The Ramsay Hunt syndrome is named after its discoverer, Dr. James Ramsay Hunt, an American Neurologist who first described it in the year 1907 AD. In Ramsay Hunt Syndrome, the facial nerve gets infected, resulting in painful rash and weakness of the facial muscle. It is caused by the virus known as Varicella zoster. The manifestations of Ramsay Hunt syndrome can look frightening, but if the treatment is given in time, one can prevent serious complications like permanent facial paralysis and complete hearing loss.
About Ramsay Hunt Syndrome
Ramsay Hunt Syndrome is a neurological disorder, caused by the reactivation of the Varicella zoster virus which belongs to the family of herpes viruses. It is the same virus that causes chicken pox. When the initial infection occurs, the virus lies dormant in the nervous system for many years. This virus may get reactivated after many years and infect the facial nerve causing the Ramsay Hunt syndrome.
People who had chicken pox before are at high risk of developing the Ramsay Hunt syndrome. It is more common in people above the age 60 years, and is rarely found in children. People who have a weak immune system like HIV patients are also at high risk. Diabetics and pregnant women in their third trimester of pregnancy are also at risk. The reactivation of Varicella zoster virus in a person can spread chicken pox to another person who never had chicken pox before, through contact.
The Ramsay Hunt Syndrome is manifested by weakness of the face on one side which is known as palsy. There is a development of painful red rash with fluid filled blisters. These blisters appear on the outsides and insides of the ear, roof of the mouth, and tongue. There will be pain in the ear, and difficulty in hearing. The person may experience difficulty in closing one eye. There may be loss of taste and difficulty in eating. As the facial nerve is affected, there may be difficulty in making expressions and facial droop.
Diagnosis
The doctor will examine the characteristic rash and check for facial weakness. If damage to the facial nerve is detected, then a nerve conduction study is done to know the extent of damage and the possibility of recovery. To check for lesions of Varicella zoster virus in the ear, an otoscope is used by which the insides of the ear can be seen. To detect Viral DNA in the skin, a lab test called ‘Polymerase chain reaction test’ (PCR) is done in which fluid is taken from the blisters of the rash; fluid from the ear; blood or tear sample and tested for the Varicella zoster virus. MRI scan or Magnetic resonance imaging technique is done to check for any swelling of the facial nerve in which radio waves and a magnetic field are used to generate images of the facial nerve and its surrounding structures. Electromyography (EMG) may be conducted by the doctor to check for the health of the muscles and nerves. When diagnosis is not clear, a spinal tap may be done in which the cerebrospinal fluid is tested for the Varicella zoster virus.
Treatment
As Ramsay Hunt Syndrome is cause by virus, the doctor prescribes 7 to 10 days course of antiviral medications like valacyclovir (valtrex), famciclovir 500mg thrice daily (Famvir), and Acyclovir 800mg , 5 times daily (Zovirax). For relief from vertigo or spinning sensation, diazepam (valium) is given. To reduce swelling, corticosteroids like prednisone (60mg daily for 3to 5 days) is given. To relieve pain, pain killers can be taken. For facial muscle weakness that causes difficulty in closing one eye, a Botox injection is given into the upper eyelid that will allow the closing of the eye. To improve the strength of facial muscles, physical therapy is recommended in which facial exercises are done to improve the condition of the facial muscles. In case of complete facial palsy (no facial movement at all), surgical decompression (reduction of pressure surgically) of the fallopian canal is done which is the canal through which the facial nerve passes. Recovery may take several weeks to several months; in severe cases complete recovery may not be possible.
Prevention
To prevent Ramsay Hunt Syndrome, a Varicella virus vaccination known as Varivax (chicken pox vaccine) is recommended in children between the ages 12 months to 18 months. This vaccination can also be given to kids of other age groups and also adults who never had chicken pox in their lives. Another vaccination known as Zostavax which is approved by the Food and Drug administration is administered in adults who are 60 years and above. This vaccination helps in preventing other conditions like Shingles also that occurs due to Varicella zoster virus.
Outlook
If the treatment is started within 3 days after the symptoms appear, there is 70% chance of recovery. The recovery is only 50% if the treatment is started after 3 days of the appearance of symptoms. If treatment is taken at the right time, one can prevent serious complications.
