Benign Essential Blepharospasm
What is Benign Essential Blepharospasm?
Blepharospasm, a form of dystonia, is a chronic, remitting, bilateral, forcible closure of the eyelids. It is variably progressive neurologic dysfunction in the motor control center of the brain. It is due to involuntary muscle contractions caused by misfiring of neurons within the central nervous system and involves the fifth and seventh cranial nerves.
What are the symptoms of Benign Essential Blepharospasm?
Early symptopms include occasional increased winking, blinking or squinting of both eyes, or simply an increasing difficulty in keeping the eyes open.
Light sensitivity and dry eye seem to be symptoms with most blepharospasm patients.
If the condition progresses, eyelid spasms become increasingly frequent until they are unremitting. Both eyelids may clamp shut and the eyebrows pull down. All cases do not have the same symptoms.
This condition is usually progressive in the sense that the periods of spasms may become more frequent and the periods of relaxation shorter. However, in some cases, the patient’s spasms reach a plateau and remain at that level with no further progression.
Who does it effect?
Symptoms occur most frequently in the fifth and sixth decade of life and is more prevalent among women than men. Recent findings indicate increasing numbers in the third and fourth decades.
What can be done about it?
While there is currently no known cure for the blepharospasm, there is hope. Much is being done by physicians and researchers to help patients and their families live with the problem.
Treatment aimed at relieving the symptoms of the disorder are usually effective. The overall goal of the treatment is to help the patient maintain a useful and satisfying lifestyle and help both the patient and family cope with the disorder and the possible limitations it imposes.
How is it treated?
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Neurological approach
This is done through primarily drug therapy. They are reported with some success. Occasionally, short lived responses are seen. One negative aspect is that these drugs may cause severe side effects.
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Botulinum injections (botox)
This is currently the treatment of choice. Small amounts are injected into the muscles around the eye. The injections temporarily weaken the muscles so that forced closure is more difficult. The effect of the injections wears off and repeated injections may be required. Many people have sucessfully been treated with this approach. It may be contraindicated for patients with any type of condition that causes muscle weakness.
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Surgical approach
1. Myectomy
The most commonly used surgical procedure is whereby some, or all, of the squeezing muscles around the eyes are removed. The approach is reserved for patients who have failed with both drug therapy and botulinum toxin.
2. Modified or Partial Myectomy
This surgery is less drastic and may be used as an alternative procedure. Botulinum toxin injections may still be required after a Partial Myectomy.
3. Neurectomy
An older technique which is sometimes successful. This is a resection or removal of nerves involved in the spasming.
Hemifacial Spasm
What is Hemifacial Spasm?
It is a unilateral involuntary contraction of the facial musculature. It usually begins as a twitching around one eye. It can easily be misdiagnosed as benign essential blepharospasm. The twitching and contraction of the facial muscles slowly progresses and advances down the face to include the musculature of the cheek, the mouth, and the neck. Only exceptionally is the musculature above the eyebrow involved. It is not a form of focal dystonia and is, in most instances, curable through surgery.
What causes Hemifacial Spasm?
It is believed to be caused by a small arterial loop pressed against the seventh (facial) nerve where it exits the brain stem. In rare cases, it can be caused by a small tumor or aneurysm compressing the nerve. An MRI is done to rule out these rare cases.
How is it treated?
Therapies include drug therapy, botulinum toxin injections or microvascular decompression surgery. In the aforementioned surgical procedure, using microsurgical techniques, the surgeon places a pad between the nerve and the blood vessel in an attempt to inhibit the cause of the spasm.
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