Ramsay Hunt syndrome, the second most common cause of facial nerve paralysis which affects an estimated 100,000 Brits, is frequently being misdiagnosed, according to new research published for Facial Palsy Awareness Week (1-7 March) by charity Facial Palsy UK. 1 in 2 do not receive the correct treatment in time, risking permanent facial disability.
Ramsay Hunt syndrome is commonly confused with Bell’s palsy which struck Angelina Jolie, George Clooney and Pierce Brosnan in the past. The symptoms are similar- the face suddenly droops and sufferers find within a few hours they can no longer smile, blink, drink or eat. Whereas there’s no identified cause for Bell’s palsy, with Ramsay Hunt syndrome (RHS), the shingles attacks the facial nerve often causing severe headache or earache. Shingles is often associated with older people but with RHS, people in their twenties and thirties are more likely to be affected. Anyone who has had Chickenpox can get shingles. For the best chance of a full recovery people should be prescribed antivirals and a course of steroids within 72 hours.
Facial Palsy UK’s recent survey found that more than half of those with Ramsay Hunt syndrome were initially misdiagnosed and more than a third were not routinely followed up by their GP or hospital. 37 year old Deborah Lack from Shenley, Hertfordshire, says:
“In May 2013 I was diagnosed with Bell’s palsy by A&E and sent home with a low dose of steroids. A rash developed on the left side of my face a few days later but it took 12 days to be prescribed antivirals and it was another month before they diagnosed Ramsay Hunt syndrome. I still don’t have much movement on one side. I still dribble on that side, I can’t eat or smile properly. It is like your skin has been pulled tight all the time.”
44 year old Claudine Goldingham from London says:
“Stroke, tumour and viral ear infection were considered by the hospital when I was first admitted with chronic earache and facial palsy. My husband phoned a private ENT doctor with my symptoms who was the first person to suggest Ramsay Hunt syndrome. Doctors had dismissed it because they couldn’t see a rash. A spinal tap revealed masses of VZV (the shingles virus) in my spinal fluid.”
55 year old Steve Byles from Walton-on-the-Naze says:
“In December 2015 I had been unwell for four days with chronic ear pain. When I pointed out a rash on my head and around my ear to the doctor he told me to take antibiotics and sent me away. The next day my face dropped as if I had a stroke and a paramedic said it could be a reaction to the antibiotics. I went to an NHS walk-in centre and finally a young doctor diagnosed Ramsay Hunt syndrome. I was then told my face may never recover fully.”
Consultant Plastic and Reconstructive surgeon and facial paralysis expert, Katerina Anesti, says:
“Ramsey Hunt syndrome may be mistaken for Bell’s palsy, especially as the characteristic rash on the face or inside the mouth is not always present. Up to a third of seemingly Bell’s palsy cases are misdiagnosed, which means that patients are missing the antiviral treatments that could help their recovery.”
Catriona Neville, chair of Facial Therapy Specialists UK and Extended Scope Practitioner Physiotherapist says:
“In my experience approximately 1 in 3 patients are misdiagnosed with Bell’s palsy. Sometimes patients even wait years before being correctly diagnosed and treated. This lack of help has in my experience led to some patients even attempting to take their own life as they have found it so impossible to live with the long term effects of their facial palsy without appropriate support.”
As part of Facial Palsy Awareness Week (1-7 March) the charity is calling for more awareness about Ramsay Hunt syndrome, earlier diagnosis and better after care for those affected. For more information visit www.facialpalsy.org.uk
For further information call 0300 030 9333 or email Karen Johnson on firstname.lastname@example.org. Website www.facialpalsy.org.uk. Facial Palsy UK is a charity registered in England and Wales (1148115) and Scotland (SC045086)
Images available from: Facial Palsy UK, email@example.com Tel: 0300 030 9333
Notes to editors
RHS literature review 2016
Overall, patients with Ramsay Hunt syndrome achieved a high rate of complete recovery of the facial nerve function (70.4%) after the different proposed treatments. The combination of steroids and acyclovir reached better recovery rates than steroids in monotherapy. Dosage and period of treatment greatly varied among studies.
About Ramsay Hunt syndrome
Ramsay Hunt syndrome (RHS) is the name given to describe the symptoms of a shingles infection which causes a facial palsy, usually on just one side of the face. The facial nerve supplies the muscles used to control different areas of the face. As a result of the shingles virus, the facial nerve becomes inflamed and irritated. Shingles is caused by the same virus that causes chickenpox (varicella zoster virus, or VZV).
