Neurological Disorders: Ramsay Hunt Syndrome

Ramsay Hunt Syndrome is a phrase used in neurology for few different disease conditions, but on majority occasions it means facial nerve (7th cranial nerve) paralysis due to infection with a herpes family virus called as Varicella Zoster.

The other conditions carrying the same name as this syndrome above are, a condition with cerebellar degeneration with myoclonic seizures; and a neuropathy related with a branch of ulnar nerve.

Ramsay Hunt syndrome related with the facial nerve is also called as herpes zoster oticus.

The acute infection with Varicella Zoster virus causes the familiar chicken pox. But these herpes group of viruses have a tendency to live in a dormant manner in the body for long time and when an opportunity strikes like for example when the body immunity weakens due to any reason they get reactivated and cause some neurological disease.

With the above chicken pox virus, when the reactivation of the virus happens later it will produce the familiar shingles (herpes zoster) condition. The virus usually stays dormant in the ganglia of the nervous system and in the Ramsay-Hunt syndrome the virus staying in the geniculate ganglion of facial nerve gets reactivated. It is called as herpes zoster oticus because structures inside and outside the ear are involved.

As mentioned above facial nerve paralysis occurs and it is usually rapid in onset. Ear pain is common. You may see characteristic herpes vesicular eruptions in the outer ear. Facial paralysis will present as facial droop and inability to close the eye on the affected side. Taste function of the tongue on the affected side may get affected.

The 8th cranial nerve (vestibulocochlear) lies near to facial nerve so sometimes the inflammation may spread to that nerve as well. The 8th cranial nerve carries nerve signals related with hearing and balance so the involvement of this nerve may affect the hearing and may produce ringing sound in the ear (tinnitus), balance problem, vertigo (spinning type of dizziness) etc.

Note that the arms and legs are not expected to be involved; it is just the head/ear/face area that is involved. If the limbs are involved then we are most likely not dealing with herpes zoster oticus, rather pathology in the brain/brainstem area like multiple sclerosis or stroke etc.

It is most often done based on the characteristic symptoms and examination findings. If rash is seen then the fluid inside the rash can be sent to lab for microscopic examination to demonstrate characteristic herpes inclusion bodies.

An MRI of the brain sometimes ordered to rule out any brainstem pathologies. A CT scan is somewhat inferior alternative to MRI in this regard.

Once Ramsay Hunt syndrome is suspected then medications acyclovir and a steroid like prednisolone are given. The earlier the treatment is started the better is the prognosis. If the above regime is instituted within the first 2 or 3 days then >75% of patients may recover completely or almost completely,.

For vertigo medications like meclizine, diazepam etc are used.

Patients may require exercise program to the facial muscles.

A neurologist is commonly involved in the management of patients with ramsay hunt syndrome.