Neurological Manifestations of Varicella Zoster 

  • Author: Wayne E Anderson, DO; Chief Editor: Karen L Roos, MD   more...
 
Updated: May 11, 2011
 

Background

Herpes zoster presents in many ways. It should not be considered simply a self-limited dermatomal rash with pain. Varicella-zoster virus (VZV) infection is an acute neurologic disease that warrants immediate evaluation. A common misperception is that VZV always is a benign disorder.

Following initial infection, usually as chickenpox in childhood, VZV remains dormant in the dorsal spinal root ganglion neurons and the fifth cranial nerve ganglion neurons. Upon reactivation, a spectrum of signs and symptoms can occur, including a self-limited painful rash, pain without skin manifestations, keratitis, vertigo, and spinal cord disease with weakness. Once VZV infection resolves, many individuals continue to suffer pain, a condition known as postherpetic neuralgia (PHN).

Herpes zoster in the ophthalmic (V1) distribution Herpes zoster in the ophthalmic (V1) distribution of the trigeminal nerve. Note the unilateral distribution of the rash and how the V1 distribution may extend to the tip of the nose. Though at risk for keratitis with zoster in this distribution, the patient had a normal ocular examination. Patient consented to picture distribution for educational use; written permission on file; contributed by JS Huff.
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Pathophysiology

VZV remains latent in the dorsal root ganglia after an initial infection (chickenpox). Host immunologic mechanisms suppress replication of the virus, but VZV reactivates when the host mechanisms fail to contain the virus. This occurs from a wide spectrum of issues, from stress to severe immunosuppression. Occasionally this follows direct trauma. VZV viremia occurs frequently with chickenpox but also may occur with herpes zoster, albeit with a lower viral load. The virus migrates from the sensory root and produces a dermatomal sensory loss and a characteristic painful rash.

Inflammatory involvement may include the leptomeninges. When the cervical and lumbar roots are involved, motor involvement (which often is overlooked) may be seen, depending on the virulence and/or extent of migration.

VZV is a DNA virus. The viral genome encodes approximately 70 proteins. Once activated at the spinal root or cranial nerve neurons, an inflammatory response occurs that also encompasses the leptomeninges. Both plasma cells and lymphocytes are noted. In at least one case of motor neuron involvement, lymphocytic infiltration and myelin breakdown were observed with preservation of axons.

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Epidemiology

Frequency

United States

Incidence is approximately 5 per 1000 per year. Immunosuppression increases risk. The incidence of PHN increases with age. Half of patients older than 60 years may have a temporary or prolonged pain syndrome. Although 50% or more of patients with PHN may have resolution of the pain within 1-2 years, the remainder may have a continuing pain syndrome. Fortunately newer treatments are available for PHN.

Although the frequency of varicella zoster may decrease as the vaccinated children grow to adulthood, the present frequency of varicella zoster in adults may be increasing.

International

The international frequency is the same as for the United States.

Mortality/Morbidity

Ninety-five percent of patients with zoster experience severe pain. The acute pain and insomnia are most bothersome.

  • Variant presentations of zoster (eg, keratitis, myelitis) may carry additional morbidity.
  • Duration of symptoms, incidence of complications and duration of post-herpetic neuralgia if it should occur, are longer in immunocompromised patients.
  • Complications may occur. For example, staphylococcal and streptococcal bacteria may superinfect the skin lesions and require treatment.

Race

Although the pathophysiology is uncertain, the vesicular eruption may be less frequent in African Americans. The reason is not clear.

Sex

  • Prevalence in males and females is equal.
  • Ertunc and colleagues suggested both that zoster frequency is higher in right-handed patients and that the rash appears more frequently on the left side in females.[1] The pathophysiology for these differences is uncertain.

Age

Although zoster can occur at any age after primary infection, incidence increases with age. Also troublesome is the increasing incidence of PHN with advancing age.

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Contributor Information and Disclosures
Author

Wayne E Anderson, DO  Assistant Professor of Internal Medicine/Neurology, College of Osteopathic Medicine of the Pacific Western University of Health Sciences; Clinical Faculty in Family Medicine, Touro University College of Osteopathic Medicine; Clinical Instructor, Departments of Neurology and Pain Management, California Pacific Medical Center

Wayne E Anderson, DO is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Society of Law, Medicine & Ethics, California Medical Association, and San Francisco Medical Society

Disclosure: Cephalon Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; King Honoraria Speaking and teaching; Forest Honoraria Speaking and teaching

Specialty Editor Board

J Stephen Huff, MD  Associate Professor of Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia School of Medicine

J Stephen Huff, MD is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Neurology, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Florian P Thomas, MD, MA, PhD, Drmed  Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University School of Medicine

Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, and National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

Chief Editor

Karen L Roos, MD  John and Nancy Nelson Professor of Neurology, Professor of Neurological Surgery, Department of Neurology, Indiana University School of Medicine

Karen L Roos, MD is a member of the following medical societies: American Academy of Neurology and American Neurological Association

Disclosure: Nothing to disclose.

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Herpes zoster in the ophthalmic (V1) distribution of the trigeminal nerve. Note the unilateral distribution of the rash and how the V1 distribution may extend to the tip of the nose. Though at risk for keratitis with zoster in this distribution, the patient had a normal ocular examination. Patient consented to picture distribution for educational use; written permission on file; contributed by JS Huff.
 
 
 
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