Neurological Manifestations of Varicella Zoster Clinical Presentation

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

History

During the prodromal phase, paresthesias and pain may present a diagnostic dilemma; however, the dermatomal distribution helps clarify the diagnosis.

  • Goh and Khoo studied 164 patients with zoster and recorded the frequency of prodromal symptoms: 41% had pain (more common in patients older than 50 years), 27% had itching, and 12% had paresthesias.[2]
  • Once the typical rash erupts, the diagnosis is easier, but zoster has various presentations. Goh and Khoo reported the frequency of active disease symptoms: 90% had pain, 20% had feelings of helplessness and depression, and 12% had flu-like symptoms.[2]
  • Zoster may have atypical presentations. Cases of unilateral headache, the appearance of an ocular chemical burn, and facial palsy have been reported in the literature.
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Physical

  • Shingles
    • Shingles refers to the painful rash associated with reactivated VZV infection; it typically affects a single dermatome, most commonly a thoracic dermatome. After a prodromal illness, erythematous macules and papules appear and progress to vesicles within 1 day. The lesions eventually crust and resolve. Patients may experience pain and sensory loss in the distribution of the rash. Motor weakness, especially in lumbar and cervical radicular distributions, is often present but not appreciated and represents viral activity beyond the sensory root. Because the weakness often is not diagnosed accurately, the incidence and prevalence are uncertain.
    • Note that zoster may present in multiple dermatomes and possibly bilaterally (ie, zoster multiplex). The frequency of multiple, disseminated, and visceral zoster is increased in the immunocompromised population. Occasionally, patients experience paresthesias and pain in a dermatomal distribution without a rash (ie, zoster sine herpete).
  • Zoster multiplex
    • Zoster occurring in noncontiguous dermatomes is rare, even in immunocompromised individuals. Terminology depends on whether the condition is unilateral or bilateral and on the number of involved dermatomes.
    • For example, zoster duplex unilateralis refers to the involvement of 2 unilateral dermatomes. Vu and colleagues report one case of zoster multiplex involving 7 noncontiguous dermatomes.
  • Myelitis and encephalitis
    • Be aware of possible VZV myelitis. Devinski and colleagues report spinal cord involvement in immunosuppressed persons.[3] The condition emerges as the expected dermatomal rash.
    • Spinal cord involvement becomes apparent within 2-3 weeks from the initial rash with myelopathic findings on examination. Manifestations are usually bilateral. The disease may progress for 3 weeks, although a few cases of progression for as long as 6 months have been reported in patients with AIDS. Recurrent zoster myelitis is rare, although one case has been reported of a previously healthy young woman who developed recurrent myelopathy at the same spinal level. The condition resolved fully with intravenous (IV) acyclovir treatment.
    • Rarely, VZV may cause encephalitis. Westenend and Hoppenbrouwers reported fatal hemorrhagic encephalitis in an otherwise healthy woman.[4]
    • Myelitis and encephalitis may occur in previously healthy individuals, both adults and children.
  • Ramsay-Hunt syndrome
    • If the geniculate ganglion is involved, this syndrome of peripheral facial palsy with pain and vesicles in the ear may occur.
    • The vesicles are noted in the external ear canal or on the tympanic membrane; additional auditory and vestibular symptoms may be present.
  • Keratitis
    • Ocular involvement occurs when the ophthalmic division of the trigeminal nerve is involved in the viral reactivation (see image below). 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.
    • Complications range from corneal ulcers to conjunctivitis to blindness.
    • Occasionally, no vesicles are seen (ie, zoster sine herpete).
    • Svozilkova and colleagues report one case of varicella-caused retinal necrosis after ocular trauma.[5]
  • Zoster sine herpete
    • VZV reactivation may occur without cutaneous vesicles. In such cases, patients may experience pain and weakness in a dermatomal distribution but have no visible signs of shingles.
    • Furuta and coworkers noted that VZV may be demonstrated in 8-25% of patients with acute peripheral facial palsy without cutaneous vesicles.[6]
    • This condition presents a diagnostic dilemma; however, VZV DNA may be detected by polymerase chain reaction (PCR) from oropharyngeal swabs in patients with zoster peripheral facial palsy. Because such studies are not routine, the true incidence and prevalence are unknown.
  • Brachial plexus neuritis
    • Fabian and colleagues reported one patient who had a left upper arm monoplegia after a C4, C5, and C6 zoster multiplex.[7]
    • The authors believed that the brachial plexus inflammation was an extension of a dorsal ganglionitis. They found that the motor neuropathy was an inflammatory demyelinating process.
  • Guillain-Barre syndrome
    • Guillain-Barre syndrome is a rare complication from reactivation of latent VZV.
    • Cresswell and colleagues report Guillain-Barre also occurring from primary VZV infection in an adult.[8]
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Causes

Risk of typical shingles and atypical presentations (eg, myelitis, encephalitis, disseminated disease, visceral involvement) is increased in immunosuppressed patients.

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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.

References

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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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