The
Ramsay Hunt Syndrome
What
is Ramsay Hunt Syndrome?
It is a neurological disorder, a rare
one that is characterized by the partial paralysis of the facial nerves (facial
palsy). An erythematous vesicular rash of the skin affecting the ear or mouth may
also be present.
Also accompanying the disorder could
be ringing in the ears (tinnitus) and hearing loss. The disorder is also
sometimes known as herpes Zoster oticus due to the rash around the ears that
accompanies the disorder.
Discovery
of Ramsay Hunt Syndrome
The disorder is named after James Ramsay
Hunt, a physician, who first described it in 1907, through observations of a
patient presenting mucosal and cutaneous rashes.
What causes Ramsey Hunt Syndrome?
Ramsay Hunt syndrome is caused by a
virus, the same type that causes the chickenpox virus, known as the
Varicella-Zoster Virus (VZV). After the chicken pox has cleared up, the virus
are still present in the nerves in a dormant state.
Years later, the virus may be
reactivated causing shingles (herpes zoster) and infecting the facial nerves
and leading to irritation, swelling, and partial paralysis.
Signs and Symptoms of Ramsay Hunt Syndrome
The symptoms can vary from individual
to individual, but usually, only one side of the face is affected. The main
symptoms of the disorder are -
· Red
painful blisters that may be fluid filled breaking out in and around either
ear.
· Paralysis
or facial weakness on the side of the face of the affected ear (unilateral
paralysis).
·
Facial
drooping
·
Sensorineural
hearing loss (where sound is not properly transmitted to the brain) in the
affected ear
·
Ringing
in the ear (tinnitus)
·
Difficulty
closing one eye (usually the eye on the affected face side
·
Develops
a crooked smile
·
Dryness
of the mouth and eyes
·
A
sensation that you or your surrounding is in motion or you are spinning (vertigo)
·
Painful
reddish rash on affected side of face
·
Blisters
on the tongue and ear canal
·
Dizziness
·
Ear
ache
·
Change
in taste or lose of
·
Nausea
and vomiting
·
Hyperacusis
- condition where sounds appear louder than they really are
·
Slurred
speach
How to diagnose Ramsay
Hunt Syndrome
There is a possibility of
misdiagnoses of this disorder, more so as it not very common, like some
ailments that Doctors are familiar with. In fact most doctors will never come
across it in the course of their practice.
Ways in which Ramsay Hunt Syndrome
can be diagnosed include
· Taking
a sample of the fluid in the blisters that usually forms around the ear on one
side of the face
· A
sample of the patient’s blood can also
be taken and analyzed
· A
magnetic resonance imaging scan is also used to detect inflammation in the
facial nerves, indicative of the syndrome
· Otoscope
ear examination to check foe inflammation in the ear canal
· All
the above coupled with physical examination.
Risk factors for Ramsay Hunt Syndrome
· Anyone
that had previously had chicken pox infection can become afflicted with the
syndrome. Adults, usually above 50 years are at a higher risk that children.
· Ramsay
Hunt syndrome in not contagious, however anyone that has not been previously
infected with the chicken pox virus or been vaccinated can contract it from
someone with an active Ramsay Hunt Syndrome because the virus has become
reactivated.
· During
the blister stage, babies, young children and pregnant women should avoid
contact with an infected person. People with a weak or compromised immune
system should also stay away.
Treatment for Ramsay Hunt Syndrome
Beginning treatment as soon as
possible after the onset of the disorder is important, so as to minimize the
risk of permanent nerve damage. Medications used include-
· Antiviral
drugs – Famciclovir (famvir), Zovirax or Valacyclovir (Valtex), can be used to
fight the disorder for between 7 to 10 days.
· Corticosteroids
– Like prednisone help increase the efficacy of the antiviral drugs used, for 3
to 5 days.
· Pain
relievers- these are used to alleviate the pain, such as seizures associated
with the syndrome. Medication for these include carbamazepine, an anti-seizure
medicine.
· Anti-anxiety
drugs – antihistamines and anticholinergics can help to suppress vertigo.
· Because
the patient may find it difficult to close their affected eye properly, dryness
and irritation (from dust) can cause injury to the cornea. Artificial tears and
eye ointment should be used to minimize this risk.
Prevention of Ramsey
Hunt Syndrome
Since it is a reactivation the
chickenpox virus that had remained dormant in the nervous system after the
infection and treatment of the virus, probably due to a compromised immune
system, vaccination against the chicken pox virus in the first place will help
to prevent the Ramsay Hunt syndrome.
Prognosis
Treatment just after the onset of
symptoms gives a good chance of recovery. If the damage to the nerves is
minimal, then a high degree of recovery is expected. More serious damage to the
nerves reduces the chance of full recovery even months after treatment.
The younger the patient, the better
the chances of making a full recovery with little or no complications. Complications
include synkinesis, a situation where the damaged nerves grow and connect back
to the wrong areas. This can lead to inappropriate responses to certain actions.
Related disorders
Bell’s palsy is a neurological
disorder that presents symptoms similar with Ramsay Hunt syndrome. It start
with a fever, stiffness on the affected face side, and a stiff neck.
Additional information
· How
long does Ramsay Hunt syndrome last – with proper treatment, the disorder lasts
between 6 weeks and 3months before recovery.
· Can
Ramsay Hunt syndrome relapse or re-occur? – No, it does not usually re-occur.
Take
Away
The Ramsay Hunt Syndrome can cause
pain, discomfort and anxiety in affected persons. To greatly reduce the chance
of developing the disorder, vaccination against the chickenpox Virus is key.
This is so because the chickenpox virus that goes dormant after the treatment of
and recovery from the virus getting reactivated is the precursor to the development
of the Ramsay Hunt Syndrome.