Ramsay-Hunt Syndrome

I. What every physician needs to know.

Ramsay-Hunt syndrome (RHS), first described in 1907, is the triad of acute unilateral facial nerve palsy with ipsilateral otalgia and an erythematous vesicular rash of the auricle or oral mucosa. It is caused by reactivation of latent varicella-zoster virus (VZV) involving the geniculate ganglion of 7th cranial nerve (CN VII) and is also referred to as herpes zoster oticus with facial palsy. There is often involvement of CN VIII as well, manifested as hearing loss and vertigo. Occasionally, RHS can be associated with cranial polyneuropathy of CN IX, V and VI and X (in order of frequency), as these cranial nerves can have anastomotic connections to the facial nerve.

The facial paralysis associated with RHS is usually more severe than that of Bell’s palsy and has a worse prognosis. Early treatment with antiviral therapy and steroids may improve clinical outcomes.

II. Diagnostic Confirmation: Are you sure your patient has Ramsay-Hunt syndrome?

Typical patients present with a painful vesicular rash of the auricle, ear canal or oropharynx, as well as ipsilateral facial weakness. The pain is usually described as sharp, burning or stabbing and may precede the development of a visible rash by several days to a week. The facial weakness is unilateral and may progress over the initial days to first week of presentation. Patients may also complain of altered taste perception, saliva and tear production as well as hearing loss and vertigo.

A. History Part I: Pattern Recognition:

Patients with RHS, as with other patients with herpes zoster, often complain of pain prior to the development of a rash in the distribution of the CN VII. The facial weakness usually develops soon after, reaching a maximum by about 1 week after onset. The facial weakness is unilateral and affects all muscles of facial expression.

B. History Part 2: Prevalence:

The incidence of Ramsay-Hunt syndrome is estimated at 5 per 100,000 in the US population. It accounts for about 18% of unilateral facial palsies in adults and 16% in children. It is estimated that as many as 20% of cases diagnosed as Bell’s palsy actually are RHS without a rash (herpes sine herpete). The incidence increases with age. The incidence in immunosuppressed patients is unknown. However, in patients with HIV, a history of organ or hematopoietic transplant, as well as those with lymphoproliferative malignancies are at higher risk for varicella-zoster reactivation and thus are at higher risk for RHS.

C. History Part 3: Competing diagnoses that can mimic Ramsay-Hunt syndrome.

Stroke and Bell’s palsy are the most common causes of abrupt onset of unilateral facial palsy. Establishing presence of peripheral nerve palsy and the characteristic rash help clinically distinguish RHS from these diagnoses.

Other considerations in patients with facial nerve paralysis (but without characteristic rash) include Lyme disease, HIV infection, sarcoidosis, parotid-nerve tumors and amyloidosis, but in general these present more insidiously and often have other associated symptoms.

D. Physical Examination Findings.

• Unilateral lower motor neuron facial palsy. Weakness of all muscles of facial expression can be noted.

• Ipsilateral vesicular rash on an erythematous base, located on the auricle, ear canal, anterior 2/3 of the tongue, or soft palate (areas innervated by the facial nerve). Lesions usually begin as erythematous papules which quickly evolve into grouped vesicles. The lesions are usually 3-5mm in diameter and may coalesce.

• Ipsilateral sensorineural hearing loss (in up to 50% of patients).

E. What diagnostic tests should be performed?

• Distinguish between lower motor neuron (peripheral) and upper motor neuron (central) facial muscle weakness. This can be accomplished by asking patients to “close your eyes” (which tests the upper facial area) and “show me your teeth” (which tests the lower facial area). Patient with lower motor neuron weakness will have weakness or paralysis of all muscles of facial expression.

• Full cranial nerve testing should be performed.

1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?

Diagnosis is made on the basis of clinical presentation and lab testing is rarely required. However, VZV can often be isolated from fluid from the vesicular lesions by Tzanck test, PCR and/or DFA Isolation. Serologic confirmation of VZV can be established by four-fold increase in IgG titer.

2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?

No imaging studies are required for diagnosis in patients with a typical presentation. Imaging studies may be necessary in patients with cranial polyneuropathy or a more insidious clinical course. MRI, if performed to rule out structural lesions, may show gadolinium enhancement of the facial nerve.

