Blepharospasm

Q:  I am suffering from blepharospasm, also referred to as Meige's syndrome. As a result, I am unable to look at people when they are speaking to me, watch TV or read. When I attempt to walk, I literally have to hold one of my eyes open with my hand and walk looking down at the floor cautiously.

I have received Botox shots and it doesn't help me. I took Artane and was taken to the hospital for slow heart beat. I need your advice on how to further treat this.

A:  Blepharospasm is uncontrollable blinking of the eyelid which makes it appear as if the person is continually winking. Besides being annoying and making life difficult, it can also be painful. Botox injections, which you received, are the treatment of choice and usually 90-95% effective. Since this is one those ironic, fascinating twists in medicine where a substance goes from "threat to cure," let me briefly explain what it is.

Botulinum toxin, made by a bacterium called Clostridium botolinum, is one of the most potent poisons known to man. The bacteria, which are found in soil, untreated water, and intestines of animals and fish, produce spores which multiply in the absence of air. These spores thrive in improperly preserved or canned food, like cured ham, smoked fish, canned vegetables (That's why those of you who are doing a lot of home canning this summer need to be sure you sterilize the items well -- by pressure cooking at 250 degrees F for 30 minutes). Within eight to 36 hours after eating contaminated food, a person may develop difficulty swallowing or speaking, see double, have nausea and vomiting. The toxin causes progressive paralysis of the nerves and can lead to death if untreated.

The great news is that Botox, when injected into nerves will paralyze them and bring relief to people with head tremors, facial spasm, wry neck, etc. (Another interesting sidelight is that in addition to these neurological uses, Botox is used for cosmetic purposes and injected into facial wrinkles to relax them.)

So back to you, why didn't the Botox work for you? Are you one of 5-10% who don't get relief or could there be another reason? I spoke with Lorri Lobeck, MD, Associate Professor of Neurology at the Medical College of Wisconsin, who has treated patients successfully with Botox. She wonders if you got a high enough dose since there is a very wide dosage range. She begins with very small units for the eyelid or face (eg 3-4 units) and increases the dose as needed, but may use as much as 300 units for the neck. Usually the paralysis lasts for about 3 months, and the injection needs to be repeated.

According to Dr. Lobeck some people develop antibodies and become resistant to the Botox. Although this is rare with low doses, antibodies against the Botox could be another explanation for your lack of results.

Side effects, other than bruising at the injection site, are pretty rare, but include itching, nausea, and a flu-like illness. The worst effect is weakening of the eyelid muscle to the point where it droops or doesn't completely close. This can lead to dry eyes, blurred vision, or excessive secretion of tears.

Gerald J. Harris, MD, an oculoplastic surgeon and Professor of Ophthalmology at the Medical College of Wisconsin, also agrees with Dr. Lobeck that Botox is by far the best option for management of blepharospasm. The great majority of patients that he has injected have had excellent results. Prior to the availability of Botox, surgery was done more frequently, but it is reserved now for only the most refractory cases. The procedure involves graded removal of the muscle surrounding the eye, which contracts excessively. It has to be performed very carefully to decrease the contraction, but also retain enough muscle to permit closure of the lid to protect the eyeball. Another surgical option involves resection of a portion of the facial nerve to reduce the intensity of spasm, but the patient is more likely to have problems with excess corneal exposure. Dr. Harris advised that you be sure that the diagnosis is correct. There are other eyelid problems that may cause symptoms similar to yours.

I know I've been very long-winded in this answer, but I've tried to lay out your 3 options: Botox, surgery, and medication. Medications like Artane, Baclofen, and Tegretol have also been used, but in addition to having side effects (some dangerous as you discovered), they are not all that effective.

"Dear Doctor" is a compilation of patient questions answered by doctors from the Medical College of Wisconsin.