Combined Therapy for Anti-N-methyl D-aspartate Receptor Encephalitis
نویسندگان
1 Department of Child Health, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
2 National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, Shizuoka, Japan.
3 Department of Child Health, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
4 Department of Pediatrics, University of Tsukuba Hospital, Ibaraki, Japan, AND Department of Emergency and Critical Care Medicine, University of Tsukuba Hospital, Ibaraki, Japan.
5 Department of Child Health, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
6 Department of Child Health, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
doi
10.22038/ijp.2017.24465.2087چکیده
Background Anti-N-methyl- d-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune neurological disorder that usually occurs as a paraneoplastic syndrome and is particularly associated with ovarian teratoma. Standard therapy for severe cases is not established and the prognosis in patients who do not respond to first-line treatment is poor. Case Report An 11-year-old boy complained psychiatric symptoms and rapidly lost consciousness. CT scan revealed mediastinal teratoma and serum/spinal fluid was positive for anti-NMDAR antibody. He kept comatose and his brain stem function was profoundly disturbed. His symptoms were refractory to first-line therapy, which involved tumor resection, methylprednisolone (mPSL) pulse, Intravenous immunoglobulin (IVIG), and plasma exchange. We administered a combination therapy of rituximab and cyclophosphamide as second-line therapy and achieved complete recovery without adverse effects related to treatment. Conclusion We consider early intensive treatment with a combination of rituximab and cyclophosphamide to be a safe and effective option for severe cases of anti-NMDAR encephalitis.
