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Ask the Doctor 2014 Number 1 (January/February)

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 been getting BOTOX® injections since the mid 1980s (I participated in clinical trials in California). I am now 87 years old and about two years ago, I moved to live near my daughter. My new doctor injects less sites and uses more BOTOX® in each site. He also insists on using EMLA before the injections, which I've never needed before. The injections used to last 4 months but now they are only lasting about 3 months. The last couple of times after the injections, I've developed headaches that seem to start around my eyes. Could the BOTOX® be causing this? What about the EMLA? I've never had this problem before.

A: EMLA cream is a local anesthetic cream that can decrease the pain of injections. I have not found it necessary for most patients. One study found that using the EMLA cream may decrease the efficacy of botulinum toxin injections. It is unlikely that the EMLA cream is causing your headaches. I would recommend that you find an experienced injector who would be willing to try your old injection pattern that worked well for you in the past. There are many causes for headaches, and it is possible that your current injections are a trigger for you.

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

Q: I have had blepharospasm for many years. Along with the spasms of the muscles around the eyes, and the sensation of heavy eyelids, is the problem of constant blinking. The blinking seems to be triggered by the stinging sensation on the surface of the eye itself. The use of artificial tears and dark glasses only slightly help. I have noticed though, when having my eyes dilated during an eye exam, the constant stinging sensation stops and I get some relief. Is there a medication that numbs the surface of the eye without dilating it?

A: The reason the blinking stops is because before dilating drops are instilled in the eye, a numbing drop is used. This probably decreases the stinging. You should NEVER use numbing drops on a regular basis since they are very toxic to the eye and can cause loss of the eye. The stinging likely is due to dry eye. Please consult a Cornea/Dry Eye specialist. This needs vigorous treatment, not the occasional drop of artificial tears.

Peter J. Savino, MD, Division of Neuro-Ophthalmology, Shiley Eye Center, University of California, San Diego

Q: I am in the process of completing an application for Long-Term Care Insurance. There are many questions including do I have a neurological disorder and /or visual disorder. When completing medical forms for insurance purposes, in what category does blepharospasm fall?

A: Blepharospasm should be classified as a Neurological Disorder, particularly, a focal dystonia.

Mark Stacy, MD, Director, Parkinson's Disease and Movement Disorders, Duke University Medical Center, Durham, North Carolina

Q: For many, dry eyes are a part of BEB. Are there any advantages to installing punctual plugs as opposed to the frequent use of eye drops?

A: Dry eye is not only visually disabling, but it can also be extremely painful and threaten the very survival of the eye. It is also considered a trigger in benign essential blepharospasm (BEB), as you so correctly identified. Your question about punctal plugs is a very good and valid one and, in order to address it fully, I will first briefly mention some key points about tears.

Tears are made of mostly water but there is also a small but vital component in tears that comprises of oils. This oil component is essential for decreasing tear evaporation and, when absent, causes the eye to dry out too fast. Certain conditions such as rosacea and chronic eyelid inflammation contribute to dry eyes by resulting in decreased oil in the tear film and can also decrease the aqueous or water component as well. These situations result in not only decreased tear production but also the tears that are present are poor "quality" and lubricate the surface of the eye inadequately. In these situations we advise tear supplementation as well as other measures to improve, over a period of time, the quality of tears.

Now, coming back to your question about punctal plugs. Absolutely, if tear quality is normal or optimal but the amount is decreased due to various factors, punctal plugs can certainly play a role in keeping the eye lubricated by virtue of preventing them from draining out of the eye and forming a longer-lasting tear lake.
However, some people with eyelid inflammation may develop a condition known as "toxic tear syndrome." This is a problem where the thick, poorly diluted and rancid oils in someone with a poor quality tear film cause problematic irritation and inflammation of the eye surface that in turn could trigger BEB. As you can imagine, in these situations, placement of punctal plugs often exacerbates the problem as the irritating oils are then unable to drain away and merely further concentrate on the surface of the eye as the water from the tear film evaporates.
So, to put it in a nutshell, the treatment of dry eyes in BEB, or otherwise, depends largely on the quality and quantity of tears made by the individual. In deciding if someone would benefit from punctal plugs so that they can make use of their own tears versus constantly relying on artificial tears, your ophthalmologist should take all these factors into account such as tear production, quality of tears, eyelid inflammation and skin disorders in order to formulate the best treatment plan.

