A rare neurological disorder that causes facial paralysis is in the spotlight after singer Justin Bieber revealed he couldn’t blink or smile.
The 28-year-old postponed his world tour and told fans in an Instagram video that he was being treated for Ramsay Hunt syndrome, a complication of shingles.
If you’ve never heard of it, you’re not alone.
Ramsay Hunt syndrome is a rare condition that is too often misdiagnosed as Bell’s palsy, a condition that also causes facial paralysis.
It’s actually so rare that it’s unclear exactly how many people have it, and those who are diagnosed often lean on each other for support and information.
It’s something Elizabeth Robinson has worked to change since being struck with it seven years ago.
What causes Ramsay Hunt syndrome?
Ramsay Hunt syndrome occurs in people who have had chickenpox because it is caused by the same virus.
The varicella zoster virus stays in your body and is harmless unless it is reactivated, usually due to stress.
This causes a painful rash called shingles.
Ramsay Hunt syndrome is a complication of shingles because the shingles travels to the inner ear, up the cranial nerves, and down the facial nerves, inflaming them.
This causes facial paralysis.
Shingles can also inflame or crush the auditory nerves, leading to hearing loss.
Ms Robinson, then a French teacher, was going through a particularly stressful time when she was diagnosed with Ramsay Hunt syndrome in January 2015.
She still hasn’t recovered from her serious condition, which paralyzed 95 percent of the left side of her face and left her left ear deaf.
“I haven’t been able to work as a teacher since,” she said.
“It affects my speech, my hearing, my balance.”
What are the symptoms of Ramsay Hunt syndrome?
Ms Robinson said her first symptom was an excruciating toothache.
“Not just one tooth, on all teeth on the left side,” she said.
“Then I had an excruciating earache like I had never experienced before – it felt like my ear was on fire.”
The doctor who checked her inner ear noted that it was slightly pink but her lymph nodes were not swollen, so she was referred to the dentist for an infected tooth.
That evening, Ms Robinson’s sister said her eye was starting to “look a little funny”.
“I said, ‘No, I’m just really tired, my ear hurts’, so I went to bed.
“At 4am I woke up and went to splash my face in the bathroom and get some water because my mouth was incredibly dry.
“[The water] just ran down my face.
After using the FAST method (Falling Face, Arm Weakness, Slurred Speech and Time), Ms Robinson ruled out the possibility of a stroke and waited to see the dentist, who referred her to a service emergency.
“By the time I got to the ER at nine in the morning, the shingles had grown so bad my ear was on fire and it was bright red,” she said.
Ramsay Hunt syndrome is often misdiagnosed as Bell’s palsy due to facial paralysis, and not all patients present with a rash immediately.
But Ms Robinson said the biggest difference between the two was the pain.
Mikaela Pretorius was 35 weeks pregnant with her second child in November 2014 when she developed a very bad headache.
Having had pre-eclampsia in her first pregnancy, she knew it was a possible sign of the pregnancy-related condition and went to the hospital.
“There was like, ‘Oh, there you go, have some Panadeine Forte. You know it’s just a headache, we checked it out – it’s fine,'” Ms Pretorius told the ABC.
“And I’m sitting on the bed [at hospital] and my husband is like, ‘You’re blinking funny.’
“We told the nurses and midwives and they said, ‘It’s just the headache and the stress, you’re fine.’
“They sent me home. The next day, still blinking weirdly.”
Ms Pretorius said she went to a medical clinic where she was told she needed an ambulance.
She spent three days in hospital and was warned that the steroids she was taking to treat the infection could cause her to go into early labor.
“I have permanent facial paralysis,” Ms Pretorius said.
“My right side of my face has a bit of movement, like I can blink again, but it’s not the same.
“I was lucky in that sense because it wasn’t in my ear, [it was in the back of my throat]so I haven’t had any long-term balance and hearing issues.”
What is the treatment for Ramsay Hunt syndrome?
Facial Palsy UK, a charity that supports people with facial palsy from any cause, says patients with suspected Ramsay Hunt syndrome should be given antivirals and steroids within 72 hours of symptom onset .
It says if this is done, around 70% of people will experience a “virtually complete recovery”.
Otherwise, the chances of a full recovery are reduced to 50%.
“The more severe the damage, the longer it will take to recover and the less likely you are to fully regain normal function,” the organization says.
Ms Pretorius said she had not seen anyone in support groups who had made a full recovery.
“If they had it in their ear, their face might have recovered, but not their hearing or their balance,” she said.
“In the semester [mark]they’re like, ‘This is as good as it gets.'”
The mother-of-two receives botox injections every three months to “unify” her face.
“I will have pain, like just muscle stiffness on both sides, because it throws the other side out as well,” Ms Pretorius said.
Ms Pretorius said she felt for Justin Bieber because she knew what he was going through.
“I’m sure there will be so many horrible, ‘It’s Justin Bieber before, it’s him now’ comparisons.
“It’s a terrible, terrible disease because it’s your face.
“You walk up to someone and say hello and you know they’re looking at you weirdly. It’s awful.”
There is no cure for Ramsay Hunt syndrome, but the severity of shingles can be lessened by getting the shingles vaccine.
Ms Robinson was given antivirals within 72 hours, but her paralysis was so severe she needed further treatment, which she did not receive.
Since then, she has been passionately committed to raising awareness of Ramsay Health Syndrome.
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