Toll-like receptor 3 deficiency in autoimmune encephalitis post-herpes simplex encephalitis

The Toll-like receptor 3 (TLR3) pathway is a key component of the innate immunity that prevents replication of viruses in the CNS. Inborn errors of this pathway (TLR3-pathway deficiency), which includes defects in the genes TLR3, UNC93B1, TRIF, TRAF3, TBK1 and IRF3, occur in 10% of patients with herpes simplex encephalitis (HSE),1,2 and about 66% of these patients develop relapses of HSE.1 A recent study showed that 27% of patients with HSE develop autoimmune encephalitis (AE) in the weeks or months ensuing the infection.3 It is unknown whether TLR3-pathway deficient patients can also develop AE post-HSE. Here we report a patient with TLR3-pathway deficiency who developed HSE and a relapse of the viral infection followed by AE post-HSE, highlighting the fact that TLR3-pathway deficient patients should be carefully followed for both HSE relapses and AE.
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: Autoimmune diseases, Encephalitis, Viral infections, Post-infectious, Psychosis Clinical/Scientific Notes Source Type: research