Combined Therapy for Anti-N-methyl D-aspartate Receptor Encephalitis

Autor: Takahiro Kido, Chie Kobayashi, Tatsuyuki Ohto, Yukitoshi Takahashi, Ryo Sumazaki, Takashi Fukushima
Jazyk: angličtina
Rok vydání: 2017
Předmět:
Zdroj: International Journal of Pediatrics, Vol 5, Iss 9, Pp 5687-5691 (2017)
ISSN: 2345-5055
2345-5047
Popis: 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.
Databáze: OpenAIRE