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Autoimmune Encephalitis

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Cerebrospinal Fluid in Clinical Neurology

Abstract

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.

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Authors and Affiliations

  1. Department of Neurology, Erasmus Medical Center, Rotterdam, The Netherlands

    Agnes van Sonderen & Maarten J. Titulaer

  2. Department of Neurology, Haga Hospital, The Hague, The Netherlands

    Agnes van Sonderen

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  1. Agnes van Sonderen
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  2. Maarten J. Titulaer
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Correspondence to Maarten J. Titulaer .

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Editors and Affiliations

  1. Department of Neurology, Innsbruck Medical University, Innsbruck, Austria

    Florian Deisenhammer

  2. Department of Neurology, Danish Multiple Sclerosis Center Rigshospitalet, University of Copenhagen, Copenhagen, Denmark

    Finn Sellebjerg

  3. Department of Clinical Chemistry, VU University Medical Center Amsterdam Neurochemistry Laboratory and Biobank, Amsterdam, The Netherlands

    Charlotte E Teunissen

  4. Department of Neurology, University of Ulm, Ulm, Germany

    Hayrettin Tumani

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van Sonderen, A., Titulaer, M.J. (2015). Autoimmune Encephalitis. In: Deisenhammer, F., Sellebjerg, F., Teunissen, C., Tumani, H. (eds) Cerebrospinal Fluid in Clinical Neurology. Springer, Cham. https://doi.org/10.1007/978-3-319-01225-4_16

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