BEBRF Summer 2023 Newsletter

Summer 2023 • Vol 43 • Issue 3

INTERNATIONAL DYSTONIA SYMPOSIUM The hall of research posters, which stretched further than this photo (lower left) can indicate, was not only posted for the whole Symposium, but also the individual researchers were available to answer any questions during the breaks between lectures. Some posters of particular interest to blepharospasm patients include:

The Samuel Belzberg 6th International Dystonia Symposium was held in Dublin, Ireland on June 1-3, 2023. Executive Director Charlene Hudgins attended on behalf of BEBRF. There were about 500 medical practitioners, including many Fellows, as well as representatives from a few patient advocacy groups such as BEBRF. There were 3 days of lectures by some of the most renowned doctors and researchers in the dystonia field. The lectures were on a wide range of topics including genetics, treatments, and research. Dozens of researchers had produced posters detailing the results of their individual research projects, including a few in which BEBRF patients were participants. While the presentations were not recorded, a detailed synopsis will be provided later this year, and BEBRF will post how to access these when they are available. What follows is a photo journal of the activities.

This is a poster created by BEBRF Medical Advisory Board (MAB) Member Dr. Sarah Pirio Richardson and her team. Several BEBRF patients participated in this research which was to develop a smart phone app to chronicle botulinum toxin injection cycles. The app will soon be available for patients to use. The poster had an actual phone so that people could experiment with the app.

Many BEBRF patients may recall that the Newsletter asked for participants for a research project led by MAB Chair Brian Berman in Virginia, and several patients got to participate directly. The following poster (on page 4) is the result of that research. It was a pilot study, but it is hoped that this could eventually lead to new treatments.

In addition to attending the lectures and networking with colleagues, Charlene also CONTINUED ON PAGE 4

The Benign Essential Blepharospasm Research Foundation (BEBRF) is a nonprofit, 501 (c) (3) organization founded in 1981 by Mattie Lou Koster, a blepharospasm patient.

Blepharospasm (BEB) means eyelid spasm. The eyelids unpredictably and involuntarily clamp shut in both eyes, leaving the victim functionally blind until the spasm ceases in a few seconds or a few minutes.

Cranial dystonia (Meige) is a similar condition in which involuntary muscle spasms in the lower face and jaw cause grimacing and jaw movements.

Blepharospasm and cranial dystonia (Meige) are classified as movement disorders and are described as focal dystonias.

Hemifacial spasm generally begins as an involuntary contraction around one eye that gradually progresses down one side of the face to the cheek, mouth, and neck.

It is not a form of dystonia.

BEBRF is a member of the National Organization For Rare Disorders (NORD), American Brain Coalition,Dystonia Advocacy Network, Dystonia Coalition ORDR, and The Harvard Brain Tissue Resource Center (Laurie Ozelius, PhD is the BEBRF Representative).

Benign Essential Blepharospasm Research Foundation 755 S. 11th St, Ste 211 Beaumont TX, 77701 P.O. Box 12468 Beaumont, Texas 77726-2468 Phone: 409-832-0788 Fax: 409-832-0890 E-mail: bebrf@blepharospasm.org Web site: www.blepharospasm.org Contributions may be sent to: BEBRF P.O. Box 12468 Beaumont, Texas 77726-2468 The Editorial Staff reserves the right to edit any and all articles. It is our editorial policy to report on developments regarding blepharospasm, cranial dystonia and hemifacial spasm, but we do not endorse any of the drugs or treatments in the Newsletter. We urge you to consult with your own physician about the procedures mentioned.

The Blepharospasm Newsletter is published quarterly and mailed to patients, families, doctors, friends of the Foundation, and health care providers around the world.

FROM THE

EXECUTIVE DIRECTOR

MEET ME IN ST. LOUIS! We are quickly approaching the 2023 BEBRF Symposium set for September 23, 2023 at the beautiful Hyatt Regency St. Louis at the Arch (see the picture on page 14, along with a list of fun and interesting activities right in the area).

There is no charge to attend the Symposium or the Meet & Greet discussed below, and BEBRF provides complimentary breakfast and lunch.

On Friday, September 22nd, BEBRF will host the annual pre-Symposium Meet & Greet from 5:00 – 8:00 pm. There will be terrific appetizers and a cash bar. This is a great opportunity to meet with fellow blepharospasm patients and share experiences, as well as ideas, tips, and tricks that many find helpful. Then on Saturday is a full-day of presentations by expert CONTINUED ON NEXT PAGE

PROTECTING PATIENT INFORMATION: The Benign Essential Blepharospasm Research Foundation respects the privacy of patients.

Patient information is not shared outside the BEBRF organization.

TABLE OF CONTENTS International Dystonia Symposium. . . . . . . . . . . . . . . . . . . . . 1 From The Executive Director. . . . . . . . . . . . . . . . . . . . . . . . . . 2 2023 BEBRF Symposium Q&A Session 1. . . . . . . . . . . . . . . . 6 2023 BEBRF Symposium Q&A Session 2. . . . . . . . . . . . . . . . 8 Patient Story - Jana Fisette . . . . . . . . . . . . . . . . . . . . . . . . . . 10 Celebrating Our Donors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12 Things To Do At Gateway Arch National Park - Missouri. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14

Newsletter suggested donation $20.00 USD

Support Group Meetings . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15

NEWSLETTER DEADLINE DATES

Support Group Photos . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16

ISSUE

Fall

COPY DUE DATE

October 1, 2023

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The opinions expressed in the articles in this newsletter are those of the authors and do not necessarily reflect the views of the BEBRF, publisher of the BEBRF newsletter.

physicians and researchers on a wide variety of topics (see agenda, below). There are even a few new topics this year. In addition, you will be given an opportunity to ask questions of all the presenters.

