Genetic predisposition to HSV-1 encephalitis

Abstract

In this issue of The Journal, Abel et al report clinical data from 85 sporadic cases of HSV-1 encephalitis (HSE) in children from several centers in France. Fifty-one families were interviewed to explore the history of HSV and other infections in relatives, as well as consanguinity. Samples for serologic and genetic testing also were collected from 49 patients. Although no familial cases of HSE were identified, 20% of cases had parental consanguinity or history of a relative with herpes keratitis at a young age. These findings, as well as young age of cases and identification of specific genetic mutations in two patients resulting in impaired interferon production, led investigators to propose that childhood HSE, although sporadic and seemingly random, may be the result of genetic predisposition in some cases. These investigations add to a body of evidence, which suggests that specific “glitches” in innate immune function, rather than bad luck, are responsible for some sporadic, uncommon manifestations of infectious diseases, especially in children. Article page 623 ▸ Age-Dependent Mendelian Predisposition to Herpes Simplex Virus Type 1 Encephalitis in ChildhoodThe Journal of PediatricsVol. 157Issue 4PreviewTo test the hypothesis that predisposition to childhood herpes simplex virus (HSV) type 1 encephalitis (HSE) may be determined in part by human genetic factors. Full-Text PDF