Abstract
Reactivation of varicella zoster virus (VZV) from latently infected human ganglia usually produces herpes zoster (shingles), characterized by dermatomal distribution pain and rash. Zoster is often followed by chronic pain (postherpetic neuralgia or PHN) as well as meningitis or meningoencephalitis, cerebellitis, isolated cranial nerve palsies that produce ophthalmoplegia or the Ramsay Hunt syndrome, multiple cranial nerve palsies (polyneuritis cranialis), vasculopathy, myelopathy, and various inflammatory disorders of the eye. Importantly, VZV reactivation can produce chronic radicular pain without rash (zoster sine herpete), as well as all the neurological disorders listed above without rash. The protean neurological and ocular disorders produced by VZV in the absence of rash are a challenge to the practicing clinician. The presentation of these conditions varies from acute to subacute to chronic. Virological confirmation requires the demonstration of amplifiable VZV DNA in cerebrospinal fluid (CSF) or in blood mononuclear cells, or the presence of anti-VZV IgG antibody in CSF or of anti-VZV IgM antibody in CSF or serum.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Abbreviations
- AIDS:
-
Acquired immune deficiency syndrome
- CSF:
-
Cerebrospinal fluid
- CT:
-
Computerized tomography
- HSV:
-
Herpes simplex virus
- Ig:
-
Immunoglobulin
- MNCs:
-
Mononuclear cells
- MRI:
-
Magnetic resonance imaging
- PORN:
-
Progressive outer retinal necrosis
- RHS:
-
Ramsay Hunt syndrome
- SSPE:
-
Subacute sclerosing panencephalitis
- VZV:
-
Varicella zoster virus
References
Akpel EK, Gottsch JD (2000) Herpes zoster sine herpete presenting with hypema. Ocul Immunol Inflamm 8:115–118
Amlie-Lefond C, Mackin GA, Ferguson M et al (1996) Another case of virologically confirmed zoster sine herpete, with electrophysiologic correlation. J Neurovirol 2:136–138
Case Records of the Massachusetts General Hospital (Case 5-1995) (1995) N Engl J Med 332:452–459
Chiappini E, Calabri G, Galli L et al (2002) Varicella-zoster virus acquired at 4 months of age reactivates at 24 months and causes encephalitis. J Pediatr 140:250–251
Cohrs RJ, Mehta SK, Schmid DS et al (2008) Asymptomatic reactivation and shed of infectious varicella zoster virus in astronauts. J Med Virol 80:1116–1122
Connolly AM, Dodson WE, Al P et al (1994) Course and outcome of acute cerebellar ataxia. Ann Neurol 35:673–679
Dangond F, Engle E, Yessayan L et al (1993) Pre-eruptive varicella cerebellitis confirmed by PCR. Pediatr Neurol 9:491–493
Dueland AN, Martin JR, Devlin ME et al (1992) Acute simian varicella infection: clinical, laboratory, pathologic, and virologic features. Lab Invest 66:762–773
Easton HG (1970) Zoster sine herpete causing acute trigeminal neuralgia. Lancet 2:1065–1066
Echevarria JM, Martinez-Martin P, Tellez A et al (1987) Aseptic meningitis due to varicella-zoster virus: serum antibody levels and local synthesis of specific IgG, IgM and IgA. J Infect Dis 155:959–967
Friedman SM, Mames RN, Sleasman JW et al (1993) Acute retinal necrosis after chickenpox in a patient with acquired immunodeficiency syndrome. Arch Ophthalmol 111:1607–1608
Funakawa I, Terao A, Koga M (1999) A case of zoster sine herpete with involvement of the unilateral IX, X and XI cranial and upper cervical nerves. Rinsho Skinkeigaku 39:958–960
Galindez OA, Sabates NR, Whitacre MM et al (1996) Rapidly progressive outer retinal necrosis caused by varicella zoster virus in a patient infected with human immunodeficiency virus. Clin Infect Dis 22:149–151
Gilden DH, Dueland AN, Cohrs R et al (1991) Preherpetic neuralgia. Neurology 41:1215–1218
