Benign essential blepharospasm is a chronic, unremitting, variably progressive neurologic disorder involving the motor control center of the brain. It is characterized by involuntary contractions of the muscles that close the eyes, resulting in visual disability and in some cases even functional blindness. Blepharospasm is classified as a localized form of dystonia, or movement disorders. The causes of blepharospasm and related disorders are unknown at this time. The symptoms are believed to be of neurological origin involving a subtle and poorly defined chemical imbalance within motor centers of the brain.

Meige Syndrome is another a form of dystonia. There is a difference of opinion among physicians on whether Meige Syndrome is an extension of blepharospasm or a separate disease. Unlike blepharospasm, which involves only the closing muscles of the eyes, Meige Syndrome may also involve muscle spasms of the mid and lower face, mouth, tongue, throat, neck and sometimes the respiratory system, anyone of which may affect the voice. Meige Syndrome is a neurological disorder of unknown cause.

Early symptoms of blepharospasm may be occasional increased winking, blinking or squinting of one or both eyes, or simply an increasing difficulty in keeping the eyes open. As the disease progresses, the eyes close more frequently, and eventually remain closed for some period of time. Patients may have to use their fingers to keep their eyes open in order to read, drive, or perform simple household tasks.

All patients do not have the same symptoms. In most cases light sensitivity (photophobia) seems to be a major symptom. In some cases the eyelids just twitch; in others they remain closed for minutes at a time. Some patients find that certain tasks such as reading or watching TV sets off the spasms; in other cases stressful situations stimulate eyelid squeezing. This condition is usually progressive in the sense that the periods of spasms 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.

While it is true that there is no known cure at this time for blepharospasm, there is hope and much is being done by physicians to help patients and their families live with this 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 the family cope with the disorder and the possible limitations it imposes.

The most important treatment available at this time is Botulinum Toxin (Botox). Botox is the toxin that causes botulism. When it is injected into the spastic muscles around the eyes, it blocks the nervous impulse from the nerve to the muscle. This reduces the intensity and frequency of eyelid squeezing. The onset of effect is about three to five days, and the benefit lasts about three months. The only significant side effects may be some drooping of the upper eyelid, seen in about 10% of injections, and double vision seen in one out of 200 injections. Beneficial results are seen in about 95% of treatments. Botox has become the single most important treatment for blepharospasm over the last ten years.

In addition to Botox, there are a number of drugs that may add to the effectiveness of Botox. Most important in this group is Klonopin and Baclofen. We have helped develop a new surgical procedure where the eyebrows are fixed to the deep tissues to prevent them from weighing down on the eyelids, and the eyelid are suspended with a silicone sling to help resist the eyelid spasms. While this procedure is not appropriate for every patient, we have had success in controlling spasms in many patients.

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