BEBRF Spring 2024 Vol.44 Issue 2

Spring 2024 • Vol 44 • Issue 2

MYECTOMY FOR BLEPHAROSPASM: RICHARD L. ANDERSON, MD TRIBUTE LECTURE PRESENTED BY JOHN HOLDS, MD, AN OPHTHALMIC PLASTIC SURGEON WITH OPHTHALMIC PLASTIC AND COSMETIC SURGERY IN ST. LOUIS • REPORTED BY HILLIARD KELLY PRUITT The medical community recently mourned the loss of a distinguished colleague, Rick Anderson, a luminary in the field of oculoplastic surgery, ushering in a time of reflection and appreciation for his significant contributions. Dr. John Holds recently delivered a heartfelt tribute to Dr. Anderson, John Holds, MD underscoring his profound impact on the field. Anderson’s journey began at Grennel College, followed by medical school and residency at the University of Iowa. He pursued further education through fellowships with Crowell Beard in San Francisco and Orkan Stasior in Albany, both influential figures in their own right. Holds and Anderson collaborated in Salt Lake City during the late ‘80s, working together to navigate the evolving landscape of blepharospasm. Anderson’s impact on the field is profound, evident in his extensive publication record and pioneering work in treating blepharospasm.

During his prolific career, Anderson produced over 300 peer-reviewed publications; one of which was a seminal paper on the anatomic approach to blepharospasm which laid the fundamental groundwork for understanding and treating the condition. His early exploration of botulinum toxin therapy, despite initial skepticism about its FDA approval, revolutionized the field and offered hope to countless patients.

Throughout his career, Dr. Anderson’s dedication to teaching and mentorship cultivated a legacy of skilled practitioners, evidenced by the impressive lineage depicted in a symbolic American Society of Ophthalmic Plastic Reconstructive Surgeons

(ASOPRS) tree. This lineage, which includes 180 fellows, grandfellows, and great-grandfellows, continues to make significant contributions to the field, underscoring Dr. Anderson’s enduring influence and the deep impact of his training on over one-third of oculoplastic surgeons in the US. These practitioners continue to serve as an indispensable resource for patients suffering from blepharospasm.

Symptoms of blepharospasm, marked by involuntary blinking or eye closure, can sometimes be managed through supportive measures such as wearing goggles or hats. Although systemic medications offer some relief, their effectiveness is often limited. Myectomy surgery, involving the removal of eyelid closure muscles, remains relevant for select cases, albeit less frequent. Neurectomy surgery, once used alongside myectomy, is now rarely performed due to limited efficacy and risks. Patients considering myectomy should consult a specialist for optimal outcomes.

Dr. Anderson’s expertise in radical myectomy, though challenging and extensive, provided a lifeline for patients resistant to other forms of therapy. However, the advent of botulinum toxin injections marked a paradigm shift in treatment approaches, offering a less invasive yet effective alternative. Unfortunately, a subset of patients may not achieve satisfactory results with botulinum toxin alone, necessitating surgical intervention to optimize outcomes.

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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.

FROM THE

EXECUTIVE DIRECTOR

JOIN US AT DUKE UNIVERSITY FOR 2024 BEBRF SYMPOSIUM!

We are pleased to announce that this year’s Symposium will be held on Saturday, November 2, 2024 at The Albert Eye Research Institute Auditorium at Duke Medical School. The Symposium is free to attend, and BEBRF will provide breakfast and lunch that day. We have made special arrangements with the Hilton Durham at Duke University for a reduced room rate of $134/night. On Friday night, Nov. 1st, we will have the annual Meet and Greet at the hotel from 5:00 – 8:00 pm with delicious appetizers and a cash bar. You will find a link to reserve your hotel rooms on the BEBRF Website under Events -> Symposium. Or you can make reservations by calling the hotel at 919-383-8033, and be sure to mention the special BEBRF rate to get the discount. This special room rate is good through October 9, 2024.

