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Ask the Doctor 2013 Number 4 (July/August)

Disclaimer: neither the BEBRF nor members of the BEBRF Medical Advisory Board has examined these patients and are not responsible for any treatment.

Q: I have Meige, and am injected by an excellent neurologist. as far as the facial injections go, he does the forehead for headaches, the eyes, the muscles, and beneath the lower lip (to control the lower lip tremor). However, I am unable to smile. This affects me deeply because people respond to one very differently when there is never a smile. The corners of my lip do not go up. We discontinued injections near the nose thinking that was the culprit. We have tried many variations of locations and quantity of BOTOX® and Xeomin® but cannot resolve this issue after trying for 7 years! Do all other Meige patients have this problem? Any suggestions?

A1: Botulinum toxin is considered the treatment of choice for Meige syndrome, better known among neurologists as "cranial dystonia," meaning involuntary spasms of muscles of the upper and lower face and jaw. The injections must be tailored to the needs of each patient. Without examining you it is impossible to know which facial muscles are affected and which need to be injected. Loss of facial expression, including smile, is a potential, but avoidable complication of botulinum toxin injection. I suggest you work with your neurologist by describing your concerns so that he can make the appropriate adjustments in dose and site of injection to achieve the most optimal result.

Joseph Jankovic, MD, Director, Parkinson’s Disease Center and Movement Disorders Clinic, Baylor College of Medicine, Houston, Texas

A2: I have seen this develop in several patients who were injected into the lower cheek area or along the orbital rim (cheek bone) and the lip elevators were permanently paralyzed. I try to avoid injections below the orbital rim for this reason. As far as treatment, Botox can be injected into the depressor anguli oris muscle (the "cry baby muscle") which will improve the appearance of the down-turned smile.

Andrew Harrison, MD,Director, Oculoplastic and Orbital Surgery, University of Minnesota, Minneapolis, Minnesota

Q1: Blepharospasm is a neurological disorder, so I do not understand why on heavy rain days when the barometric pressure is high, my eyes feel so heavy. I have spoken with other patients and some agree with me. Can you please shed some light on this subject?

Q2: once the botulinum toxin has kicked in, there are days when I am very busy, and I go about my normal business with no down time. But often the next day my eyes feel particularly dry, strained and tired as if I have overdone it. Why does this happen and why is it cumulative?

A: Barometric pressure is the force exerted on objects by the weight of the atmosphere above them. In other words, the air around us has real mass and weight, and this is typically measured as "atmospheric" or "barometric" pressure. If you doubt this, hold a piece of paper at one end and slowly move your arm back and forth through the air. The mass of the air will bend the paper backward as you move it. The weight of everything, including people and the air around them, changes with distance from the center of the earth. Thus, barometric pressure (the weight of air) decreases at higher altitudes and greater distances from the center of the earth, or focal point of gravitational force. Dry air and cooler air have higher densities, or higher barometric pressure. As a storm approaches, the barometric pressure typically falls, not rises. So, the question is why does an approaching rainstorm or a falling barometric pressure make benign essential blepharospasm (BEB) worse for some people? A parallel question might be why do some people with damaged or arthritic joints have more pain when the weather changes? Although we can make up theoretical explanations regarding changing tissue volumes with changes in surrounding air pressure, the reality is that western medicine today does not have a clear, proven answer to these questions. Recall, however, that the current thinking is that BEB is caused by an inability of the brain to turn off reactive spasm or blinking in response to something that irritates the eye. As botulinum toxin therapy begins to work, the muscles that cause BEB are weakened, but these are also the muscles that allow the eyes to normally close and blink. A normal blink helps to lubricate the eyes. One of the challenges in treating BEB is to weaken the spasms, but maintain enough muscle movement to prevent eye drying. If this is not achieved, a change in injection dose and/or pattern may be indicated.

