Autoimmune Encephalitis

  • Agnes van Sonderen
  • Maarten J. TitulaerEmail author


This chapter gives an overview of the syndromes associated with antibodies directed to membrane-bound or synaptic proteins. In contrast to the classical syndromes, disease occurs in younger patients as well; patients are also seen by non-neurologists like psychiatrists and pediatricians, and patients tend to have a more favorable response to immunotherapy. The focus in this chapter is on the clinical characteristics which distinguishes these disorders and on the relevance of cerebrospinal fluid (CSF) analysis. Comparison of sensitivity and specificity for serum and CSF is extensively discussed. N-methyl-D-aspartate receptor (NMDAR) encephalitis is relatively common and discussed first. Alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis and encephalitis associated with antibodies to the metabotropic glutamate receptors mGluR1 and mGluR5 are rare and will be reviewed briefly. Subsequently, syndromes associated with antibodies to the glycine receptor, VGKC complex, and dipeptidyl-peptidase-like protein-6 (DPP6) are described. Encephalitis with antibodies directed to the γ-aminobutyric acid (GABA) receptor type B and type A are reviewed last.


Encephalitis Antibodies NMDA receptor VGKC GABA receptor AMPA receptor Glycine receptor 


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© Springer International Publishing Switzerland 2015

Authors and Affiliations

  1. 1.Department of NeurologyErasmus Medical CenterRotterdamThe Netherlands
  2. 2.Department of NeurologyHaga HospitalThe HagueThe Netherlands

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