While the presentations will, no doubt, be invaluable, many blepharospasm patients say that the quality time spent with other patients is their favorite reason for attending in person. Blepharospasm can often feel like a ‘lonely’ disorder, but the Symposium is a chance to feel a lot less lonely. In order to have an accurate count for food and other matters, it is very important to register for the event(s) you will be attending. Contact the BEBRF office to get registered.

BEBRF has arranged for a special hotel room rate of $159 per night (rooms at this hotel are usually $200+ per night). This special room rate is available only through September 7th. A link to reserve at the special room rate is on the BEBRF Website. If you prefer to reserve by phone, the number is 1-800-233-1234, and you must reference the group code BEBS to get the special rate. The special rate is available for reserved rooms from Wednesday, September 20th through Sunday, September 24th.

Once the number of rooms set aside for the BEBRF room block have been reserved, you will have to pay full price, so don’t hesitate to reserve!

Hopefully, I’ll meet you soon in St. Louis!

BEBRF BOARD OF DIRECTORS

Heidi Coggeshall, President/Treasurer* Tishana Cundiff, Vice PresidentEducation & Support* Peter Bakalor, Vice PresidentDevelopment* Charlene Hudgins, Executive Director/Secretary Cynthia Clark* Gina Duvalsaint* Jonathan Healy Carlas Powell* Bryan Renehan* Christopher West

EX OFFICIO

Brian Berman, MD, Advisor *Patients

MEDICAL ADVISORY BOARD

Brian Berman, MD, Chair, Richmond, VA Mark Hallett, MD, Bethesda, MD Andrew Harrison, MD, Minneapolis, MN Joseph Jankovic, MD, Houston, TX Hyder A. “Buz” Jinnah, MD, PhD, Atlanta, GA Laurie Ozelius, PhD, Boston, MA Sarah Pirio Richardson, MD, Albuquerque, NM Charles Soparkar, MD, PhD, Houston, TX Mark Stacy, MD, Greenville, NC Julie Woodward, MD, Durham, NC

DISTRICT DIRECTORS & EMAIL

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Bryan Renehan, Eastern C T, DE, DC, FL, GA, MA, MD, ME, NH, NJ, NY, NC, PA, PR, RI, SC, VT, VA, WV dir-e@blepharospasm.org Tishana Cundiff, Central AL, AR, IL, IN, IA, KS, KY, LA, MI, MN, MS, MO, ND, OH, SD, TN, TX, WI dir-c@blepharospasm.org Cynthia Clark, Western AK, AZ, CA, CO, HI, ID, MT, NE, NV, NM, OK, OR, UT, WA, WY dir-w@blepharospasm.org

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COVER ARTICLE CONTINUED

represented BEBRF to provide information about the research grant opportunities, to enlist registrations for the BEBRF international treating doctor database, and to distribute a flash drive containing a lecture delineating the different facial movement disorders and how to tell them apart.

The lecture on the flash drive was given by Dr. Laura Scorr, a neurologist at Emory University, and it was recorded through a grant BEBRF received.

BEBRF, in conjunction with other patient advocacy groups provided scholarships through the Dystonia Medical Research Foundation for Fellows studying treatment and research of dystonia to attend the Symposium. Below are the testimonials of two of the Fellows:

Dana Sugar, MD

I was generously awarded the Travel Scholarship to attend the Samuel Belzberg International Dystonia Symposium in Dublin, Ireland. As a second year movement disorders fellow at Rush University, I have developed a strong interest in the field of dystonia, both as a clinician and as a researcher. This scholarship afforded me the resources necessary to participate in this incredible conference. The scientific content was exceptional, and the short, rapid-fire format of the sessions allowed for a captivating experience as a member of the audience. I enjoyed the first day of clinically-

relevant material and learned many diagnostic and management pearls that I will take with me into my clinical practice.

The next two days of biomarker, imaging, and neurogenetics content opened my mind to the depth and complexity of the current landscape in dystonia research.

It was an inspiring event that sparked new interests and questions that will undoubtedly guide my future research pursuits. It is clear that the entire audience enjoyed this conference as much as I did, as there was fierce and friendly debate, and excellent questions asked of the speakers. The sessions included topics in non-motor symptoms of dystonia, and it was represented in several posters. The exposure to the active research in this area will allow me to further develop future research endeavors in my work in dystonia as I graduate from fellowship and begin my professional career.

I have spent the past two years of fellowship working on dystonia research, specifically, assessing non-motor symptoms and their response to botulinum toxin. I have poured over the available literature on this topic in developing this study.

In doing so, I have become acquainted with the great writers in the field. Attending this conference allowed me to meet some of the authors of this esteemed work in person, to learn from their ongoing work and experiences, and I left Dublin inspired to further my own research. Furthermore, the networking opportunities and exposure to other scientists and clinicians that this conference provides is pivotal for development in this field. I was able to connect other colleagues who may never have met. For example, my division chief and mentor from residency at the University of Utah, and a colleague across the country at Northwestern in Chicago who both are working in neurogenetics and would benefit from each other’s expertise and resources. The examples can go on and on!

I have accepted a position as Assistant Professor in Neurology at the University of New Mexico, in CONTINUED ON NEXT PAGE

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Albuquerque. I hope to continue my research in the field of dystonia, and participate as a principal investigator in clinical trials pertaining to dystonia with Dr. Sarah Pirio Richardson*, one of the many excellent speakers at the conference. I am filled with gratitude for the opportunity to attend the symposium, and I extend my deepest thanks to the Dystonia Medical Research Foundation for making this all possible. *Editor’s note: Dr. Pirio Richardson serves on the BEBRF MAB.

Matthew Woodward, MD

I am one of the awardees of the travel scholarship for the International Dystonia Symposium from June 1st to the 3rd, 2023. I attended with my mentor, Dr. Jeanne Feuerstein, as we presented a poster at the conference together titled “Form vs Function: Inappropriate Behaviors in Cervical Dystonia Beyond Deficits Predicted by Social Cognition Testing”​. It was wonderful to be able to speak more on social cognition in dystonia and have some interest generated by it.

