INFORMATION ON RAMSAY HUNT SYNDROME I

Ramsay Hunt syndrome Type I consists of facial paralysis, ear pain, vertigo, ringing in the ears and blistering rash on the ear on one side of the face, all caused by infection with herpes virus The virus spreads to the nerves in the face and causes the symptoms. It is rare in healthy people, but occurs more commonly in people with weakened immune systems, such as the elderly.The disorder generally occurs in early adulthood. Tremor may begin in one extremity and later spread to involve the entire voluntary muscular system. Arms are usually more affected than legs. Some of the cases are due to mitochondrial abnormalities.

SYNONYMS OF RAMSAY HUNT SYNDROME I

Dyssynergia Cerebellaris Progressiva,
Dentate Cerebellar Ataxia,
Dentatorubral Atrophy,
Primary Dentatum Atrophy,
Herpes Zoster Oticus geniculate neuralgia,
Herpes zoster oticus,
Nervus intermedius neuralgia.

CAUSES OF RAMSAY HUNT SYNDROME I

Ramsay Hunt syndrome caused by infection with herpesvirus 3, the virus that causes Ramsey-Hunt syndrome has been conclusively identified. It is varicella zoster virus which is the virus that causes chicken pox, and is a strain of the Herpes virus. Varicella zoster typically remains dormant for decades. The incidence of Ramsey Hunt syndrome increases significantly after age 50. This virus is a relative of human herpes viruses, which cause fever sores on the mouth and genital herpes.

SYMPTOMS OF RAMSAY HUNT SYNDROME I

Basic Symptoms of Ramsay Hunt syndrome:

1. Pain: Bell's palsy patients may complain of pain behind the ear
2. Swollen and tender lymph nodes near the affected area.
3. Facial weaknesses with difficulty closing one eye -- smile may look crooked
4. Painful rash on the ear drum, tongue, or palate on the same side of the facial weakness
5. Hearing loss on one side
6. Sensation of things spinning around vertigo.

DIAGNOSIS OF RAMSAY HUNT SYNDROME I

Diagnosis of Ramsay Hunt syndrome
Diagnosis is based on the symptoms, especially the rash, which has the same appearance as chickenpox.
Research has shown that prompt treatment with antiviral medications such as acyclovir and famcyclovir lessens the symptoms and improves recovery
Corticosteroids such as prednisone may be used, and scopolamine or antihistamines such as meclizine will reduce vertigo and tinnitus.
A nerve conduction study may be done to determine the extent of damage to the facial nerve and potential for recovery.

TREATMENT OF RAMSAY HUNT SYNDROME I

1. Corticosteroids and oral acyclovir are utilized commonly.
2. Infectious disease consultations are recommended.
3. Vestibular suppressants may be helpful if vestibular symptoms are severe.
4. As with Bell palsy, care must be taken to prevent corneal irritation and injury.
5. If a structural lesion is discovered on imaging, a neurosurgical or otolaryngologic consultation is recommended.
6. Temporary relief of otalgia may be achieved by applying a local anesthetic or cocaine to the trigger point, if in the external auditory canal.
7. Carbamazepine may be helpful, especially in cases of idiopathic geniculate neuralgia.

Surgical decompression of the facial nerve has no role in this syndrome.