Please also make sure that you register for both the Symposium and Meet & Greet, so that we have an accurate head count for ordering food. On the day of the Symposium, breakfast will be served beginning at 7:00 am at the hotel, and lunch will be served at Duke. Let me know in advance if you have any special dietary needs. You can register by calling the BEBRF office at 409-832-0788, or contact Kelly at kelly@blepharospasm.org.

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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 Myectomoy For Blepharospasm: Richard L. Anderson, MD Tribute Lecture. . . . . . . . . . . . . . . . . . . . 1 From The Executive Director. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 How Medicare Administrative Contractors Impact Patient Access . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 2024 BEBRF Symposium At Duke University. . . . . . . . . . . . . . . . . 6 2024 BEBRF Duke University Symosium Program . . . . . . . . . . . . 6 Effect of Alternate Toxin Concentration in

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

Blepharospasm Therapy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9

Newsletter suggested donation $20.00 USD

Dystonia Advocacy Day 2024. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13

NEWSLETTER DEADLINE DATES

Support Group Meetings and Photos. . . . . . . . . . . . . . . . . . . . . . 14

ISSUE

Summer Fall

COPY DUE DATE

July 1, 2024 October 1, 2024

Surgery For Associated Eyelid Malpositions. . . . . . . . . . . . . . . . 10 Making Sense Of All These Botulinum Neurotoxins. . . . . . . . . . 12

Gardening – My Respite from BEB . . . . . . . . . . . . . . . . . . . . . . . . 16 2

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.

This year’s Symposium Program Director is Dr. Julie Woodward, and she has put together one of the most comprehensive programs ever! You can read all about the Program and the presenters starting on page 6 of this issue. There will also be District Meetings at the close of the presentations, along with a silent auction you can bid on at the Meet & Greet and throughout the day of the Symposium. We have already received six pairs of Avulux lenses which are next generation FL41 lenses.

We are very proud to have our 2024 Symposium sponsored by Merz Therapeutics, manufacturer of Xeomin ®. Thank you Merz!

BEBRF BOARD OF DIRECTORS

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

ONGOING MEDICARE ISSUES

We are still getting calls about the various ways that Medicare is denying medically necessary treatment to people, such as dictating where the doctors can and cannot inject, or prohibiting previously approved 10 week injection cycles. If this happens to you, please do the following: Contact me at charlene@blepharospasm.org or 409-832-0788. There are advocates who need specific examples of Medicare treatment denials.

Write or email your congressional representative and let him/her know that you are being denied medically necessary treatment.

Write or email your senators for the same reasons.

If you need help finding your representative or senators, let me know, and I will help you.

The Alliance for Patient Access (AfPA) has made an infographic on this very topic. You will find it on page 5 of this Newsletter. If you want to use that in your communications, please feel free.

EX OFFICIO

Brian Berman, MD, Advisor *Patients

MEDICAL ADVISORY BOARD

Brian Berman, MD, Chair, Richmond, VA Mark Hallett, MD, Bethesda, MD Joseph Jankovic, MD, Houston, TX Hyder A. “Buz” Jinnah, MD, PhD, Atlanta, GA Laurie Ozelius, PhD, Boston, MA Victoria Pelak, MD, Denver, CO David Peterson, PhD, San Diego, CA Sarah Pirio Richardson, MD, Albuquerque, NM Charles Soparkar, MD, PhD, Houston, TX Mark Stacy, MD, Greenville, NC Julie Woodward, MD, Durham, NC Michael Yen, MD, Houston, TX

MEDICAL ACADEMIES

I attended the North American Neuro-Ophthalmology Society (NANOS Academy) annual meeting in March. While there, I distributed numerous copies of the flash drive with the lecture on the differential diagnoses of facial movement disorders, with special emphasis on eye movement disorders. It is our goal for every blepharospasm patient to get a faster diagnosis and faster access to treatment. In addition, I signed more than thirty new treating doctors to our doctor database. About a third of them were international. I was also able to distribute information about BEBRF-funded research programs to potential researchers. In addition, I shared several videos of injection patterns with the doctors, as well as general information about the Foundation that can be shared with their patients. We hope to go to a few more academies later in the year.