Bell’s palsy (BP) is the most common disorder and affects 11–40 persons per 100,000 each year, with peak incidence between 15 and 50 years. Lesions are not required for diagnosis of RH syndrome and around 2–35% of unilateral FNP without vesicles are actually herpes zoster sine herpete.
- The reported incidence of RHS is 5 cases/100,000 people.
- Medical research states up to 35% of Bell’s palsy cases may actually be a type of Ramsay Hunt syndrome.
- It affects mostly patients between the ages of 20 and 30
- Anyone who has had chickenpox can potentially develop Ramsay Hunt syndrome.
- It is caused by the varicella-zoster virus which is the same virus that causes shingles and chickenpox
- Ramsay Hunt syndrome is where shingles affects the facial nerve
- The reason why the shingles virus reactivates and causes Ramsay Hunt syndrome is unknown
- RHS affects males and females in equal numbers
- Prompt treatment with antiviral medication and steroids is recommended within 72 hours.
- If antiviral treatment is given within 72 hours, 70% will make a full recovery. If not given in this timeframe the likelihood of making a full recovery reduces to 50%.
- RHS is often confused with Bell’s palsy but symptoms tend to be more severe.
- Symptoms include: rash/blisters in or around the ear, scalp or hair line, or in the mouth (the rash is not always seen); weakness on the affected side of the face (facial paralysis), unable to blink or close the eye; altered taste on the tongue; difficulty eating, drinking and speaking; ear, face or head pain, dizziness/vertigo or tinnitus.
- A small number of people may not have a rash. This is called Zoster sine herpete (ZSH).
About the survey
The survey was carried out between June 2017 and February 2018 across England, Scotland, Wales and Northern Ireland.
The report shows:
- More than 1 in 2 people with Ramsay Hunt syndrome are initially misdiagnosed (57%)
- 1 in 2 did not receive the correct treatment of antivirals within the critical 72 hour window. (52%).
- More than 2 in 3 people were still experiencing health issues six months later (69%)
- Less than 2 out of 3 people are routinely followed up by their GP or hospital (63%)
- Less than 1 out of 3 felt their GP was knowledgeable about the condition. (31%)
- More than 1 in 3 were not routinely followed up by their GP or hospital (37%).
The report shows that for people who have had the condition for 6 months or more:
- More than 2 in 3 are suffering with facial pain (67%)
- More than 3 in 5 had been left with facial paralysis and are unable to make facial expressions (61%)
- More than half were suffering with dry eye (59%)
- More than half still had difficulties eating and drinking (50%)
- Almost 1 in 2 are suffering with anxiety (48%)
- More than 1 in 3 are suffering with depression (36%)
- 1 in 10 changed jobs or left employment as a direct result of Ramsay Hunt syndrome (10%).
Physiotherapy to treat faces is a specialist skill that is not routinely taught as part of physiotherapy training. There is limited evidence to support the use of physiotherapy to treat this patient group so often this treatment is denied by local health authorities because it’s not deemed cost effective.
The survey found that out of those who had accessed treatment:
- More than 3 in 4 found specialist physiotherapy helpful (76%) – but only 41% accessed
- More than 6 out of 7 people found botox injections helpful (88%) – but only 21% accessed
Access to treatment
Botulinum toxin is licensed for treating facial spasticity [https://bnf.nice.org.uk/drug/botulinum-toxin-type-a.html] and studies have shown that it is effective for synkinesis [https://www.ncbi.nlm.nih.gov/pubmed/28688864]
ENGLAND: A Freedom of Information request made by Facial Palsy UK in February 2017 found that just over 1 in 3 Clinical Commissioning Groups in England routinely offer botox for facial palsy patients (38%).
NORTHERN IRELAND: A Freedom of Information request made by Facial Palsy UK in May 2017 found that just one out of the five trusts routinely offered Botox for facial palsy patients. (20%)
SCOTLAND: A Freedom of Information request made by Facial Palsy UK in May 2017 found more than 1 in 5 health authorities do not routinely offer Botox for facial palsy patients (21%).
WALES: A Freedom of Information request made by Facial Palsy UK in May 2017 found that ALL treatments for facial palsy are considered to be specialised services in Wales and individual funding requests have to go through the Welsh Health Specialised Services Committee (WHSSC).
According to a survey about chronic pain by Pain Alliance Europe in June 2017, chronic pain is considered to be one of the main causes of disability, with considerable effects on negativity at work. It directly influences the budgets of social protection systems. http://www.pae-eu.eu/wp-content/uploads/2017/12/PAE-Survey-on-Chronic-Pain-June-2017.pdf
NHS link: https://www.nhs.uk/conditions/shingles/complications/
Last reviewed: 01-03-2018 || Next review due: 01-03-2025