Ramsay Hunt Syndrome (RHS)

What is Ramsay Hunt Syndrome (RHS)

Ramsay Hunt syndrome is a viral infection that affects the nerves in the face and the nerves near the inner ear. The infection is caused by the varicella zoster virus (VZV). This is the same virus that causes chickenpox and shingles.

After a person has chickenpox, this virus may become inactive. Years later, the virus can become active again and cause Ramsay Hunt syndrome. The trigger may be something that weakens the body’s defense system (immune system), like stress.

When VZV becomes activated, it moves up the facial nerve and causes a painful rash in or around the ear canal. It may also travel up the nerve that is responsible for hearing.

Ramsay Hunt syndrome is a herpetic infection of the geniculate ganglion of the facial nerve. Symptoms include ear and facial pain, lower motor neuron facial weakness, and vesicular eruption around the external auditory canal. Treatment comprises oral acyclovir and corticosteroids.

Ramsay Hunt syndrome cannot be passed from person to person (it is not contagious). However, if a person who has never had chickenpox comes in contact with fluid from someone’s skin blisters, he or she may develop chickenpox.

Ramsay Hunt syndrome is the eponym given to acute herpes zoster involvement of the geniculate ganglion. The syndrome results from reactivation of the varicella-zoster virus (VZV) within the geniculate ganglion. VZV is also the causative agent of chickenpox (varicella). Primary infection in the nonimmune host manifests itself clinically as the childhood disease chickenpox. It is postulated that during the course of primary infection with VZV, the virus invades the geniculate ganglia. The virus remains dormant in the ganglia, producing no clinically evident disease. In some individuals, the dormant virus reactivates and travels along the pathways of the geniculate ganglion, producing the pain and skin lesions characteristic of shingles. The reason that reactivation occurs in only some individuals is not fully understood, but it is theorized that a decrease in cell-mediated immunity may play an important role in the evolution of this disease by allowing the virus to multiply in the ganglia and spread to the corresponding sensory nerves, producing clinical disease. Patients with malignancies (particularly lymphoma), patients who are receiving immunosuppressive therapy (chemotherapy, steroids, radiation), and patients with chronic diseases are generally debilitated and much more likely than healthy individuals to develop acute herpes zoster. These patients all have in common a decreased cell-mediated immune response, which may be the reason for their propensity to develop shingles.

This decreased immune response may also explain why the incidence of shingles increases dramatically in individuals older than 60 years and is uncommon in individuals younger than age 20.

The first division of the trigeminal nerve is the second-most common site for the development of acute herpes zoster after the thoracic dermatomes. Rarely, the virus may attack the geniculate ganglion, resulting in facial pain, hearing loss, vertigo, vesicles in the ear, and pain. This constellation of symptoms is called Ramsay Hunt syndrome and must be distinguished from acute herpes zoster involving the first division of the trigeminal nerve.

What are the causes?

This condition is caused by the varicella zoster virus.

What increases the risk?

You may be at risk for Ramsay Hunt syndrome if you have had chickenpox in the past.

What are the symptoms?

Symptoms of this condition include:

• A rash with blisters that breaks out around the ear. The rash may be accompanied by:
  • A rash in the inner ear, along the side of the face, or up the scalp.
  • A rash inside the mouth.
  • Deep and severe pain in the ear.
  • Severe, burning pain wherever the rash develops.
• Facial nerve weakness. This may cause:
  • Drooping on one side of the face.
  • Inability to close the eyelid on the affected side of the face.
  • Trouble eating.
  • Loss of ability to taste on the side of the tongue.

If RHS affects the inner ear nerve (auditory nerve), other symptoms may be present. These may include:

• Hearing loss.
• A spinning sensation (vertigo).
• Clumsiness.
• Ringing in the ear (tinnitus).

As viral reactivation occurs, ganglionitis and peripheral neuritis cause pain, which is generally localized to the segmental distribution of the geniculate ganglion. This pain may be accompanied by flu-like symptoms and generally progresses from a dull, aching sensation to dysesthetic neuritic pain in the distribution of the geniculate ganglion.

In most patients, the pain of acute herpes zoster precedes the eruption of rash by 3 to 7 days, often leading to erroneous diagnosis (see discussion of differential diagnosis).