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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MYECTOMY FOR BLEPHAROSPASM: RICHARD L. ANDERSON, MD TRIBUTE LECTURE Radical myectomy, characterized by extensive muscle removal, is a challenging yet potentially transformative procedure for patients with severe blepharospasm. The nature of the surgery is meticulous, emphasizing the need for postoperative care and management of side effects such as numbness and swelling.

In contrast, limited myectomy, a less extensive variation of the procedure, is a viable option for patients requiring ongoing botulinum toxin therapy.

It’s important to choose the right surgical approach based on the unique needs and expectations of each patient. Myectomy should not be associated with cosmetic procedures such as blepharoplasty, as unique objectives and results are linked with each procedure.

Potential treatment horizons were outlined, including improving surgical therapy, exploring different toxins, revisiting chemodestruction, and learning more about deep brain stimulation.

Questions about the impact of myectomy on nerves and the long-term effects of certain medications

Myectomy should not be associated with cosmetic procedures such as blepharoplasty, as unique objectives and results are linked with each procedure

Former BEBRF Board President Mary Lou Thompson with Dr. Richard Anderson

were addressed, acknowledging the complexity of these topics. This tribute to Anderson serves as a reminder of the ongoing efforts to improve the lives of patients with blepharospasm and the importance of continued research and advancements. As the field continues to evolve, through innovation and collaboration, Dr. Anderson’s legacy serves as a guiding light, inspiring future generations to push the boundaries of research and treatment.

BEBRF WOULD LIKE TO THANK

Mary Louise Borgman and John Miller

FOR REMEMBERING US IN THEIR WILLS. BEBRF EXTENDS CONDOLENCES TO THEIR LOVED ONES.

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MEDICARE ADMINISTRATIVE CONTRACTORS HOW

IMPACT PATIENT ACCESS Medicare often uses private health insurers to process medical claims.

They are called Medicare administrative contractors.

Medicare administrative contractors are assigned to specific geographic regions.

Their decisions, called local coverage determinations, can vary widely.

So, patients in one area may get treatment with no trouble...

While patients in another regions are denied coverage.

Using local contractors may be necessary.

But barriers to care are not.

All patients – no matter where they live – deserve optimal care.

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2024 BEBRF SYMPOSIUM AT DUKE UNIVERSITY Julie Woodward, MD, Chief of Oculofacial Surgery at Duke University, is this year’s Program Director. She specializes in cosmetic and reconstructive surgery of the eyelids and orbit as well as the cosmetic laser surgery of the skin. She has published extensively on her work with cosmetic lasers, injectable fillers, neuromodulators, and benign essential blepharospasm in peerreviewed journals and several textbooks. She is a Professor of Ophthalmology, Oculo-plastic Surgery, and Dermatology. She currently serves on the BEBRF Medical Advisory Board. She will be speaking on laser myectomies.

Brian Berman, MD, current chair of the BEBRF Medical Advisory Board, is a Professor of Neurology at Virginia Commonwealth University. He is also the Director of VCU’s Parkinson’s and Movement Disorder Center. He joined VCU in September 2020 after a decade on faculty at the University of Colorado Anschutz Medical Campus. During his time there, he diagnosed, cared for, and treated patients in the Movement Disorders Clinic at UCHealth and in the Neurology Clinic at the Denver Veteran’s Affairs Medical Center. Additionally, he served as the Associate Director of Research at the University of Colorado Movement Disorders Center. He will speaking on non-invasive neurostimulation for dystonia.

