Neurol. praxi. 2023;24(3):168-179 | DOI: 10.36290/neu.2023.020

Autoimmune encephalitides

MUDr. David Krýsl, Ph.D.^{1, 2}

¹ Neurologická klinika 2. LF UK a FN Motol, Praha

² Department of Clinical Neurophysiology, Sahlgrenska University Hospital, Göteborg

Autoimmune encephalitides (AIE) are autoimmune diseases of the central nervous system with predominant involvement of the cerebral cortex. The prevalence of AIE is comparable to that of encephalitis of infectious etiology. They can occur as paraneoplastic syndromes (in which the abnormal immune response is triggered by the presence of a peripheral tumor) or as non-paraneoplastic syndromes (where a combination of a viral trigger and an innate disposition seems to play a role). The most common AIEs include encephalitis with antibodies to glutamate N-methyl-D-aspartate receptors (NMDAR encephalitis), limbic encephalitis with antibodies to leucine-rich glioma inactivated protein 1 (LGI1 encephalitis) and syndromes formerly referred to as "classic paraneoplastic" - in today's terminology "high-risk phenotypes". These include limbic encephalitis with anti-Hu and anti-CV2 positivity, rapidly progressive cerebellar syndrome (usually associated with anti-Yo antibodies) and stiff-person syndrome (including its variants). In recent years, several new phenotypes have been described that deserve attention, such as anti-GFAP syndrome, anti-GABAA receptor encephalitis (relevant in the context of new-onset refractory status epilepticus - NORSE) and syndrome with anti-IgLON5 antibodies. This article provides a basic overview of current information regarding AIE.

Keywords: encephalitides, autoimmune diseases, NMDAR encephalitis, antibodies.

Received: March 16, 2023; Revised: April 19, 2023; Accepted: April 20, 2023; Published: June 14, 2023  Show citation