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 Neuromuscular Disorders (e.g., ALS/Lou Gehrig's Disease), General Pediatrics

Ramsay Hunt syndrome

Ramsay Hunt syndrome


Definition:

Ramsay Hunt syndrome is a disorder of the brain and nervous system (neurologic disorder) that occurs when a virus (varicella zoster) infects certain nerves in the head.

Alternative Names:
Hunt syndrome; Herpes oticus
Causes, incidence, and risk factors:

The varicella zoster virus causes both Ramsay Hunt syndrome and another common cause of facial weakness, Bell's palsy (in which there is no rash).

This virus is part of the family of human herpes viruses, which cause fever sores on the mouth and genital herpes. The varicella zoster virus also causes shingles and chickenpox.

In people with Ramsay Hunt syndrome, the virus is believed to infect the facial nerve near the inner ear. This leads to irritation and swelling.

Symptoms:
  • Hearing loss on one side
  • Painful rash on the eardrum, tongue, or roof of the mouth (palate) on the same side as the facial weakness
  • Sensation of things spinning around (vertigo)
  • Weakness of the face with difficulty closing one eye -- smile may look crooked
Signs and tests:

The doctor will usually make the diagnosis by looking for signs of weakness in the face and a blister-like (vesicular) rash. Some patients will have a nerve conduction study to find out how much damage has been done to the facial nerve and to see how well they will recover.

Blood tests can help determine whether you have been infected with the varicella zoster virus.

There is a promising laboratory technique called PCR, which can detect very small amounts of the virus's DNA in the skin. However, it is mostly used for research.

The use of pictures of the brain (neuroimaging), such as magnetic resonance imaging (MRI), may show swelling of the facial nerves and help the doctor find out whether the infection has spread to other nerves or the brain. A spinal tap is used in rare cases, especially when the diagnosis is not clear.


Review Date: 2/6/2008
Reviewed By: Daniel Kantor, MD, Director of the Comprehensive MS Center, Neuroscience Institute, University of Florida Health Science Center, Jacksonville, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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