Treating refractory post-herpetic anti-N-methyl-Daspartate receptor encephalitis with rituximab

Melzer N., Strippel C., Mönig C., Golombeck K.S., Dik A., Bönte K., Kovac S., Schulte-Mecklenbeck A., Wiendl H., Meuth S.G., Johnen A., Gross C.C.

Abstract

Herpes simplex virus-1 has been identified as the trigger factor in certain cases of NMDA-receptor autoimmune encephalitis.We report on a 67-year-old female patient, who was severely affected by post-herpetic NMDA-receptor autoimmune encephalitis. Her symptoms did not improve under methylprednisolone pulse therapy and plasma exchange under acyclovir prophylaxis. She received protein A immunoadsorption and a long-term immunosuppression with rituximab. Under treatment, activated T-cells as well as B- and plasma cells decreased in peripheral blood and cerebrospinal fluid, and anti-NMDA-R IgG titers in serum and cerebrospinal fluid declined with near complete cessation of intrathecal autoantibody synthesis. The patient regained near complete independence and profoundly improved on formal neuropsychological assessment. Despite reduction of antiviral defense through of lowered activated T cells and concomitantly decreasing HSVspecific IgG antibodies, no evidence of viral reactivation was detected.