Long-term and Strong Immunotherapy to Treat Anti-N-Methyl- D-Aspartate Receptor Encephalitis with Refractory Status Epilepticus.

BACKGROUND: Anti-N-Methyl-D-Aspartate receptor (anti-NMDAR) encephalitis is responsive to immunotherapy and removal of tumor, but there is no consensus in the treatment of severe anti- NMDAR encephalitis with prolonged refractory status epilepticus (SE).

CASE REPORT: A 17-year-old girl presented as acute psychosis, refractory seizures, hyperkinesia, autonomic instability, and soon progressed to a dissociative state of coma. Anti-NMDAR antibodies were positive in serum and CSF. When most of the symptoms were alleviated after repeated one-byone immunotherapy during the first four months, the patient still remained in a coma with frequent seizures despite treatment with five different anti-epileptic drugs. We then proposed a three-combined immunotherapy of high-dose steroid, intravenous immunoglobulin and rituximab. After such treatment, her SE was soon resolved and this patient regained her consciousness before resection of ovarian teratoma. Although she had suffered from a prolonged period of refractory SE and coma for six months, she still had good recovery from encephalitis after a long-term immunotherapy.

CONCLUSION: A strong and long-term course of immunotherapy is necessary in treating severe refractory anti-NMDAR encephalitis. If traditional step-by-step way of immunotherapy is not strong enough to rapidly cure severe anti-NMDAR encephalitis, combined immunosuppressive agents can be considered to shorten the clinical course.