You’ve Been Diagnosed with Benign Essential Blepharospasm

September 6, 2013


child winking

Benign essential blepharospasm (BEB) is a condition in which the muscles around the eyelids go into periodic blinking spasms that squeeze the lids shut.  Each time a spasm occurs the lids may remain tightly closed for seconds to minutes.

In the early stages, the blinking may appear to be merely a nervous twitch or a “bad habit”.  Because of this, most patients have BEB for a long time before becoming aware of it.  BEB usually comes on after the age of 50, is more common in women than men, and may be hereditary (though this has not been proven).  Estimates of the number of affected individuals in the United States range up to about 150,000.

In medical terms, “essential” means “of unknown cause”, “blepharo” means eyelid, and “spasm” means an involuntary forceful contraction of muscles.

Symptoms

Early symptoms are winking, blinking, or squeezing together of the eyelids of one or both eyes, or difficulty in keeping the eyes open.  You may be especially sensitive to bright light.  As the condition progresses, the spasms become more frequent until they are almost constant; both eyelids clamp shut and the eyebrows pull down.  There may be accompanying facial spasms as well.  The symptoms are not the same for everyone.

As the spasms increase in frequency and duration, it becomes more difficult to drive, read, watch television, or perform routine daily activities.  Because the eyelids cannot be opened at will, many individuals with BEB eventually become functionally “blind”.

What Causes BEB?

BEB is a neurological disorder (dystonia) involving the 7th (facial) cranial nerve.  The cause is unknown at this time, but the symptoms are believed to come from a chemical imbalance that occurs within the brain centers that control movement.  It is not due to any disease of the eyes themselves and it is not part of any generalized neurological condition such as Parkinson’s disease.

An unrelated but similar-appearing condition that keeps the eyelids shut occurs temporarily when there is severe eye irritation, such as from the lashes scratching the cornea, or from recent eye trauma.  But in these cases the lid spasm tends to be more constant rather than periodic.

Treatment

Although so far there is no cure for BEB, there are some treatments that reduce the severity of symptoms.  Medications are available, but they are helpful in only about 15% of cases.  A far more effective treatment, which can produce good results in almost all patients, is the injection of Oculinum (botulinum toxin, also called “botox”) into the muscles of the eyelids; the injections, however, may need to be repeated every few months.  For those who are not helped by either medications or Oculinum, there is a surgical procedure to actually remove some of the muscles and nerves to those muscles that squeeze the lids shut.

Both the injections and the surgery are meant to paralyze the lid muscles, but they can result in unpleasant side effects, such as paralysis of the facial muscles.  This may lead to drooping of the corner of the mouth (and some drooling) or excessive tearing.  Fortunately, these side effects are usually temporary.

Other treatments being used occasionally include biofeedback, acupuncture, hypnosis, chiropractic, and nutritional therapy, but these are controversial and the benefits are unproven.

Meige Syndrome

Some of these with BEB also have Meige Syndrome (named for the doctor who first identified it; also called Brueghel’s Syndrome).  This problem is more extensive and involves movements and spasms of the lower face, mouth, tongue, throat, and neck.  Sometimes even the voice is affected.  It, too, is a neurological disorder of unknown origin.

Additional Help

Ongoing research to find a cure and/or better treatment for BEB and its related disorders is sponsored by the Benign Essential Blepharospasm Research Foundation.  The Foundation also maintains support groups throughout the country that can help patients and their families cope better with the disorder and the limitations it imposes.  By offering encouragement and allowing participants to share experiences, information, you can contact the Benign Essential Blepharospasm Research Foundation, Inc., P.O. Box 12468, Beaumont, Texas 77726-2468;  (409) 832-0788, www.blepharospasm.org, email [email protected].

All About Vision