What is Ramsay Hunt Syndrome? Symptoms, causes and treatment
Ramsay Hunt syndrome (RHS) is a rare neurological disorder that causes painful rashes around the ears, face, or mouth. This rare disorder occurs when the varicella-zoster virus infects the nerves in the head. Ramsay Hunt Syndrome not only causes painful rashes around the face and ears, but also causes paralysis in the mouth, due to which one has to face serious problem of deafness in the affected ear. Ramsay Hunt syndrome is caused by varicella zoster virus (VZV), the same virus that causes chickenpox in children and shingles (herpes zoster) in adults. In cases of Ramsay Hunt syndrome, the previously inactive or weakened varicella-zoster virus becomes active again and spreads to affect the facial nerve.
What is the confusion regarding the name of Ramsay Hunt Syndrome?
Many different names have been used in the medical literature to denote this disorder, often causing confusion. The disorder is named after James Ramsay Hunt, a physician who first described this rare disorder in 1907. A few years ago, more than one disorder gave rise to the designation Ramsay Hunt syndrome. Ramsay–Hunt syndrome is now used to denote the disorder described in this report. This disorder is sometimes known as herpes zoster oticus because of the red ear rash. However, some physicians use herpes zoster oticus for the ear rash alone and Ramsay Hunt syndrome for the combination of ear rash and facial paralysis.
What are the symptoms of Ramsay Hunt syndrome?
Although many types of symptoms and signs are seen in a serious rare disorder like Ramsay Hunt Syndrome, it mainly has the following two symptoms by which it can be easily identified: –
Usually, the rash and facial paralysis occur at the same time. Sometimes one may occur before the other. Other times, the rash never occurs. Apart from the above two main symptoms, when Ramsay Hunt syndrome occurs, the patient experiences the following symptoms: –
What causes Ramsay Hunt syndrome?
Ramsay Hunt syndrome occurs in people who have had chickenpox. Once you recover from chickenpox, the virus remains in your body and sometimes reactivates years later (several years) to cause shingles, a painful rash with fluid-filled blisters. .
Ramsay Hunt syndrome is a shingles outbreak that affects the facial nerve near one of your ears. It can also cause unilateral facial paralysis and hearing loss.
What are the risk factors for Ramsay Hunt syndrome?
Ramsay Hunt syndrome can happen to anyone, especially someone who has had chickenpox or has not been exposed to chickenpox. It is more common in older adults, usually affecting people over the age of 60. Ramsay Hunt syndrome is a rare disease, but it is much rarer in children.
Ramsay Hunt syndrome is not contagious. However, reactivation of the varicella-zoster virus can cause chickenpox in people who have not previously had chickenpox or been vaccinated for it. Infection can be serious for people who have immune system problems.
Until the rash blisters go away, the following people should avoid physical contact: –
Anyone who has never had chickenpox or has not been vaccinated against chickenpox
Anyone who has a weak immune system
What complications can occur from Ramsay Hunt syndrome?
Many types of serious complications can occur due to Ramsay Hunt syndrome, which are described below: –
Permanent hearing loss and facial weakness: For most people, the hearing loss and facial paralysis associated with Ramsay Hunt syndrome is temporary. However, it may be permanent.
Eye damage:- Facial weakness due to Ramsay Hunt syndrome can make it difficult for you to close your eyelids. When this happens, the cornea that protects your eye can be damaged. This damage can cause eye pain and blurred vision.
Postherpetic neuralgia:- This painful condition occurs when shingles infection damages nerve fibers. The messages sent by these nerve fibers become confused and exaggerated, causing pain that can last long after the other signs and symptoms of Ramsay Hunt syndrome.
What are the disorders associated with Ramsay Hunt syndrome?
