Ramsay Hunt Syndrome: Causes, Symptoms And Treatment

Ramsay Hunt syndrome is a neurological disease that causes vesicles to appear on the skin around the ear canal, as well as facial paralysis, ear pain and other characteristic signs. It is associated with infection of a region of the facial nerve by the varicella-zoster virus.

In this article we will describe in detail The causes, symptoms and treatment of Ramsay Hunt syndrome To do this, we will place special emphasis on the alterations of the nervous system with which this disease is related, since they are a fundamental aspect in its understanding.

    What is Ramsay Hunt syndrome?

    Ramsay Hunt syndrome, also known as “herpes zoster oticus”, “geniculate neuralgia” and “intermediate nerve neuralgia” is a neurological disorder characterized by the appearance of vesicular erythematous eruptions on the skin, mainly in areas near the ear canal, as well as other related signs.

    The disease was described by neurologist James Ramsay Hunt in 1907. This expert attributed the appearance of the distinctive rashes to infection of the geniculate ganglion by the varicella-zoster virus.

    Although it is a relatively rare diagnosis, it is estimated that Ramsay Hunt syndrome It is the cause of between 16 and 18% of all unilateral facial paralysis Its association with Bell’s palsy (that is, any caused by problems with the facial nerve) is particularly significant.

    Ramsay Hunt syndrome is usually not life-threatening; However, the muscular alterations that characterize it can interfere very markedly in the lives of those who suffer from it. Likewise, less than half of affected people recover completely from symptoms.

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    Causes of this disease

    This disease occurs as a result of reactivation of the varicella-zoster virus in the geniculate ganglion, which is located in the facial nerve, the seventh cranial nerve. This nerve controls many of the movements of the face, such as those involved in facial expressions and chewing, as well as the perception of flavors on the tongue.

    After infection, the varicella-zoster virus remains inactive in nerve cells thanks to the action of the immune system. However, if the virus manages to reach the geniculate ganglion of the facial nerve, it interferes with its functioning, causing the symptoms that we will describe in the following section, including vesicular eruptions.

    Occasionally other cranial nerves are also affected, in particular the fifth (trigeminal), the sixth (abducens or abducens), the eighth (vestibulocochlear) and the ninth (glossopharyngeal). When this happens, signs related to the functions of the nerve in question appear.

    It is important to keep in mind that the symptoms occur only in one of the halves of the head, since the most normal thing is for the virus to affect only one of the two facial nerves.

      Main symptoms and signs

      One of the most characteristic and useful signs for the diagnosis of Ramsay-Hunt syndrome is the presence of a partial or complete paralysis of many muscles of the face This manifests itself through difficulty making movements with the mouth, such as those necessary to eat, or to close one of the eyelids, among other aspects.

      It is also very common for rashes consisting of fluid-filled vesicles, mainly on the palate, on the tongue and in the ear canal, both externally and internally. Other common symptoms are loss of taste on the outside of the tongue and dry mouth and eyes.

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      Since neurological lesions are located near the vestibulocochlear nerve, which transmits information related to sound and balance from the inner ear to the cortex of the brain, It is also common for ear pain and hearing loss to appear (hearing loss), dizziness, vertigo and tinnitus (perception of sounds such as buzzing and beeping).

      Sometimes the involvement of the geniculate ganglion by the varicella-zoster virus does not cause the appearance of the distinctive vesicles but does cause pain, paralysis of the facial muscles and other associated symptoms. When this happens the term “zoster sine herpete” is used.

      Treatment and management

      The most common way to treat the infection that causes Ramsay Hunt syndrome is by administering steroidal anti-inflammatories, among which prednisone stands out Antiviral medications such as acyclovir are also often prescribed.

      When the pain is very intense and requires specific treatment, analgesics can be administered. Sometimes very powerful drugs are prescribed since the discomfort can also be very intense.

      It has been found that l Loss of hearing ability and mobility in the muscles of the face They are largely maintained even if treatment is applied early and appropriately.

      In any case, in a large number of countries vaccination against the varicella-zoster virus is mandatory; This makes Ramsay Hunt syndrome tend to be rare in much of the world.