Herpetic encephalopathy is a cerebral infection caused by herpes simplex virus type 1 (HSV1). This disease, which affects only a small minority of HSV1-infected individuals, could result from a genetic predisposition. A mutation in the UNC-93B gene, inducing impaired production of interferon, an anti-infectious factor necessary to fight the herpetic virus infection in nervous tissue, may be responsible for the disease. Another mutation was found in a gene encoding toll-like receptor 3 (TLR3).
Alternative names
TLR3D
Influenza associated encephalopathy
HSV encephalitis, Herpes simplex encephalitis, Herpes simplex neuroinvasion, Herpetic encephalopathy Herpetic encephalopathy, idiopathic
Classification
- Defects of innate immune system, receptors and signaling components
Inheritance
Autosomal dominant
Cross references
Phenotypically related immunodeficiencies
IDR factfile for UNC-93B deficiency
IDR factfile for IFNγ1-receptor deficiency
IDR factfile for IFNγ2-receptor deficiency
IDR factfile for Interleukin-12 p40 deficiency
Incidence
1 in 250 000 and 1 in 500 000.