F. Over-utilized or “wasted” diagnostic tests associated with this diagnosis.

Blood tests and cerebrospinal fluid (CSF) studies are unwarranted in cases with typical presentation.

III. Default Management.

Although there is limited evidence-based data in patients with RHS, standard treatment includes antiviral therapy, preferably given in the first 72 hours of presentation. The treatment of patients with herpes zoster with antiviral agents has proven efficacious in reducing the risk and severity of post-herpetic neuralgia, decreasing viral shedding, and accelerating healing of skin lesions.

Use of corticosteroids is more controversial, but there is some retrospective data associating steroid use with better recovery in patients with RHS and they are usually given in the absence of contraindications. Other treatments include pain control and medications to limit post-herpetic neuralgia.

There are several small retrospective studies looking at the benefit of surgical decompression in RHS, mostly in patients at high risk for residual deficitis (i.e. complete palsy and >90% reduction in axonal response by electroneurography). There are no studies comparing medical management to surgery in such high-risk patients and at this time there is no good data for surgical decompression.

A. Immediate management.

• Antiviral therapy with either acyclovir, famciclovir, or valacyclovir

• Steroid therapy

• Analgesics for pain associated with the acute neuritis

• In patients with significant pain (particularly patients over age 60), use of gabapentin or pregabalin to decrease incidence of post-herpetic neuralgia should be considered.

• Patients with decreased tear production and inability to close the eye on the affected side are at risk for corneal ulceration and should be encouraged to use saline wetting drops frequently.

B. Physical Examination Tips to Guide Management.

The House-Brackman scale can be used to quantify the degree of facial muscle weakness from 1 – 6:

• Normal

• Mild dysfuction: normal tone at rest; slight weakness noted only on close inspection; complete eye closure

• Moderate dysfunction: obvious, but not disfiguring difference between the two sides, some forehead movement

• Moderately severe dysfunction: obvious weakness and/or disfigurement, no forehead movement, incomplete eye closure

• Assymetrical tone at rest; barely perceptive motion

• Complete paralysis

Patients are at risk for corneal ulceration and for bacterial superinfection of skin lesions and should be monitored for such complications.

C. Laboratory Tests to Monitor Response To, and Adjustments in, Management.

None

D. Long-term management.

Patients with limited recovery from the facial palsy may benefit from physical therapy. Aberrant nerve regeneration may lead to synkinesis or spasms and patients with these complications may benefit from referral to an otolaryngologist for possible botulinum injections.

Drugs and dosage

• Antiviral therapy: acyclovir (500mg po 5 times daily x 7 days), famciclovir (500mg po tid x 7 days) or valacyclovir (1000mg po tid x 7 days)

• Steroid therapy: 60mg of prednisone daily, tapered over 10-14 days

• Post-herpetic neuralgia: gabapentin (day 1 – 300mg, day 2 – 300mg bid, day 3 – 300mg tid, can be titrated to 1800mg/d, taper over 7 days to d/c) or pregabalin (start 75mg bid, max 600mg daily, taper over 7 days at d/c)

IV. Management with Co-Morbidities

N/A

A. Renal Insufficiency.

Dose reduction of antiviral therapy (acyclovir, valacyclovir or famciclovir) and medications for post-herpetic neuralgia (gabapentin and pregabalin) based on creatinine clearance is indicated in patients with renal insufficiency.

B. Liver Insufficiency.

No change in standard management.

C. Systolic and Diastolic Heart Failure

No change in standard management.

D. Coronary Artery Disease or Peripheral Vascular Disease

No change in standard management.

E. Diabetes or other Endocrine issues

Close monitoring of blood glucoses is indicated in patients with diabetes who are treated with steroids.

F. Malignancy

No change in standard management (unless significantly immunosuppressed, then see below).

G. Immunosuppression (HIV, chronic steroids, etc).

Immunosuppressed patients are at risk for severe complications from VZV including cutaneous and visceral dissemination as well as VZV pneumonitis and central nervous system (CNS) infections. Such patients are usually hospitalized and treated with intravenous acyclovir until clinical improvement in the rash is evident.

