ASHLY ALEX

GROUP 5

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.

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

Clinical manefestationsThe two main signs of Ramsay Hunt syndrome are:

1.rash or herpetic blisters in the distribution of the nervusintermedius.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 generalisedsensation of burning over the affected area.

2.Facial weakness or paralysis on the same side as the affected ear

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.

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 .

Functional anatomyFunctional 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

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.

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 fibresare 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 nervusintermedius and can be affected by local inflammation as they pass the geniculate ganglion.

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.

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 geniculateganglia and are the site of viral reactivation in classic Ramsay Hunt syndrome, causing vesicular eruptions in geniculate zones.

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

Differential diagnosis Bell Palsy

Persistent Idiopathic Facial Pain

Postherpetic Neuralgia

Temporomandibular Disorders

Trigeminal Neuralgia

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

Diagnosis Virological studies are available but usually the diagnosis is clinical.

To diagnose RHS in children, enzyme-linked immunosorbent assay (ELISA) serum anti-VZV IgGand 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.

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

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.

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

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 .

PROGNOSISRamsay 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.

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

Reference http://www.patient.co.uk/doctor/herpes-zoster-oticus-ramsay-hunt-syndrome

http://www.ramsayhunt.org/