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2024 BEBRF SYMPOSIUM AT DUKE UNIVERSITY PROGRAM INTRODUCTION 1. Edward Buckley, MD – My 40-year experience with Botulinum Toxin for Blepharospasm 2. John Dutton, MD – Movement Disorders from the Oculofacial perspective 3. Noreen Bukhari-Parlakturk, MD, PhD - Focal Adult Dystonia fMRI data and brain activity network insights for dystonia 4. Nicole Calakos, M.D., Ph.D - Cellular Processes contributing to dystonia and Updates on clinical trials for new therapeutics

TOXINS 5. Mark Stacey, MD - FDA approved toxins 6. Irene Lee, MD – New Toxins on the Horizon 7. Amy Fowler, MD – Insurance issues

MINIMALLY INVASIVE TREATMENTS 8. Amol Sura, MD – Update on Dry Eye – connections between Blepharospasm and Ocular Surface Disease 9. Burton Scott, MD - Oral Medications 10. Nathan Troy Tagg, MD – Trans Cranial Magnetic Stimulation 11. Brian Berman, MD – Non-invasive Neurostimulation Treatments for Dystonia 12. Daniel Rubenstein, MD – Protective Lenses and other Eye Treatments

SURGICAL OPTIONS 13. Mark Stacey, MD – Deep Brain Stimulation 14. Chris Demarkarian – Blepharoplasty and Ptosis Repair 15. Julie Woodward, MD – Laser Myectomy 6

Edward Buckly, MD, is a neuro-ophthalmologist at Duke University. He is the Director of Duke’s Pediatric Ophthalmology Fellowship Program. He also serves as Vice Dean of Education for Duke Medical School, as well as the Chair of the Department of Ophthalmology.

He is the past chair of both the American Board of Ophthalmology and the Ophthalmology Section of the American Board of Pediatrics. His areas of specialty include eye movement disorders, double vision, and adult strabismus. He will be speaking on the historical perspective of toxin clinical trials.

Noreen Bukhari-Parlakturk, MD, PhD, is a neurologist and movement disorder specialist at Duke University where she is an Assistant Professor of Neurology. She treats patients with movement disorders with botulinum toxin and deep brain stimulation. Her research pursuits involve finding advancing therapies for movement disorders. She also serves on the DMRF Medical and Scientific Advisory Board. She will be speaking on focal adult dystonia fMRI data and brain activity network insights for dystonia.

Nicole Calakos, MD, PhD, is a neurologist at Duke University where she is a Professor of Neurology and Cell Biology. She specializes in the treatment of patients with movement disorders like Parkinson’s, dystonia, and tremors. Her research focuses on brain plasticity mechanisms in circuits that cause movement disorders. She’s published numerous articles.

She will be speaking cellular processes contributing to dystonia and updates on clinical trials for new therapeutics.

Christopher Demarkarian, MD, is an oculoplastic surgeon at Duke University where he is an Assistant Professor of Ophthalmology. His clinical interests include medical and surgical management of many eyelid disorders such as “droopy eyelids,” eyelids that are turned in or out, and uncontrollable eyelid movements, such as twitching and facial spasms. He also offers medical and surgical treatments of blocked tear ducts. He will be speaking on blepharoplasty and ptosis repair.

Jonathan J Dutton, MD, practices oculo-plastic surgery with the University of North Carolina School of Medicine where he is Professor Emeritus of Ophthalmic and Reconstructive Surgery. His practice covers the full scope of problems related to the eyelids and orbits, including blepharospasm, eyelid malpositions, tearing disorders, thyroid eye disease, eyelid and orbital trauma, and periocular malignancies. He will be speaking on movement disorders from the oculofacial perspective.

Amy Fowler, MD, is an oculoplastic surgeon with Duke University where she is an Associate Professor of Ophthalmology and Oculo-plastic Surgery. Her clinical interests, among other things, is surgical correction of eyelid malpositions and treatment of blepharospasm and hemifacial spasm with neuralmodulators. She will be speaking on insurance issues associated with the treatment of blepharospasm.

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Irene Lee, MD, is an ophthalmologist in Durham, North Carolina and is affiliated with Duke University Hospital. She received her medical degree from Icahn School of Medicine at Mount Sinai and has been in practice between 6-10 years. Her subspecialties include oculo-plastic and orbital reconstructive surgery. She will be speaking on new toxins on the horizon.