Having the ability to attend this conference was incredibly rewarding and beneficial to me. Firstly, the range of lectures presented by the various experts in the field were helpful in many ways including clinically, academically, and researchwise. Particularly, it was interesting to see how much data there has been collected on PET imaging and the like, and what that may mean for the future for our patients. I expect my clinical ability with dystonia will only be bolstered, as well

Charlene at BEBRF Booth

With MAB Chair Dr. Brian Berman

as my ability to potentially conduct further research in dystonia, whether related to the prior poster we presented, or something new. I am especially interested in where our further diagnostic studies may lead, given the breadth of functional imaging, genetic testing, etc. that was presented.

One unexpected benefit of the conference, I believe, is the ability to connect and talk with so many different physician and patient led organizations. Particularly, having the ability to talk to the patients involved in advocacy was quite helpful to know what is important to them and how they see themselves and their diagnosis.

I would like to say thank you one last time for the travel for the symposium. It made a trip that would have otherwise not been financially feasible possible. Everyone involved was very friendly, helpful, and informative. Not to mention, the city of Dublin and country of Ireland were lovely to spend some time in, the Gaelic sports were a blast to watch, and I hope to be able to go back some day in the future to see more of it.

FINALLY, The BEBRF is pleased to announce that following the Dublin Symposium, one of the outstanding research presenters, Dr. Roy Sillitoe of Baylor College of Medicine, will discuss a summary of the current research for dystonia and blepharospasm in our next Webinar. Please stay tuned for further details/date. Also, if you would like a clearer image of the two posters pictured in this article, please contact Charlene at charlene@ blepharospasm.org.

With Dr. Sarah Pirio Richardson

With Dysphonia International Executive Director Kimberly Kuman 5

2022 BEBRF SYMPOSIUM Q & A SESSIONS REPORTED BY PETER BAKALOR

Q & A SESSION 1: DRS. MAHANT, SAMANTA, AND SUBEI

Q: Can you comment on the study that reported on the benefits of coffee in the studied cohort?

A: (Provided after the event by Dr. Mahant) Prior coffee habit was looked at as one variable among several in an Italian multicenter case control study (groups studied: one with blepharospasm compared to control groups of patients with hemifacial spasm, and another group of relatives of patients with blepharospasm). A protective effect was seen, which was proportional to the amount of coffee consumed -- one to two cups per day were needed for protective benefit. Also, the age of onset of the eye spasm was found to be later in patients who drank more coffee: 1.7 years for each additional cup per day. This may indicate a role for adenosine; caffeine blocks adenosine receptors which may play a role in the underlying cause of blepharospasm. Defazio G, Martino D, Abbruzzese G, et al. Influence of coffee drinking and cigarette smoking on the risk of primary late onset blepharospasm: evidence from a multicentre case control studyJournal of Neurology, Neurosurgery & Psychiatry 2007;78:877-879.

Q: Does having a blepharoplasty improve the results of Botox or is having a limited upper lid myectomy a better option? Part 2: I had blepharoplasty, with 100 units Xeomin every eight weeks and my lids are still closing. What else can I do?

A: Dr. Mahant: With blepharoplasty, I’m not sure it has been shown to improve blepharospasm in particular. Blepharoplasty can remove excess skin on the eyelid and if that is getting in the way, that 6

may help with vision. I don’t know that it will help with the results of the injections.

Dr. Samanta: The data for improving blepharospasm specifically is inclusive of a myectomy. Clearly, you would not expect an improvement in blepharospasm just from blepharoplasty. It might help to remove some of the excess weight which might be pushing down on the lids but other than that, no. In regard to the second question, I wouldn’t expect the blepharoplasty to have made a significant change. If it had been a myectomy, then you would be back to trying different toxins and dosages. Otherwise, deep brain stimulation might be considered a possibility and there are other non-western approaches that may help. Also, have you been evaluated for dry eye and has that been dealt with fully?

Dr. Subei: Re blepharoplasty, you have to be careful with that because oftentimes I see patients after blepharoplasty with dry eye. They can do it by putting a little sling in the eye lid and that can result in increased exposure to air. In any case, blepharoplasty can lead to increased exposure of the eye and if you have blepharospasm, an increase in the risk of dry eye is the last thing you want.

Q: Does zinc, when taken five days ahead of injections increase the effect of botulinum toxin and are there any foods with zinc you see helps injections?

A: My experience is that there hasn’t been a clear difference. A couple of studies have been published but they were small, and it’s not entirely clear what the whole mechanism for this would be.

Q: Can you damage your eyes by using outdoor FL 41 lenses indoors? Can you over massage the meibomian glands?

A: Dr. Subei: Typically not. What patients would often do is use regular sunglasses indoors and that could definitely increase the sensitivity of the eye to all wavelengths of light, so that could make the motor photophobia much worse. If you’re not going to wear the FL41 lenses, I would not wear regular sunglasses indoors. To answer the question

directly: No. You wouldn’t typically damage your eyes.

In regard to massaging the meibomian gland: as long as you’re not irritating the skin, there is no concern. If that were the case, I would stop.

Otherwise, typically warm compresses are helpful as well as massage. Also, there is intense pulse light therapy which was used initially for rosacea.

Some studies show that it can help with meibomian gland dysfunction. It stimulates the gland and decreases the inflammation that may be clogging it up or causing it to malfunction.

Q: I have taken Baclofen for blepharospasm and meige for four years with little effect. Is there another Med that you could recommend?