Mirwat S. Sami, MD, Plastic Eye Surgery Associates, Houston, Texas

Q: Is there a difference in treatment for apraxia of the eyelids and blepharospasm? My eyes want to close when I'm walking and BOTOX® injections seem to make them worse.

A 1: Apraxia is the inability to control the levator, the eyelid lifting muscle, to open the eyes or to keep them open. It is the most difficult problem in blepharospasm. Eyelid closing spasm can be eliminated by BOTOX® paralysis of the orbicularis, the eyelid closing muscle. But if apraxia is present, the eyes still won't open. Treatment-
  1. Careful placement of BOTOX® at the upper eyelid margin helps some persons with apraxia by removing the last amount of orbicularis closure force.
  2. Trihexyphenidyl ( (Artane) pills helps a few patients.
  3. Surgical tightening of the levator, the lifting muscle is often helpful.
  4. Bupivacaine injection of the levator helps tighten the levator but is not as powerful as surgery.
  5. The fact that you notice eyelid closure with walking may be due to the bright light outdoors - a hat and dark glasses with side-shades is very important to remove that stimulus to eye closure - once closed, apraxia keeps them from opening.

We have an ongoing research program of functional electrical stimulation of the levator to keep the eyelids open that looks very promising, but application in patients is a couple of years away.

Alan Scott, MD, Smith-Kettlewell Eye Research Institute, San Francisco, California

A 2: Apraxia is different from classic blepharospasm but can co-exist with blepharospasm about 10% of the time. Blepharospasm is the squeezing and contraction of the muscles around the eye that cause the eyelids to forcefully close. Apraxia, on the other hand, has no muscle contractions but is the inability to initiate eyelid opening from a closed position. This is due to an inhibition of the levator muscle which is the main elevating muscle of the upper eyelid. Apraxia may also occur with Parkinson's disease. Apraxia is notoriously difficult to treat but there are some interventions that can help. Standard BOTOX® injections may not help but a very minute amount of BOTOX® placed in the central upper eyelid near the eyelid margin can sometimes be effective. Limited myectomy combined with a levator muscle tightening may provide some relief. Also, a frontalis suspension with or without a limited myectomy can be very helpful. This is an operation that "slings" open the eyelids by connecting them to the eye brow lifting muscles and bypassing the apraxic levator muscle.

James R. Patrinely, MD, Plastic Eye Surgery Associates, Houston, Texas and Pensacola, Florida

Q: Are there any medications that patients should not use prior to being injected with BOTOX® - any that would interfere with how the BOTOX® works?

A: It has been suggested that anesthetic cream decreases the effectiveness of BOTOX®. I am not aware of any oral, intravenous, or intramuscular medications that interfere with BOTOX®.

Neil R. Miller, MD, Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, Maryland

Q: I was diagnosed with benign essential blepharospasm in 1987, however, my eyelids do not involuntarily clamp shut in both eyes. Initially I started having spasms in the left eye with pain alongside of my nose. The spasms with pain eventually moved to the right eye with similar symptoms. I have had botulinum toxin injections but they were unsuccessful in controlling the spasms. I do have a problem falling asleep and staying asleep and my neurologist believes it is due to the spasms. My problem sounds more like what your foundation describes as hemifacial spasm. I have been on trazodone and neurontin for sleep and take it nightly in order to fall asleep and sometimes it keeps me awake. Have you known of anyone that has hemifacial spasm and is dealing with pain as well?

A 1: Hemifacial spasm does not involve pain. Patients do, however, complain of fatigue in their face.

Raymond Sekula, MD, Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania

A 2: Without personally examining you I am unable to determine whether you have blepharospasm, which often starts asymmetrically, or whether you have bilateral hemifacial spasm, which is extremely rare (Tan EK, Jankovic J. Bilateral hemifacial spasm: A report of 5 cases and a literature review. Mov Disord 1999;14:345-349). I suggest you consult a movement disorder neurologist who can differentiate these two conditions or diagnose another disorder that would explain your symptoms. It is extremely unusual for a patient with either blepharospasm or hemifacial spasm not to obtain benefit from botulinum toxin injections.

