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Ask the Doctor 2013 Number 2 (March/April)

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 a ten year history of blepharospasm. My initial response to BOTOX® was good until 5 years when a limited myectomy was done by an experienced oculoplastic surgeon. For one year no BOTOX® was needed. Then BOTOX® was resumed and it required increasing dosage, most recently 100 units every 8 weeks. During the last year the associated apraxia has progressed and now is disabling, and my excellent ophthalmologist is suggesting a frontalis sling. At several of the national BEB meetings, I met patients who had had this procedure and they were not very pleased. Hence I am not very enthusiastic at the possibility, even though the eye lid closure makes any reading (my major activity) very, very difficult. Is there a better treatment option for apraxia?

A: I wish I were an expert in apraxia since it is one of the most frustrating aspects of BEB to treat. If there is one question that usually baffles the expert panels at BEBRF conferences, it is the best management of apraxia. There is no one thing that is the answer for every patient but there is a progression of things we try. It is not unusual for apraxia patients to have an inadequate response to BOTOX® since there are no spasms for the drug to block. Sometimes very small amounts (1-2 units) of pre-tarsal central upper eyelid BOTOX® near the eyelid margin can help. This placement and dosage does not usually induce ptosis. We have no drug to initiate or stimulate levator muscle contraction which is the primary problem. When myectomy has been performed by an experienced and expert surgeon, as yours has, there is a chance that removal of enough of the antagonistic closure muscles can help the apraxia patient gain some eyelid opening. Often times, however, it is necessary to go back and possibly tighten the levator muscle more or add in a frontalis sling. The sling can be made of a variety of materials. I prefer small solid silicone bands because they provide the eyelid lift but have enough elasticity to allow some, if not complete, eyelid closure. Actually I have been pleased with the clinical improvement apraxia patients have had from the slings and believe the majority of patients have found it beneficial. If there is a problem with this type of sling, it can be adjusted up or down or even totally reversed if desired. If it works you will be very happy but if it doesn't there are not a plethora of other options.

James R. Patrinely MD FACS, Plastic Eye Surgery Associates, Pensacola, Florida

Q: Deep brain stimulation (DBS) has been around since the turn of the century and dystonia patients have been candidates. Where are we today on success for benign essential blepharospasm (BEB)? They now implant one battery instead of two. Which targets in the brain do they aim for in treating BEB? Do BEB patients who have DBS surgery continue using botulinum toxin injections?

A: Deep brain stimulation (DBS) has been used a little for BEB, but usually in the context of a more extensive cranial dystonia with involvement of other parts of the face, jaw, tongue or neck. The operation has generally not been undertaken for pure BEB. The best target for improving cranial dystonia, and the BEB component, is not established. Both the globus pallidus, internal division (GPi), and the subthalamic nucleus (STN) have been targeted. Improvement with DBS has only been partial with operations so far; therefore, it might well still be necessary to continue to utilize botulinum neurotoxin injections.

Mark Hallett, MD, NINDS, NIH, Bethesda, Maryland

Q: My wife has had BEB and cervical dystonia for five years and has not yet been able to accept it very well. She is 81 and I am 84. She also suffers from chest pains (since a three way bypass operation in 10/05), chronic mucus discharge, depression and lack of energy. One of my main concerns is her worsening temper, which has greatly impaired our quality of life. I'd appreciate your take on how dystonia affects brain function, since she indicates that her feelings for family and friends have been dulled along with her ability to think.

A: Without specifically commenting on your wife's behavior, which I suspect is not directly related to her blepharospasm; I do not believe that there is a specific psychiatric component to blepharospasm. There have been some studies, however, that have indicated a higher frequency of anxiety and depression, which can be manifested by irritability and crankiness, similar to your wife's behavior. Since psychiatric symptoms, such as anxiety, depression, and obsessive-compulsive behavior, have been infrequently reported to be present even before the onset of blepharospasm (identified in 18% of 264 patients described by Grandas et al 1988), it has been suggested that the behavioral symptoms do not necessarily reflect a reaction to the blepharospasm, but are manifestations of the underlying brain (basal ganglia) dysfunction associated with blepharospasm. This possible coexistence with mild psychiatric symptoms may explain the occasional misdiagnosis of blepharospasm as a psychiatric or psychogenic problem. Overall, there is little or no evidence of any significant psychopathology specifically related to blepharospasm (Scheidt CE, Schuller B, Rayki O, et al. Relative absence of psychopathology in benign essential blepharospasm and hemifacial spasm. Neurology 1996;47:43-5).