Gilden DH, Wright RR, Schneck SA et al (1994a) Zoster sine herpete, a clinical variant. Ann Neurol 35:530–533
Gilden DH, Beinlich BR, Rubinstien EM et al (1994b) Varicella-zoster virus myelitis: an expanding spectrum. Neurology 44:1818–1823
Gilden DH, Kleinschmidt-DeMasters BK, Wellish M et al (1996) Varicella zoster virus, a cause of waxing and waning vasculitis: the New England Journal of Medicine case 5-1995 revisited. Neurology 47:1441–1446
Gilden DH, Bennett JL, Kleinschmidt-DeMasters BK et al (1998) The value of cerebrospinal fluid antiviral antibody in the diagnosis of neurologic disease produced by varicella zoster virus. J Neurol Sci 159:140–144
Gilden D, Nagel MA, Ransohoff RM et al (2009) Recurrent varicella zoster virus myelopathy. J Neurol Sci 276:196–198
Goon P, Wright M, Fink C (2000) Ophthalmic zoster sine herpete. J R Soc Med 93:191–192
Grant RM, Weitzman SS, Sherman CG et al (2002) Fulminant disseminated varicella zoster virus infection without skin involvement. J Clin Virol 24:7–12
Habib AA, Gilden D, Schmid DS et al (2009) Varicella zoster virus meningitis with hypoglycorrhachia in the absence of rash in an immunocompetent woman. J Neurovirol 15:206–208
Hato N, Kisaki H, Honda N et al (2000) Ramsay Hunt syndrome in children. Ann Neurol 48:254–256
Heller HM, Carnevale NT, Steigbigel RT (1990) Varicella zoster virus transverse myelitis without cutaneous rash. Am J Med 88:550–551
Hevner R, Vilela M, Rostomily R et al (2003) An unusual cause of trigeminal-distribution pain and tumour. Lancet Neurol 2:567–571
Hon C, Au WY, Cheng VC (2005) Ophthalmic zoster sine herpete presenting as oculomotor palsy after marrow transplantation for acute myeloid leukemia. Haematologica 90 Suppl 12:EIM04
Kakourou T, Theodoridou M, Mostrou G et al (1998) Herpes zoster in children. J Am Acad Dermatol 39:207–210
Kaufman HE, Azcuy AM, Varnell ED et al (2005) HSV-1 DNA in tears and saliva of normal adults. Invest Ophthalmol Vis Sci 46:241–247
Kleinschmidt-DeMasters BK, Mahalingam R, Shimek C et al (1998) Profound cerebrospinal fluid pleocytosis and Froin’s syndrome secondary to widespread necrotizing vasculitis in an HIV-positive patient with varicella zoster virus encephalomyelitis. J Neurol Sci 159:213–218
Koelle DM, Benedetti J, Langenberg A et al (1992) Asymptomatic reactivation of herpes simplex virus in woman after the first episode of genital herpes. Ann Intern Med 116:433–437
Lewis GW (1958) Zoster sine herpete. Br Med J 34:418–421
Ljungman P, Lönnqvist B, Gahrton G et al (1986) Clinical and subclinical reactivations of varicella-zoster virus in immunocompromised patients. J Infect Dis 153:840–847
Mancardi GL, Melioli G, Traverso F et al (1987) Zoster sine herpete causing encephalomyelitis. Ital J Neurol Sci 8:67–70
Martinez-Martin P, Garcia-Sáiz A, Rapun JL et al (1985) Intrathecal synthesis of IgG antibodies to varicella-zoster virus in two cases of acute aseptic meningitis syndrome with no cutaneous lesions. J Med Virol 16:201–209
Mayo DR, Booss J (1989) Varicella zoster associated neurologic disease without skin lesions. Arch Neurol 46:313–315
Mehta SK, Cohrs RJ, Forghani B et al (2004) Stress-induced subclinical reactivation of varicella zoster virus in astronauts. J Med Virol 72:174–179
Mehta SK, Tyring SK, Gilden DH et al (2008) Varicella-zoster virus in the saliva of patients with herpes zoster. J Infect Dis 197:654–657
Morgan M, Nathwani D (1992) Facial palsy and infection: the unfolding story. Clin Infect Dis 14:263–271
Moses H, Nagel MA, Gilden DH (2006) Acute cerebellar ataxia in a 41 year old woman. Lancet Neurol 5:984–988
Murakami S, Honda N, Mizobuchi M et al (1998) Rapid diagnosis of varicella zoster virus infection in acute facial palsy. Neurology 51:202–205