As the disease progresses, the vesicles coalesce, and crusting occurs. The area affected by the disease can be extremely painful, and the pain tends to be exacerbated by any movement or contact (e.g., with clothing or sheets). As healing occurs, the crusts fall away, leaving pink scars in the distribution of the rash that gradually become hypopigmented and atrophic.

The clinical diagnosis of shingles is readily made, however, in most patients when the characteristic rash appears. Similar to chickenpox, the rash of herpes zoster appears in crops of macular lesions, which rapidly progress to papules and then to vesicles.

In most patients, the hyperesthesia and pain generally resolve as the skin lesions heal. In some patients, pain and neurological findings may persist beyond lesion healing. This most common and feared complication of acute herpes zoster is postherpetic neuralgia. Elderly patients are affected at a higher rate than the general population suffering from acute herpes zoster.

The symptoms of postherpetic neuralgia can vary from a mild, self-limited problem to a debilitating, constantly burning pain exacerbated by light touch, movement, anxiety, or temperature change. This unremitting pain may be so severe that it completely devastates the patient’s life, even leading ultimately to suicide.

To avoid these disastrous sequelae to a usually benign self-limited disease, the clinician must use all possible therapeutic efforts for the patient suffering from acute herpes zoster in the geniculate ganglion.

How is this diagnosed?

This condition may be diagnosed based on:

• Your symptoms.
• A physical exam.
• Other tests to confirm the diagnosis. These may include:
  • Viral culture. This test is done by swabbing the rash or blister to check for VZV.
  • Blood tests to check for antibodies to VZV. Antibodies are proteins that your body produces in response to germs.
  • Nerve conduction studies (electroneurogram).
  • MRI scan.
  • Hearing tests (audiology).

Although in most instances the diagnosis of acute herpes zoster involving the geniculate ganglion is easily made on clinical grounds, confirmatory testing is occasionally required.

Such testing may be desirable in patients with other skin lesions that confuse the clinical picture, such as patients with acquired immunodeficiency syndrome who have Kaposi’s sarcoma.

In such patients, the diagnosis of acute herpes zoster may be confirmed by obtaining a Tzanck smear from the base of a fresh vesicle, which reveals multinucleated giant cells and eosinophilic inclusions.

To differentiate acute herpes zoster from localized herpes simplex infection, the clinician can obtain fluid from a fresh vesicle and submit it for immunofluorescent testing.

Differential Diagnosis

Careful initial evaluation, including a thorough history and physical examination, is indicated in all patients suffering from acute herpes zoster involving the geniculate ganglion to rule out occult malignancy or systemic disease that may be responsible for the patient’s immunocompromised state and allow early recognition of changes in clinical status that may presage the development of complications, including myelitis or dissemination of the disease.

Other causes of pain in the distribution of the geniculate ganglion include trigeminal neuralgia, sinus disease, glaucoma, retroorbital tumors, inflammatory diseases such as Tolosa-Hunt syndrome, and intracranial pathology, including tumors.

How is this treated?

This condition may be treated with:

• An antiviral medicine to treat the virus.
• NSAIDs or prescription pain relievers to control pain.
• An anti-inflammatory medicine (steroid) called prednisone.
• Antibiotic medicine, if the rash becomes infected.

If treatment starts within the first 3 days of having symptoms, it will shorten the course of the pain and rash that is caused by RHS. Treatment will also prevent your facial nerve from continuing to weaken. Without treatment, it is possible that you may not recover full use of your facial nerve.

The therapeutic challenge of a patient with acute herpes zoster involving the geniculate ganglion is twofold: (1) to provide immediate relief of acute pain and symptoms and (2) to prevent complications, including postherpetic neuralgia.

It is the consensus of most pain specialists that the earlier in the natural course of the disease that treatment is initiated, the less likely it is that the patient will develop postherpetic neuralgia. Because older patients are at highest risk for developing postherpetic neuralgia, early aggressive treatment of these patients is mandatory.