Q: I've been getting BOTOX® shots for 20 years (I am currently getting 100 units). They used to work quickly, and I would be able to see again. But lately after the injections, I just can't see at all. My eyes continue to spasm shut. This has been going on for the last four sets of injections. I also take Klonopin (1 tablet at night). I have been under a lot of stress this past year with several illnesses and deaths in my family and wondered if it could it be stress alone or if I am getting immune to the BOTOX®?

A: Botulinum toxin injections, such as BOTOX®, are considered the treatment of choice for blepharospasm. I have been injecting some of my patients for over 25 years with continued benefit and virtually no side effects. Rarely, some patients lose their full benefit or stop responding altogether, but this is extremely rare. Since some patients who have been treated for long time had received the "old" BOTOX®, used before 1998, which contained a much high protein load than the "current" BOTOX®, it is possible that they have developed "blocking antibodies," which means that they stop responding because of immunoresistance. This used to occur in up to 10% of patients but is now extremely rare, particularly in blepharospasm patients who require a relatively low dose (e.g. 100 units). In such individuals, an alternative form of botulinum toxin, such as MYOBLOC®, may be helpful. Another reason why some patients lose their response to botulinum toxin is because they are taking certain antibiotics that may interfere with the action of the drug. Rarely, stress can also compromise the response to botulinum toxin, but the mechanism of this phenomenon is not well understood. Without knowing more about the exact circumstances, it is difficult to provide a specific answer to the question, but I hope the above explanation helps.

Q: Does high blood pressure have any relationship to dystonia?

A: No, there is no relationship.

Q: At a recent disorder meeting, a doctor suggested anyone with dystonia should try Sinemet. What is your take on this?

A: L-dopa is a component of Sinemet, a medication used most frequently to treat symptoms of Parkinson's disease (PD). L-dopa replaces dopamine, a missing chemical in the brains of patients with PD. Because dopamine does not cross the blood brain barrier and, therefore, it does not get into the brain it cannot be used in the treatment of PD. L-dopa, however, readily replaces brain dopamine and improves PD-related tremor, slowness of movement, stiffness of muscles and other motor aspects of PD. Another condition that improves with L-dopa (or Sinemet) is dopa-responsive-dystonia (DRD). This condition usually affects children who develop involuntary spasms of muscles and abnormal postures (dystonia) often involving initially the legs (but later progresses to other parts of the body), as a result of which they frequently have trouble walking and experience problems with their balance. They are often initially misdiagnosed as cerebral palsy. About half of these patients have marked worsening of their symptoms as the day progresses and some of them become bedridden by the end of the day (so called "diurnal variation") as a result of dystonia, slowness of movement, rigidity of muscles and other parkinsonian features. Many also have family members with adult-onset PD. In contrast to PD, a neurodegenerative disease associated with dopamine deficiency due to loss of dopamine-producing neurons in the midbrain (substantia nigra), DRD is due to genetic, metabolic abnormality that results in impaired synthesis of dopamine without degeneration of neurons. In both conditions, PD and DRD, L-dopa (in Sinemet) can be very effective in controlling the symptoms by replacing brain dopamine.

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

Q: I am 82 years old and have been getting BOTOX® injections for about 20 years. I am also taking Restasis for dry eye. I've had some tearing in the past after I got the injections, but it did not last very long. I got my last injections, and the tearing has been heavy and has lasted for 3 weeks. When I called the doctor about it, he says it is to be expected. I am wondering what caused it to be so bad this time and what I could do to alleviate it or avoid it happening again.

A: The eye surface must be always lubricated for the eye to remain healthy and pain-free. To achieve this, tears are constantly made, sweep across the eye with each blink, bathe the eye, and then normally depart by either evaporation into the air or drainage down the tear drains into the back of the nose and throat. Now you understand, in large part, why when someone cries hard, making lots of tears, their nose runs and they may choke, as tears flow both down the throat and down the nose. This drainage of tears is not facilitated by just gravity alone. There is an actual "pump" mechanism powered by normally blinking eyes that pumps the tears down the tear drain system. In fact, some have argued (although there has been heavy debate over this issue) that the "dry eye" in blepharospasm is due in part to excessive, forceful blinking and the rapid clearance of tears from the eye surface. So, when you use botulinum toxin to decrease the ability of the eyes to squeeze closed (forcefully blink), you decrease the normal tear drainage pump, and the tear production exceeds the rate of tear evaporation, and tears pour down the face.

