LAUSR

Objective: To describe the role of immunotherapy in refractory status epilepticus (SE) due to atypical Rasmussen encephalitis. Background: Hemispherectomy is the most effective treatment option for medically refractory epilepsy due to Rasmussen’s encephalitis (RE) and is typically performed when the patients are hemiplegic. In atypical patients without dense hemiparesis, hemispherectomy is only rarely considered when the seizure burden is extreme. The role of immunotherapy in SE due to RE is limited. Method: A 19-year-old male with refractory epilepsy was transferred to our hospital for treatment of SE. His epilepsy started at 11 years of age and has remained refractory since the onset. MRI findings and brain biopsy at age 14 supported the diagnosis of RE. Despite mild progressive right hemisphere atrophy over 8 years, he had intact motor, cognitive and visual function. At baseline, he had multiple seizures weekly despite medications. He developed SE which was managed with propofol and pentobarbital burst suppression. As seizures recurred on weaning of medications, he was transferred to our center. Seizures occurred at a rate of 10-15 per hour, each lasting for 30-180 seconds despite 7 anti-epileptic medications. He also developed moderate left hemiparesis after the recent increase in seizures. In view of resistant SE, hemispherectomy was considered. Further aggressive immunotherapy was attempted prior to proceeding with surgery. Patient was treated initially with high dose intravenous methylprednisolone and immunoglobulin, and Tacrolimus was added. Results: Seizures markedly decreased after initiation of combined immunotherapy. Both clinical and EEG seizures stopped 8 days after Tacrolimus therapy. At discharge after 30 days of hospitalization and rehabilitation, he had only subtle left hand weakness and discharged with 5 anti-epileptic medications. Conclusion: Aggressive immunotherapy with methylprednisolone and immunoglobulin, and Tacrolimus may be tried for patients with refractory SE due to RE. Disclosure: Dr. Cho has nothing to disclose. Dr. Zeft has nothing to disclose. Dr. Pestana Knight has nothing to disclose. Dr. Kotagal has received research support from UCB Pharma, Ortho-McNeil Pharmaceuticals, Inc., and Ovation Pharmaceuticals, Inc. Dr. Wyllie has nothing to disclose. Dr. Naduvil Valappil has nothing to disclose.