Justin Bieber and Ramsay Hunt Syndrome

Maui, Hawaii. (Dr. Noorali Bharwani)
Maui, Hawaii. (Dr. Noorali Bharwani)

Canadian singer, Justin Bieber, recently announced he has taken time off from his tour to recover from Ramsay Hunt syndrome. We hope his recovery is quick and complete.

What is Ramsay Hunt syndrome?

Ramsay Hunt syndrome was first described in 1907 by James Ramsay Hunt in a patient who had ear pain associated with skin and mucosal rashes. Ramsay Hunt (1872 – 1937), was an American neurologist.

Ramsay Hunt syndrome is a viral infection. The varicella zoster virus, which also causes shingles and chicken pox, typically infects facial nerve near the inner ear, causing weakness, inflammation and sometimes pain in the surrounding areas.

After chickenpox clears up, the virus still lives in the nerves. Years later, it may reactivate. When it does, it can affect the facial nerve causing one-sided facial paralysis and hearing loss.

According to a report published in the Canadian Medical Association Journal (cmaj.ca/content/189/8/e320), Ramsay Hunt syndrome has an annual incidence rate of 3.2 to 4.2 cases per 1,000 people.

Ramsay Hunt syndrome can occur in anyone who has had chickenpox. It’s more common in older adults, typically affecting people older than 60. It is rare in children.

What is Bell’s palsy?

Ramsay Hunt syndrome can be confused with Bell’s palsy. A famous example of Bell’s palsy sufferer who experienced permanent effects is former Prime Minister of Canada, Jean Chretien, who had Bell’s palsy as a child.

Bell’s palsy is named after Sir Charles Bell, a Scottish doctor and surgeon in the 19th century. In 1821, Bell described the anatomy of the facial nerve and its association with the unilateral facial paralysis. Historically, the descriptions of the facial distortion were first told by early Greek and Roman physicians.

The cause of Bell’s palsy is unknown. Swelling and inflammation of the cranial nerve VII is seen. Most scientists believe that reactivation of an existing (dormant) viral infection may cause the disorder.

Bell’s palsy can strike at any time, and although most symptoms can improve within a few weeks and a complete recovery is usually anticipated, some people have lingering effects, and others may be burdened with symptoms for life.

What is the difference between Bell’s palsy and Ramsay Hunt syndrome?

Compared with Bell’s palsy (facial paralysis), patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely. Overall, the chances of recovery are better if the treatment is started within three days.

Prevention and Management of Ramsay Hunt syndrome:

Shingles vaccination is the only way to protect against shingles and postherpetic neuralgia, the most common complication from shingles.

Shingrix, the shingles vaccine, can provide protection against both shingles and Ramsay Hunt syndrome. It’s available for adults age 50 and older, given in two doses two to six months apart, and is more than 90 per cent effective in preventing shingles.

Children are now routinely vaccinated against chickenpox, which greatly reduces the chances of becoming infected with the chickenpox virus. A shingles vaccine for people age 50 or older also is recommended.

Time is of the essence when it comes to treatment of Ramsay Hunt syndrome. If treatment begins within three days of the diagnosis, partial paralysis of the face resolves itself by 75 per cent. If treatment starts four to seven days after diagnosis, or more than eight days, paresis is resolved by 48 and 30 per cent, respectively.

Medical treatment may include: antiviral drugs, corticosteroids, and anti-anxiety medications. Drugs such as diazepam (Valium) can help relieve vertigo. The pain associated with Ramsay Hunt syndrome can be severe. Prescription pain medications may be needed.

Complications:

Complications of Ramsay Hunt syndrome may include permanent hearing loss and facial weakness.  Usually, it is temporary but can be permanent. There may be eye damage because of the weakness of eye-lids. There may be postherpetic neuralgia.

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Shingles Vaccine may be Viewed as a Quality of Life Vaccine

An example of shingles. (iStockphoto/Thinkstock)
An example of shingles. (iStockphoto/Thinkstock)


A man with shingles of the face.

Shingles is caused by chickenpox virus called varicella zoster virus. The first indications that chickenpox and shingles were caused by the same virus were noticed at the beginning of the 20th century.

The incidence of shingles is mainly in adults. There are approximately four cases per 1000 population per year and a lifetime risk of 20 to 30 per cent.

Chickenpox generally occurs in children. Once the child gets over the illness the virus does not disappear from the body. Virus can settle down in one of the nerve cell bodies and lay dormant for many years.