Although Ramsay Hunt syndrome is a rare disorder, there are other related disorders that cause similar symptoms as Ramsay Hunt syndrome. Symptoms of the following disorders may be similar to Ramsay Hunt syndrome: –
Bell’s palsy is a non-progressive neurological disorder of the facial nerve (7th cranial nerve). This disorder is characterized by the sudden onset of facial paralysis, which may be preceded by a mild fever, pain behind the ear on the affected side, stiff neck, and weakness and/or stiffness on one side of the face. The paralysis is believed to be caused by loss of blood supply (ischemia) and compression of the 7th cranial nerve. The exact cause of Bell’s palsy is not known. Viral and immune disorders are often implicated as the cause of this disorder. There may also be an inherited tendency to develop Bell’s palsy.
Acoustic neuroma is a benign tumor of the 8th cranial nerve. This nerve is located within the inner ear canal. The early symptoms of acoustic neuroma result from pressure on this nerve; Ringing in the ears (tinnitus), and/or hearing loss may occur. An associated compression of the facial nerve (7th cranial nerve) can cause muscle weakness; Pressure on the trigeminal nerve (fifth cranial nerve) can cause facial numbness. Extension of the tumor to different areas may result in impaired coordination of the legs and arms (ataxia), numbness in the mouth, slurred speech (dysphagia), and/or hoarseness.
Trigeminal neuralgia, also known as tic dolorex, is a disorder of the 5th cranial nerve, which innervates the mouth, cheeks, nose, and/or other nerves on one side of the face. Characterized by attacks of intense, stabbing pain affecting the areas. The exact cause of trigeminal neuralgia is not completely understood.
How is Ramsay Hunt Syndrome Diagnosed?
A doctor will probably make a diagnosis after noticing symptoms of facial weakness and a blister-like rash. A nerve conduction study may be performed to determine the extent of damage to the facial nerve as well as the likelihood of recovery.
Ramsay Hunt syndrome is commonly misdiagnosed because many doctors have never encountered the condition, due to it being a very rare disease.
A sample of fluid in one of the blisters in the ear may be taken to confirm the diagnosis. The doctor may also use a tear or blood sample.
An imaging scan, such as an MRI, can reveal swelling of the facial veins. The scans can also help the doctor determine whether the virus has reached the brain or other nerves. Yes, if paralysis occurs on the face, it can be quite easy to confirm or diagnose.
How is Ramsay Hunt syndrome treated?
Prompt treatment of Ramsay Hunt syndrome can reduce pain and reduce the risk of long-term complications. Medicines are used for its treatment, in which the following medicines are included in the treatment: –
Antiviral drugs:- Medicines like Acyclovir (Zovirax), Famciclovir (Famvir) or Valacyclovir (Valtrex) often help in dealing with the chickenpox virus.
Corticosteroids: A short regimen of high-dose prednisone appears to boost the effect of antiviral drugs in Ramsay Hunt syndrome.
Anti-anxiety drugs:- Medicines like diazepam (Valium) can help in relieving dizziness.
Painkiller: Pain associated with Ramsay Hunt syndrome can be severe. Prescription pain medications may be needed.
Lifestyle changes and home remedies :-
The following can help in reducing the discomfort of Ramsay Hunt Syndrome:-
Keep areas affected by rashes clean.
Apply a cool, wet compress to the rash to reduce pain.
Take an over-the-counter pain reliever or anti-inflammatory medication, such as ibuprofen (Advil, Motrin IB, others).
If facial weakness makes it difficult for you to close one eye, take the following measures to protect your vision:-
If your eyes become dry then use moisturizing eyedrops during the day as per doctor’s advice.
At night, apply eye ointment and close your eyelids or blindfold your eyes.
Give your eyes as much rest as possible.
Is it possible to prevent Ramsay Hunt Syndrome?
Yes, it is possible to prevent Ramsay Hunt syndrome. For this, it is most important that you get all the prescribed vaccinations for your children on time, so that they can be protected from all rare diseases. Keep in mind that these vaccines also include vaccination of your child against chicken pox i.e. both small and large mumps. Maintain proper cleanliness of your body so that the possibility of getting ringworm anywhere in the body can be eliminated. Especially when you are above 50 years of age, take special care of this. If any ringworm is seen on the body, then immediately talk to the doctor. For more information in this regard, you can also take necessary advice from your doctor.