Objective: To describe the effect of rituximab on seizure burden in patients with Rasmussen encephalitis treated at Texas Children’s Hospital. Background: Rasmussen encephalitis (RE) is an inflammatory disease affecting one cerebral hemisphere, manifesting as focal, intractable seizures, cognitive decline, and unilateral neurologic deficits. While hemispherectomy is the gold standard of treatment, attempts to delay surgery may be made when the dominant hemisphere is affected or early on in the disease course when neurologic deficits are mild. 1 Rituximab is a monoclonal anti-CD20 antibody used in a variety of autoimmune disorders. Two prior case reports found that rituximab improved seizure burden for up to 18 months in RE patients. 2,3 Design/Methods: We retrospectively reviewed charts of patients diagnosed with RE who received rituximab from the years 2007 to 2017. Patients who did not have a brain biopsy consistent with RE were excluded. Progress notes by the treating epileptologist were reviewed to determine the patient’s seizure burden at baseline and at various time points after receiving rituximab, up to two years later. Results: Five patients met inclusion criteria. All had undergone hemispherectomy, lobectomy, or focal cortical resection, but continued to have focal seizures or epilepsia partialis continua (EPC) prior to rituximab infusions. All five patients had improvement in seizure burden initially after treatment, which was sustained at 1–2 years posttreatment in three patients. Patients 1 and 4 initially saw improvement in seizure frequency, but declined 1–2 years later. Patient 2 only had EPC, which had completely resolved one year later. Patient 3 had complete resolution of seizures sustained two years after rituximab. Patient 5 had seizure reduction without complete resolution sustained at one year. Conclusions: Rituximab may improve seizure burden in RE, although the effect may only be of short duration in some patients. Larger studies are needed to further characterize its utility. Disclosure: Dr. Herring has nothing to disclose. Dr. Messinger has nothing to disclose. Dr. Coorg has nothing to disclose.