1
Department of Pediatrics, University of Tsukuba Hospital, Ibaraki, Japan, AND Department of Emergency and Critical Care Medicine, University of Tsukuba Hospital, Ibaraki, Japan.
2
Department of Child Health, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.
3
National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, Shizuoka, Japan.
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.
Kido T, Kobayashi C, Ohto T, Takahashi Y, Sumazaki R, Fukushima T. (2017). 'Combined Therapy for Anti-N-methyl D-aspartate Receptor Encephalitis', Journal of Pediatric Perspectives, 5(9), pp. 5687-5691. doi: 10.22038/ijp.2017.24465.2087
CHICAGO
T Kido, C Kobayashi, T Ohto, Y Takahashi, R Sumazaki and T Fukushima, "Combined Therapy for Anti-N-methyl D-aspartate Receptor Encephalitis," Journal of Pediatric Perspectives, 5 9 (2017): 5687-5691, doi: 10.22038/ijp.2017.24465.2087
VANCOUVER
Kido T, Kobayashi C, Ohto T, Takahashi Y, Sumazaki R, Fukushima T. Combined Therapy for Anti-N-methyl D-aspartate Receptor Encephalitis. JPP; J Ped Perspect. 2017;5(9):5687-5691. doi: 10.22038/ijp.2017.24465.2087
