title: Encephalitis related to primary varicella-zoster virus infection in immunocompetent children.
abstract: BACKGROUND
  Encephalitis is a rare complication of primary varicella-zoster virus (VZV) infection in immunocompetent children.
  
  
  METHODS
  The clinical and laboratory findings of two girls with VZV-related encephalitis are reported.
  
  
  RESULTS
  Both children presented with focal epileptic seizures, corresponding to cortical/subcortical as well as white matter lesions. The first showed a typical vesicular skin rash. She was easily diagnosed and made a rapid recovery during acyclovir and steroid treatment. In the second girl, a preceding measles-mumps-rubella virus vaccination and the absence of skin vesicles were misleading with respect to the diagnosis, which was finally proven by IgG seroconversion and intrathecal synthesis of IgG antibodies to VZV. She developed left parieto-occipital tissue necrosis and recovered only transiently during initial acyclovir/steroid treatment. Eight weeks after onset, progressive white matter demyelination and the occurrence of erythema nodosum in the lower limbs necessitated a second 4-month course of oral steroids. The VZV PCR from cerebrospinal fluid was negative in both children.
  
  
  CONCLUSIONS
  Primary VZV infection may cause severe encephalitis that may occur without skin vesicles and lead to a chronic course with systemic vasculitis. The coincidence of vaccination and neurologic diseases offers no proof per se of a causal relationship.
authors.affiliation: Department of Pediatrics, Pediatric Neurology Division, University Hospital, RWTH Aachen, Pauwelsstr. 30, D-52074, Aachen, Germany. Haeusler@RWTH-Aachen.de
authors.forename: M
authors.initials: M
authors.lastname: Häusler
authors.forename: L
authors.initials: L
authors.lastname: Schaade
authors.forename: S
authors.initials: S
authors.lastname: Kemény
authors.forename: K
authors.initials: K
authors.lastname: Schweizer
authors.forename: C
authors.initials: C
authors.lastname: Schoenmackers
authors.forename: V Th
authors.initials: VT
authors.lastname: Ramaekers
mesh_terms: D000212:Acyclovir / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000914:Antibodies, Viral / Q000097:blood; D000998:Antiviral Agents / Q000627:therapeutic use; D001921:Brain / Q000473:pathology* / Q000503:physiopathology / Q000821:virology*; D002675:Child, Preschool; D003937:Diagnosis, Differential; D020804:Encephalitis, Varicella Zoster / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006423:Hemianopsia / Q000473:pathology / Q000503:physiopathology / Q000821:virology; D014645:Herpesvirus 3, Human / Q000472:pathogenicity*; D006801:Humans; D007223:Infant; D012008:Recurrence; D012640:Seizures / Q000473:pathology / Q000503:physiopathology / Q000821:virology; D013256:Steroids; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D000914:Antibodies, Viral; D000998:Antiviral Agents; D013256:Steroids; D000212:Acyclovir