Ramsay Hunt Syndrome

Three different conditions, first described by Ramsay Hunt, are called by the same name – Ramsay Hunt Syndrome.
Most important one is Ramsay Hunt cerebellar syndrome, is a rare form of cerebellar degeneration which involves myoclonic epilepsy, progressive ataxia, tremor, and a dementing process.

Synonyms: dyssynergia cerebellaris myoclonica, dyssynergia cerebellaris progressiva, dentatorubral degeneration, Ramsay Hunt syndrome (RHS) type 1
Cause: the impairment of a regulatory mechanism between cerebellar and brainstem nuclei.
Treatment: Treatment of Ramsay Hunt Syndrome Type 1 is specific to individual symptoms.

Ramsay Hunt syndrome type 2 is the reactivation of herpes zoster in the geniculate ganglion. It has a variable presentation which may include a lower motor neuron lesion of the facial nerve, deafness, vertigo, and pain.
Synonyms: herpes zoster oticus, geniculate neuralgia, nervus intermedius neuralgia, zoster sine herpete(when without rashes)

Clinical Features

A triad of ipsilateral facial paralysis, ear pain, and vesicles on the face, on the ear, or in the ear is the typical presentation.
Taste loss on the front of the tongue, dry eyes and mouth is a classical presentation with tinnitus, hearing loss, and vertigo in some cases when the Vestibulocochlear nerve is affected.

Pathogenesis

After initial infection, varicella-zoster virus lies dormant in nerve cells, where it is kept in check by the immune system. During an illness that suppresses the immune system, the virus travels to the end of the nerve cell, where it causes the symptoms.
Ramsay Hunt syndrome is thought to be the cause of as many as 20% of clinically diagnosed cases of Bell palsy.

Prevention & Treatment

Shingles can be prevented by immunizing against the causal virus, varicella zoster. Treatment with the steroid prednisone and the antiviral drug acyclovir is prevalent though controversial as some studies report only 22% effectiveness in recovery from paralysis and have no effect on hearing loss.

Ramsay Hunt syndrome type 3 is a less commonly referenced condition, an occupationally induced neuropathy of the deep palmar branch of the ulnar nerve. It is also called Hunt’s disease or Artisan’s palsy.

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