A shingles outbreak will affect the facial nerve next to one of your ears and can result in Ramsay Hunt Syndrome. Ramsay Hunt Syndrome can result in acute facial paralysis and hearing loss or make an impact on the ear in addition to the excruciating shingles rash. Herpes Zoster Oticus is another name for the illness.
1. Why is it Called So?
James Ramsay Hunt, an American army officer, and Neurologist, first described the disorder in 1907, which is how it got its name.
A rare form of Cerebellar Degeneration known as Ramsay Hunt Syndrome type 1, also known as Ramsay Hunt Cerebellar Syndrome, causes shock-like jerks in one or more muscles, a number of uncommon neurological conditions, a trembling effect, and memory and thinking process loss.
Herpes zoster reactivation in the Geniculate Ganglion, also known as Herpes Zoster Oticus, causes Ramsay Hunt syndrome type 2.
Neuropathy affects the palmar portion of the ulnar nerve, which is referred to as Ramsay Hunt Syndrome type 3. The condition is also known as Hunt’s Disease and Artisan’s Palsy.
3. Ramsay Hunt Cerebellar Syndrome- Type 1
When analyzing the clinical neurologic findings, Ramsay Hunt syndrome will be taken seriously. Then add brain Magnetic Resonance Imaging, Varicella Zoster Polymerase chain reaction, and cerebrospinal fluid analysis to the diagnostic workup. During encephalitis, steroids and high-dose intravenous Acyclovir must be given for a longer period of time.
Delays in receiving treatment may frequently result in unsatisfactory outcomes with significant residual deficits.
Image by creativepic from Unlimphotos
3.1 Let’s see a case study- To know more about this Syndrome!
A 62-year-old man with a 3-day history of left ear pain and accompanying nausea and vomiting.
3.1.2 Suspected Causes
- The region of the throat used for breathing, swallowing, and talking, which contains the vocal cords, had squamous cell laryngeal cancer, but it was in remission,
- History of having a tracheostomy, which is an incision made in the windpipe to relieve a breathing obstruction,
- Type 2 diabetes mellitus, which causes the body to not be able to control the amount of glucose in the blood and causes the kidneys to produce a lot of urine,
3.1.3 Initial Investigations
Early laboratory studies discovered elevated levels of an unspecific acute phase reactants. On a CT scan, the left middle ear was enlarged, and the left soft palate was enhanced. Negative fungal cultures were discovered. The appropriate intravenous and topical antimicrobial treatments were started following the confirmation of the diagnosis of left malignant otitis externa.
The patient’s condition continued to deteriorate the following day, and a neurological exam revealed conductive hearing loss, skew deviation, persistent herpetic lesions on the hard palate and the outer ear, horizontal diplopia of the left eye, and other conditions. Nystagmus wasn’t present, and the clinical head impulse exam came back negative.
Brain MRI scan revealed a hyperintense lesion along the inferior and middle cerebellar peduncles of the fourth ventricle. Cerebrospinal fluid contained higher-than-normal levels of protein, glucose, and nucleated cells. Varicella-Zoster virus was found, according to the results of a quantitative polymerase chain reaction test. Herpes Simplex Virus type 1 and type 2 titers were unfavorable.
Finally, Ramsay Hunt Cerebellar Syndrome, a condition characterized by swelling of the brain and spinal cord, was discovered at this point, along with concurrent Varicella encephalitis.
The course of treatment included Empiric Antibiotic and Antifungal drugs.
Acyclovir 800 mg intravenously five times per day and Prednisone 70 mg orally every day, were given.
After one day, the patient’s visual deficits were significantly improved. The whole left facial weakness, abnormal reactions and vesicular lesions on the hard palate and on ear were still present. In addition to the mild lateral gaze palsy. He was given Acyclovir 20 mL five times daily for 14 days after spending some days in the hospital.
After four-month follow up, he had improved balance but continued to have left facial droop and left-side hearing loss. The patient was discharged from the hospital after receiving, course of 1 g of Valacyclovir three times per day due to the recurrence of left otic discharge, which is present in the region of the ear.
