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Benign Essential Blepharospasm Meige and Other Related Disorders
Although benign essential blepharospasm (BUB) was first described in a North American medical journal in 1895, only in recent years have physicians begun to recognise it as a physical (as distinct from psychological) disorder. It is often misdiagnosed because of a relative lack of awareness among physicians. Neurologists, neuro-ophthalmologists and ophthalmologists are the exceptions.
Benign essential blepharospasm (BEB) – what is it?
Blepharospasm is a chronic, unremitting, bilateral (both eyes), forcible closure of the eyelids. It is a variably progressive neurologic dysfunction in the motor control centre 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 does BEB mean?
Benign means not fatal
Essential, in medical terms, means of unknown cause.
Blepharo is derived from a Greek word meaning eyelid.
Spasm means involuntary, forceful contraction of muscles.
Little is known about the cause of BEB and related disorders at this time. Essential blepharospasm is an involuntary spasmodic blinking or squinting that is not due to a primary eye disorder.
What is Apraxia of Lid Opening?
Apraxia of lid opening associated with blepharospasm is the most common cause for failure or minimal improvements with BOTOX. These patients have difficulty opening their eyelids even after the spasms of the eyelids have been reduced by BOTOX. They use their brows, forehead, neck and face to try and help open the eyelids. After eyelid spasms, a time lag is required before the eyelids will open and the eyelids may again droop closed without obvious spasm.
Meige Syndrome – what is it?
Meige is also a form of dystonia. There is a difference of opinion among physicians on whether Meige is an extension of BEB or a separate syndrome. Meige may involve muscle spasms around the eyes, lower face, mouth, tongue, throat, neck and sometimes the respiratory system, any one of which may affect the voice.
What does Meige Mean?
Meige is a neurological disorder of unknown cause at this time. It is also known as Brueghel’s Syndrome. It was identified as a possible dystonia in 1910 and has since been referred to as Meige Syndrome, so named after Henri Meige, a French doctor.
What are the symptoms of BEB?
Early symptoms of BEB may be occasional increased winking, blinking or squinting of both eyes, or simply an increasing difficulty in keeping the eyes open. Light sensitivity (photophobia) and dry eye seem to be symptoms associated 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 gets BEB and/or Meige?
Symptoms occur most frequently in the fifth and sixth decades of life and is three times more prevalent among women than men. Recent findings indicate increasing numbers in the third and fourth decades.
Is BEB/Meige Hereditary?
There is no proven scientific data available on this important topic. However, there are some families in which more than one person has BEB/Meige or other forms of dystonia. This suggests that there is at least a genetic predisposition in some families.
What are the Effects of BEB / Meige?
The spasms may increase in frequency and duration until a patient becomes functionally blind. Facial spasms may become more severe interfering with speech or eating. The ability to drive, read and watch television, or perform other necessary daily activities can become increasingly difficult.
How is BEB/Meige Diagnosed?
There is no specific test for diagnosing BEB, Meige and related disorders. In the beginning a patient may feel that his/her problem is merely an annoying ‘habit’ and may fail to seek immediate medical attention. When seeking diagnosis from a knowledgeable physician, he/she will take the patient’s history and make a determination based on his/her experience.
BEB and/or Meige – what can be done about it?
While it is true that there is no known cure at this time for BEB and Meige, there is hope and 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 may be effective. The overall goal of 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.
Botulinum Injections: This is currently the treatment of choice for BEB. Small amounts of Botulinum toxin 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 in weeks or months and repeated injections may be required. Botulinum toxin A (Botox) was approved by FDA in 1989 for the treatment of blepharospasm. Many people have been successfully treated for years with this approach. Botulinum toxin B (Myobloc) was approved by FDA in December 2000 for the treatment of cervical dystonia and can be used to treat BEB.
Myectomy: The most commonly used surgical procedure is a ‘myectomy’ whereby some, or all, of the squeezing muscles around the eyes are removed. This approach is reserved for patients who have failed with both drug therapy and Botulinum toxin.
Modified or Partial Myectomy: This surgery is less drastic and your physician may recommend this as an alternative procedure. Botulinum toxin injections may still be needed after partial myectomy.
What Treatment is Available for Apraxia of Lid Opening?
Myectomy and ptosis repair are the only treatments that can provide significant relief for apraxia of lid opening. Botox cannot be injected in the central portion of the upper eyelid without inducing ptosis (droopy eyelids). If the orbicularis muscle (squeezing muscle of the eyelid) still has even minimal function in the upper eyelid then the levator muscle (elevating muscle of the eyelid) cannot contract and elevate the eyelid. Removing the upper eyelid orbicularis muscle (myectomy) and tightening the tendon of the levator (ptosis repair) greatly helps most of these patients. Frontalis suspension or frontalis sling (placing a sling from the eyelid with brow elevation) is a last resort and is only required in approximately 10% of patients with blepharospasm and apraxia of lid opening.
Hemifacial Spasm – what is it?
Hemifacial spasm is a unilateral (one side of the face) involuntary contraction of the facial musculature. It usually begins as a twitching around one eye. At this stage, it can be, and very often is, misdiagnosed for benign essential blepharospasm. However it is always unilateral. 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?
Hemifacial spasm 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 tumour or aneurysm compressing the nerve. An MRI is run to rule out these rare causes.
How is Hemifacial Spasm Treated?
herapies include drug therapy, Botulinum toxin injections, or microvascular decompression (MVD) surgery. In this 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.
What can you do if you have BEB, Meige or related Disorders?
Your attitude toward the disorder will be one of the most important factors in deciding how it affects you. It will not kill you, but if you let it get you down it may spoil your life. It is quite natural, when you learn of the nature of your disorder to go through stages of shock, anger, despair and depression. Acceptance may take time.
Until a more effective treatment is discovered, people with BEB and/or Meige and
related disorders must learn that it is they who still control their lives
rather than allowing the symptoms to control them. Contacting other patients
who have successfully adopted this philosophy and attending support group
meetings are great motivational assets to newly diagnosed BEB and/or Meige
patients as well as to those who are still struggling for acceptance. Remember,
help is available – after all, happiness and beauty come from within.
Acknowledgement: Leaflet adapted from patient information from the Benign Essential Blepharospasm Research Foundation, Inc.
P.O. Box 12468
Web Site; http://www.blepharospasm.org