H. Primary Lung Disease (COPD, Asthma, ILD)

No change in standard management.

I. Gastrointestinal or Nutrition Issues

No change in standard management.

J. Hematologic or Coagulation Issues

No change in standard management.

K. Dementia or Psychiatric Illness/Treatment

Consider the risks and benefits of steroid use in patients at high risk for steroid psychosis.

V. Transitions of Care

A. Sign-out considerations While Hospitalized.

Patients with RHS rarely require hospitalization, except in cases in which IV antivirals are required, such as in immunosuppressed patients with disseminated varicella-zoster rash or in patients who are unable to tolerate medications by mouth.

B. Anticipated Length of Stay.

1 week.

C. When is the Patient Ready for Discharge.

Patients can be discharged when no new skin lesions are forming and there is no evidence of further dissemination of VZV infection.

D. Arranging for Clinic Follow-up

N/A

1. When should clinic follow up be arranged and with whom.

• General medicine clinic

• Ophthalmology referral should occur for any patient with poor eye closure (House-Brackman scale 3 and above) or any patient in whom concerns for corneal damage/ulceration occur.

• Ear, nose and throat (ENT) clinic can be considered in patients with poor recovery of function and significant nerve impairment or development of complications such as synkinesis to consider any possible surgical options (such as nerve grafting or botulinum injections).

2. What tests should be conducted prior to discharge to enable best clinic first visit.

None

3. What tests should be ordered as an outpatient prior to, or on the day of, the clinic visit.

None

E. Placement Considerations.

Not applicable

F. Prognosis and Patient Counseling.

Only approximately 50% of patients with RHS will have complete recovery of motor function of the facial nerve, although there is some evidence that early treatment with antiviral therapy portends a better prognosis. Up to 38% of those patients who suffer from hearing loss will have residual hearing deficits. Improvement in facial nerve function occurs slowly, usually over several months.

Poor prognostic factors include age over 60, complete paralysis of the facial nerve and lack of nerve excitability by electroneurography (if performed). The incidence of post-herpetic neuralgia (residual pain > 90 days after disease onset) correlates with age and may be persistant in 13-40% of patients over age 60. RHS is not recurrent in immunocompetent patients.

VI. Patient Safety and Quality Measures

A. Core Indicator Standards and Documentation.

Not applicable

B. Appropriate Prophylaxis and Other Measures to Prevent Readmission.

Not applicable

What's the evidence?

Donald, H. “Bell's Palsy”. NEJM. vol. 351. 2004. pp. 1323-31.

Goldani, LZ, da Silva, LF, Dora, JM. “Ramsay Hunt syndrome in patients infected with human immunodeficiency virus”. Clin Exp Dermatol.. vol. 34. 2009. pp. 552-4.

Kim, YH. “Prognosis of Ramsay Hunt Syndrome Presenting as a Cranial Polyneuropathy”. The Laryngoscope. 2010. pp. 2270-2276.

Lustig, LR, Niparko, JK. “Disorders of the Facial Nerve”. CURRENT Diagnosis & Treatment in Otolaryngology – Head & Neck Surgery.

Murakami, S. “Treatment of Ramsay Hunt syndrome with Acyclovir-Prednisone: Significance of Early Diagnosis and Treament”. Annals of Neurology. vol. 41. 1997. pp. 353-7.

Morrow, MJ. “Bell's Palsy and Herpes Zoster Oticus”. Current Treatment Opinions in Neurology. vol. 2. 2000. pp. 407-416.

Uscategui, T, Dorée, C, Chamberlain, IJ, Burton, MJ. “Antiviral therapy for Ramsay Hunt syndrome (herpes zoster oticus with facial palsy) in adults. Cochrane Database Syst Rev”. 2008.

Uscategui, T, Dorée, C, Chamberlain, IJ, Burton, MJ. “Corticosteroids as adjuvant to antiviral therapy in Ramsay Hunt syndrome (herpes zoster oticus with facial palsy) in adults. Cochrane Database Syst Rev”. 2008.

Whitley, RJ. “A seventy year old woman with shingles: review of herpes zoster”. JAMA. vol. 302. 2009. pp. 73-80.

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