If you take a twig and stick it in your eye (don't try this at home), your eyelids will squeeze shut (forceful blink) and then the eye produces copious tears to wash the foreign material out of the eye. The eye is not very smart. It will have this same kind of reaction to varying degrees whether you poke it with a finger, hit it with a softball, splash in vinegar, or allow it to dry out, all of which are irritating to the eye. When you get a botulinum toxin treatment, you decrease forceful blinking, and decrease the ability for the eyelids to spread the tears nicely across the eye. This allows the eye to dry out, and the eye's response is to make a huge quantity of tears, because the not-so-smart eye doesn't know whether it is trying to lubricate itself or wash out a twig. It turns out that the tears made in this situation are mostly water-tears, and not the complex, multi-component tears essential for keeping the eye surface healthy and safe.

So now you know two reasons why your eyes might tear after botulinum toxin treatments. What's interesting to me is that the vast majority of my blepharospasm patients DON'T make excess tears when their eyes severely dry out. Somehow this response to eye irritation is interrupted. In my experience, it tends to be the people with more mild forms of blepharospasm that experience tearing. The trick to finding the right toxin dose and injection pattern (which again in my experience must be individualized for each person) is to disturb the forceful blink of blepharospasm, but leave enough "normal" blink to keep the eyes from burning or tearing. As another aside, if your eyes burn and tear EARLY AFTER your injections, you are getting a bit too much toxin effect and adjustment to dose or location of injections may be warranted, but balanced against not getting enough effect, a little too much is often preferable, since eye drops and ointments can help. If your eyes burn and tear shortly BEFORE your next injection, you may be getting too little toxin or not getting it frequently enough (see previous article in this Newsletter on FABS, Frontalis Antagonist Blink Syndrome - March/April 2012 BEBRF Newsletter - Page 9).

The next part of your question is why did this happen this time more than ever before? This is the same as asking why is there sometimes variation in the effect of botulinum treatments? Unfortunately, MANY things can influence each treatment. Please understand the list below is a mixed bag of things that are well established to have a significant effect as well as those that are more speculative or controversial.
  1. Toxin Potency. The companies that make the toxins are now making such vast quantities in each batch, that there is no longer the huge variability (we think) in bottle to bottle potency. The toxins are also more stable now compared to decades ago, both before and after being reconstituted (solution added to the powder in which it is stored). However, the toxin seems to be quite sensitive to how it is reconstituted. If the vial is shaken vigorously, some of the toxin may be degraded. So if someone new is making up the toxin, it may make quite a difference. Alcohol applied in abundance immediately before an injection may get dragged into the injection site and can denature the toxin.
  2. Injection Precision. Although you may have an experienced injector, we are not machines, and we may not put in EXACTLY the same amount of toxin in EXACTLY the same place each time.
  3. Injection Spread. The depth of the injection (the tissue plane), the dilution of the toxin, the brand of toxin used (competing companies are constantly offering physicians special deals), and whether or not there is any bruising all may change how much the toxin spreads away from the site of injection.
  4. Host Influences. Diet may influence the toxin effect, especially zinc levels. Although "aminoglcyocide" antibiotics are frequently mentioned as influencing the effects of botulinum toxin type A, anecdotal experience with MANY patients is that other newly started antibiotics and other non-antibiotic medications may have an impact as well. We know that local anesthetics will impact toxin effect as will cold compresses applied immediately before or after treatment. Extreme outdoor temperatures (freezing cold or burning hot) may play a role if one goes outside immediately after injection, since the uptake of the toxin into the nerves is temperature dependent. Some data also supports forceful blinking within the first 30 minutes after injection, as this possibly increases toxin uptake into the nerves before it is degraded extracellularly.
So, to answer your question more succinctly, your doctor was right. But instead of saying "it's to be expected," a better answer might be, "It's not surprising. It sometimes happens."

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

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