When your resistance is low and this can be due to any reason, the virus may break out of the nerve cell and travel down the nerve causing viral infection of the skin in the area supplied by that nerve. This can happen decades after the chickenpox infection. Exactly how the virus remains latent in the body, and subsequently re-activates is not understood.

Shingles starts with burning pain, itching and tingling followed by painful rash and blisters in the area supplied by the affected nerve. The pain and rash most commonly occurs on the torso, but can appear on the face, eyes or other parts of the body. If the nerve to the eye is involved then a person may suffer loss of vision. It usually affects one nerve on one side of the body.

The rash and blisters heal within two to four weeks but some sufferers experience residual nerve pain for months or years. This condition is known as postherpetic neuralgia. About 20 per cent of patients with shingles suffer from this.

If the diagnosis of shingles is made early then it helps to start antiviral medications within 72 hours of the appearance of the rash. This reduces the severity and duration of the illness. The antiviral medications should be used for seven to ten days. The blisters crust over within seven to ten days, and usually the crusts fall off and the skin heals. But sometimes after severe blistering, scarring and discolored skin remains.

Until the rash has developed crusts, a person is extremely contagious. During the blister phase, direct contact with the rash can spread the virus to a person who has no immunity to the virus. This newly infected individual may then develop chickenpox, but will not immediately develop shingles.

Since 2008-2009, a vaccine for shingles is available for adults age 60 and over. The vaccine is used to boost the waning immunity to the virus that occurs with aging. The effectiveness of the vaccine is about 60 per cent. It is kind of a “quality of life” vaccine. It does not prevent death from shingles (an extremely rare event) but does help with postherpetic neuralgia (pain).

Booster doses of the vaccine are not recommended for healthy individuals. The efficacy of protection has not been assessed beyond four years and it is not known whether booster doses of vaccine are beneficial. This recommendation may need to be revisited as further information becomes available.

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Shingles is an Ugly and Painful Condition

Recently, a relative sent me a picture of his face, full of blisters, on the right side, below the lower eyelid. The blisters went all the way to his upper lip. He said it was very painful and he had lost sensation in the right side of the face and upper lip. He was feeling lousy and had no appetite. He could not drink anything hot or too cold because of the loss of sensation. There was constant headache.

This is a classic case of herpes zoster, commonly known as shingles. Now, do not confuse this with the shingles on the roof of your house or a short hair cut on a pretty lady. Shingles does not look pretty and unlike your shingles on the roof it does not provide any protection.

Shingles is caused by varicella zoster virus (VZV). This is the same virus which causes chickenpox. The first indications that chickenpox and shingles were caused by the same virus were noticed at the beginning of the 20th century.

This is what happens. The virus causes chicken pox which generally occurs in children. Once the child gets over the illness the virus does not disappear from the body. Virus can settle down in one of the nerve cell bodies and lay dormant for many years.

When your resistance is low and this can be due to any reason, the virus may break out of the nerve cell and travel down the nerve causing viral infection of the skin in the area supplied by that nerve. This can happen decades after the chicken pox infection. Exactly how the virus remains latent in the body, and subsequently re-activates is not understood.

Early symptoms of shingles are non-specific like headache, fever and malaise. Then there is burning pain, itching and tingling followed by painful rash and blisters in the area supplied by the affected nerve. The pain and rash most commonly occurs on the torso, but can appear on the face, eyes or other parts of the body. If the nerve to the eye is involved then a person may suffer loss of vision.

The rash and blisters heal within two to four weeks but some sufferers experience residual nerve pain for months or years. This condition is known as post-herpetic neuralgia. About 20 per cent of patients with shingles suffer from this.

If the diagnosis of shingles is made early then it helps to start anti-viral medications within 72 hours of the appearance of the rash. This reduces the severity and duration of the illness. The anti-viral medications should be used for seven to ten days. The blisters crust over within seven to ten days, and usually the crusts fall off and the skin heals. But sometimes after severe blistering, scarring and discolored skin remains.

Although a person of any age can suffer from shingles, aging population and individuals with poor immunity (AIDS, renal failure, stress) are more prone to shingles if they have had chickenpox in their younger days.

Is it contagious? Yes, to a point. Until the rash has developed crusts, a person is extremely contagious. During the blister phase, direct contact with the rash can spread the virus to a person who has no immunity to the virus. This newly-infected individual may then develop chickenpox, but will not immediately develop shingles.

Start reading the preview of my book A Doctor's Journey for free on Amazon. Available on Kindle for $2.99!