Nau R, Lantsch M, Stiefel M et al (1998) Varicella zoster virus-associated focal vasculitis without herpes zoster: recovery after treatment with acyclovir. Neurology 51:914–915
Noda Y, Nakazawa M, Takahashi D et al (2001) Retinal periphlebitis as zoster sine herpete. Arch Ophthalmol 119:1550–1552
Orme HT, Smith AG, Nagel MA et al (2007) VZV Spinal cord infarction identified by diffusion-weighted MRI (DWI). Neurology 69:398–400
Osaki Y, Matsubayashi K, Okumiya K et al (1995) Polyneuritis cranialis due to varicella-zoster virus in the absence of rash. Neurology 45:2293
Powell KF, Wilson HG, Corxson MO et al (1995) Herpes zoster meningoencephalitis without rash: varicella zoster virus DNA in CSF. J Neurol Neurosurg Psychiatry 59:198–199
Rasoul-Rockenschaub S, Zielinski CC, Müller C et al (1990) Viral reactivation as a cause of unexplained fever in patients with progressive metastatic breast cancer. Cancer Immunol Immunother 31:191–195
Ratzka P, Schlachertzki JC, Bahr M et al (2006) Varicella zoster virus cerebellitis in a 66-year-old patient without herpes zoster. Lancet 357:182
Ross JS, Fanning WL, Beautyman W et al (1980) Fatal massive hepatic necrosis from varicella-zoster hepatitis. Am J Gastroenterol 74:423–427
Schott GC (1998) Triggering of delayed-onset postherpetic neuralgia. Lancet 351:419–420
Silverstein BE, Chandler D, Neger R et al (1997) Disciform keratitis: a case of herpes zoster sine herpete. Am J Ophthalmol 123:254–255
Taylor GR, Janney RP (1992) In vivo testing confirms a blunting of the human cell-mediated immune mechanism during space flight. J Leukoc Biol 51:129–132
Terada K, Niizuma T, Kawano S et al (1998) Detection of varicella-zoster virus DNA in peripheral mononuclear cells from patients with Ramsay Hunt syndrome or zoster sine herpete. J Med Virol 56:359–363
Vafai A, Mahalingam R, Zerbe G et al (1988) Detection of antibodies to varicella-zoster virus proteins in sera from the elderly. Gerontology 34:242–249
Vartdal F, Vandvik B, Norrby E (1982) Intrathecal synthesis of virus-specific oligoclonal IgG, IgA and IgM antibodies in a case of varicella-zoster meningoencephalitis. J Neurol Sci 57:121–132
Weber FP (1916) Herpes zoster: its occasional association with a generalized eruption and its occasional connection with muscular paralysis—also an analysis of the literature of the subject. Int Clin 3:185–202
Widal (1907). Med Chiropractic Pract 78:12
Yamamoto S, Tada R, Shimomura Y et al (1995) Detecting varicella-zoster virus DNA in iridocyclitis using polymerase chain reaction: a case of zoster sine herpete. Arch Ophthalmol 113:1358–1359
Acknowledgements
This work was supported in part by Public Health Service grants AG006127, NS032623 and AG032958 from the National Institutes of Health. Maria Nagel is supported by Public Health Service grant NS007321 from the National Institutes of Health. The authors thank Marina Hoffman for editorial review and Cathy Allen for manuscript preparation.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2010 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Gilden, D., Cohrs, R.J., Mahalingam, R., Nagel, M.A. (2010). Neurological Disease Produced by Varicella Zoster Virus Reactivation Without Rash. In: Abendroth, A., Arvin, A., Moffat, J. (eds) Varicella-zoster Virus. Current Topics in Microbiology and Immunology, vol 342. Springer, Berlin, Heidelberg. https://doi.org/10.1007/82_2009_3
Download citation
DOI: https://doi.org/10.1007/82_2009_3
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-12727-4
Online ISBN: 978-3-642-12728-1
eBook Packages: Biomedical and Life SciencesBiomedical and Life Sciences (R0)