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Autoimmune encephalitis as differential diagnosis of infectious encephalitis

Armangue, Thaísa; Leypoldt, Frankb; Dalmau, Josepa,b,c

Current Opinion in Neurology: June 2014 - Volume 27 - Issue 3 - p 361–368
doi: 10.1097/WCO.0000000000000087
INFLAMMATORY DISEASES AND INFECTION: Edited by Jérôme Honnorat and Thomas de Broucker
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Purpose of review This review describes the main types of autoimmune encephalitis with special emphasis on those associated with antibodies against neuronal cell surface or synaptic proteins, and the differential diagnosis with infectious encephalitis.

Recent findings There is a continuous expansion of the number of cell surface or synaptic proteins that are targets of autoimmunity. The most recently identified include the metabotropic glutamate receptor 5 (mGluR5), dipeptidyl-peptidase-like protein-6 (DPPX), and γ-aminobutyric acid-A receptor (GABAAR). In these and previously known types of autoimmune encephalitis [N-methyl-D-aspartate receptor (NMDAR), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), γ-aminobutyric acid-B receptor (GABABR), leucine-rich glioma inactivated protein 1 (LGI1), contactin-associated protein-like 2 (CASPR2)], the prodromal symptoms or types of presentations often suggest a viral encephalitis. We review here clues that help in the differential diagnosis with infectious encephalitis. Moreover, recent investigations indicate that viral encephalitis (e.g., herpes simplex) can trigger synaptic autoimmunity. In all these disorders, immunotherapy is usually effective.

Summary Autoimmune encephalitis comprises an expanding group of potentially treatable disorders that should be included in the differential diagnosis of any type of encephalitis.

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aAugust Pi i Sunyer Biomedical Research Institute (IDIBAPS), Service of Neurology, Hospital Clinic, University of Barcelona

bCatalan Institution for Research and Advanced Studies (ICREA), Barcelona, Spain

cDepartment of Neurology, University of Pennsylvania, Philadelphia, USA

Correspondence to Josep Dalmau, MD, PhD, Centre de Recerca Biomèdica, CELLEX, Facultat de Medicina, Universitat de Barcelona, C/Casanova 143, Recepció CELLEX, 08036 Barcelona, Spain. Tel: +34 932 271 738; e-mail:

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