Ram Say Hunt is also called Herpes zoster oticus. here oticus means affecting the ears.
It is a rare peripheral facial neuropathy.
It is caused due to reactivation of the Varicella Zoster
Virus in the geniculate ganglion of cranial nerve VII which supplies the facial
nerve.
Besides facial paralysis, Ramsay Hunt syndrome can cause painful
shingles rash, and hearing loss in the affected ear.
These two symptoms typically occur at the same time but can also
happen before the other and this rash is not always present in which case the infection is known as zoster sine herpete.
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It is mainly due to the reactivation of the
previous latent virus present in the ganglion
- The approximate incident is 5 per 1,00, 000
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It is however more common in females than in
males
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Talking about age, it is mainly seen in adults,
particularly in people over 60 years of age.
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It is rarely seen in children
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About 12 percent of the facial nerve palsy is
due to RHS
And it is also the second most
common cause of non-traumatic peripheral facial paralysis
- After a person is infected with the virus ( VZV ), there is a latent period where the virus remains dormant in the neurons of the cranial nerves.It mainly resides in the dorsal root ganglion or autonomic ganglion.The reactivation of this virus in the geniculate ganglion of cranial nerves VII leads to the condition
The virus can also spread down the axon of
other cranial nerves that share the common blood supply with the same ganglion
Reactivation needs some fertile conditions
like
- The person with immunodeficiency which may be due to HIV or any malignancy
- The person might be taking immunosuppressive medications like steroids, chemotherapy, or radiation therapy
- The virus can also reactivate if a person experience infections or physical or emotional stress.
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As I already told you, the main features are
unilateral facial nerve palsy and painful rash on the affected ear, oropharynx, or palate.
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In about 20 percent, facial palsy occurs
several days before the blisters appear while in 10% there are no blisters
Other non-specific symptoms can be
- - Flu-like symptoms ( fever /headache before developing the rash )
- - Aching or painful sensations in the ear
- - Macules/papules / collapsing vesicles at the painful sites which then rupture producing a crust
- - There can be a loss of taste in the anterior 2/3 of the tongue
- - Dry eyes and dry mouth can be seen
- - Tinnitus, hearing loss, hyperacusis, vertigo, nausea, and vomiting
How to diagnose it?
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Diagnosis is mainly done clinically and is
confirmed by testing with a PCR for the virus from the ear exudate
- It is necessary to distinguish it from Bells palsy as the treatment varies.
What is the treatment?
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If antiviral is started within 72 hours of the onset
of symptoms, there are more chances of full recovery.
The treatment includes
- Aciclovir 800 mg Orally five times daily for 7 days
- Valaciclovir 1 g three times daily for 7 days
- Famciclovir 500 mg three times daily for 7 days
- Combination therapy of antiviral treatment and prednisone (60 mg orally daily for 5 days).
Other treatment includes a symptomatic approach
- This includes corticosteroids, opioid analgesics, and convulsants such as gabapentin or pregabalin
- Ice packs on bolsters
- Eye path if unable to close the eyes
- Artificial tear and lubrication can help to reduce dry eye symptoms
- TENS – Transcutaneous electrical nerve stimulations and vibrations
The prognosis is poor than that of bels palsy and complete
recovery is less likely if antivirals are started beyond 72hours
Poor prognostic factors include:
- Diabetes mellitus
- Advanced age (> 60)
- Hypertension
- Vertigo.
Complications may include:
- Permanent facial paralysis
- Hearing loss
- Synkinesis (unwanted facial movements linked to deliberate movements)
- Polycranial neuropathy
- Myelitis (inflammation of the spinal cord)
- Post-herpetic neuralgia.
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