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Ramsay Hunt Syndrome: The rare, often misdiagnosed condition afflicting Justin Bieber

KUALA LUMPUR: Canadian pop singer Justin Bieber, who was on his 'Justice World Tour' - which began in February - has postponed a series of shows in the United States due to a virus that has caused "full paralysis" on one side of his face.

The 28-year-old two-time Grammy winner on June 11 shared a video on his verified Instagram page which boasts over 241 million followers, in which he explained he had been diagnosed with Ramsay Hunt Syndrome (RHS), forcing him to take a break from performing to focus on recovery.

Amid the outpouring of "get well soon" wishes, the announcement left Beliebers (Bieber fans) around the globe, including Malaysia anxious as Bieber is scheduled to take the stage in Bukit Jalil National Stadium on Oct 22 as part of the Justice tour.

"I really hope he (Bieber) won't cancel his tour in Malaysia. Speedy recovery please #prayforjustin," a fan posted on Twitter.

"Will Bieber's concert in Malaysia be cancelled due to his condition or will it be postponed instead?", wrote another, while others have resorted to selling the tickets priced from RM288 to RM2,088.

The announcement of Bieber's condition had also shed light on the rare disease as there was an immediate global spike in search volume for RHS.

"What is RHS? Is it contagious? Can it be treated? Can people with RHS fully recover from it? "What is the recovery time?", were among the most asked questions.

Experts prominent senior consultant neurologist, Emeritus Professor Datuk Dr Raymond Azman Ali of the Medicine Faculty, Universiti Teknologi Mara (UiTM) and molecular virologist Dr Vinod Balasubramaniam of the Jeffrey Cheah School of Medicine and Health Sciences at Monash University Malaysia spoke to the New Straits Times on this.

RHS, AN UNWELCOME 'SOUVENIR' OF CHILDHOOD CHICKENPOX

Ramsay Hunt syndrome (RHS), also known as herpes zoster oticus (zoster or 'kayap' in Malay), is a neurological disorder in which the varicella zoster virus (VZV) causes inflammation of the facial nerve, said Dr Raymond.

 

RHS is a rare disease in which a neurologist may see at most five cases in their lifetime, or zero to two cases every five years, he said.

He said primary VZV infection causes chickenpox (varicella) and when it is reactivated after decades, it produces shingles in adults (herpes zoster, stinging rash on one side of the body), as well as a number of subacute, acute and chronic neurological conditions, including RHS.

"RHS is shingles of the facial nerve. RHS can occur in anyone who has had chickenpox and it is more common in the elderly aged above 60 as their immunity tends to get weaker with age, and rarely seen in children because they get infected with chickenpox first.

"When a person recovers from chickenpox, which usually clears in a week or two, the VZV virus does not disappear, but it 'hibernates' in nerve cells for decades."

STRESS, A TRIGGER FACTOR

"In times of stress - emotional or physical - this (VZV) virus can be reactivated and when it reappears, it is called zoster or herpes zoster," Dr Raymond explained.

"I am sure Bieber had this virus dormant in his nervous system. It is a place called 'geniculate ganglion', which is a collection of 'pseudounipolar sensory neurons' of the facial nerve located in the facial canal of the head."

He described a glandular ganglion as a bony structure in the skull where the virus hides and can be awoken under stress, leading to inflammation of the facial nerve, death of cells, and eventually paralysis.

"RHS is ubiquitous as many people have this (zoster) virus in them. It is just a matter of time. It is the same as Bell's palsy (unexplained episode of facial muscle weakness or paralysis which begins suddenly and worsens over 48 hours)."

Dr Raymond chairman of the Epilepsy Council, Malaysian Society of Neurosciences has been invited to more than 350 lectures locally and internationally and published over 100 full journal papers related to neurology.

According to a paper published in the National Library of Medicine, of zoster cases involve the facial nerve and result in RHS.

The syndrome is named after James Ramsay Hunt (1872-1937), an American neurologist and army officer in World War I who described three different syndromes, the most famous of which is the second, which is RHS.