4. Ramsay Hunt Syndrome- Type 2
4.1 Who Will be Affected?
Anyone with chickenpox or shingles is susceptible to Ramsay Hunt Syndrome. People over 60 years old account for the majority of cases. Even though kids are rarely affected by the condition, children as young as three have had their condition identified. Immunodeficient individuals are more susceptible to this condition and typically display more severe symptoms. A complete recovery is also less likely for them.
4.2 Cause of the Syndrome
4.2.1 Varicella Zoster Virus Infection
One of nine herpes viruses known to infect humans is the Varicella Zoster virus, also known as Human Herpesvirus 3 (HHV-3, HHV3) or Human Alphaherpesvirus 3.
Ramsay Hunt syndrome is a form of acute herpes zoster caused by the Varicella-Zoster virus reactivating at the Geniculate Ganglion. For better understanding, this virus is the same one that causes Shingles and Chickenpox. The virus stays in your nerves even after chickenpox has healed. Years from now, it might start up again. The facial nerves could get impacted if this happens.
In another case, if a person comes in contact with the fluid from the blister of an infected person, the virus may spread to others. People who have never experienced the illness or received the chickenpox vaccine are most susceptible to developing chickenpox from it.
4.2.2 Is stress a factor in Ramsay Hunt Syndrome?
Stress is frequently a trigger. Stress is a state of tension that can be either physical or emotional.
Numerous studies have shown that stress can impair immunity and raise the risk of infection in those who experience high levels of stress. Because of this, it is believed that stress and shingles outbreaks are closely related, and that stress-related shingles may lead to Ramsay Hunt Syndrome.
Depending on which part of the facial nerve is affected by Ramsay Hunt syndrome, one side of your face may experience weakness or stiffness. It might be challenging to shut your eye as a result. It might be difficult to frown, smile or even wrinkle your forehead. Additionally, it can result in acute facial paralysis or drooping on one side. Speaking may become slurred, and eating may become challenging.
The nerves on one side of your face are typically the only ones affected by Ramsay Hunt syndrome. As a result, you might only notice symptoms on that side of your face. Other signs of the Ramsay Hunt syndrome could be:
- Throbbing rash on your earlobe, ear canal, and eardrum.
- The ear canal and earlobe blisters are filled with fluid.
- On the affected part, you may experience a rash on your tongue, throat, and mouth’s roof.
- Severe ear pain.
- Hearing ringing.
- Greater sensitivity to noise.
- One-sided hearing loss.
- Mouth and Eyes are dry.
- Taste sense declines.
- Social phobia brought on by facial anomalies.
- Vertigo might also result from Ramsay Hunt syndrome. The erroneous impression that the world is spinning or moving causes Vertigo.
The following conditions can have symptoms that are similar to Ramsay Hunt syndrome.
4.3.1 Bell’s Palsy
Bell’s palsy is a neurological condition affecting the facial nerve that does not get worse. Another name for Bell’s palsy is acute peripheral facial palsy of unknown origin. The symptoms of this condition include weakness and stiffness on one side of the face, a stiff neck, and abrupt onset of total facial paralysis. The affected side may also experience a mild fever and pain behind the ear.
In contrast to Ramsay Hunt syndrome, there is no rash, and the paralysis is less severe at first. Paralysis is thought to be caused by non-infectious inflammation and swelling of the 7th cranial nerve. Immune and viral disorders may be involved in Bell’s palsy, even though the exact cause is unknown. Additionally, Bell’s palsy may be an inherited propensity to develop.
4.3.2 Acoustic Neuroma
The vestibular nerve that connects your inner ear to your brain can develop a noncancerous tumor called an Acoustic Neuroma, also known as a Vestibular Schwannoma. The pressure from an Acoustic Neuroma can cause hearing loss, ringing sound inside the ears, and unsteadiness of ears.
Schwann cells that covers this nerve give rise to Acoustic Neuromas, which typically grow slow or not at all. Rarely, it may develop quickly and grow big enough to press-up against the brain and obstruct vital processes. Regular monitoring, radiation therapy, and surgical removal are all options for treating Acoustic Neuromas.
4.3.3 Trigeminal Neuralgia
An illness on the fifth cranial nerve known as Trigeminal Neuralgia is characterized by an episode of excruciating, stabbing pain that affects the mouth, cheek, nose, and other areas on one side of the face. It is unclear what causes trigeminal neuralgia exactly.
