Ramsay Hunt Syndrome

  • Medical Author:
    Charles Patrick Davis, MD, PhD

    Dr. Charles "Pat" Davis, MD, PhD, is a board certified Emergency Medicine doctor who currently practices as a consultant and staff member for hospitals. He has a PhD in Microbiology (UT at Austin), and the MD (Univ. Texas Medical Branch, Galveston). He is a Clinical Professor (retired) in the Division of Emergency Medicine, UT Health Science Center at San Antonio, and has been the Chief of Emergency Medicine at UT Medical Branch and at UTHSCSA with over 250 publications.

  • Medical Editor: Melissa Conrad Stöppler, MD
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

What is Ramsay Hunt syndrome? What causes it?

  • Ramsay Hunt syndrome (also termed Hunt's Syndrome and herpes zoster oticus) is a herpes zoster virus infection of the geniculate ganglion of the facial nerve.
  • It is caused by reactivation of herpes zoster virus that has previously caused chickenpox in the patient.
  • Ramsay Hunt syndrome results in paralysis of the facial muscles on the same side of the face as the infection. So, the virus infects the facial nerve that normally controls the muscles on one side of the face.
  • Ramsay Hunt syndrome is typically associated with a red rash and blisters (inflamed vesicles or tiny water-filled sacks in the skin) in or around the ear and eardrum and sometimes on the roof of the mouth or tongue.

What are the symptoms of Ramsay Hunt syndrome?

The classic symptom that clinically distinguishes Ramsay Hunt syndrome is a red painful rash associated with blisters in the ears and facial paralysis (for example, eyelid or mouth) on one side of the face. Other symptoms are: 

Genital herpes virus infection

Definition of Ramsay Hunt Syndrome

A herpes virus infection of the geniculate nerve ganglion that causes paralysis of the facial muscles on the same side of the face as the infection. The geniculate ganglion is a sensory ganglion associated with the VIIth cranial nerve.

The Ramsay Hunt syndrome is usually associated with a rash and blisters.

There are three variations or types of Ramsay Hunt syndromes, including:

  • Ramsay Hunt syndrome I
  • Ramsay Hunt syndrome II
  • Ramsay Hunt syndrome III

How is Ramsay Hunt syndrome diagnosed?

Diagnosis of the syndrome is most often made by observing the symptoms described above (red painful rash with ear and or mouth blisters and one-sided facial paralysis). Also, a PCR test (polymerase chain reaction) can be performed on the fluid from the blisters to demonstrate the viral genetic material, but this test is not done routinely.

Is Ramsay Hunt syndrome contagious?

The syndrome is not contagious; however, the herpes zoster virus that can be found in the blisters of Ramsay Hunt syndrome can be transmitted to other people and cause chickenpox in those who are unvaccinated against chickenpox and who have never had chickenpox. Individuals with Ramsay Hunt syndrome should avoid contact with newborns, pregnant women, immunodepressed individuals, and people with no history of chickenpox, at least until all the blisters change to scabs.

How does Ramsay Hunt syndrome compare with Bell's palsy?

Bell's palsy also is a result of injury to the facial nerve by a viral infection, but the suspected viral cause of Bell's palsy has not been identified. Ramsay Hunt syndrome is caused by the Varicella virus (Herpes zoster) that also causes chickenpox and shingles (a painful, blister-producing Herpes zoster reinfection that usually occurs on one side of the body). There is no red rash associated with Bell's palsy as there is with Ramsay Hunt syndrome. Additionally, Ramsay Hunt syndrome is commonly more painful than Bell's palsy. However, both can cause eyelid and mouth paralysis on one side of the face.

Dyssynergia cerebellaris myoclona is a rare degenerative disease of the nerves characterized by epilepsy, muscle spasms, and gradually increasing tremors. Like Bell's palsy, this disease complex mimics many symptoms of Ramsay Hunt syndrome. Some investigators term the disease complex Ramsay Hunt syndrome type 2.

How is Ramsay Hunt syndrome treated or prevented?

Treatment consists of antiviral agents (for example, acyclovir, valacyclovir or famciclovir) for about one week, steroids (prednisone), and pain medications. Best results are reported when treatment protocols are started within three days after symptoms appear. Early treatment usually results in a better prognosis (see below). For children, the varicella vaccine can reduce the chance of getting chickenpox from which the syndrome comes (reactivation of the virus). However, once a person gets chickenpox, the person is susceptible to reactivation of the virus and thus can develop shingles and/or Ramsay Hunt syndrome. Fortunately, there is another vaccine, Zostavax, which is helpful in preventing viral reactivation. Consequently, shingles and Ramsay Hunt syndrome can be either prevented or their symptoms reduced if the vaccine is administered. Usually, this vaccine is given to individuals that have had chickenpox as children and are now age 60 or older. The CDC (U.S. Centers for Disease Control and Prevention) suggests the vaccine be routinely given to individuals aged 60 or older, as about 90% of the population has been exposed to chickenpox and about 20% of people that had chickenpox are likely to get shingles without the vaccine.

What is the outlook (prognosis) for patients with Ramsay Hunt syndrome?

The prognosis for Ramsay Hunt syndrome is not as good as that for Bell's palsy. There is good clinical evidence to suggest that treatment with steroids, pain medications, and antiviral agents (like acyclovir, valacyclovir or famciclovir) improve recovery and lessen the extreme facial discomfort. However, infrequent complications can develop, such as synkinesis (inappropriate nerve responses such as blinking or tear formation while trying to talk), eye damage, or rarely, viral spread to other nerves causing many other problems (for example, pain, confusion, weakness). Postherpetic neuralgia (pain due to nerve fiber damage by the virus) may also develop and persist for months to years.

Medically reviewed by Robert Cox, MD; American Board of Internal Medicine with subspecialty in Infectious Disease

REFERENCE:

Albrecht, Mary A., MD. "Clinical manifestations of varicella-zoster virus infection: Herpes zoster." UptoDate.com. Updated Aug. 2, 2016.

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Reviewed on 9/7/2016
References
Medically reviewed by Robert Cox, MD; American Board of Internal Medicine with subspecialty in Infectious Disease

REFERENCE:

Albrecht, Mary A., MD. "Clinical manifestations of varicella-zoster virus infection: Herpes zoster." UptoDate.com. Updated Aug. 2, 2016.

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