Ramsay Hunt Syndrome

What is Ramsay Hunt Syndrome?

Ramsay Hunt Syndrome is an acute peripheral neuropathy commonly affecting the facial nerves and the internal ear canal functions. This disease is also knowns as the Hunt’s Syndrome which consists of erythematous vesicular rashes. A form of herpes zoster that is usually caused by the Herpes Zoster virus.

Image 1 source – en.wikipedia.org

The virus is also the same family as of Chickenpox (varicella viruses) causing vesicular eruptions in the skin. (Figure 1: A Painful Expression)

Who are at risk?

Patients who have a current condition or recurrent Bell’s palsy are prone and at risk for acquiring this syndrome. It is also common to patients who have been exposed or who are suffering from HIV infections. Making them at risk for varicella zoster infections.

Signs and Symptoms

The following physical manifestation and classic symptoms can also be assessed. ^[1][2][3]

• Vesicular rash of the ear or mouth
• Facial paralysis
• Ipsilateral lower motor neuron facial paresis/palsy
• Vertigo and ipsilateral hearing loss (CN VII)
• Tinnitus or ringing sensation in the ears
• Otalgia or ear pain
• Headaches
• Dysarthria or problem in articulating words and delivering speeches
• Gait ataxia or staggering gait
• Fever
• Cervical adenopathy

Spinal damages and brain nerve endings may cause the following signs that needs abrupt medical attention:

• Confusion
• Lightheadedness or drowsiness
• Headaches
• Limb weakness
• Nerve pain
• Nausea and vomiting
• Sensational alterations – The patient may be at risk for suffering from burns because of altered response to heat or cold.

Causes

The common causes of Ramsay Hunt Syndrome are the following reasons:

• Suppressed immune system
• Exposure to Chickenpox varicella
• HIV patients who are exposed with Chickenpox
• Stress and anxiety
• Damage in the facial nerve endings
• Infections and sepsis

Bell’s Palsy, Trigeminal Neuralgia & Acoustic Neuroma from Ramsay Hunt Syndrome?

The disorders and diseases when it comes to neuropathy and peripheral functions are very much alike and similar when it comes to their manifestations and clinical signs and symptoms: ^[2][3][5]

• Bell’s palsy is a non-progressive neurological disorder of the facial nerve (7th cranial nerve). However Bell’s palsy is characterized by the sudden onset of facial paralysis unlike Ramsay Hunt Syndrome where there is already damage in the facial nerve endings.

• Acoustic neuroma on the other hand is a benign tumor of the 8th cranial nerve. The nerve affected is the hearing nerve endings. Pressure on this nerve results in the early symptoms of acoustic neuroma where there is  a ringing sound in the ear (tinnitus), and/or hearing loss may occur.
• Trigeminal neuralgia also known as tic douloureux, is a disorder of the 5th cranial nerve (trigeminal nerve) characterized by attacks of intense pain and very much sensitive to cold to hot temperature which causes and triggers the stabbing pain affecting the mouth, cheek, nose, and/or other areas on one side of the face. Causing too much stress on the patient.

Diagnosis & Tests

Series of tests and examination are done to assess and diagnose this syndrome. The following laboratory studies are done: ^[2][3]

• PCR test or polymerase chain reaction
• Blood tests for varicella zoster virus – The blood is being extracted and test for any possible multiplication of viruses in the blood.
• Electromyography (EMG)
• Lumbar puncture – To diagnose if there are damaged nerve endings and problem affecting the brain waves and activities.
• Magnetic Resonance Imaging (MRI) of the head – To tests and see an image of possible injuries and scarring on the neurons and brain linings.
• Nerve conduction (to determine the amount of damage to the facial nerve)
• Skin tests for varicella zoster virus

Treatment

The patient who suffers from Ramsay Hunt’s Syndrome usually takes tons and series of medications to palate and recover from the signs and symptoms. ^[1][2][3]

Medications and Drugs

Corticosteroids and oral acyclovir – This drug is used in the treatment of Ramsay Hunt syndrome. To alleviate the erythematous vesicular rashes.

• Alacyclovir
• Famciclovir
• Vestibular suppressants may be helpful if vestibular symptoms are severe.
• Carbamazepine – Treating the idiopathic geniculate neuralgia.
• Analgesics and painkillers
• Anti-viral medications
• Anti-seizure medications

If possible movement is affected, the patient can also undergo series of physical therapy to assist and aid in the activities of daily living (ADLs) of the patient.

Prognosis and Recovery rate

Only less than 50% of the total population can be affected and can recover from this syndrome. Since the disease is a self-limiting one, it can only cause morbidity cases and not of mortality. Unlike other neuropathy disorders that can be manifested and can last for a lifetime. ^[1]

Complications

The series of signs and symptoms can also cause various complications on the physical and systemic aspect of the patient. The patient may experience:

• Facial paralysis
• Facial disfigurement where the physical appearance of the skin is affected and altered.
• Taste palettes are altered. May confuse the different taste types.
• Optical damages due to affected facial nerve endings. Eye complications such as corneal ulcers and infections can lead to loss of vision.
• Abnormal nerve degeneration.
• Ear abnormalities (similar to Acoustic neuroma)
• Postherpetic neuralgia or the severe and persistent pain
• Muscle spasm of the face or eyelids
• Brain damage and nerve damages caused by the virus.
• If these symptoms occur, a hospital stay may be needed. A spinal tap may help determine whether other areas of the nervous system have been infected.
• Burns due to sensational alteration from heat and cold stimulus.

Pictures

References:

1. http://emedicine.medscape.com/article/1166804-overview
2. http://www.medicinenet.com/ramsay_hunt_syndrome/article.htm
3. https://www.nlm.nih.gov/medlineplus/ency/article/001647.htm
4. http://www.facialpalsy.org.uk/about-facial-palsy/causes-diagnoses/ramsay-hunt-syndrome/55
5. http://rarediseases.org/rare-diseases/ramsay-hunt-syndrome/