Varicella Zoster Disease of the Central Nervous System: Epidemiological, Clinical, and Laboratory Features 10 Years after the Introduction of the Varicella Vaccine

• Published in: The Journal of infectious diseases 2011-02, Vol.203 (3), p.316-323
• Main Authors: Pahud, Barbara A., Glaser, Carol A., Dekker, Cornelia L., Arvin, Ann M., Schmid, D. Scott
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Applied microbiology; Biological and medical sciences; Central nervous system; Central nervous system diseases; Chickenpox; Chickenpox Vaccine - adverse effects; Chickenpox Vaccine - immunology; Child; Child, Preschool; Encephalitis; Encephalitis, Varicella Zoster - pathology; Encephalitis, Varicella Zoster - prevention & control; Epidemiology; Female; Fundamental and applied biological sciences. Psychology; Genotypes; Human viral diseases; Humans; Infant; Infectious diseases; Major and Brief Reports; Male; Medical sciences; Meningitis, Viral - pathology; Meningitis, Viral - prevention & control; Meningitis, Viral - virology; Microbiology; Middle Aged; Nervous system diseases; Time Factors; Vaccination; Vaccines, antisera, therapeutical immunoglobulins and monoclonal antibodies (general aspects); Varicella zoster encephalitis; Varicella-zoster virus; Viral diseases; Viral diseases with cutaneous or mucosal lesions and viral diseases of the eye; Viral meningitis; VIRUSES; Young Adult
• ISSN: 0022-1899; 1537-6613; 0022-1899; 1537-6613
• DOI: 10.1093/infdis/jiq066

Background. Since the introduction of live attenuated varicella zoster virus (VZV) vaccine in 1995 there has been a significant reduction in varicella incidence and its associated complications, but the impact on VZV-associated central nervous system (CNS) disease has not been assessed. Methods. In this descriptive study we evaluated patients referred to the California Encephalitis Project from 1998 to 2009 with VZV PCR-positive cerebrospinal fluid (CSF). Epidemiological, clinical, and laboratory data were collected using a standardized case form. Specimens were genotyped using multi-single nucleotide polymorphism (SNP) analysis. Results. Twenty-six specimens were genotyped from patients 12–85 years of age (median, 46 years). Clinical presentations included meningitis (50%), encephalitis (42%), and acute disseminated encephalomyelitis (ADEM) (8%). Only 11 patients (42%) had a concomitant herpes zoster rash. Genotype analysis identified 20 European Group (Clade1, Clade 3) strains; 4 Asian (Clade 2) strains, and 2 Mosaic Group (Clade 4, Clade VI) strains. One specimen was recognized as vaccine strain by identifying vaccine-associated SNPs. Conclusions. VZV continues to be associated with CNS disease, with meningitis being the most frequent clinical presentation. CNS VZV disease often presented without accompanying zoster rash. Sequencing data revealed multiple genotypes, including 1 vaccine strain detected in the CSF of a young patient with meningitis.