2. Ramsay Hunt syndrome also termed Hunt's Syndrome
and herpes zoster oticus.
Ramsay Hunt syndrome is a complication of shingles.
Shingles is an infection caused by the varicella-zoster
virus, which is the virus that causes chickenpox.
Shingles occurs in people who have had chickenpox and
represents a reactivation of the dormant varicella-zoster
virus.(geniculate ganglion)
If the virus reactivates and affects your facial nerve, the
result is Ramsay Hunt syndrome.
3. Herpes zoster oticus or Ramsay Hunt syndrome is a
disease that affects cranial nerves 7 and 8.
It is caused by the spread of the varicella-zoster virus
to facial nerves near one of your ears .
As a result of this infection, the facial nerve becomes
inflamed and irritated.
Hearing loss in the affected ear occurs.
Hearing loss is reported in 24.4% of affected children
4. Clinical manefestations
The two main signs of Ramsay Hunt syndrome are:
1.rash or herpetic blisters in the distribution of the nervus
intermedius.
The distribution of the rash varies, as does the area
innervated by the nervus intermedius. It may include the
following:
The anterior two thirds of the tongue.
The soft palate.
The external auditory canal.
The pinna
The rash/blisters are often painful with a generalised
sensation of burning over the affected area.
2.Facial weakness or paralysis on the same side as the
affected ear
5.
6.
7. Usually, the rash and the facial paralysis develop at the
same time.
But in some cases, the rash will occur before the facial
paralysis or the paralysis before the rash.
Sometimes the rash never materializes.
8. Other symptoms
Weakness on the affected side of your face which
causes the facial muscles to droop.
Difficulty closing the eye or blinking on the affected
side.(drying or irritation of cornea)
Altered taste on the affected half of the tongue.
Loss of facial expression on the affected side.
Difficulty eating, drinking and speaking as a result of
weakness in the lip and cheek on the affected side.
Ear, face or head pain.
Hearing loss on the affected side
Dizziness/vertigo
Tinnitis (ringing in the ear) on the affected side.
Nausea and vomiting .
9.
10.
11. Functional anatomy
Functional anatomy
The neuroanatomy involved in Ramsay Hunt
syndrome is more complex .
There are four cranial nerve nuclei involved in facial
nerve functions.
They are the motor nucleus of VII,
the nucleus of the solitary tract
the superior salivatory nucleus
the spinal nucleus of V
12. Motor nucleus of VII -
special visceral efferent motor fibres from the motor
nucleus of VII leave the brain stem and travel through
the internal acoustic meatus to the bony facial canal to
supply facial muscles.
In Ramsay Hunt syndrome, these fibres are affected as
they pass through the geniculate ganglion, impairing
motor supply of the facial nerve.
13.
14. Nucleus of the solitary tract -
the solitary tract receives special visceral afferent taste
fibres from the anterior two thirds of the tongue.
These fibres travel with the chorda tympani.
The cell bodies of these special visceral afferent fibres
are in the geniculate ganglion which is the site of virus
reactivation when vesicles erupt on the tongue.
The fibres reach the brain stem via the nervus
intermedius and can be affected by local inflammation
as they pass the geniculate ganglion.
15. Superior salivatory nucleus
- special visceral efferent parasympathetic fibres to the
lacrimal and salivary glands come from the superior
salivatory nucleus.
Travel in the nervus intermedius and branch at the
geniculate ganglion into the greater petrosal and
chorda tympani nerves.
Decreased lacrimation may result from involvement of
these fibres as they branch at the level of the
geniculate ganglion.
16. Spinal nucleus of V - this receives general somatic
afferent fibres from the geniculate zone of the ear via
the chorda tympani.
Cell bodies of these neurons lie in the geniculate
ganglia and are the site of viral reactivation in classic
Ramsay Hunt syndrome, causing vesicular eruptions in
geniculate zones.
17. Epidemiology
The disease of older people (most commonly over 60
years old)but it can affect all ages, including children.
There has been a case report of varicella infection in
utero and presentation in infancy with this syndrome.
The incidence and severity increase with age.
In one study about 50% of cases were aged 60 or older.
This may be due to a decline in cellular rather than
humoral immunity
19. Difference between bells palsy and
ramsay hunt syndrome .
Injury to the facial nerve by virus
infection, but the suspected cause of
Bell's palsy (viral) has not been
identified
Is caused by the Varicella virus
(herpes zoster) that also causes
chickenpox and shingles
No red rash associated with Bell's palsy Rash present .
Less painful than ramsay hunt Ramsay Hunt syndrome is commonly
more painful than Bell's palsy
Bells palsy Ramsay hunt syndrome
20. Diagnosis
Virological studies are available but usually the
diagnosis is clinical.
To diagnose RHS in children, enzyme-linked
immunosorbent assay (ELISA) serum anti-VZV IgG
and IgM antibody titers are recommended
Audiometry may be performed.
Occasionally, nerve conduction studies may be done
to determine the extent of damage to the facial nerve
and potential for recover.
Structural lesions can be ruled out by CT scan, MRI, or
magnetic resonance (MR) angiography.
21. Treatment
The key to recovery from Ramsay Hunt syndrome is the
prompt and effective treatment of the varicella zoster virus.
Starting steroids and antiviral agents in the early phase of
the disease is recommended.
Antivirals prevent VZV replication and facial nerve
involvement, and the steroids prevent inflammation and
edema.
The recovery of facial nerve function after starting
treatment in the first 3 days, at 3–7 days, or later than 7
days
And found that the recovery was best when treatment was
started within 3 days of disease presentation
22. Anti-viral medications such as Valtrex, Acyclovir, or
Famciclovir are recommended for 7 to 10 days along
with strong anti-inflamatory steroids (such as
Prednisolone/Prednisone) for 3 to 5 days which are
tapered off in about a week or more.
23. In children
Oral acyclovir at a dose of 800 mg for 7–10 days and
prednisone 1 mg/kg/day for 5 days, followed by
weaning over the next 6-7 days .
The combination of acyclovir and steroid treatment
was superior to treatment with acyclovir alone
24. PREVENTION
VACCINATION IS RECOMMENDED FOR ALL
PATIENTS OLDER THAN 60 YEARS EVEN IF THEY
HAVE HAD CHICKEN POX OR ZOOSTER IN THE
PAST
This age group suffers significant morbidity from
zooster and may therefore benefit fro the vaccine .
Contraindications :
Younger than 60 yrs
Current use of antivirals .
Pregnancy
And certain immunosuppressive conditions .
25. PROGNOSIS
Ramsay hunt syndrome accounts for upto 12% of facial
paralysis.
The prognosis of the facial paralysis in RHS is worse
than that in Bell’s palsy, and only 10% of complete
facial paralysis in RHS recovers completely .
lower rate of recovery in RHS versus Bell’s palsy (73%
versus 100%) .
The prognosis for RHS in children is better than that
in adults.
Audiovestibular findings, presentation including
advanced facial paralysis, and starting treatment late
result in a bad prognosis.
26. Early diagnosis and initiation of treatment within 72
hours of the onset of symptoms improves outcome.
Scarring of deep lesions may occur.
Hearing loss usually recovers well but prognosis is
poorer in elderly males.
Age, diabetes and hypertension appear to be poor
prognostic features
