Case Report
Volume 10 Issue 8 - 2018
N-Methyl-D-Aspartate Receptor Encephalitis Masking Creutzfeldt-Jakob Disease
Anikó Papp1,2*, András Horváth1,2, Dániel Fabó1, GáborGéza Kovács3, Anita Kamondi1 and Anna Szűcs1
1Department of Neurology, National Institute of Clinical Neurosciences, Budapest, Hungary
2School of PhD Studies, Semmelweis University, Budapest, Hungary
3Institute of Neurology Medical University of Vienna, Vienna, Austria
*Corresponding Author: Anikó Papp, Department of Neurology, National Institute of Clinical Neurosciences, Budapest, Hungary.
Received: April 23, 2018; Published: July 10, 2018
Citation: Anikó Papp., et al. "N-Methyl-D-Aspartate Receptor Encephalitis Masking Creutzfeldt-Jakob Disease”. EC Neurology 10.8 (2018): 653-657.
Introduction: N-Methyl-D-aspartate receptor antibody encephalitis is an autoimmune disorder presenting with psychiatric symptoms, dementia, orofacial dyskinesia, myoclonus, epileptic seizures, and central alveolar hypoventilation. The co-occurrence of Nmethyl- D-aspartate receptor antibodies with Creutzfeldt-Jakob disease has been described, but the mechanism and significance of their presence is unclear.
Our Case: We present a 63-year-old woman with cognitive decline, orofacial dyskinesia, ataxia and central hypoventilation, leading to death in 13 months. She had plasma anti-N-methyl-D-aspartate receptor antibodies, and transitorily improved after plasmapheresis. Brain-autopsy did not find inflammation but spongiform changes with kuru-type amyloid plaques compatible with sporadic Creutzfeldt-Jakob disease MV-2 subtype.
Discussion: The diagnosis of autoimmune encephalitis needs careful check-up for underlying conditions. In our case N-methyl-Daspartate receptor antibody encephalitis masked the underlying Creutzfeldt-Jakob disease calling attention to the need of careful diagnostic interpretation in such cases. In addition to onconeuronal antigens, autoantibodies might emerge against receptors released from the neurons damaged by a prion disease as well; the longer the neuronal damage exists, the more likely the development of autoantibodies.
Keywords: NMDAR-Ab; Encephalitis; Creutzfeldt-Jakob Disease; MV Type 2; Plasmapheresis
Copyright: © 2018 Anikó Papp., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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