4.3.4 Congenital Facial Paralysis
Before or at the time of birth is considered congenital facial paralysis. The causes of this could be traumatic, environmental, or genetic. Forceps use or compression molding while in the birth canal can cause birth trauma.
4.3.5 Traumatic Facial Paralysis
The most common Traumatic cause of facial nerve injury is temporal bone fractures. The facial nerve passes through the temporal bone and behind the ear before emerging from the bone below the ear as it leaves the brain. The face muscles then reaches after it enters the parotid salivary gland. Temporal bone fractures are typically caused by high-velocity head trauma from falls and accidents. The facial nerve may swell or become cut if the fracture affects the bone that surrounds it.
The abnormal regeneration of facial nerve fibers is known as facial synkinesis, which leads to some muscles contracting unintentionally during other intended facial movements.
Patients with Ramsay-Hunt syndrome (30%) experience facial synkinesis more frequently than Bell’s palsy (10%) patients do. After the removal of a brain tumor, salivary gland tumor, or both, synkinesis may also appear if the facial nerve is injured or damaged due to head or facial trauma. Rather than just one area of the face, synkinesis frequently affects the mouth, neck, and eye. It has a different impact on every patient.
Treatment for facial muscle synkinesis requires a multidisciplinary approach, which may include a facial plastic surgeon, physical/occupational therapists, and speech-language pathologists.
For some patients with synkinesis, Botulinum toxin injections can be used as the sole form of treatment. Botulinum toxin’s ability to temporarily relieve some patients’ symptoms can help their surgeon recommend longer-lasting surgical options. These operations are referred to as selective Neurectomy. Your facial movement pattern and how you react to botulinum toxin injections are taken into account when creating an individualized treatment plan that includes these procedures.
4.4 The diagnosis of Ramsay Hunt Syndrome
Because the Ramsay Hunt syndrome symptoms such as earache, permanent facial paralysis, and the distinctive rash do not always manifest at the same time. So, diagnosing this condition can be challenging.
A thorough clinical examination, a thorough patient history, and the recognition of recognizable symptoms such as one-sided facial palsy and/or an ear rash are used to make the diagnosis. The diagnosis can be verified using a sample from the fluid-filled blistering rash that surrounds the ear. Ramsay Hunt facial paralysis, rather than Bell’s palsy, acoustic neuroma, or trigeminal neuralgia, is likely the cause of this rash.
Although viral tests can find the varicella-zoster virus in blood, tears, and saliva, they are not required to make a Ramsay Hunt syndrome diagnosis.
4.5 The Possible Difficulties of this Illness
Ramsay Hunt syndrome complications can include nerves that regrow to the wrong facial muscles structures. This condition may result in unusual responses to specific movements. For instance, your eye might close when you smile.
The following conditions may also cause complications,
- Loss of movement causes changes in facial appearance (disfigurement).
- Your sense of taste will change.
- Corneal ulcers, abrasions, and infections can cause eye damage and vision loss.
- Muscle spasms on the face.
- Social phobia.
You might also experience additional issues if the virus reactivates in other nerves, like your brain or spinal cord. These may consist of
- Your arms and legs are weak.
- Painful Nerves.
The House-Brackmann facial nerve palsy grading scale can be used to grade the severity of nerve palsy.
Grade 1 – Normal
Grade 2 – Mild Dysfunction
Grade 3 – Moderate Dysfunction
Grade 4 – Moderately Severe dysfunction
Grade 5 – Severe Dysfunction
Grade 6 – Total Paralyze Rehabilitation
Antiviral drugs like Acyclovir or Famciclovir and Corticosteroids like Prednisone are used for treating Ramsay Hunt syndrome. Together, the medications increase the effectiveness of treatment for the Varicella-Zoster virus. In particular, steroids or anti-inflammatory drugs may help to lessen pain by reducing nerve inflammation.
Despite treatment, some people may experience permanent hearing loss and facial paralysis. Specific symptoms can be noticeable in each person are the focus of additional treatment. This treatment includes painkillers, carbamazepine, an anti-seizure drug that may help lessen neuralgic pain, and vertigo-suppressing drugs like antihistamines and anticholinergics. Pain and vertigo can be effectively treated with anti-anxiety medications like diazepam (Valium).
