What is Ramsay Hunt Syndrome?
Ramsay Hunt Syndrome is an infection caused by the herpes virus of geniculate nerve ganglion. This leads to paralysis of facial nerves on the similar side as that of the infection. The disorder also leads to development of rash in ears or the area surrounding. The syndrome has been named after James Ramsay Hunt, an American neurologist.
Ramsay Hunt Syndrome Type I
Ramsay Hunt Syndrome Type I is a rare type of progressive cerebellar dyssynergia. The main characteristics include tremors, convulsions or myoclonic epileptic jerks. The tremors usually starts at one point in extremity and gradually spread to other parts. Arms are affected more than the legs even though the gait turns unsteady. Patients are noticed to make errors in estimation of direction and range. Some other characteristics associated with the syndrome include muscular hypotonia, adiadochokinesia and asthenia.
This syndrome is also known by various names like Hunt’s ataxia, Dentate cerebellar ataxia, dyssynergia cerebellaris progressive, sive myoclonica, dyssynergia cerebellaris myoclonica, primary dentatum atrophy, Hunt’s syndrome I and primary dentatum atrophy.
Ramsay Hunt Syndrome Type II
Also known as Herpes Zoster Oticus or Ramsay Hunt Syndrome Shingles generally, it is a commonly noticed complication of Shingles. The varicella-zoster virus which causes chickenpox is also the reason for Shingles. Though rarely affecting the children, it affects elders of both genders equally and isn’t contagious. It affects those suffering from chickenpox and occurs on the reactivation of latent varicella-zoster virus. The Herpes zoster oticus caused by the spreading of varicella-zoster virus to the facial nerves leads to intense ear pain and development of rashes around mouth, ear, face, scalp and neck. It can also lead to paralysis of facial nerves, hearing loss, tinnitus and vertigo. Some patients also complain of loss of taste and dry eyes and mouth.
Ramsay Hunt Syndrome Causes
Ramsay Hunt syndrome is caused by the varicella zoster virus which lies dormant in the affected person’s body for many years until the reactivation takes place. This reactivation leads to Shingles and Ramsay Hunt syndrome is in fact a complicated form of Shingles. The varicella-zoster actually affects the 7th cranial nerve for a number of days.
Ramsay Hunt Syndrome Symptoms
The various symptoms associated with Ramsay Hunt Syndrome are:
- Severe ear pain
- Formation of rashes around mouth, neck, face, scalp and ears.
- Hearing loss.
- Vertigo
- Paralysis of facial nerves
- Loss of taste.
- Dry eyes
- Dry mouth
- Ringing in the ears.
Ramsay Hunt Syndrome Diagnosis
The syndrome can be diagnosed by closed observing various signs of facial weakness or rash. In persons affected with the syndrome, an otoscope shows the inflamed ear canal along with the presence of lesion on the pinna of ear. These are some signs of varicella-zoster virus’ presence. PCR or Neuroimaging like MRI can show any inflammation of facial nerves and also determine the severity of the condition.
Ramsay Hunt Syndrome Pictures
Picture 1 : Ramsay hunt Syndrome Signs
Image source : uwgi.org
Picture 2 : Ramsay hunt syndrome Image showing affected areas
Image source : Mayo Foundation
Ramsay Hunt Syndrome Facial Exercises
For treating Ramsay hunt syndrome, facial exercises can be very helpful even though these are not mandatory. Some of the facial exercises that improve the recovery rate are as follows:
- In the initial stages of the syndrome, warm and gentle massage on the face in circulatory motions.
- Raising the eyebrows to form horizontal wrinkles on the forehead as in surprise.
- Drawing the eyebrows together and in downward direction giving rise to vertical wrinkles between eyebrows. This is similar to the expression noticed while frowning.
- Drawing lips together as if kissing.
- Protruding the upper lip
- Smiling with/without the teeth showing.
- Lifting the outer borders of nostrils to form diagonal wrinkles along the nose’s bridge.
- Pursing the lips and also attempting to whistle.
- Puffing out the cheeks and holding air.
- Shutting eyes tightly and then opening them wide.
- Winking each eye alternatively.
Never rush or force exercises assuming that constant workout may speed recovery. Many neurologists are of the belief that this forced stimulation of facial muscles can lead to synkinesis. For patients with long term facial paralysis, professional assistance is needed for these methods. Most of the recovered patients have reportedly never opted for these facial exercises.
Ramsay Hunt Syndrome Recovery
Ramsay Hunt Syndrome Prognosis depends on the severity of damage caused by the syndrome. The successful and efficient treatment of varicella-zoster virus is the key to the syndrome’s recovery. Though less commonly seen in children, they have more chances of recovering fully when compared to the adults. If the nerve damage is minimal, then only a few weeks are sufficient for a full recovery. However, severe nerve damage may lead to longer time like several months.
The treatment should be started as early as possible since a delay can cause worsening of the condition. The recovery gets complicated if synkinesis develops as it causes inappropriate responses. Some of those affected, experience troubles like blinking of eye while eating or talking. 50% patients are reported to have a long-term hearing damage. However, all these can be recovered by following the right treatment procedure and medications.
Ramsay Hunt Syndrome Treatment
For Ramsay Hunt Syndrome Type I, there isn’t any cure but measures can be taken to treat the symptoms associated with the disorder. These measures include the following methods:
- Myoclonus medication – Clonazepam, Benzodiazepines and Valproic Acid.
- Anti-seizure medication – Clonazepam, Phenobarbital and Valproic Acid.
- Various other supportive measures including speech, hearing and physical therapy.
For Ramsay Hunt Syndrome Type II, the treatment varies a little. It includes the following measures:
- Anti-viral medications like Famciclovir or Acyclovir in addition to anti-inflammatory steroids like Prednisolone.
- Corticosteroids
- Strong pain killers
- Diazepam to treat vertigo
- Eye-drops are used to reduce the dryness in eyes.
- Facial exercises also help in quicker recovery
- Anti-emetic drugs like Domperidone, Cyclizine or Prochlorperazine to control vomiting or nausea.
- The blood pressure, blood glucose and electrolytes level should be monitored regularly.
In some cases, these treatment measures lead to side effects like headaches, flushing, indigestion, insomnia, fatigue, twitching and constipation.
References
http://en.wikipedia.org/wiki/Ramsay_Hunt_syndromes
http://emedicine.medscape.com/article/1166804-overview
Also Read:
- Cornelia de Lange Syndrome
- Klinefelter syndrome
- CHARGE Syndrome
- Marfan Syndrome
- Cauda Equina Syndrome
by on 05. Apr, 2011 in Syndromes
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