A: Dr. Mahant: first, it’s important that you consult your primary physician about your blepharospasm care, but outside of Baclofen, as we’ve discussed here, there are some other options. For example, benzodiazepines are an option that work a little bit differently by working on a different GABA receptor. Benzodiazapines include Xanax, Valium, Ativan, and others. They can be more effective but there is risk because they are controlled substances, and there are risks of tolerance and dependency, so you have to be very careful with that. I have to say that within my practice, the use of clonazepam (another benzodiazepine), particularly at night, seems to lessen some of the severity on a daily basis that patients experience. My patients have done quite well and not had dependency issues – again, that’s within my patient population, and that doesn’t mean that it’s not a risk. There are other options but keep in mind that as far as benefits are concerned maybe 20 to 25% of the patient population benefit from these medications. You shouldn’t rely on that as your sole source of improvement.

Q: Any progress on long-lasting botulinum toxin?

A: Dr. Samanta: There is a formulation based on type A strain that has concluded its clinical trial and has recently filed with the FDA. Typically getting approvals and the process through the FDA will delay the availability of any drug or therapy by six months or more. Until it’s officially approved it’s just guesswork as to when it might come out. I would hope that it would be available by some time

perhaps the middle of next year. The short answer is yes. There is at least one longer acting botulinum toxin that is in development and the approval process. So we hope that we will see something within a year. As far as duration of benefit is concerned: six months has been mentioned but probably between four to five months, so perhaps a five month window is the most likely result.

In the real world, that would be a substantial improvement, getting people down to two or maybe three treatments a year whereas within this room now there are probably patients who are getting five or more treatments a year.

Q: Please comment on the eye drop (Upneeq) that temporarily opens eyelids.

A: Dr. Subei: It’s a newer medicine. I haven’t really used it or rather haven’t prescribed it for my patients yet. It sounds promising, but I do have very limited experience with it. In general, with new medications, they have to be on the market for a while before you see accurate results.

Q: For surgery such as myectomy or deep brain stimulation, do you see any difference in coverage for Medicare or Medicare Advantage versus typical workplace insurance?

A: Dr. Samanta: There are as many answers as there are plans, so it’s not an easy question to give a straightforward answer to.

Q: I like bourbon and a good cigar, so the question is how much does the smoke affect my dry eye?

A: Dr. Subei: It depends on the bourbon I guess.

Smoking can definitely worsen dry eye. There are not many things that smoking improves beyond perhaps your mood. It’s not that great for your eyes especially if you’re indoors or in an enclosed environment. If you have dry eye, I would say goggle up before you go into the smoke room.

There is at least one longer acting botulinum toxin that is in development and the approval process. So we hope that we will see something within a year.

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Q & A SESSION 2: DRS. MAHANT, SAMANTA, AND SUBEI

(one in each side of the head, one behind the ear and one on the chest). The short answer is: it’s reversible, and they can be removed very safely, and what’s leftover is basically a little dent behind the forehead where the four small bur holes in the skull were.

Q: Have you heard of muscle activation therapy applied to dystonia?

A: Dr. Subramanian: In the paper I referred to, most of those patients had focal dystonias: neck Q: Can you have jaw opening and jaw closing dystonia, torticollis, and some with hand and foot when speaking at the same time? or musicians dystonia. With musicians dystonia, A: Dr. Evidente: That’s a tough question to the sense is that people use the body part a lot answer. The answer is yes, but it is very rare. and really have to be precise. Sometimes the Dystonia can be at rest or action induced. Action brain is hyperactivated because it is using this induced is when a certain action is done, the area so much, and so sometimes, in these sorts dystonia appears. For jaw opening dystonia, the of problems, we’ll send people to physical therapy most common thing you see is jaw opening at rest to retrain the brain in some way. Obviously with and yet a very small percentage of my patients with the eyes that’s going to be hard. For example, jaw opening dystonia have symptoms when they with a violinist and vibrato, if we use that hand in a speak, when they chew, or when they eat. That different way such as training them to read braille, action now triggers the jaw closing muscle. So and by doing that, reorient the same body parts in opening is at rest; closing is action induced. It’s a different way, the brain is remapped. We don’t kind of paradoxical, and the only way to prove that really understand what causes dystonia, where is to do electrophysiology. The last thing you want there is some mismatch between sensory input to do is to inject both muscles at the same time because that would negate the gains from injecting and motor output. Again, in musicians’ dystonia the movement is very refined, and the question one versus the other because those are opposing is can we retrain these areas? In response to the muscles. In that case, you have to ask the patient which one is more bothersome - jaw opening or jaw question: if you’re working with a physical therapist, the idea is they work with your physician to assess closing? It is really tough to tackle both because if what’s needed and then give it say two weeks you do both the agonist and antagonistic muscles, the effect will be zero. It’s like patients who haven’t where you are not distracted and give it a try. Work with the person, give them feedback and assess had any injections. with them. There are situations where too much Q: If DBS does not work, is it easily exercise or too much use can actually worsen the removable and if so, are there any issues left movement. after the removal?

Q: I have jaw clenching and have experienced A: Dr. Ponce: Yes, it is reversible. If you turn it very dry mouth from injections in the off, it’s like it’s not there. I’ve had patients who’ve elected to have the battery, the pacemaker and the masseters which affected the salivary glands. wires down the neck removed, but for me not to do Temporalis injections are helping. Is there a danger of impacting the salivary glands when anything with their brain, so that’s an option. Then I’ve had people who had an infection. The standard injecting the medial pterygoid?

A: Dr. Evidente: Yes. Absolutely. Anytime you of care for an infection is to remove the entire do injections around the face or the neck or the system. So, once I release the electrodes from the jaw, there is potential for dryness of mouth and fixation point behind the forehead, it basically just indirectly, dysphagia which is difficulty swallowing. slides right out. So the hardware is out, but there’s We try to minimize that in several ways. One is still the bur hole. Those kind of heal up overtime, to use the least dose, and the second is to be as but you still feel a dent and you’d have the scars 8

precise as possible therefore using EMG; third, use the most concentrated solution. The more you dilute the toxin, the more it spreads to structures that you don’t want to influence. Those are the strategies to reduce the degree of spread of the toxin and minimize the dryness of mouth.