Charles N.S. Soparkar, MD, PhD, Plastic Eye Surgery Associates, Houston, Texas

Q: I have been getting BOTOX injections since 1989, but with terrible soreness and dryness. This was resolved when I had punctual occlusion. Recently my doctor started putting the injections really close to the eyes, right in the corners. After several sets of injections, I began to have pain and redness in the eyes about four or five days after the injections, and I now have this problem every time I have injections. The pain has changed from a general soreness to a burning feeling around the eyeball, which comes and goes. The last time I also had blurred vision as well.

A: With your dry eyes, it sounds like the BOTOX nearer the lid margin may be making the dryness worse. This could be from further weakness, or the BOTOX can also decrease tear production if injected over the tear glands. I would try increased dry eye treatments and try moving the injections farther from the lid margin.

Richard L. Anderson, MD, Medical Director, Center for Facial Appearances, Salt Lake

Q: I have blepharospasm, had BOTOX injections, eyelift, myectomy, and a frontal sling. None of these things have helped me. The doctor also said I have Meige and I suffer with Obsessive Compulsive Disorder (OCD). I am wondering if OCD is connected to the blepharospasm. I think it is because when the OCD is bad, the eyes get worse. Do you think it might have something to do with serotonin? I read that blepharospasm might be a brain disease.

A: First, blepharospasm is indeed primarily "a brain disease" rather than an "eye disease". There is much evidence that blepharospasm results from abnormal function of the part of the brain called the basal ganglia, brainstem, and adjacent brain areas. Although not conclusively demonstrated, patients with blepharospasm may have a higher prevalence of obsessive-compulsive disorder (OCD). Drugs that act on this neurotransmitter may play a role not only in OCD, but may play a role also in blepharospasm. The serotonin uptake inhibitors such as Prozac and related drugs, however, have not been demonstrated to improve blepharospasm. Botulinum toxin remains the treatment of choice for blepharospasm and; therefore, I would recommend that you seek this treatment for your condition. Adjustments in the dosage or in the technique may be needed before optimal results can be achieved.

Joseph Jankovic, MD, Director, Parkinson’s Disease Center and Movement Disorders Clinic, Baylor College of Medicine, Houston, Texas

Q: I am a dispensing optician practicing in the UK and have a patient with BEB who is receiving BoToX® injections regularly under the national Health service. I provided her with some tinted lenses. i was wondering if there is any link (proven or unproven) with ocular myasthenia? I've looked at the info on myasthenia and it makes no mention of blepharospasm but my optometrist colleague had mentioned that the facial and eyelid twitches which are characteristic of both conditions receive the same treatment.

A: Simplified, benign essential bepharospasm (even the pseudoapraxic variant) is characterized by an OVER-action of muscles, whereas myasthenia causes an UNDER-action of muscles. There are sporadic reports in the medical literature of patients with benign essential blepharospasm (BEB) or Meige syndrome who also have myasthenia. There is even a report of a patient with myasthenia initially misdiagnosed with blepharospasm. These rare reports, however, find their way into the literature precisely because they are extremely rare curiosities, not common associations. I think that most physicians with much experience managing patients with BEB as well as other patients with myasthenia would not easily confuse these disorders. Although myasthenics may show a "flutter" of the eyelids and a sign called "Cogan's Twitch" these appear very different from the spastic form of BEB. Even pseudo-apraxic BEB looks quite different from the myasthenic eyelid droop with the former being far more intermittent, more rapidly variable, more likely to show normal eyelid elevation, and more symmetric in the simultaneous involvement of both eyes. I would hesitate to say that eyelid and facial twitches are "characteristic" of myasthenia and would strongly disagree that BEB and myasthenia receive the same treatment. In general, botulinum toxin injections are the first line treatment for BEB, whereas botulinum toxin injections are relatively contraindicated in myasthenia, since the toxins may make myasthenics worse. Edrophonium, the medication often used to treat myasthenia, is not an effective treatment for BEB. Some of the surgical eyelid opening procedures (NOT MYECTOMIES) used in many different disorders causing eyelid droop might be employed in the management of either patients with BEB or those with myasthenia; however, the overall medical and surgical approach to patients with BEB and myasthenia remains quite different.

Charles N.S. Soparkar, MD, PhD, Plastic Eye Surgery Associates, Houston, Texas

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