This article includes discussion of varicella-zoster virus infections of the nervous system, herpes zoster, shingles, VZV infections of the nervous system, zoster sine herpete, disseminated zoster, herpes zoster myelitis, herpes zoster ophthalmicus, herpes zoster ophthalmicus and contralateral hemiplegia, herpes zoster oticus, postherpetic neuralgia, Ramsay Hunt syndrome 1, varicella-zoster virus encephalitis, VZV encephalitis, and zoster sine herpete. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
Varicella-zoster virus causes chickenpox (varicella) in childhood, becomes latent in dorsal root ganglia, and can reactivate years later to produce shingles (zoster) in adults, as well as postherpetic neuralgia, central nervous system vasculopathy, myelitis, and meningoencephalitis. Diagnosis of varicella-zoster virus infection of the central and peripheral nervous system is critical as antiviral therapy can suppress productive infection with clinical benefit.
Historical note and terminology
Varicella-zoster virus causes chickenpox (varicella) in childhood, becomes latent in dorsal root ganglia, and can reactivate years later to produce shingles (zoster) in adults. Chickenpox is a generalized exanthem, whereas shingles is usually limited to 1 or more adjacent sensory dermatomes. Herpes zoster was described in medieval literature, and it was recognized as caused by varicella-zoster virus by the end of the 19th century (Weller and Witton 1958). The relationship between varicella and zoster was not recognized until the end of the 19th century partially because varicella was not differentiated from variola (smallpox) until then (Osler 1892). In 1888 Von Bokay suggested that chickenpox and shingles were related after observing that susceptible children could acquire chickenpox after exposure to adults with shingles (Von Bokay 1909). Varicella was shown to be infectious in 1875 by Steiner, and shingles in 1925 by Kundratitz (Steiner 1875; Kundratitz 1925). The characteristic intranuclear inclusion bodies seen in varicella-zoster virus infections were described in chickenpox lesions by Tyzzer in 1906 and in shingles lesions by Lipschultz in 1921 (Tyzzer 1906; Lipschultz 1921). In 1943 Garland suggested that shingles was due to the reactivation of latent varicella-zoster virus (Garland 1943). The tissue culture and serologic studies of Weller and colleagues firmly established that chickenpox and shingles were due to a single etiologic agent (Weller and Witton 1958; Weller et al 1958).
Developments include the use of PCR and in situ hybridization techniques to demonstrate that varicella-zoster virus DNA is latently present in trigeminal and thoracic ganglia (Mahalingam et al 1990). Specific prophylactic and therapeutic agents have also become available. Intravenous acyclovir has been shown to reduce varicella-zoster virus dissemination and neurologic complications when herpes zoster occurs in immunocompromised patients (Shepp et al 1986). It has also been effective for varicella-zoster virus encephalitis in immunocompetent patients (Broman and Overall 1994). Although not indicated for neurologic involvement, varicella-zoster virus immune globulin can prevent varicella infection in exposed seronegative pregnant women and immunocompromised children and newborns (Arvin 1991; Anonymous 1996). A report of 2 neonates with severe varicella, despite intravenous immunoglobulin, suggests that these infants should also receive acyclovir (Reynolds et al 1999). In 1995 the U.S. Food and Drug Administration approved a live attenuated varicella-zoster virus vaccine (Varivax-Merck); this vaccine has been recommended for people 12 months of age or older, who are in good health and without a history of prior varicella-zoster virus infection (Anonymous 1996). Modified chicken pox has occurred in 2% to 3% of vaccinated patients per year (Johnson et al 1997). It remains to be seen if this vaccine will affect the incidence of herpes zoster.
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