Neuro-ophthalmology: Case ten

Figure 1

Figure 2

This 70 year old woman was referred by the ENT (ear, nose and throat) department because of the above appearance.

a. What is the diagnosis?

Ramsey Hunt syndrome due to herpes zoster.

Figure 1 shows a right lower facial nerve palsy with failure of lid closure; and figure 2 shows vesicular rashes in the ipsilateral pinna which is supplied by the sensory branch of the facial nerve.

In Ramsey Hunt syndrome, there is herpes zoster of the geniculate ganglion with resultant lower facial nerve palsy and loss of taste to the naterior 2/3 of the tongue. Other features may include sensorineural hearing loss, vertigo and rarely encephlaitis.

b. How would you manage this patient?
The main goals of treatment are to control the herpes zoster and protect the cornea from exposure keratopathy:
  • oral acyclovir 800 mg fives times a day is useful if the vesicular rash occurs within 72 hours
  • regular artificial tear or lubricating ointment, strapping of the lids at night can reduce exposure keratitis. If there were severe exposure keratopathy despite the above treatment, lids closure should be performed. This can be either with surgical tarsorrhaphy or botulinum toxin-induced ptosis.
Compared with Bell's palsy (idiopathic lower seventh nerve palsy), patient with Ramsey Hunt syndrome tends to have a less satisfactory recovery (85% vs 50%).
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