Encephalitis related to primary varicella-zoster virus infection in immunocompetent children
Introduction
Primary varicella-zoster virus (VZV) infection may cause various neurologic complications. In immunocompetent children, its frequency is considered to be below 1%, with postinfectious ataxia, a usually benign disease, being the most frequent manifestation [1], [2], [3]. Myelitis, encephalitis or stroke, in contrast, are considered rare events. During recent years, however, increasing data suggest an underestimated impact of VZV on pediatric neurologic disease. Recently, Askalan et al. [4] showed a close epidemiologic link between recent VZV infection and childhood stroke, and according to our own experience, patients with VZV-related cerebral vasculitis may present even up to 4 years after the primary infection [5]. Therefore, at our hospital, all patients with acute, otherwise unexplained neurologic symptoms, are routinely investigated for acute primary VZV infection and intrathecal synthesis of anti-VZV antibodies. Between January 2000 and March 2001, two children presented with severe VZV-related encephalitis, a disorder that has hitherto been reported in 15 cases only [3], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19]. The clinical data of these two children, the diagnostic approach and the treatment response are summarized below.
Section snippets
Methods
Specific antiviral antibody titers were measured using Enzygnost enzyme immune assays and the BEP III instrument (Dade Behring, Marburg, Germany). Peripheral blood lymphocyte subsets were analyzed by means of two-color flow cytometry (FACScan™) according to standard protocols (Becton Dickinson Immunocytometry Systems). The following subsets were investigated: B cells (CD19)-, helper T cells (CD3CD4)-, NK cells (CD16CD56)-, cytotoxic/suppressoric T cells (CD3CD8), HLA-DR antigen and
Results
Basic clinical and serologic data are described in the patient reports. Table 1 summarizes the virologic data. Table 2 shows detailed laboratory data referring to the prolonged course of Patient 2.
Discussion
This report describes two children with encephalitis related to primary VZV infection. In the first, a typical skin rash rapidly established the diagnosis, whereas in the second, the preceding vaccination might have suggested post-vaccination acute demyelinating encephalomyelitis (ADEM) [21], [22]. ADEM, however, was unlikely as it commonly occurs within 4 weeks of the vaccination, rapidly responds to steroids, rarely causes cortical/subcortical lesions and usually shows a monophasic course.
References (39)
- et al.
Acute cerebellar swelling in varicella encephalitis
Pediatr. Neurol.
(1988) - et al.
Leucoencéphalite avec hémiplégie au cours d'une varicelle
Arch. Pédiatr.
(1997) - et al.
Protein transfer at the blood CSF barrier and the quantitation of the humoral immune response within the central nervous system
Clin. Chim. Acta
(1987) - et al.
Postinfectious and postvaccinial encephalomyelitis
Neurol. Clin.
(1984) - et al.
The value of cerebrospinal fluid antiviral antibody in the diagnosis of neurologic disease produced by varicella zoster virus
J. Neurol. Sci.
(1998) - et al.
The patterns of varicella zoster virus encephalitis
Hum. Pathol.
(1996) - et al.
Varicella-zoster virus infections of the nervous system
Arch. Pathol. Lab. Med.
(2001) - et al.
Neurologic complications of the reactivation of varicella-zoster virus
N. Engl. J. Med.
(2000) - et al.
Postvaricella encephalitis
Arch. Neurol.
(1984) - et al.
Chickenpox and stroke in childhood. A study of frequency and causation
Stroke
(2001)
Early and late onset manifestations of cerebral vasculitis related to varicella zoster
Neuropediatrics
Nonsuppurative encephalomyelitis accompanying chickenpox
Arch. Neurol. Psychiatry
Contribution clinique et anatomique a l'etude des manifestations neurologiques et psychiatriques de l'infection varicelleuse
J. Neurol. Psychiatry
Documents personnels inédits des rapporteurs: complications neurologiques des exanthèmes
J. Neurol. Psychiatry
Studie über Enzephalitis bei Varizellen mit besonderer Berücksichtigung der Spätprognose
Acta Paediatr.
A case of post-varicella encephalitis showing bilateral softening of the neostriatum and terminal “tetanoid chorea”
Med. J. Aust.
Varicella encephalitis
Arch. Dis. Child.
Encephalitis complicating chickenpox
Am. J. Dis. Child.
Varicella encephalitis
N. Y. State J. Med.
Cited by (51)
Virus Infections of the Nervous System
2023, Manson's Tropical Diseases, Fourth EditionInfectious encephalitis: Management without etiological diagnosis 48 hours after onset
2017, Medecine et Maladies InfectieusesVirus Infections of the Nervous System
2013, Manson's Tropical Diseases: Twenty-Third EditionAcute varicella zoster encephalitis without evidence of primary vasculopathy in a case-series of 20 patients
2012, Clinical Microbiology and InfectionCitation Excerpt :The recommended regimen is acyclovir at the same dosage as herpes simplex virus encephalitis treatment despite the lack of paediatric data [14,31,32]. Unlike herpes simplex virus, few studies have been published on VZV encephalitis [33]. In our study, 3 years after discharge, half of the survivors still presented with moderate to severe sequelae.
Management of suspected viral encephalitis in children - Association of British Neurologists and British Paediatric Allergy, Immunology and Infection Group National Guidelines
2012, Journal of InfectionCitation Excerpt :In immunocompetent children, VZV can cause CNS disease through three mechanisms; a post-VZV cerebellitis, an acute VZV encephalitis and a VZV vasculopathy. In cerebellitis caused by VZV, antiviral treatments are not normally used because the disease is usually self limiting, resolving in one to three weeks, and the primary pathogenic process is thought to be immune-mediated demyelination, rather than viral cytopathology.178 Although there are no good studies in primary VZV encephalitis, this condition is usually treated with antiviral drugs and, possibly corticosteroids.178
- 1
These authors have contributed equally.