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

Q: It is 30 days from successful botulinum toxin injections. At what point in the 90 day period, is it recommended to get reading glasses? The ophthalmologist said I had mild astigmatism, or blurriness from improper use of eye drops. I will step up my eye drops, and go in at 60 days from the injections for another exam, and get reading glasses, and computer tinted glasses. I have just been using drugstore readers for 5 years, because I thought it was futile, with my eyes changing during the 90 days.

A: For most patients with BEB who are well-controlled with botulinum neurotoxin injections (BoNT), there should be very little difference in refraction (i.e. change in the power of glasses needed) throughout a treatment cycle. If your BEB is not well-controlled and your eyelids are squeezing hard, the pressure of the eyelids can distort the shape of the eye and change the measured refraction. Prying or holding the eyes open in an attempt to obtain a refraction can also put pressure on the eye, confounding the examiner's measurements.

Everyone's response to BoNT is different. Not everyone is on a 90 day cycle. In fact, most of our patients are not. If you chart your BoNT effect, you will find a "Lag Time" (the time for the BoNT to have their full effect) and a "Duration" (the time that your BoNT lasts before your spasms start again). For most people with BEB who are not significantly changing their diet and who receive the same toxin provided by the same experienced injector with an individualized treatment pattern, Lag Time and Duration is pretty consistent from treatment to treatment for that individual, but these metrics are different from person to person. If you receive your injections at a Frequency = (Duration - 1/2(Lag Time)), then there should be minimal spasms and minimal eye drying at the start of your treatment cycle, and then it doesn't really matter when you get a refraction (exam to check for the power of glasses needed). If you find that your vision is more blurry shortly after receiving BoNT, then your eyes are probably drying out when your injections are most effective, and you need to lubricate more. If you find that your vision is most blurry just before the next BoNT injections, then you are probably trying hard to keep your eyes open, getting an incomplete blink, and thus causing eye drying, and you might wish to decrease the time between your treatments. It is difficult for an examiner to provide you with a good refraction if your eyes are dry, since drying distorts the optical surface of the eye. Short answer: You can obtain a refraction any time if your BEB is well-controlled. If your BEB is not well-controlled, then get a refraction in the middle of your cycle when your eyes are most open, least dry, and have the best vision.

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

Q: I recently had my first BOTOX® injections around my eyes, but I chose not to have them around my nose. My right nostril constantly feels like it has dust in it and is twitching and causing my right eye to twitch, too. Will botox help this nostril problem? Is that part of blepharospasm, too?

A: Without personally examining you it is difficult to be certain about the nature of your nose twitching. It is, however, possible that it is related to the blepharospasm as most patients with blepharospasm also have involuntary muscle contractions of other facial muscles, including the paranasal muscles. These facial (and mouth and jaw contractions) are a manifestation of cranial dystonia. Blepharospasm is considered one component (categorized as focal dystonia).

Q: After 20 years of having blepharospasm, I have recently been diagnosed with fortification spectrum. Is there any connection?

A: Fortification spectra, also known as scintillating scotoma, is a medical term that is used to describe visual disturbance that is often experienced before the onset of a migraine headache as part of "migraine aura." It typically starts as a tiny flickering of light that moves to the peripheral vision of one or both eyes where it expands and often assumes different shapes and patterns such as zig zag lines resembling fortification of a castle. The symptoms typically last only a few minutes, rarely more than one hour, before the migraine headache emerges. Although patients with blepharospasm often complain of increased light sensitivity (photophobia) there is no evidence that fortification spectra (or migraine headaches) are related to blepharospasm.

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

Q: I have had focal dystonia for over a decade now. Over many years I have tried many, many medications and therapies, none of which helped much. BOTOX® shots into my tongue have been beneficial, but the results are often inconsistent from month to month.

