Bieber diagnosed with Ramsay Hunt syndrome: Read to know what health experts say about this rare neurological condition

By Shalini Bhardwaj
New Delhi [India], June 11 : American pop star Justin Bieber, who has been diagnosed with Ramsay Hunt Syndrome, which has caused him partial face paralysis, is a rare neurological condition characterized by paralysis of the facial nerve and a rash affecting the ear or mouth.
Speaking about the same, health experts from India said that this Syndrome damages the facial nerve near the ear causing hearing loss in addition to the severe shingles outbreak.
Dr Rajnish Kumar, Unit Head and Senior Consultant, Neurology at Paras Hospitals Gurugram told ANI that the same virus which causes chickenpox causes Ramsay Hunt syndrome and it can harm facial nerves.
"When a shingles outbreak damages the facial nerve near one of your ears, Ramsay Hunt syndrome develops. Ramsay Hunt syndrome can include facial paralysis and hearing loss in the afflicted ear, in addition to the severe shingles outbreak. The same virus that causes chickenpox causes it. The virus remains in your nerves after chickenpox has healed. It could rejuvenate years later. It can harm your facial nerves," said Dr Kumar.
Speaking about the signs and symptoms of the Ramsay Hunt syndrome, Dr Kumar said it has two primary signs and symptoms by which one can detect it.
"Ramsay Hunt syndrome has two primary signs and symptoms: 1. A painful red rash on, in, and around one ear with fluid-filled blisters and 2. Weakness or paralysis of the facial muscles on the same side as the afflicted ear," he said.
He further suggested seeing a doctor immediately if someone detects facial paralysis or a shingles rash.
"If you have facial paralysis or a shingles rash, see your doctor right away. Treatment that begins three days after the onset of symptoms may assist to prevent long-term problems," Dr Kumar added.
Another health expert, Dr Deepak Gupta, Professor Neurosurgery, AIIMS Delhi said that Ramsay Hunt syndrome is the second most common cause of atraumatic peripheral facial paralysis.
He said that the first most common disease of atraumatic peripheral facial nerve palsy is idiopathic Bell's palsy (most often caused by Herpes simplex virus type 1).
"Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy. Compared with Bell's palsy (facial paralysis without rash), patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely," said Dr Gupta.
He further said that the early treatment of all patients with Ramsay Hunt syndrome or Bell's palsy with a seven to ten days course of Famciclovir (500 mg, three times daily) or Acyclovir (800 mg, five times daily), as well as oral Prednisone (60 mg daily for 3-5 days) is recommended.
"Strict definition of the Ramsay Hunt syndrome is peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth," said Dr Gupta.
James Ramsay Hunt (1872-1937) received his MD from the University of Pennsylvania School of Medicine in 1893.
Facial palsy (traumatic) is seen by neurosurgeons mostly after head trauma and before/after acoustic neuroma (brain tumour) surgeries. Nowadays with improved DTI (diffuse tensor imaging), intraoperative facial nerve monitoring, improved surgical skills and operating timely before a tumour reaches big size, outcomes have improved and the incidence or falsy/injury has become significantly less.
The history and neurological examination remain the bases for diagnosing Ramsay Hunt syndrome. Examination of CSF and gadolinium-enhanced MRIs have had no diagnostic or prognostic value. Polymerase chain reaction (PCR) to detect VZV in exudates from the geniculate zone of the ear is more sensitive than VZV PCR performed on tears or blood mononuclear cells.
J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus.
He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal.
Hunt's analysis of clinical variations of the syndrome now bearing his name led to his recognition of the general somatic sensory function of the facial nerve and his defining of the geniculate zone of the ear. It is now known that varicella-zoster virus (VZV) causes Ramsay Hunt syndrome.
Studies suggest that treatment with prednisone and acyclovir may improve outcomes. Some patients develop peripheral facial paralysis without ear or mouth rash, associated with either a fourfold rise in antibody to VZV or the presence of VZV DNA in auricular skin, blood mononuclear cells, middle ear fluid, or saliva.
This indicates that a proportion of patients with "Bell's palsy" have Ramsay Hunt syndrome zoster sine herpete. Treatment of these patients with acyclovir and prednisone within 7 days of onset has been shown to improve the outcome of recovery from facial palsy.
Beyond steroids or combined steroid-antiviral treatments, various drugs (e.g. Nimodepine, mycophenolate mofetil) and new treatment options have also been explored. For long-standing facial paralysis and postparetic synkinesis sequelae after facial palsy, facial reanimation has been highlighted and the necessity of new paradigms has been raised.
Earlier in the day, Justin Bieber revealed that he is suffering partial face paralysis after he cancelled shows this week. The 28-year-old pop singer in a video on his Instagram account said that he has been diagnosed with Ramsay Hunt Syndrome, which has caused his condition.
Bieber announced that he will be taking a break from work as he needs time to recover. Apologising to his fans, he said that his condition is "pretty serious" and that is the reason why some of his shows were cancelled in recent times.
In the video, Justin explained, "As you can see, this eye is not blinking, I can't smile on this side of my face, this nostril will not move. So, there's full paralysis on this side of my face."
"So for those who are frustrated by my cancellations of the next shows, I'm just physically, obviously, not capable of doing them. This is pretty serious, as you can see."
Bieber said that he has been doing facial exercises to recover but that he does not know how long it will take to recover.