‘Ramsay Hunt syndrome is not completely new to Mumbai’: Experts on Justin Bieber’s video about the disorder

20 June,2022 10:32 AM IST |  Mumbai  |  Sarasvati T

After singer Justin Bieber brought attention of the world to the Ramsay Hunt syndrome, there has been greater curiosity among people about the neurological disorder. Though RHS is rare, city neurologists say they have treated other conditions closely related to it

Justin Bieber during a performance. Image credit: AFP

In a message to his fans about the cancellation of his upcoming concerts, Canadian singer Justin Bieber opened up about a viral infection ‘Ramsay Hunt syndrome* (RHS) he was suffering from, in an Instagram post on June 11. In the video, the performer showed how the condition had paralysed one side of the performer*s face and that he will need time to rest and recover.

Ever since Bieber highlighted the effects of the viral infection, which started from his ear, there has been an increased discussion about the condition which has a prevalence of around five cases per one lakh persons a year according to several medical journals. According to Dr. Arun B Shah Director, Neurosciences at Sir HN Reliance Foundation Hospital, it is a relatively rare neurological disorder found among Indians. "However, a similar illness bell*s palsy is common and mostly found in people across Mumbai. We see four out of the five patients being treated for Bell*s palsy in a period of one-two month," he adds.

While both indicate similar symptoms, the severity of RHS exceeds that of Bell*s palsy. Mid-Day Online reached out to Dr Shah, Dr Pavan Pai, Consultant Interventional Neurologist, Wockhardt Hospitals, and Dr. Pradyumna J. Oak, director and head – Neurology at Nanavati Max Super Speciality Hospital to know more about the disorder and its treatment.

What is Ramsay Hunt Syndrome?

Shah: RHS is a rare viral infection. In fact, you could say that it*s a reactivation of a virus lying dominant in the body, particularly affecting the nerve cells. It causes pain in the auditory meatus and the pinna of one ear. As the pain gets severe, vesicles may appear in the ear and facial paralysis is noted on the affected side. Vesicles may appear on the hard palate as well. This virus is similar to the chickenpox virus, so when you get an infection the virus remains dormant in the body and when your resistance power goes down it gets reactivated and may affect other parts of the body.

What are the causes of RHS?

Pai: RHS is seen in those people who have had chickenpox. Once the person recovers from chickenpox, the virus stays in your body and can reactivate in later years to cause shingles (a painful rash). Hence, this syndrome is a shingles outbreak that impacts the facial nerve near one of your ears. It can happen to those who have had chickenpox in the past. This syndrome is commonly seen in older people. You need to know that it is not contagious. But, reactivation of the varicella-zoster virus can cause chickenpox in people who haven*t had chickenpox or didn*t get vaccinated for it. This infection can be problematic for those with low immunity. It can affect people of any age group but is rarely seen in children.

Shah: It can affect people above the age of 30-40, but this illness is more likely to be detected among the elderly aged group i.e. mostly above 50 plus as well as in children. Additionally, patients with a past history of diabetes, or any illness that causes suppression of immunity have more chances of being diagnosed with this illness.

How common is it in India?

Oak: RHS is not the rarest of rare diseases and we do see and treat these patients on a regular basis. Unfortunately, since there is no reportage of the disease, there is no centralised data on RHS. However, on an average, less than one percent of zoster cases concern the facial nerve and cause RHS and by those standards, about 6000-7000 cases of RHS must be reported in India every year.

What are the early signs of the syndrome and when should one consult a doctor?

Pai: The symptoms of this condition are painful rashes with fluid-filled blisters on, in, and around the ear, facial weakness or paralysis on the same side as the affected ear, ear pain, hearing loss, vertigo, and inability to close the eye, dry mouth and eyes and tinnitus. You need to consult the doctor once you notice the red flags.

To diagnose this condition, your treating doctor will suggest some tests. You will be asked to opt for blood tests for varicella-zoster virus, an MRI of the head, nerve conduction (to check for the damage to the facial nerve), and skin tests for varicella-zoster virus. The doctor will thoroughly evaluate you, understand the symptoms, and then only suggest an appropriate test. Once you have been detected with this syndrome then your doctor will decide a proper line of treatment for you. You need to follow all the instructions given by the doctor.

Shah: Generally, it would start with probably extreme pain in the pinna, the area near the ears followed by redness or spots that can be observed growing over a span of days and then developing facial pain or facial paralysis. The patient should immediately visit or consult his general physician to understand the further treatment process. In this case, the patient may be unable to blink his one eye or observe his speech get altered, the checks are also becoming weak angles of the mouth, excessive lacrimation of eye and saliva dripping from one side of his mouth, and overall the face looks different as it appears crooked.

Is it curable? How long does it take for a patient to fully recover?

Oak: We have observed excellent treatment results in RHS patients who visited the hospital immediately after the onset of symptoms. The condition is primarily managed medically, using antivirals in conjunction with steroids. In most patients, the symptoms relieve after seven days and a follow-up module of physiotherapy puts them on the path to recovery. However, the damage of facial paralysis or hearing loss can be permanent in patients, if the diagnosis and treatment are delayed.

How do environmental conditions and lifestyle changes influence the risk of getting the illness?

Pai: Various studies confirm that people often recall cooling of the face or a cold draught just before the outburst of facial palsy. Bell*s palsy following airplane trips has also been reported. Viruses that have been linked to Bell*s palsy include those causing Respiratory illnesses (adenovirus), Flu (influenza B). The risk factors of BP are having an upper respiratory infection, such as the flu or a cold, diabetes, high blood pressure, obesity, and all of these can happen due to lack of exercise and sleep, stress and poor eating habits.

Do you think lack of RHS awareness among people is a major obstacle?

Oak: Awareness about RHS is extremely low in society and most individuals mistake it as a primary stage of full-body paralysis. This is also the reason why most patients seek timely medical care and recover successfully. Some try home remedies and avoid treatment, mistaking the condition as a temporary anomaly. This can be reduced by creating awareness about the condition and its treatment success rate.

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(Disclaimer: This article is not intended as a substitute for professional medical advice, diagnosis or treatment. Mid-day Online does not in any way endorse the accuracy, completeness, efficacy or timeliness of any advice or line of treatment mentioned in this article. Readers must always seek the advice of a certified medical practitioner and/or a mental health professional before deciding on or starting any course of treatment.)

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