Ramsay Hunt Syndrome, afflicted by singer Justin Bieber, is a rare condition that paralyzes one side of the face and is caused by reactivation of the chickenpox virus. What do we know about this disease?
What is that?
Discovered in 1907 by neurologist Ramsay Hunt, who is named after him, the syndrome is a rare neurological disorder characterized by paralysis of the facial nerve (facial nerve palsy) and a rash affecting the ear or mouth.
What are the symptoms?
They differ from case to case. Those affected usually suffer from facial paralysis and a rash on the ear. These two symptoms do not always appear at the same time. In most cases, only half of the face is affected (on one side), according to the American organization NORD on its website, which aims to support people with rare diseases. Facial muscles affected by nerve palsy may be weak or stiff, which can result in people being unable to smile, frown, or close their eyes on the affected side. In some cases, the language can get muddled.
In most cases, there is a reddish (erythematous), painful, blistering (vesicular) rash that affects the outside of the ear (pinna) and often the outer ear canal. “The diagnosis is often made because of this blistering rash around the ear or in the ear canal,” he saysAFP Benjamin Davido, infectiologist at Garches Hospital (AP-HP Hôpitaux de Paris). In some cases, the rash, including painful blisters, can also affect the mouth, roof of the mouth, and upper throat.
Other symptoms such as ringing in the ears (tinnitus), earache (otalgia), sensorineural hearing loss, hyperacusis (intensification of sounds) are possible. Sometimes those affected also experience vomiting or dizziness.
What are the causes?
Ramsay Hunt syndrome is caused by the varicella-zoster virus, the same virus that causes chickenpox and shingles. The virus can lie dormant for decades in a person who had chickenpox as a child. Reactivation of the varicella-zoster virus leads to shingles and, in some cases, leads to Ramsay Hunt syndrome. Why the virus reactivates in Ramsay Hunt syndrome and affects the facial nerve is unknown.
Which population groups are affected?
This syndrome affects men and women equally. According to the NORD organization, an estimated five out of every 100,000 people in the United States develop this syndrome each year. However, some researchers believe cases go undiagnosed or misdiagnosed, making it difficult to determine the true frequency of the disorder in the general population.
Anyone who has had chickenpox can potentially develop Ramsay Hunt syndrome. However, most cases occur in the elderly, particularly those over 60 or those who are immunocompromised. “It’s amazing doing a Ramsay hunt at Justin Bieber’s age,” says Benjamin Davido. But “an imperfect lifestyle or a link to overwork can come into play because then we’re more susceptible to viral infections,” he continues.
What are the treatments?
Treatment for Ramsay Hunt syndrome usually involves antiviral medications, such as aciclovir or famciclovir, in conjunction with corticosteroids, such as prednisone. Rehabilitation, which is “essential to start early,” generally allows for a good recovery, according to Benjamin Davido. But the consequences remain in “about 30% of the cases”.
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