Q: to Dr. Ponce: What’s the oldest person you’ve implanted for DBS? A: Dr. Ponce: We’ve had patients in their early 90s. Unilateral essential tremor would probably be the condition. It’s a family decision making sure that family members who might advocate for them have a chance to speak up and are part of that decision making process.

Q: Given the success rate of DBS, how long do you need to keep tweaking the Botox before you go for the DBS? A: Dr. Evidente: I would want to use at least two toxins, for example Botox and Xeomin, first. I would rarely use the only type B toxin, Myobloc,

because if injected in the face it really hurts. I rarely use Dysport. I want to try at least two of these toxins and at least two injection cycles each and of course oral medications if possible before I say let’s start talking about deep brain stimulation and things that are more invasive and radical.

Q: If you have DBS, can it help both blepharospasm and cervical dystonia?

A: Dr. Ponce: Yes it can help those segmental dystonias that affect multiple parts of the body.

Q: Can injections at the lash line affect the meibomian glands?

A: Dr. Evidente: The answer is yes, because all the botulinum toxins affect glands, and nerves are also affected not just the muscles. It also affects the autonomic nervous system or the nerves around the muscles so anything that’s driven by autonomic nerves such as the glands will be potentially affected.

Q: What is the relationship between blepharospasm and Parkinson’s?

A: Dr. Evidente: Blepharospasm is more common in patients with Parkinson’s than the normal population. It can sometimes be driven by the medicines for Parkinson’s. I’ve seen patients with blepharospasm as a dyskinesia from levodopa which is the most common drug used for Parkinson’s. What’s even more common in Parkinson’s and Parkinson’s syndrome is apraxia of eyelid opening. That’s very common. The main difference between apraxia of eyelid opening and blepharospasm is a difficulty opening the eyelids. There’s no spasm or forceful closure. It’s like your eyes are just so heavy. We see apraxia more commonly in Parkinson’s than we see blepharospasm. There is no doubt blepharospasm is seen in Parkinson’s patients. Sometimes it responds to levodopa, but also can be worsened by Parkinson’s medicines. The important thing is that if you have blepharospasm or apraxia in the setting of Parkinson’s, DBS will make those symptoms disappear.

Dr. Subramanian: I have seen apraxia of eyelid caused by DBS as a side effect but agree with everything Dr. Evidente said.

Q: If you get DBS can you still get botulinum toxin injections if you still have blepharospasm?

CONTINUED ON NEXT PAGE 9

A: Dr. Evidente: It depends on whether the DBS treats the blepharospasm or not. DBS is really effective for these conditions. The tricky thing is the programming. If your blepharospasm doesn’t respond but your Parkinson’s does, that doesn’t mean that programming by another doctor might not be able to solve both. It’s really tricky. When we published the first paper on blepharospasm, one of the things that we wrote is that you really have to stimulate the deepest contact. For instance, the Medtronic lead has four contacts from up to down so from dorsal to ventral. In that paper, we wrote the most effective contacts for blepharospasm were the deepest contacts. Usually when Dr. Ponce targets, it’s the middle contacts as the sweet spot for hand dystonia, whereas for blepharospasm it’s the deepest contact where we usually have better success. The programming can be set to program three contacts or four contacts as needed to ensure the right contacts for each problem are programmed.

Q: What were the names of the two books Dr. Subramanian referenced?

A: Dr. Vivek Murthy’s book is Together, and Noreena Hertz’s book called The Lonely Century. I urge you to read both.

Q: My doctor says I’m not a good candidate for DBS because of fear of seizures. Can you

comment on that?

A: Dr. Ponce: the gatekeeper for DBS is the neurologist, and different neurologists have different ideas and different preferences for treatment.

For example, there are neurologists that manage Parkinson’s disease and never incorporate DBS as part of their strategy. They may not have been trained in it in their Fellowship and may not want to deal with the programming. A second opinion is always a very reasonable thing to ask for. I’m more comfortable when the referral comes from a given neurologist than from a generalist. As far as seizures go, the risks from the operation are things like bruising, confusion, or a speech problem that goes away as the swelling goes down. It can manifest in a seizure. I used to see around one a year around five years ago, but don’t see them very often right now. Typically, if a seizure occurs, we would give medication, and if we don’t see another seizure, then the neurologist would stop the seizure medication eventually. Epilepsy is extremely rare - I would go so far as to say it’s not a risk and you’re not going to have permanent seizures. Under the neurologist care, discontinuing any epileptic medication for that seizure should be an inevitability.

So a seizure is a risk but it is kind of a recovery event that should be temporary.

PATIENT STORY - JANA FISETTE REPORTED BY HEIDI COGGESHALL

Jana Fisette has blepharospasm and oromandibular dystonia (“OMD”) but has seen this year significant symptom improvement via her dentist Dr. David Olson in Bridge City, TX. Jana was diagnosed and treated for blepharospasm three years ago. She had difficulty speaking, swallowing and was unable to drive, read, walk by herself, or watch 10

TV. She was treated by eyelid Frontalis Sling Suspension surgery and received Botox® with intermediate success and a poor prognosis of remission.

Along with blepharospasm symptoms, Jana presented to her dentist with sore back teeth, constant pain in her left ear and multiple headaches. Dr. Olson thought these symptoms were separated from her blepharospasm and successfully treated them with a custom made lower balanced dental occlusal splint (Tanner Splint). More significant to our community, Jana also noted some mild improvement in her dystonia.

Could Dr. Olson improve her dystonia further?

