Heterogeneity of clinical features and corresponding antibodies in seven patients with anti-NMDA receptor encephalitis

Sühs,Kurt‑Wolfram; Wegner,Florian; Skripuletz,Thomas; Trebst,Corinna; Tayeb,Said  Ben; Raab,Peter; Stangel,Martin

doi:10.3892/etm.2015.2689

Heterogeneity of clinical features and corresponding antibodies in seven patients with anti-NMDA receptor encephalitis

Authors:
- Kurt‑Wolfram Sühs
- Florian Wegner
- Thomas Skripuletz
- Corinna Trebst
- Said Ben Tayeb
- Peter Raab
- Martin Stangel
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Affiliations: Department of Neurology, Hannover Medical School, Hannover D-30625, Germany, Department of Neuroradiology, Hannover Medical School, Hannover D-30625, Germany
Published online on: August 19, 2015 https://doi.org/10.3892/etm.2015.2689
Pages: 1283-1292
Copyright: © Sühs et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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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.

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