BELL'S PALSY vs RHS

Research has shown that Bell's palsy most likely occurs due to the reactivation of herpes simplex virus type 1 (HSV-1), said Dr Raymond.

According to the World Health Organisation, HSV-1, known as herpes, is common globally and is mainly transmitted by oral-to-oral contact, causing oral herpes (including symptoms known as cold sores), but it can also lead to genital herpes.

"HSV-1 is the virus that causes cold sores, like ulcers on the lips and Bell's palsy is the reactivation of that virus in the facial nerve, whereas RHS is reactivation of the chicken pox (zoster) virus in the facial nerve.

"HSV-1 and zoster are from the same family of viruses called human neurotropic alphaherpesviruses, notoriously clever at hiding in the nerves and there is no way to get rid of them. They cause lifelong infections in the ganglia.

"We think we can get rid of them (virus) by giving patients all these antiviral drugs, but once they hide it is difficult to get to them, so both are similar in a way and both require almost similar treatment."

Dr Raymond said those who have received the chickenpox (varicella) vaccine may still develop RHS since the vaccine is not 100 per cent effective, like all other vaccines.

"Some people claim they have never had chickenpox, but I believe they are unable to recall having it. Some people call it measles or mumps, but one of those episodes might have been chickenpox.

"Besides, chickenpox is generally a mild disease in children hence some parents do not take their children to hospitals at all."

According to the Health Ministry, chickenpox is a contagious viral infection that over 90 per cent of people get during childhood unless they have been immunised.

RHS: IS IT CONTAGIOUS?

Dr Raymond said while RHS is not contagious, a person with the condition can pass chickenpox to people who have never contracted the disease before.

Therefore, he said people with RHS should wait until they fully recover before coming in contact with the immunosuppressed (individuals with weakened immune systems), those who have never had chickenpox, infants, and pregnant mothers, even if they have been vaccinated against the virus.

Immunosuppressed people include those undergoing HIV or cancer treatment, are diabetic, and have certain genetic disorders, he said.

"Often people with zoster have red rash or vesicular rash. These small, fluid-filled blisters can start as papules (raised red spots). When these blisters pop or rupture, it can release the virus to others who are prone to infection."

Dr Vinod concurred saying that factors that increase the incidence of RHS, include stress, chemotherapy, immunocompromised, infection, and malnutrition.

"RHS affects both immunocompetent and immunocompromised patients and has an incidence of about five per 100,000 people per year (an estimation)," Dr Vinod said.

TETRAD OF SIGNS

Dr Raymond said there are four main signs and symptoms of RHS - ear pain (otalgia) due to swelling of the nerve compressing against the bony facial canal; ipsilateral facial paralysis (paralysis on the same side of ear pain); loss of taste over the anterior two-thirds of the tongue (sweet and salty); and vertigo (sensation of feeling off balance).

"These are the typical tetrad but not all patients exhibit all four signs. In some cases, patients only have paralysis and painful rash in the ear.

 

"In fact, it is mandatory to look for rash if people have facial paralysis, which typically appears in the ear canal, soft palate (roof of the mouth) and the tongue (the front where the taste receptors for salty and sweet are found). All on the same side of the paralysis."

Dr Raymond explained that there are a total of 12 cranial nerve pairs in a human body (each nerve pair splits to serve the two sides of the brain and body) that work to send electrical signals between the human brain and different parts of the neck, head and torso. These signals help people smell, taste, hear and move facial muscles.

The facial nerve, he said is the seventh cranial nerve and carries nerve fibres that control facial movement and expression. It also carries nerves that are involved in taste to the anterior two-thirds of the tongue where taste buds for sweetness and saltiness are located.

The facial nerve also travels close to the eighth cranial nerve, the vestibulocochlear nerve, which is responsible for hearing and balance, thus causing ear pain and vertigo in RHS patients, Dr Raymond said.

"The inflammation caused by the zoster virus makes the nerve ineffective."