Quite by accident I tried Ambien or (zolpidem) for some sleep issues I was having, and my dystonia improved quite dramatically. I tried it again with the same results, my focal dystonia in my jaw and tongue diminished by around 70 to 80 percent. I did some research online and found some case studies where Ambien had been used to treat focal dystonia too. In combination with BOTOX® shots - it helps me enormously and I feel compelled to share this information with as many people as possible who have this seemingly intractable disease.

A: There have been a number of reports of individual patients with different forms of dystonia who have responded to zolpidem (Ambien is the brand name). The drug is being tried now for other patients; it is likely that a proper trial is warranted. Zolpidem may also have severe side-effects of sleepiness and interfering with ability to think clearly, so it must be used carefully. It is primarily used as a sleeping pill! There does seem to be a deficit of inhibition in dystonia and zolpidem might improve this.

Q: A few weeks ago, your office sent me a list of "Drugs That May Cause Dystonia". I am wondering if the BEBRF doctors would recommend an antidepressant drug that will not make my eyes worse.

A: It is difficult to give generic advice about drugs since patients do seem to differ significantly one to another. If patients have some experience with certain drugs that cause problems, then they might try drugs of a different class. The patient's personal physician should guide her.

Q: I was diagnosed with Meige in 2006 and endured the full blown symptoms affecting my eyelids, mouth, and neck muscles. I have been getting BOTOX® injections as well as oral medications. During the past 2 years, my symptoms have gradually become more controllable. I voluntarily discontinued my BOTOX® injections last April, but I am still on one oral medication (I plan to try to get off that as soon as possible). There is no question that BOTOX® was the first line of help for me as I would have never improved without it. I do not feel my Meige is in remission as the symptoms are still present. However, I can suppress the abnormal symptoms. My eyelids are well under voluntary control, my breathing and speech muscles are in need of improvement, but they are improving.

Since I ran track/cross country in college, I was able to easily start running and lifting weights again and also incorporate Pilates classes for balance and flexibility. I learned how to use diaphragmatic breathing and some biofeedback training to influence stress levels. When I run, I am constantly thinking about each step and adjusting as necessary for balance, breathing and what my body tells me. Running is not an automatic thing for me now like my younger days. Breathing while running is still a limiting factor, but it is improving. We already know that patients can use sensory tricks to temporarily help their situation. Maybe there is more to this technique than we know. Would it not be good research to investigate the effect of exercise and cuing systems on these movement disorders?

A: There are remissions, both full and partial, in patients with focal dystonias including Meige (cranial dystonia) and blepharospasm. Why and how this happens is not known. Remissions may only be temporary and it is difficult to predict what would happen in future. Perhaps the exercise is useful; we just don't know. As to the botulinum toxin, it is only symptomatic, and there is no problem in discontinuing it (temporarily or permanently).

Q: It seems there are many of the same characteristics of BEB present in Parkinson's disease (raised dopamine levels for one). Are we sure there is no connection? Are there other shared treatments besides Artane? What is the relationship if any?

A: There are connections between dystonia and Parkinson's disease. Specifically, there are often dystonic symptoms in Parkinson patients. Dystonia patients may have some Parkinsonian features, but this is not as common, and patients with blepharospasm generally do not. Exactly why there are the relationships is not completely clear, and this is the subject for ongoing research.

Q: If one accepts that many BEB patients are elderly and prone to balance problems, is ataxia more prevalent among them, and should BEB patients prepare for such?

A: Ataxia is not more prevalent in blepharospasm. Balance does become more difficult for everyone as they age, and, of course, it is difficult to balance if you are not seeing well. So blepharospasm might make balance more difficult by virtue of interfering with vision.

Q: I got a cortisone injection in the hip and noticed that my blepharospasm/apraxia improved. Why would this happen and would it be possible to get a cortisone injection once a month as a treatment for BEB?

A: There is no reason to think that a hip injection of steroid would improve blepharospasm. I suspect that it is a chance occurrence. The severity of blepharospasm does vary from time to time for unclear reasons. Perhaps if the hip was less painful, then the body would have less stress, and the blepharospasm might be better for that reason.

Mark Hallett, MD, Chief, Human Motor Control Section, NINDS, NIH, Bethesda, Maryland

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