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Encephalitis related to primary varicella-zoster virus infection in immunocompetent children
M Häusler1, L Schaade, S Kemény
1Department of Pediatrics, Pediatric Neurology Division, University Hospital, RWTH Aachen, Pauwelsstr. 30, D-52074, Aachen, Germany. Haeusler@RWTH-Aachen.de
Insights
Varicella-zoster virus (VZV) encephalitis can occur in children without a rash and may lead to chronic illness. Diagnosis requires careful evaluation, as vaccination history can be misleading.
Area of Science:
- Neurology
- Infectious Diseases
- Pediatrics
Background:
- Encephalitis is a rare but serious complication of primary varicella-zoster virus (VZV) infection in immunocompetent children.
- This report details two pediatric cases of VZV-related encephalitis.
Observation:
- Two girls presented with focal epileptic seizures and white matter lesions.
- One child had a typical rash and recovered well with treatment; the other lacked a rash and had a complicated, chronic course.
- Diagnostic challenges arose due to a prior measles-mumps-rubella vaccination and absence of skin vesicles in the second patient.
Findings:
- Diagnosis was confirmed by VZV-specific IgG seroconversion and intrathecal antibody synthesis.
- Cerebrospinal fluid VZV PCR was negative in both cases.
- The second patient developed progressive demyelination and erythema nodosum, requiring prolonged steroid treatment.
Implications:
- Primary VZV infection can cause severe encephalitis without a rash, potentially leading to chronic neurological conditions like vasculitis.
- Coincidental vaccination does not imply a causal link to neurological disease.
- Highlights the importance of considering VZV in pediatric encephalitis, even without characteristic skin manifestations.
Introduction:
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.
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