After consulting with a colleague, Dr. Steven CONTINUED ON NEXT PAGE

Broderson, and using his process of treating patients with headache pain by using orthopedic dental appliances along with cranial osteopathic adjustments, Jana’s blepharospasm and OMD symptoms began to subside. These appliances are the May Pivot appliance on the lower jaw (which replaced her Tanner Splint) and a Broderson ALF appliance on the maxilla.

After 3 months of treatment with reports of good and bad days, continued symptom resolution occurred until she could enjoy a normal quality of life. She can now drive, read, talk, and participate in social activities. Is this a cure? No! But there is hope that the dystonia may completely resolve over time. Jana still receives Botox injections for her eyes and this may also be a factor in her symptom reversals. Maybe some reduction in frequency of her Botox injections will be a test of the efficacy of this mechanical way of treatment.

Dr. Olson continues her orthopedic therapy and biweekly cranial orthopedic releases as she reports nearly complete remission.

(I, as a fellow BEBRF Meige patient visited Dr. Olson for my OMD and felt an immediate improvement in my speech with my May Pivot device he provided. Because I am not nearby, I do not have his weekly cranial adjustments, which Dr. Olson and Jana believe are critical to her success.

I also continue to receive Botox in both my upper and lower face.)

Could other patients be helped? Dr. Olson’s suggestion for potential treatment research would include an academic neuroanatomist PhD, a cranial osteopath DO, a vascular neurologist MD, and a dentist who is experienced in treatment of myofacial pain, orthodontics and gnathologic orthopedics.

From Dr. Olson: “I believe that the switch that activates the neuropathway to dystonia symptoms can be reversed. The question arises as to what it is and where it is. It could be mechanical, biochemical or physiological to a mechanical event.

Is it at the nerve source or peripheral? Why is it bilateral?

There is no question that it involves the fifth and seventh cranial nerves, and possibly the tenth as well as it sometimes affects the voice and swallowing. The temporal cranial bone is intimately related to these three cranial nerves as well as the occlusal function of the teeth. This articulation of the Jaw Joint is exposed to multiple different force directions and strengths due to chewing and bruxing.

An injury to the chin directly affects this bone, normal functional chewing affects this bone, and abnormal function of the teeth (Bruxing) also affects this bone.

A lifetime of stress and parafunctional occlusion often is related to frequent severe headaches.

Cumulative functional trauma to the temporalis David Olson, DDS bone, its neighbor bones and soft tissue may be a factor in any dystonia, therefore there may be some relief simply by decreasing the Jaw Joint pressure, massaging the cranial sutures and balancing the direction of chewing forces. Hopefully improving the cranial environment by micro adjustments to the sutures of the skull may facilitate fluid movement of blood and cerebral spinal fluid. The goal is a normal balanced organic function of the Jaw-Cranial relationship. It may be that simple.

During Jana’s orthopedic treatment with blepharospasm/miege symptom subsidence and reversal, in order to try to understand the complexity of this disease, I have been studying the dental texts by Dr. Jack Hockel, Dr. Harold Gelb, and Dr. Stephen Broderson, and videos by Brendan Stack. Along with the tempero mandibular joint texts and extensive historical literature review, I have spent significant time studying cranial osteopathic texts by Dr. McGown, Dr. Sutherland, Dr. Upledger, and the Upledger cranial sacral therapy study guide.

Integrating the information with neuro-anatomy texts and Cunningham’s Manuel of head, neck, and brain dissection is overwhelming. So when I asked Dr. Brendan Stack (who was very successful treating dystonia symptoms using orthopedic dental appliances). “What is happening?” His answer was “I don’t know but it works.” Parallels of the peripheral nervous systems and their cross communication should be investigated as there being demonstrated by sensory tricks used to offset dystonia symptoms.”

PLEASE REMEMBER BEBRF IN YOUR WILL

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BEBRF would like to publicly thank, recognize, and acknowledge our generous donors from 2022. We have tried to contact all donors for permission to list their names, but unless a donor responded with that permission by the beginning of 2023, other donors are listed anonymously.

Whether you are listed by name or not, we most sincerely thank you for your generosity.

DIAMOND SUPERSTAR DONORS ($1000 +)

Peter Bakalor Elisabeth Bathgate Charles Beckett Howard Bigelow Pamela Goss B Kelly Graves Gaines Hutcheson Kenneth Keyser Roselle Lazarov Joanne Matuzas Tracey McKinzie John C. Miller Larry Phelps Frances Shuping Joan & Steve Sullivan Nancy Wolf

$1000 +

14 Anonymous Donors

PLATINUM SUPERSTAR DONORS ($500-$999)

Ruth Anderson Sandra Chupick Heidi Coggeshall Florence Duggan-Bayne Eva Dyer Kathryn E Krug Frances McCabe Matt & Marjorie Nydell Mary Ann Olson Bryan Renehan 12

Nona Sansom Stephanie Shimada Ena Wilmot

$500-$999

8 Anonymous Donors

EMERALD SUPERSTAR DONORS ($200-$499)

Sally A Acuff Shirley Barr William Bender Vera Brazeau Elizabeth Calcutt Barbara Chestovich Cynthia Clark Julie Crothers Virginia Darrow Patricia Ellestad Elisabeth Evans Judith Feilen-Kocsis Donald Hamm Alan & Joellen Hartley Janice Hitchcock Carol Horton Al and Carol Intintoli Virgil J. Koski Elizabeth Lamar Helene Lauffer Jeanne LaVana Mark LeDoux Pam Lloyd Sherwin Low Gloria Merritt

Carolyn Muchhala Cathy Palmer Dorothy Paulsen Michel Pilon Kim Pirger Anne Reschak Susan Roser Torre Shanks Jeannetta Tignor Joseph A Tronco, Jr. Joe Trumbly Barbara Wiegel Nancy Williams Richard S Winslow MD Monica Wong

