Encephalopathy, acute, infection-induced (herpes-specific), susceptibility to, 10

Susceptibility to infection-induced acute encephalopathy-10 (IIAE10) presents as neurologic deficits in response to acute infection, particularly with herpes simplex virus-1 (HSV-1), which leads to inflammation of the brain and herpes simplex encephalitis (HSE). The age at onset ranges from infancy to adulthood. However, some mutation carriers do not develop encephalopathy even if exposed to the virus, indicating incomplete penetrance (summary by Lafaille et al., 2019). For a phenotypic description of herpes simplex encephalitis (HSE) and a discussion of genetic heterogeneity of susceptibility to acute infection-induced encephalopathy, see 610551. [from OMIM]