To treat Postherpetic Neuralgia-related neuropathic pain, Capsaicin is the recommenced one. For the best effect, treatment should be started as soon as the onset of symptoms.
Ramsay Hunt syndrome patients must take extra precautions to avoid corneal damage because they have trouble closing one eye. These can put the cornea at risk of abnormal drying and irritation from foreign objects. The Cornea may be protected by using lubricating ointments and artificial tears. It might be advised to wear an eye patch for some patients.
Both the chickenpox vaccine for children and the shingles vaccine for those over 50 can protect against the Varicella-Zoster virus. These immunizations can significantly lower the risk of contracting the Varicella-Zoster virus, which lowers the risk of developing Ramsay Hunt syndrome.
It should be noted that the benefits and effectiveness of antiviral medications have not been proven yet.
5. Ramsay Hunt Syndrome- Type 3
Other names for the disorder include Artisan’s palsy and Hunt’s Disease.
5.1 Ulnar Nerve
The little finger is connected to the shoulder by the ulnar nerve. The muscles that allow you to precisely move the fingers are controlled by the ulnar nerve. The shock-like sensation you feel when you hit your elbow’s funny bone is prompted by the ulnar nerve.
5.2 Ulnar Nerve Palsy
If there is any damage to the ulnar nerve, there is a risk of losing feeling and developing hand muscle weakness. The medical term for this is Ulnar Nerve Palsy or Ulnar Neuropathy. The ability to perform a wide range of routine tasks and make precise movements may get impacted by this condition. When ulnar nerve palsy is severe, the muscles can atrophy and waste away the hands appears of a claw. The solution is surgery may be required, occasionally.
Ulnar nerve palsy is frequently a chronic condition that deteriorates over time. Ulnar nerve palsy signs and symptoms include,
- A loss of feeling in hand, particularly in the ring and little fingers.
- A decline in finger coordination.
- Severe Pain in hand that feels tingly or burning, hand weakness that might get worse with exercise, and a loss of grip power.
For Example, the ability to hold things or object can be hampered by a hand that lacks strength. The muscles in your hand may gradually tighten due to a lack of control and sensation, resulting in a claw-like deformity. Typically, ulnar nerve palsy only manifests itself in severe cases.
5.4 Causes of Ulnar Nerve Palsy
A disease that damages the nerve, an injury to the nerve, excessive pressure on the nerve from swelling and an elbow fracture or dislocation are the causes of Ulnar Nerve Palsy. Brain’s signals cannot reach their intended recipients in your arm and hand, nor can the hand properly receive them, which is similar to the function of Telephone. If the cord/cable of the telephone damage, then the receiver won’t work.
5.5 Ways for Early Diagnosis Ulnar Nerve Palsy
Following a physical examination, the doctor will inquire about symptoms whether the symptoms started before or after a hand injury. It can make it easier for the examiner to rule out potential causes of the condition. They’ll inquire about the nature of the injury, the duration of symptoms and what makes them worse.
Tests that may be performed in addition to a physical examination include,
- Imaging examinations like MRI and CT scans and nerve conduction tests
- The degree of dysfunction can be determined with the aid of a nerve conduction study.
5.6 How to Treatment this Palsy?
The Normal healing times for nerve tissues are much longer than those for other types of tissues. However, some ulnar nerve palsy symptoms might improve on their own.
The following are a few possible ulnar nerve palsy treatments,
- Medications to treat nerve spasms, such as Gabapentin, Carbamazepine or Phenytoin.
- Over-the-counter painkillers.
- Corticosteroids to treat inflammation.
- A splint to support the hand and ease uncomfortable symptoms.
- Physical therapy to strengthen and function muscles.
- To prevent further injury, occupational therapy.
Among the three different types of Ramsay hunt syndrome, the type 2 is very common.
6. Real World Cases of Ramsay Hunt Syndrome
Anupam Kher is a Indian Actor and Film Producer.
In 1994, during the filming of “Hum Aapke Hain Koun,” Anupam Kher claimed to have experienced facial paralysis. He immediately went to the director for help. The veteran actor informed the director of his condition. Although his face is twisted, he is prepared to shoot despite facial paralysis. He also said that facing problems increases self-confidence.
Apurva Asrani, a Indian writer, and National Award-winning filmmaker.
