Ramsey Hunt Syndrome (RHS), forced by singer Justin Bieber to cancel the World Tour, is a rare and painful complication of the virus that causes shingles and chickenpox.
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RHS was discovered in 1907 by a neurologist of the same name. This is a rare neuropathy that can inflame and paralyze the facial nerve, causing a painful rash around the ears and mouth.
Symptoms vary from person to person, but can cause severe discomfort and pain. According to the National Rare Disease Organization (NORD), most patients are paralyzed on one side of the face and develop an ear rash. The affected facial muscles can become weak or stiff, preventing the patient from smiling, frowning, or closing the eyes on the paralyzed side.
In some cases, their speech may be obscured. Often, a reddish and painful bullous rash appears in the outer ear and ear canal. “This ear rash is often diagnosed,” French infectious disease specialist Benjamin David told AFP. Blisters may spread to the mouth, and pain in the soft palate, nasopharynx, and ears may spread to the neck. Other possible symptoms include tinnitus (tinnitus), earache, deafness, hyperacusis (much louder than normal), nausea and dizziness.
RHS is caused by the varicella-zoster virus, which also causes chickenpox in children and shingles in adults. For those who have had chickenpox as a child, the virus can stay dormant for decades. When it is reactivated, the carrier develops shingles and, in some cases, RHS. It is unknown why the virus reactivates and affects the facial nerve.
RHS has equal effects on men and women. According to one estimate quoted by NORD, about 5 out of 100,000 people in the United States develop the syndrome each year. However, some researchers believe that cases are undiagnosed or misdiagnosed, making it difficult to determine the true frequency of disability in the general population.
Anyone who has had chickenpox can develop Ramsay Hunt Syndrome, but it is very rare in children, NORD said. In most cases, it affects the elderly, especially adults over the age of 60 or have weakened immunity. “Getting a Ramsey Hunt at Justin Bieber’s age is very amazing,” said David. “But unhealthy lifestyles and excessive fatigue can contribute to the predisposition to viral infections.”
RHS is commonly treated with antiviral drugs such as acyclovir and famciclovir, along with corticosteroids such as prednisone. According to David, physiotherapy (“need to start early”) usually allows patients to fully recover, but “about 30 percent” experience sequelae. – AFP