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The aim of this study was to evaluate the efficacy and safety of protein A immunoadsorption (IA) for anti- N -methyl- d -aspartate receptor (NMDAR) encephalitis resistant to intravenous methylprednisolone (IVMP) and intravenous immunoglobulin (IVIG). We prospectively evaluated patients with refractory anti-NMDAR encephalitis, treated with protein A IA. Demographic data, clinical characteristics, modified Rankin Score (mRS), and anti-NMDAR antibodies were documented before and after IA and at follow-up. Clinical improvement was defined as a decrease of mRS ≥1. Adverse events were recorded throughout the study. Ten patients with mRS ≥3 were enrolled and treated with protein A IA; treatment was performed for an average of 5.2 times per patient. Among the nine patients with positive serum anti-NMDAR, the titer decreased in seven patients, of which two became negative. The cerebrospinal fluid (CSF) anti-NMDAR titer decreased in all patients, and one became negative. Anti-NMDAR levels were tested in two patients at follow-up and found to have declined continuously. All patients exhibited clinical improvement with a mRS decline ≥1 after IA treatment (median mRS: 5.0 [range, 3.0–5.0] vs. 4.0 [range, 2.0–4.0], p = 0.014), and the median mRS decreased to 1.0 (range, 0–3.0) at follow-up. After IA, all patients exhibited accelerated recovery. No adverse events were observed during IA treatment. Protein A IA may be effective for treating IVMP/IVIG-resistant anti-NMDAR encephalitis and well tolerated. It is necessary to initiate larger-scale prospective controlled studies to validate the efficacy and safety of protein A IA in anti-NMDAR encephalitis. • Efficacy and safety of protein A immunoadsorption (IA) was evaluated in this study. • Patients with refractory anti- N -methyl- d -aspartate receptor encephalitis were enrolled. • No adverse events were observed during IA treatment. • Protein A IA was effective for treating IVMP/IVIG-resistant anti-NMDAR encephalitis. [ABSTRACT FROM AUTHOR]

Anti-N-methyl- d -aspartate receptor (anti-NMDAR) encephalitis is a treatable but often misdiagnosed autoimmune encephalitis. Diagnosis depends on NMDAR antibody testing, which may not be readily available. Alternatively, the electroencephalogram (EEG) extreme delta brush pattern may provide a valuable immediate indicator for the diagnosis of anti-NMDAR encephalitis. A 32-year-old female (case 1) presented with fever, headache, behavioral changes, confusion, intractable seizures, central hypoventilation, dysautonomia, facial and limb dyskinesias, and comorbid ovarian teratoma. Cerebral spinal fluid (CSF) testing revealed mild lymphocytic pleocytosis while brain MRI results were normal. A 45-year-old male (case 2) presented with major behavioral changes and rare seizures. Results of routine CSF testing and brain MRI scanning were unremarkable. In both cases, EEG initially revealed the extreme delta brush (EDB) pattern of beta bursting on the peaks and/or the troughs of delta waves, which led to subsequent NMDAR antibody testing and the confirmative diagnosis. Thus, EDB may be a readily accessible sign for suspected anti-NMDAR encephalitis. [ABSTRACT FROM AUTHOR]

The objective of this study was to determine the incidence of anti-NMDAR encephalitis in patients in whom a teratoma was removed. As far as we know, there has been no report on the incidence of anti-NMDAR encephalitis in patients in whom a teratoma was removed. This study was a single-institutional observational study. We enrolled patients who were diagnosed with teratoma in the Department of Pathology, Sapporo City General Hospital during a nine-year period between January 2008 and December 2016. In Sapporo City General Hospital, 6 NMDAR encephalitis cases were detected during the 9-year period. In the same 9-year period, a pathological diagnosis of teratoma was made in 343 cases in the hospital. Anti-NMDAR encephalitis patients with a teratoma accounted for only 1.17% of all teratoma patients. Three of the 4 anti-NMDAR encephalitis patients with a teratoma underwent second removal of a teratoma, and no nervous tissue was detected pathologically. In this study, we determined the association between teratoma with anti-NMDAR encephalitis and teratoma without anti-NMDAR encephalitis in cases in a single institution. As far as we know, this report is the first report on the incidence of anti-NMDAR encephalitis in teratoma patients. • We determined the association between teratoma with anti-NMDAR encephalitis and teratoma without anti-NMDAR encephalitis. • In this study, anti-NMDAR encephalitis patients with a teratoma accounted for only 1.17% of all teratoma patients. • In this study, 3 of the 4 anti-NMDAR encephalitis patients with a teratoma underwent second removal of a teratoma. • Our results suggested that there is an association between anti-NMDAR encephalitis and teratoma relapse. [ABSTRACT FROM AUTHOR]