Mendelian predisposition to herpes simplex encephalitis
Herpes simplex encephalitis (HSE) is the most common sporadic viral encephalitis in the
Western world. The pathogenesis of HSE, which affects a small minority of HSV-1-infected
individuals, has long remained elusive. Mendelian defects in the TLR3-interferon (IFN) and
IFN-responsive pathways were recently shown to predispose to HSE, at least in some
children. Autosomal recessive STAT-1 deficiency and X-linked NEMO deficiency were found
in children with both mycobacterial disease and HSE. Autosomal recessive UNC-93B …
Western world. The pathogenesis of HSE, which affects a small minority of HSV-1-infected
individuals, has long remained elusive. Mendelian defects in the TLR3-interferon (IFN) and
IFN-responsive pathways were recently shown to predispose to HSE, at least in some
children. Autosomal recessive STAT-1 deficiency and X-linked NEMO deficiency were found
in children with both mycobacterial disease and HSE. Autosomal recessive UNC-93B …