title: Age-dependent Mendelian predisposition to herpes simplex virus type 1 encephalitis in childhood.
abstract: OBJECTIVE
  To test the hypothesis that predisposition to childhood herpes simplex virus (HSV) type 1 encephalitis (HSE) may be determined in part by human genetic factors.
  
  
  METHODS
  A genetic epidemiologic survey of childhood HSE (onset at age 3 months to 15 years) over a 20-year period (1985-2004) was conducted throughout France (comprising 29 university hospital neuropediatric centers). A total of 85 children fulfilled the diagnostic criteria for inclusion. Family and personal histories were obtained by face-to-face interview for 51 patients.
  
  
  RESULTS
  No familial cases of HSE were identified in our survey; however, a high proportion (20%) of the children interviewed had a relevant family history: parental consanguinity (12% of patients), early-onset herpetic keratitis in a first-degree relative (6%), or both (2%). The narrow window of high susceptibility to HSE before age 3 years (62% of patients) further indicates that predisposition to HSE is tightly age-dependent.
  
  
  CONCLUSIONS
  This survey suggests that childhood HSE, although sporadic, may result from Mendelian predisposition (from autosomal recessive susceptibility in particular), at least in some children. There likely is incomplete penetrance, however, which may reflect, at least in part, the impact of age at the time of HSV-1 infection.
authors.affiliation: Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM (National Institute of Health and Medical Research), Paris, France. laurent.abel@inserm.fr
authors.forename: Laurent
authors.initials: L
authors.lastname: Abel
authors.forename: Sabine
authors.initials: S
authors.lastname: Plancoulaine
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Jouanguy
authors.forename: Shen-Ying
authors.initials: SY
authors.lastname: Zhang
authors.forename: Nora
authors.initials: N
authors.lastname: Mahfoufi
authors.forename: Nathalie
authors.initials: N
authors.lastname: Nicolas
authors.forename: Vanessa
authors.initials: V
authors.lastname: Sancho-Shimizu
authors.forename: Alexandre
authors.initials: A
authors.lastname: Alcaïs
authors.forename: Yiqi
authors.initials: Y
authors.lastname: Guo
authors.forename: Annabelle
authors.initials: A
authors.lastname: Cardon
authors.forename: Soraya
authors.initials: S
authors.lastname: Boucherit
authors.forename: Dorothée
authors.initials: D
authors.lastname: Obach
authors.forename: Thomas
authors.initials: T
authors.lastname: Clozel
authors.forename: Lazaro
authors.initials: L
authors.lastname: Lorenzo
authors.forename: Daniel
authors.initials: D
authors.lastname: Amsallem
authors.forename: Patrick
authors.initials: P
authors.lastname: Berquin
authors.forename: Thierry
authors.initials: T
authors.lastname: Blanc
authors.forename: Cécile
authors.initials: C
authors.lastname: Bost-Bru
authors.forename: Stéphane
authors.initials: S
authors.lastname: Chabrier
authors.forename: Brigitte
authors.initials: B
authors.lastname: Chabrol
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Cheuret
authors.forename: Olivier
authors.initials: O
authors.lastname: Dulac
authors.forename: Philippe
authors.initials: P
authors.lastname: Evrard
authors.forename: Bénédicte
authors.initials: B
authors.lastname: Héron
authors.forename: Leila
authors.initials: L
authors.lastname: Lazaro
authors.forename: Josette
authors.initials: J
authors.lastname: Mancini
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Pedespan
authors.forename: François
authors.initials: F
authors.lastname: Rivier
authors.forename: Louis
authors.initials: L
authors.lastname: Vallée
authors.forename: Pierre
authors.initials: P
authors.lastname: Lebon
authors.forename: Flore
authors.initials: F
authors.lastname: Rozenberg
authors.forename: Jean-Laurent
authors.initials: JL
authors.lastname: Casanova
authors.forename: Marc
authors.initials: M
authors.lastname: Tardieu
mesh_terms: D000212:Acyclovir / Q000627:therapeutic use; D000293:Adolescent; D000367:Age Factors; D017668:Age of Onset; D000998:Antiviral Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D020803:Encephalitis, Herpes Simplex / Q000188:drug therapy / Q000235:genetics* / Q000821:virology*; D005260:Female; D020022:Genetic Predisposition to Disease; D014644:Genetic Variation / Q000235:genetics; D006801:Humans; D007223:Infant; D008297:Male; D012307:Risk Factors; D018139:Simplexvirus; D051196:Toll-Like Receptor 3 / Q000235:genetics*; D055815:Young Adult
chemical_list: D000998:Antiviral Agents; C495344:TLR3 protein, human; D051196:Toll-Like Receptor 3; D000212:Acyclovir