Dr Raymond noted that it's "very rare" for paralysis to occur on both sides of the face.

Dr Vinod said symptoms include decreased movement of one side of the face, a painful rash with fluid-filled blisters, hearing loss, ringing in the ear, vertigo, dry mouth, dry eyes and change in taste.

FULL RECOVERY POSSIBLE, BUT...

Dr Raymond shared that the mainstay treatment for RHS is antiviral therapy and corticosteroids.

Acyclovir (800 mg orally five times daily) and valacyclovir (1g orally three times daily) tablets are the two recommended antivirals for RHS often administered for a duration of between seven and 10 days, he said.

At the same time, he said patients would be given steroids in the form of prednisolone to reduce inflammation.

He added that patients would undergo physiotherapy to retrain the facial muscles to become stronger with facial grimacing exercises to hasten recovery.

"Many of my patients complain of food or drink spilling off from their mouth on the side of the facial weakness and that annoys and frustrates them because they can't close their mouth.

"They think they are closing their mouth, but when they look in the mirror, the lips are parted."

The four main face muscles are - frontalis (responsible for elevating the eyebrow), orbicularis oris (controls movements of the mouth and lips), orbicularis oculi (closes the eyelids), buccinator (holds the cheek to the teeth and assists with chewing), and zygomaticus (controls how people smile).

In the three-minute video Bieber posted on Instagram, he said: "As you can see, this eye is not blinking. I can't smile on the side of my face.

"This nostril will not move. So there's full paralysis in the side of my face," he said gesturing to the right side of his face.

Dr Vinod said antiviral therapy is effective in decreasing VZV replication to prevent its proliferation and spread, but it does not eradicate the existing virus.

"Antivirals help to reduce the duration of viral shedding and new lesion formation while hastening rash healing.

"RHS can be a very painful condition, too. Pain control with multiple types of medications, including steroids is important during treatment and even after the rash clears and facial movement returns.

"Because patients have difficulty closing the eye due to facial nerve paralysis, eye moisturisation and lubrication are very important. Medication for nausea and dizziness is also helpful during treatment to support recovery," Dr Vinod added.

Dr Vinod however, noted that the therapy has a short therapeutic window and must be initiated within 72 hours of rash onset for it to be effective.

MISDIAGNOSED, DELAYED TREATMENT: EDUCATING GPs, MOs

Dr Raymond said while people generally seek treatment upon noticing facial asymmetry, the problem could lie with junior or inexperienced doctors, who are often the first point of contact, who fail to rightfully diagnose zoster.

He said diagnosis is often missed or delayed, which can lead to an increased incidence of long-term complications.

"The first thing which comes to people's minds when they notice facial asymmetry is stroke, so they will visit a doctor. They will also come in if they spot painful rash.

"Treatment must start within 48 to 72 hours of symptom onset. A lot of patients think that they had a stroke and would have gone to the emergency department and sometimes junior doctors or medical officers might say it is Bell's palsy and send them home, often with a suboptimal dose of oral steroids.

"They miss the zoster. So I am more worried about that. We have to educate doctors as well, not just the public."

RECOVERY TAKES WEEKS TO A YEAR

Dr Raymond said patients may take from a few weeks to a year to recover from RHS, depending on the severity of the condition.

"Recovery is certainly possible. This is also based on my personal experience of being infected with zoster. I took prednisolone and Acyclovir and it disappeared fast.

"Recovery takes a few weeks to months for mild or partial paralysis (e.g. upper and lower eyelids meet but they can't shut tightly), but if it is total paralysis (e.g. eyelids don't meet at all), it might take up to a year.

"If Bieber had received treatment within two to three days of symptom onset and followed the doctor's advice, he would have a 100 per cent recovery rate, sometimes within a few weeks of treatment.

"If Bieber is suffering from complete (or total) paralysis, full recovery might take up to a year. It is best not to put him under any stress at the moment," Dr Raymond said.