Nerve Blocks

Sympathetic neural blockade with local anesthetics and steroids via stellate ganglion block seems to be the treatment of choice to relieve the symptoms of acute herpes zoster involving the geniculate ganglion and to prevent the occurrence of postherpetic neuralgia. Sympathetic nerve block is thought to achieve these goals by blocking the profound sympathetic stimulation that results from the viral inflammation of the nerve and geniculate ganglion. If untreated, this sympathetic hyperactivity can cause ischemia secondary to decreased blood flow of the intraneural capillary bed. If this ischemia is allowed to persist, endoneural edema forms, increasing endoneural pressure and causing a further reduction of endoneural blood flow with irreversible nerve damage.

As vesicular crusting occurs, the addition of steroids to the local anesthetic may decrease neural scarring and decrease further the incidence of postherpetic neuralgia.

These sympathetic blocks should be continued aggressively until the patient is pain free and should be reimplemented at the return of pain. Failure to use sympathetic neural blockade immediately and aggressively, especially in elderly patients, may sentence the patient to a lifetime of suffering from postherpetic neuralgia.

Occasionally, some patients suffering from acute herpes zoster involving the geniculate ganglion may not experience pain relief from stellate ganglion block, but they do respond to blockade of the trigeminal nerve. Ultrasound needle guidance may improve the accuracy of needle placement and decrease needle-related complications when performing stellate ganglion block.

Opioid Analgesics

Opioid analgesics may be useful in relieving the aching pain that is often present during the acute stages of herpes zoster as sympathetic nerve blocks are being implemented. They are less effective in the relief of the neuritic pain that is often present. Careful administration of potent, long-acting opioid analgesics (e.g., oral morphine elixir or methadone) on a time-contingent rather than as-needed basis may represent a beneficial adjunct to the pain relief provided by sympathetic neural blockade. Because many patients with acute herpes zoster are elderly or may have severe multisystem disease, close monitoring for the potential side effects of potent opioid analgesics (e.g., confusion or dizziness, which may cause a patient to fall) is warranted. Daily dietary fiber supplementation and Milk of Magnesia should be started along with opioid analgesics to prevent the side effect of constipation.

Adjuvant Analgesics

The anticonvulsant gabapentin represents a first-line treatment in the palliation of neuritic pain of acute herpes zoster involving the geniculate ganglion. Studies suggest that gabapentin also may help prevent the development of postherpetic neuralgia. Treatment with gabapentin should begin early in the course of the disease, and this drug may be used concurrently with neural blockade, opioid analgesics, and other adjuvant analgesics, including the antidepressant compounds if care is taken to avoid central nervous system side effects. Gabapentin is started at a bedtime dose of 300 mg and is titrated in 300-mg increments to a maximum dose of 3600 mg given in divided doses as side effects allow. Carbamazepine should be considered in patients with severe neuritic pain who have failed to respond to nerve blocks and gabapentin. If this drug is used, rigid monitoring for hematological parameters is indicated, especially in patients receiving chemotherapy or radiation therapy. Phenytoin also may be beneficial to treat neuritic pain, but it should not be used in patients with lymphoma because the drug may induce a pseudolymphoma state that is difficult to distinguish from the actual lymphoma itself.

Antidepressants also may be useful adjuncts in the initial treatment of patients with acute herpes zoster. On an acute basis, these drugs help alleviate the significant sleep disturbance that is commonly seen with acute herpes zoster. In addition, antidepressants may be valuable in helping ameliorate the neuritic component of the pain, which is treated less effectively with opioid analgesics. After several weeks of treatment, the antidepressants may exert a mood-elevating effect that may be desirable in some patients. Patients must be observed closely for central nervous system side effects. These drugs may cause urinary retention and constipation that may be mistakenly attributed to herpes zoster myelitis.

Antiviral Agents

A few antiviral agents, including famciclovir and acyclovir, have been shown to shorten the course of, and may help prevent the development of, acute herpes zoster. They are probably useful in attenuating the disease in patients with immunosuppression. These antiviral agents can be used in conjunction with the treatment modalities mentioned earlier. Careful monitoring for side effects is mandatory with these drugs.