$200-$499

28 Anonymous Donors

GOLDEN SUPERSTAR DONORS ($1-$199)

Lewis Alberswerth Samuel Alvarez Robert Amort Wanda Anderson Nancy Atkinson Elizabeth Avery Karla Bailey Kenneth Baker William Baker Arnold Balsam Pauline M Barnes Linda Barnes Constance Barton

Sally Baum Constance Belcher Stuart Benas Barbara Benton Carol Benyi Marcia Berk Chandi Biswas Jane Blaho William Blue Linda Bobkoskie Frances Boothe Mary Louise Borgman Sharon Brello Frances Bruney Cynthia Buchanan Betty Burns Dr John A Burns Margaret Campbell Patricia Carew Janet M Carey Nancy Carroll Helen Rose Chestnut Norma Chisnell Margery Clancy Katherine W Clarke Velma Clement Rocky Colwell Linda Corley Anne D Craver PhD Ginny Dalton Ellen Daneke Delann De Benedetti Linda DeCubellis Pat DeMars

Beverly Derish Andrew Diamond Nancy Diebel-Downing Theresa Doss Sara Draper Mary Earnhart Liisa Ecola Beverly Edwards Jill Edwards MD Judy Eilken James Eison Diane Estep Norma Evans Thomas Fagans Sharon Falese Ellen Fealey John Feeney Korine Ferraro Andrea Fisher Margaret Fitzgerald Peggy Foster Leonard Freeman Chery Fry Mary Faith Gardiner-Ferretto William Garrett Norman Garrey Marian O Garrity Paul Gendron Mario and Linda Giacoio Peggy Gifford Diane Gouliamos Helen Greany Sue Green Gail Grueneberg Peggy Guichard Janet Guthridge Jewel Hainer Carolyn Halbert Ester Hallerman Willie Hampton Ella Hankins

Teresa Hargis Donna Harrison Margaret Ann Hartman Mary-Ellen Harwood Susan and Jim Hasselle Nancye Henkle-McPeek Annie Henson Wilma Hoffman Mark Howland Nicki Hunt Anne Janeway Burton Jaynes Mary W Johnson David S Johnson Dr David M Katz Dana Kelleher Cynthia Kester Marilyn Kibilowski Theresa Kinscherf Janice Kitchens Dr. Marty Knepper Igor Kocev C. K. Konas Jill Kostrinsky Debbie Krause Nancy Kuo Myrna Landherr Paula Lardiere Mae Lee Dorothea Leick Jaimie Levin Sarita Levine Cynthia Lewis Dee Linde Ann Loch Gregory Luzecky David Lyon Larry Mabry Nancy Maertens Jane Maher Carole Masa

Angela McClain Laurie McGee Virginia McKay Jill McLaren Ria Medendorp Sonya Miller Lynnea Miller Jo Mintzer Thomas Mollo Nadine Moore Dianne Nadler Nancie Near Janet Necessary Robert C Nelson Carol Nelson Paula Nichols Joan Nikelsky Sharon Nowlin Diane Oesterwind Kathleen Ohl Toni Okada Luisa Ontog Bonnie O’Rourke Linda Overton Kathryn Parnell Anthony Passarello Joan Perdue Jane Plantholt Sheila Pollack Laverne C Pope Francesca Judith Postemsky Marilyn J Radaz Nita Rausch Debi Rickard Jim Rienhardt Angel Roberts Gayle Robertson Eleni Rongos Gary Royse Eddie Ruiz Vonnie Rutherford

Deanna Scharf Dr Barry Scher Jeanette Schlidt Otis Scott Connie Secor Robin Serody Page M Shurley C. Miller Sigmon Martha Sinquefield Harlan Smith Sue Snyder Dale Sorenson Lawrence Stahl Steve Stephens Anna Ruth Stetenpohl Jesse Stiller Constance K Swearingen Yvonne Taczak Cathy Thomas Edna Thompson Barbara Tobias-Cowell Louise Todd-Stoll Lisa Van Sant Margaret Veedock Beatrice Vinson-Foster Magda Von Schoenebeck Eileen Wagshul Shoshana Wallach Kenneth Walls Jean Ward Noriko Watanabe Shirley Weber Renate Weber Starlene Williams Nell Williams Viola Williams Mary Wilmarth

$1-$199

334 Anonymous Donors 13

THINGS TO DO AT GATEWAY ARCH NATIONAL PARK – MISSOURI Prior to February 2018, the Gateway Arch and grounds were known as the Jefferson National Expansion Memorial. The park offers the opportunity to learn more about Thomas Jefferson and the westward expansion of the United States.

Construction of the Gateway Arch began on February 12, 1963, and it was completed on October 28, 1965.

One of the most popular things to do at Gateway Arch National Park is the tram ride to the top. The north tram was opened to the public on July 24, 1967, and the south tram was completed in 1968.

The tram ride transports you 630 feet to the top of the tallest man-made structure in the United States. You will want to plan for the tram ride to take at least an hour not counting waiting in line.

Does the Arch move?

It takes a 50-mile-an-hour wind to move the top 1 1/2 inches on each side of the center. The Arch was designed to move as much as 18 inches and can withstand an earthquake. On a normal good weather day, you should not feel the Arch moving.

How far can you see from the top of the Arch?

On a clear day, you can see up to 30 miles in each direction. It is worth noting that St. Louis is a hazy city so you will want to be prepared for a minimal view. Dense fog can surround the Arch creating zero visibility at the top.

Gateway Arch Museum The Gateway Arch Museum was re-opened in 2018 with brand new displays. The interactive museum is dedicated to telling the story of America’s pioneering spirit and the westward expansion of the United States. Exhibits span from 1764 to 1965 and include

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Colonial St. Louis, Jefferson’s Vision, Manifest Destiny, The Riverfront Era, New Frontiers, and Building the Gateway Arch.