Dr Raymond shared that doctors will also conduct Nerve Conduction Studies on patients with paralysis to determine the degree of nerve damage.

"This test helps find the extent of diseases that damage the nerves and muscles and doctors can be informed of the chances of recovery."

HOW SEVERE IS BIEBER'S CONDITION?

Professor Derick Wade, an expert in neurological rehabilitation at Oxford Brookes University, recently told Sky News that although most people recover fully, Bieber appears to have a serious case of the virus.

Wade said Bieber appears to have suffered "quite severe loss" of movement in his face and could be left with paralysis "long term".

In the video Bieber shared that his condition is "pretty serious".

"For those who are frustrated by my cancellations of the next shows, I'm just physically not capable of doing them.

"I wish this wasn't the case. But obviously, my body's telling me I gotta slow down. I hope you guys understand. And I'll be using this time to just rest and relax and get back to 100 per cent so that I can do what I was born to do. But in the meantime, this ain't it.

"I'm doing all these facial exercises to get my face back to normal and it will go back to normal, just that we don't know how much time that's going to be but it's going to be okay," Bieber added.

The 'Justice' world tour has already been rescheduled thrice thus far. Further delays happened earlier this year after Bieber tested positive for Covid-19.

Justice World Tour on Instagram shared a post noting that: "Justin is receiving the best medical care possible and is determined to resume the tour as soon as he and the doctors feel he is able to continue."

On June 14, Bieber took to Instastory to share an update on his condition.

"Each day has gotten better and through all of the discomfort, I have found comfort in the one who designed me and knows me. I'm reminded he knows all of me… this perspective has given me peace during this horrific storm that I'm facing. I know this storm will pass…," he wrote.

FAILURE, DELAY IN TREATMENT-SEEKING: HOW DOES THE DISEASE PROGRESS?

Dr Raymond said chickenpox is regarded as a mild, self-limiting viral illness with occasional complications but it often takes a long time to recover.

"But when the virus resurfaces as zoster and if the vesicles pop, it can cause secondary bacterial infection if the zoster is extensive, particularly in immunosuppressed people.

"This can go into the blood and cause septicemia (blood poisoning by bacteria), which if not recognised early and managed promptly can lead to death."

He said delayed RHS treatment could also lead to permanent facial paralysis for life or 'aberrant reinnervation' (well-known complication of facial nerve injury).

"(Aberrant reinnervation) this is where fibres that regrow do not grow back to the correct target which is supposed to be the facial muscle, some of the fibres get targeted to the wrong organ, like the tear gland or sweat gland.

"Hence why some patients complain they tear up or sweat while eating when the food is not even spicy."

RHS PREVENTION

"There is none. It is almost like cancer, like how do you prevent cancer? You can minimise your risk of cancer such as by not smoking," Dr Raymond said.

For RHS, he said stress ought to be managed as it depresses the immune system.

"But today we are living in a world where everybody is stressed.

"A local public university had conducted a study on Orang Asli in Malaysia and it found that they are probably the least stressful people because unlike us, they do not have deadlines or assignments. The study showed that none of them had migraine.

"However, migraine is almost a common disease for us city dwellers. Women have a 20 per cent chance of getting a migraine in a lifetime, while men have five per cent. Migraine never existed before. We invented this disease, a disease of city dwellers," Dr Raymond said. 

MINIMISING ZOSTER RISK WITH VACCINATION

Dr Vinod said since the varicella-zoster vaccine was first introduced in 1997, the risk of getting chickenpox has been dramatically reduced.

Today, there are two different vaccines used to immunise against chickenpox: Varivax (varicella virus vaccine), which is a single vaccine and ProQuad (MMRV), a combination vaccine that is also used to prevent measles, mumps, and rubella.

Both vaccines, he said are live attenuated vaccines, meaning that they contain live weakened viruses that cannot cause disease.

When delivered as prescribed, two doses of the chickenpox vaccine can reduce the risk of chickenpox by 88 per cent to 98 per cent, Dr Vinod added.

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