Adjunctive Treatments

The application of ice packs to the lesions of acute herpes zoster may provide relief in some patients. Application of heat increases pain in most patients, presumably because of increased conduction of small fibers, but it is beneficial occasionally and may be worth trying if application of cold is ineffective. Transcutaneous electrical nerve stimulation and vibration also may be effective in a few patients. The favorable risk-to-benefit ratio of all these modalities makes them reasonable alternatives for patients who cannot or will not undergo sympathetic neural blockade and do not tolerate pharmacological interventions.

Topical application of aluminum sulfate as a tepid soak provides excellent drying of the crusting and weeping lesions of acute herpes zoster, and most patients find these soaks soothing. Zinc oxide ointment also may be used as a protective agent, especially during the healing phase when temperature sensitivity is a problem. Disposable diapers can be used as an absorbent padding to protect healing lesions from contact with clothing and sheets.

Follow these instructions at home:

Medicines

• Take over-the-counter and prescription medicines only as told by your health care provider.
• If you were prescribed an antibiotic or antiviral medicine, take or apply it as told by your health care provider. Do not stop using the antibiotic or antiviral medicine even if your condition improves.
• Do not drive or use heavy machinery while taking prescription pain medicine.
• If you are taking prescription pain medicine, take actions to prevent or treat constipation. Your health care provider may recommend that you:
  • Drink enough fluid to keep your urine pale yellow.
  • Eat foods that are high in fiber, such as fresh fruits and vegetables, whole grains, and beans.
  • Limit foods that are high in fat and processed sugars, such as fried or sweet foods.
  • Take an over-the-counter or prescription medicine for constipation.

General instructions

• If told by your health care provider, use artificial tears and wear an eye patch to protect your eye until you can close your eyelid again.
• Do not scratch or pick at the rash.
• Put a cold, wet cloth (cold compress) on the itchy area as told by your health care provider.
• If you have blisters in your mouth, do not eat or drink spicy, salty, or acidic things. Soft, bland, and cold foods and beverages are easiest to swallow.
• Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if:

• Your pain medicine is not helping.
• You have chills or fever.
• Your symptoms get worse.
• Your symptoms have not gone away after 2 weeks.
• You have any changes in vision.

Summary

• Ramsay Hunt syndrome (RHS) is a viral infection that affects the nerves in the face and the nerves near the inner ear. The infection is caused by the varicella zoster virus (VZV), which also causes chicken pox and shingles.
• After a person has chickenpox, this virus may become inactive. If the inactive VZV virus becomes activated, it moves up the facial nerve and causes a painful rash in or around the ear canal. It can also cause hearing loss and facial nerve weakness, with drooping on one side of the face.
• If treatment starts within the first 3 days of having symptoms, it will shorten the course of the pain and rash that are caused by RHS. Treatment will also prevent your facial nerve from continuing to weaken.
• Treatments may include antiviral, steroid, and pain medicines.

Complications and Pitfalls

In most patients, acute herpes zoster involving the geniculate ganglion is a self-limited disease. In elderly and immunosuppressed patients, complications may occur, however. Cutaneous and visceral dissemination may range from a mild rash resembling chickenpox to an overwhelming, life-threatening infection in patients already suffering from severe multisystem disease. Myelitis may cause bowel, bladder, and lower extremity paresis. Ocular complications from trigeminal nerve involvement may range from severe photophobia to keratitis with loss of sight.

Clinical Pearls

Because the pain of herpes zoster usually precedes the eruption of skin lesions by 5-7 days, erroneous diagnosis of other painful conditions (e.g., trigeminal neuralgia, glaucoma) may be made. In this setting, the astute clinician advises the patient to call immediately if a rash appears because the diagnosis of acute herpes zoster is a possibility.

Some pain specialists believe that in a few immunocompetent patients, when reactivation of virus occurs, a rapid immune response may attenuate the natural course of the disease and the characteristic rash of acute herpes zoster may not appear. This pain in the distribution of the geniculate ganglion without associated rash is termed zoster sine herpete and is, by necessity, a diagnosis of exclusion.

Other causes of head pain must be ruled out first before invoking this diagnosis. Because of the potential for hearing loss in Ramsay Hunt syndrome, patients should be warned of this possibility to avoid erroneously blaming this complication on a therapeutic intervention, such as stellate ganglion block.