He revealed that he suffers from Ramsay Hunt Syndrome, a type of facial paralysis that affects muscle control on the afflicted side. The right side of his face was paralyzed when he first awoke. He was in pain for almost two months. Out of concern that he might be having a stroke, he was taken directly to the hospital. This was disregarded following an MRI scan. The root of this condition is excessive stress.
He found it difficult to eat, drink without spilling, close his right eye, smile, and even close his mouth. It was difficult to walk without tripping. His face sagged to one side, his mouth twisted, and one eye was wide and unblinking.
It is possible to reverse this condition, but it may take weeks, months, or even years in some circumstances. Severe vertigo was also present, and he was unable to stand upright. There was still no thaw even after using steroids, anti-viral, and weeks of physiotherapy and acupuncture. One senior neurologist warned him to be ready for the possibility that he might never fully recover from this if there was nerve damage.
Fortunately, the face started to thaw after a terrifying month. There is a small amount of progress every day, tiny twitches that promise to become something more significant tomorrow. And he was patiently waiting. In the next few months, there is an significant improvement. He admit himself that the fear has been indescribable.
Angelina Jolie is an American actress, Filmmaker, and Humanitarian.
Bell’s Palsy is a temporary facial paralysis similar to Ramsay Hunt Syndrome brought on by injury or trauma to the facial nerves. Her face drooped on one side as a result of the illness. Additionally, she was diagnosed with hypertension. Since then, with the aid of acupuncture, she has fully recovered.
George Timothy Clooney is an American Actor and Filmmaker was affected with facial nerves which leads him to facial paralysis during the early stage of his life.
Pierce Brendan Brosnan is famously known for playing James Bond in a series of films. He is an Irish American Actor who also affected with facial nerve palsy mainly known as Ramsay hunt syndrome presenting in 1980’s of his life. With the help of a steroid, Prednisone he recovered fully.
Justin Drew Bieber is a Canadian pop singer.
He was given a Ramsay Hunt Syndrome diagnosis in 2022. Through a video on Instagram, he was describing his disability. Ramsay Hunt Syndrome, a rare neurological condition, is to blame for the facial paralysis he experiences. The right side of his face is experiencing right-sided facial nerve paralysis as a result of this virus attack. He added that it is apparent that his right eye isn’t blinking in the same video. He can’t smile, at least not on the right side of his face.
7. Facts Box
- Ramsay Hunt Syndrome is reporting 5 cases per 100,000 people.
- According to medical research, Ramsay Hunt syndrome could be the cause of up to 35% of Bell’s palsy cases.
- Patients, mostly between the ages of 20 and 30, are affected by it.
- Ramsay Hunt syndrome is a condition that could affect anyone who has ever had chicken pox.
- It is brought on by the same virus that causes shingles, and chickenpox, varicella-zoster.
- The facial nerve is impacted by shingles in Ramsay-Hunt syndrome.
- The cause of Ramsay-Hunt syndrome, which is caused by the shingles virus reactivating, is unknown.
- RHS affects both men and women equally.
- Within 72 hours, prompt administration of steroids and antiviral medication is advised.
- 70% of patients will fully recovered if antiviral therapy is started within 72 hours. The chance of making a complete recovery drops to 50% if treatment is not provided within this time frame.
- Despite having more severe symptoms, RHS is frequently mistaken for Bell’s palsy.
- One or more of the following symptoms may be present- rash or blisters in or around the ear, scalp or hairline, or mouth; weakness on the affected side of the facial muscles, facial paralysis; inability to blink or close the eye; altered tongue taste; difficulty in eating, drinking, and speaking; severe pain on ear, face, or head; dizziness/vertigo; or tinnitus.
- There is a chance that some people won’t have a rash. Zoster sine herpete is the name for this condition(ZSH).
Ramsay Hunt syndrome is characterized by severe dysfunction and a worse prognosis for facial nerves than Bell’s palsy. If the nerve is not severely damaged, this condition can be fully reversible in a few weeks. Even after several months, if the damage is more severe, you might not fully recover.
In general, if you begin treatment within three days of the onset of the symptoms, your chances of recovering are better. Ramsay Hunt syndrome cannot be prevented, but it can be better managed by taking medication as soon as symptoms appear. Maintaining social distance from the person suffering from chicken pox is another important step in removing infection from their blister fluid.
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