Ramsay Hunt Syndrome

The Ramsay Hunt syndrome is herpes zoster affecting the geniculate ganglion. Herpes zoster, also called shingles, is discussed in its own article. This syndrome develops when the varicella virus becomes reactivated from the geniculate ganglion.

As a general rule, shingles is a disease of sensory nerves but the Ramsay Hunt syndrome is distinctive in that there is a motor component. J Ramsay Hunt described the various clinical presentations of facial paralysis with a rash and also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal. He recognised the general somatic sensory function of the facial nerve that we usually think of as purely motor.

Herpes zoster is seen as a disease of older people but it can affect all ages including children. The incidence and severity increase with age. This may be due to a decline in cellular rather than humoral immunity.¹ The incidence of all types of herpes zoster is:

• Under 20 years - 0.4 to 1.6 cases per 1000 persons.
• Age 20 to 50 years - 2.0 to 3.0 cases per 1000 persons.
• Over 80 years - 4.5 to 11.0 cases per 1000 persons.
• About 20% of people will have shingles at some stage in their lives.
• Of patients presenting with lower motor neurone facial palsy, 12% have Ramsay Hunt syndrome² but the diagnosis can be missed if there is no rash.

People who are immunocompromised are much more susceptible to shingles in all forms. It tends to be more severe and may present at a comparatively young age. If shingles affects more than one dermatome, consider immune compromise.

The Ramsay Hunt syndrome does not represent such clear anatomy as most cases of shingles of a single dermatome. There are 4 cranial nerve nuclei involved in facial nerve functions. They are the motor nucleus of VII, the nucleus of the solitary tract, the superior salivatory nucleus, and the spinal nucleus of V. Special visceral efferent motor fibres from the motor nucleus of VII leave the brain stem and travel through the internal acoustic meatus to the bony facial canal to supply facial muscles. In Ramsay Hunt syndrome, these fibres are affected as they pass through the geniculate ganglion, impairing motor supply of the facial nerve. The solitary tract receives special visceral afferent taste fibres from the anterior two thirds of the tongue. These fibres travel with the chorda tympani. The cell bodies of these special visceral afferent fibres are in the geniculate ganglion which is the site of virus reactivation when vesicles erupt on the tongue. The fibres reach the brain stem via the nervus intermedius and can be affected by local inflammation as they pass the geniculate ganglion. Special visceral efferent parasympathetic fibres to the lacrimal and salivary glands come from the superior salvitory nucleus, travel in the nervus intermedius, and branch at the geniculate ganglion into the greater petrosal and chorda tympani nerves. Decreased lacrimation may result from involvement of these fibres as they branch at the level of the geniculate ganglion. Special visceral efferent sympathetic fibres originate from the carotid plexus on the internal carotid artery and join the greater petrosal nerve as they pass through the foramen lacerum. The sympathetic fibres supply the same areas as the parasympathetic. The spinal nucleus of V receives general somatic afferent fibres from the geniculate zone of the ear via the chorda tympani. Cell bodies of these neurons lie in the geniculate ganglia and are the site of viral reactivation in classic Ramsay Hunt syndrome causing vesicular eruptions in geniculate zones.

Symptoms

The presenting feature is often pain deep within the ear. It may be paroxysmal at first but after a day or two the pain often radiates outward into the pinna and there is a more constant, diffuse, and dull background pain.

The following may also be presenting features:

• Vertigo and ipsilateral hearing loss.
• Tinnitus.
• Facial weakness or face drop.
• The patient also complains of rash or blisters.
• They may be on the skin of the ear canal, auricle, or both may become infected secondarily, causing cellulitis.

Signs

• There is a rash or herpetic blisters in the distribution of the nervus intermedius.
• The distribution of the rash varies, as does the area innervated by the nervus intermedius. It may include the following:
  • Anterior two thirds of the tongue
  • Soft palate
  • External auditory canal
  • Pinna
• An ipsilateral face drop or weakness may be obvious or it may need testing.
• There may be hyperacusis on that side due to paralysis of the stapedius and tensor tympani.
• The patient may have associated ipsilateral hearing loss and balance problems.

The weakness of the facial nerve will show a lower motor neurone pattern as with Bell's palsy. Ask the patient to give a big grin showing his teeth. Ask him to screw up his eyes. Ask him to raise his eyebrows. The upper motor neurone innervation of the forehead is bilateral, so that in an UMN lesion of the face, the muscles of the forehead are spared.

• The unilateral facial weakness is very similar to Bell's palsy but the rash is the characteristic diagnostic feature to differentiate the two.
• There is not always a rash, especially in younger patients. In children aged 5 to 15, acute facial palsy, like a Bell's palsy and without a rash, may be produced by the varicella/zoster virus.³
• Making the diagnosis is very difficult when the presentation is simply pain in the ear. If vesicles are seen in the auditory canal or on the palate, this will help make the diagnosis.
• If it presents as vertigo, viral labyrinthitis will be considered, or possibly a stroke of the posterior inferior cerebellar artery region.
• Trigeminal neuralgia is paroxysmal and tends to be precipitated by a stimulus such as a cold wind or washing the face.

Virological studies are available but usually the diagnosis is clinical. Audiometry may be performed.

Beware of possible immune compromise.

The management of herpes zoster was discussed in the article, including when it is appropriate to use antiviral agents to reduce the risk of postherpetic neuralgia. The relative merits of adding corticosteroids to antivirals is also discussed.

If there is problem with closing the eye, a pad will protect the cornea.

A proportion of patients with "Bell's palsy" have Ramsay Hunt syndrome zoster sine herpete (without a rash). Treatment of these patients with acyclovir and prednisone within 7 days of onset has been shown to improve the outcome for recovery from facial palsy.⁴ In view of this occurrence, especially if paresis appears to be complete rather than partial, a case can be made for adding antivirals to the treatment of such apparent cases of Bell's palsy. The latter is thought to be caused by herpes simplex. The number of patients with peripheral facial nerve palsy who really have zoster sine herpete appears to be small but varies between series. However, they do have a better outcome if given antivirals too.

• If closure of the eye is compromised, abrasions may occur.
• Lesions may acquire secondary bacterial infection.
• Post herpetic neuralgia may occur. The condition, risk factors and management are described in the article on herpes zoster.

• The shingles will resolve but unlike Bell's palsy, the rate of complete recovery is less than 50%.²
• Scarring of deep lesions may occur.
• Hearing loss usually recovers well but prognosis is poorer in elderly males.⁵
• Age, diabetes and hypertension appear to be poor prognostic features.⁶

James Ramsay Hunt was born in Philadelphia in 1872 and graduated M.D. from the University of Pennsylvania in 1893. After studying in Paris, Vienna, and Berlin, he returned to New York to practice neurology at Cornell University Medical School from 1900 to 1910. He described the syndrome that bears his name in 1907.⁷ His research interests were the anatomy and disorders of the corpus striatum and the extra-pyramidal system. He was consulting physician at several New York hospitals and was appointed professor of neurology at Columbia University College of Physicians and Surgeons, New York, in 1924. He died in 1937.