2019 年 1 巻 1 号 p. 11-17
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disease with multiple neurologic symptoms with or without a tumor, including ovarian teratoma. In the present study, a national inpatient database in Japan was used to investigate the characteristics, treatment, and outcomes of hospitalized patients with anti-NMDAR encephalitis who received initial treatment.
Using the Diagnosis Procedure Combination database, we identified all patients who were diagnosed with anti-NMDAR encephalitis and received initial first-line treatments (methylprednisolone, intravenous immunoglobulin, plasma exchange, and tumor removal) and second-line treatments (cyclophosphamide and rituximab) from July 2010 to March 2017. We excluded patients who received no immunotherapy or surgical treatment and those for whom data were missing. We investigated the characteristics, treatment, and outcomes of eligible patients.
In total, 163 eligible patients were identified. Of these patients, 116 (71%) were female and 44 (28%) were ≤19 years of age. Among the female patients, 44 (38%) had a tumor. Thirty-nine patients (24%) were admitted to the intensive care unit during their hospitalization. Methylprednisolone, intravenous immunoglobulin, and plasma exchange were used as initial therapy in 82%, 56%, and 34% of patients, respectively. Second-line treatments were rarely used. Most patients were alert or had a slight disturbance of consciousness upon discharge. The proportion of patients discharged to home was 55%.
The results of this Japanese study on anti-NMDAR encephalitis suggest that patients’ characteristics and outcomes including the male-to-female ratio, proportion of associated tumors, treatment options, and consciousness disturbance may differ from those in previous reports from other countries.