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Ramsay Hunt syndrome, a rare neurological disorder

What is that ?

Discovered in 1907 by the neurologist Ramsay Hunt after whom it is named, the syndrome is a rare neurological disorder characterized by paralysis of the facial nerve (facial palsy) and a rash affecting the ear or mouth.

What are the symptoms ?

They vary from case to case. Those affected usually suffer from facial nerve paralysis and a rash affecting the ear. These two symptoms do not always occur simultaneously. In most cases, only one side of the face is affected (unilateral), indicates on its website the American organization NORD, which aims to provide support for people with rare diseases.

Facial muscles affected by nerve palsy may be weak or stiff, which may cause affected individuals to be unable to smile, wrinkle their foreheads, or close their eyes on the affected side. In some cases, speech may become muddled.

In most cases, a reddish (erythematous), painful, blistered (vesicular) rash appears that affects the outer part of the ear (pinna) and often the external auditory canal.

“The diagnosis is often made because of this vesicular eruption around the ear or inside the ear canal,” Benjamin Davido, infectious disease specialist at Garches hospital (AP-HP Hôpitaux de Paris).

In some cases, the rash, including painful blisters, may also affect the mouth, palate, and upper throat.

Other symptoms such as ringing in the ear (tinnitus), ear pain (otalgia), sensorineural hearing loss, hyperacusis (an increase in sounds) are possible.

Sometimes those affected also suffer from vomiting or dizziness.

What are the causes ?

Ramsay Hunt syndrome is caused by 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 varicella zoster virus results in shingles and in some cases progresses to Ramsay Hunt syndrome. Why the virus reactivates and affects the facial nerve in Ramsay Hunt syndrome is unknown.

Which populations are affected?

This syndrome affects men and women in equal numbers. It is estimated that five out of 100,000 people develop this syndrome each year in the United States, according to the NORD organization. Some researchers believe, however, that 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 older people, especially those over 60 or immunocompromised.

“It’s amazing to do a Ramsay Hunt at Justin Bieber’s age,” says Benjamin Davido. But “an imperfect lifestyle or a context of overwork can come into play, because we are then more susceptible to viral infections”, he continues.

What are the treatments ?

Treatment for Ramsay Hunt syndrome usually involves antiviral drugs, such as acyclovir or famciclovir, in conjunction with corticosteroids, such as prednisone.

Rehabilitation, which is “essential to start early”, generally allows good recovery, according to Benjamin Davido. But sequelae persist in “about 30% of cases”.

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