The Monument to the Dream Film plays at the Tucker Theater located in the Gateway Arch Visitor Center. The 28-minute film details the building of the Gateway Arch.

Mississippi River Boat Cruise The “Riverboats at the Gateway Arch” is a part of the oldest Mississippi excursion boat company that has called St. Louis its home port since 1917. The Riverboats are replica 19th-century paddle-wheel riverboats named Becky Thatcher and Tom Sawyer, and they transport riders back in time to when steamboats ruled the mighty Mississippi and fueled the nation’s westward expansion, making St. Louis truly the Gateway to the West. The riverboats are located on the St. Louis Riverfront below the south leg of the Gateway Arch at 50 S. Leonor K. Sullivan Blvd., St. Louis, MO 63102.

Visit the Old Court House The Old Courthouse was the location of the Dred Scott Case along with hundreds of other civil rights suits, and it features restored courtrooms and free exhibit galleries telling the story of Lewis & Clark, Native Americans, and colonial St. Louis. You can shop for memorabilia and gifts at the Old Courthouse Shop. Entrance to the Old Courthouse is free.

SUPPORT GROUP MEETINGS To get your support group meeting in the next issue of the newsletter, please notify the Foundation office, before October 1, 2023, the next newsletter deadline. If you are interested in attending an online meeting but are not currently in a location with a support group leader, please contact us, and we will try to find you a meeting.

CENTRAL DISTRICT Houston TX Area Date: Friday, September 15, 2023 Time: 1pm – 3pm central time Location: Zoom (Please contact Carlas for invitation instructions to meeting.)

Contact Person: Carlas Powell Phone: 409-363-2206 Email: carlas.powell@gmail.com Dallas/Fort Worth Date: Tuesday, October 17, 2023 Time: Noon – 2pm Central Time Location: Zoom (Please contact Ena for invitation instructions to meeting.)

Contact Person: Ena Wilmot Phone: 817-488-0445 Email: enamwa@hotmail.com WESTERN DISTRICT Greater Los Angeles Area Date: Saturday, October 7, 2023 Time: 10am, Pacific Time Location: Zoom (Please contact Cynthia for invitation instructions to meeting.)

Contact Person: Cynthia Clark Phone: 650-678-4132 Email: clark.cyl@gmail.com Greater Los Angeles Area Date: Saturday, October 7, 2023 Time: 4:30pm, Pacific Time Location: Walter’s Restaurant, 310 Yale Ave Claremont, CA 91711 Contact Person: Cynthia Clark Phone: 650-678-4132 Email: clark.cyl@gmail.com

Western District Support Group Meeting Date: Thursday, August 10, 2023 Time: 3pm, Pacific Time Location: Zoom (Please contact Cynthia for invitation instructions to meeting.)

Contact Person: Cynthia Clark Phone: 650-678-4132 Email: clark.cyl@gmail.com Western District Support Group Meeting Date: Thursday, November 9, 2023 Time: 3pm, Pacific Time Location: Zoom (Please contact Cynthia for invitation instructions to meeting.)

Contact Person: Cynthia Clark Phone: 650-678-4132 Email: clark.cyl@gmail.com NATIONAL / INTERNATIONAL SUPPORT National Support Group Meeting Date: Friday, August 18, 2023 Time: 1pm – 3pm, Central Time Location: Zoom (Please contact Charlene for invitation instructions to meeting.)

Contact Person: Charlene Hudgins Phone: 409-832-0788 Email: charlene@blepharospasm.org AUSTRALIA Support Group Meeting Date: Monday, August 28, 2023 Time: 7pm, Central Time Location: Zoom (Please contact Charlene for invitation instructions to meeting.)

Contact Person: Charlene Hudgins Phone: 409-832-0788 Email: charlene@blepharospasm.org

National Support Group Meeting Date: Wednesday, September 13, 2023 Time: 1pm – 3pm, Central Time Location: Zoom (Please contact Charlene for invitation instructions to meeting.)

Contact Person: Charlene Hudgins Phone: 409-832-0788 Email: charlene@blepharospasm.org UNITED KINGDOM Support Group Meeting Date: Thursday, October 5, 2023 Time: 1pm – 3pm, Central Time Location: Zoom (Please contact Charlene for invitation instructions to meeting.)

Contact Person: Charlene Hudgins Phone: 409-832-0788 Email: charlene@blepharospasm.org National Support Group Meeting Date: Friday, October 20, 2023 Time: 1pm – 3pm, Central Time Location: Zoom (Please contact Charlene for invitation instructions to meeting.)

Contact Person: Charlene Hudgins Phone: 409-832-0788 Email: charlene@blepharospasm.org

IF YOU ARE INTERESTED IN LEARNING MORE ABOUT STARTING A SUPPORT GROUP, CONTACT THE BEBRF OFFICE AT 409-832-0788

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It is our editorial policy to report on developments regarding BEB/Meige and related disorders, but we do not endorse any of the drugs or Ittreatments is our editorial to report developments regarding BEB/Meige and related but domentioned. not endorse any of the drugs or treatin thepolicy Newsletter.

Weon urge you to consult with your own physician about disorders the procedures ments in the Newsletter. We urge you to consult with your own physician about the procedures mentioned.

bENIGN ESSENTIAL bLEPHAROSPASM RESEARCH FOuNDATION, INC.

Non Profit Org. U.S. Postage PAID Beaumont, Texas Permit #299

P.O. BOX 12468 BEAUMONT, TEXAS 77726-2468 409-832-0788 l Enclosed

Dated Materia

Support Group Meeting Photos DALLAS FORT WORTH SUPPORT GROUP MEETING

UPSTATE NEW YORK SUPPORT GROUP MEE

JUNE 21, 2023 • NATIONAL SUPPORT GROUP MEETING

TING JULY 14, 2023 • NATIONAL SUPPORT GROUP

MEETING