title: Heterogeneity of clinical features and corresponding antibodies in seven patients with anti-NMDA receptor encephalitis.
abstract: Anti-N-methyl D-aspartate (NMDA) receptor encephalitis is the most common type of encephalitis in the spectrum of autoimmune encephalitis defined by antibodies targeting neuronal surface antigens. In the present study, the clinical spectrum of this disease is presented using instructive cases in correlation with the anti-NMDA receptor antibody titers in the cerebrospinal fluid (CSF) and serum. A total of 7 female patients admitted to the hospital of Hannover Medical School (Hannover, Germany) between 2008 and 2014 were diagnosed with anti-NMDA receptor encephalitis. Among these patients, 3 cases were selected to illustrate the range of similar and distinct clinical features across the spectrum of the disease and to compare anti-NMDA antibody levels throughout the disease course. All patients received immunosuppressive treatment with methylprednisolone, intravenous immunoglobulin and/or plasmapheresis, followed in the majority of patients by second-line therapy with rituximab and cyclophosphamide. The disease course correlated with NMDA receptor antibody titers, and to a greater extent with the ratio between antibody titer and protein concentration. A favorable clinical outcome with a modified Rankin Scale (mRS) score of ≤1 was achieved in 4 patients, 1 patient had an mRS score of 2 after 3 months of observation only, whereas 2 patients remained severely impaired (mRS score 4). Early and aggressive immunosuppressive treatment appears to support a good clinical outcome; however, the clinical signs and symptoms differ distinctively and treatment decisions have to be made on an individual basis.
authors.affiliation: Department of Neurology, Hannover Medical School, Hannover D-30625, Germany.
authors.forename: Kurt-Wolfram
authors.initials: KW
authors.lastname: Sühs
authors.affiliation: Department of Neurology, Hannover Medical School, Hannover D-30625, Germany.
authors.forename: Florian
authors.initials: F
authors.lastname: Wegner
authors.affiliation: Department of Neurology, Hannover Medical School, Hannover D-30625, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Skripuletz
authors.affiliation: Department of Neurology, Hannover Medical School, Hannover D-30625, Germany.
authors.forename: Corinna
authors.initials: C
authors.lastname: Trebst
authors.affiliation: Department of Neurology, Hannover Medical School, Hannover D-30625, Germany.
authors.forename: Said Ben
authors.initials: SB
authors.lastname: Tayeb
authors.affiliation: Department of Neuroradiology, Hannover Medical School, Hannover D-30625, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Raab
authors.affiliation: Department of Neurology, Hannover Medical School, Hannover D-30625, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Stangel
keywords: anti-N-methyl-D-aspartate receptor antibodies; cerebrospinal fluid; encephalitis