Dan Rubinstein, MD, is an ophthalmologist and oculo-plastic surgeon affiliated with the University of North Carolina School of Medicine where he is an Assistant Professor of Ophthalmology. Dr. Rubinstein has extensive training and experience in a broad array of cosmetic and reconstructive facial procedures, focusing on eyelid, lacrimal, and orbital surgery. He will be speaking on protective lenses and other eye treatments.

Burton Scott, MD, is a neurologist and movement disorder specialist with Duke University where he is a Professor of Neurology and Movement Disorders. His areas of expertise, among others, are Parkinson’s and Huntington’s diseases, dystonia, and movement disorders. He will be speaking on oral medications for blepharospasm and its related disorders.

Mark Stacey, MD, is a neurologist with a subspecialty in Parkinson’s and movement disorders. He is currently affiliated with Medical University of South Carolina. Prior to that he served as Vice Dean of Clinical Research at Duke University. Dr. Stacy has more than 30 years of experience in caring for people with Parkinson’s Disease and Parkinsonian disorders, tremor, dystonia and other movement disorders. He has published more than 200 manuscripts and the textbook, Handbook of Dystonia. He has served as Editor of the Movement Disorders Society Newsletter: Moving Along and is currently an Associate Editor for the journal: Movement Disorders. In addition, he is currently on the BEBRF Medical Advisory Board. He will be speaking on the different botulinum toxins and deep brain stimulation.

Amol Sura, MD, is an ophthalmologist with Duke University where he is an Assistant Professor of Ophthalmology and Corneal Diseases. His areas of expertise focus on the health of the eye, especially severe dry eye. He will be speaking on the connections between blepharospasm and ocular surface disease.

Nathan Troy Tagg, MD, is a neuro-ophthalmologist with Duke University where he is an Associate Professor of Neurology, Ophthalomology, and Neuro-ophthalmology. His areas of expertise include, among other things, optic nerve and cranial nerve disorders. He will be speaking on trans-cranial magnetic stimulation.

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EFFECT OF ALTERNATE TOXIN CONCENTRATION IN BLEPHAROSPASM THERAPY PRESENTED BY KALLA A. GERVASIO, MD, NEURO-OPHTHALMOLOGIST AFFILIATED WITH SEVERAL ST. LOUIS AREA HOSPITALS • REPORTED BY CARLAS POWELL The main objectives of the presentation are 1) to discuss risks and benefits of double concentration botulinum toxin (100 units/millimeter of saline vs 50u/ml), 2) review results of double concentration botulinum toxin for blepharospasm in our practice, and 3) explore patientreported side effects, duration, Kalla A. Gervasio, MD and efficacy compared to “regular” concentration. In other words, the study was looking at high versus low concentrations of botulinum toxin and whether dilution makes a difference.

got the lower concentration on the right side of their forehead, the toxins affected a greater area of tissue because of a greater volume, and there were fewer wrinkles because the toxins traveled more of the tissue.

On the left side with higher concentration, you can see more wrinkles because the toxin stays more focal at the point of injection.

Other Studies This study consisted of 16 patients in a randomized controlled clinical trial of low concentration (10 u/ml) vs high concentration (100 u/ml). The studied treatment injected one side with a low concentration and the other side with a high concentration. The survey considered pain, bruising, and redness after injection and once the patient returned for the follow up visit, they were surveyed with whether there were complications, duration of effect and injection side preferred. Many of the patients were female (81%) with an average age of 65 years old (range from 45-92).

One of the main findings was that the injection side of the higher concentration, the pain was significantly less (58%). The average pain score 4.59 out of 10 on low concentration versus 1.94 out of 10 on high concentration. The concept is if it’s diluted with more volume and it’s possibly stretching the tissue and causing more pain. In terms of reporting from the patients, it was reported that there was no difference in bruising/redness from injections or complications (ptosis, diplopia, tearing); similar length of relief of symptoms between high vs. low concentration; and 80% reported no difference in duration of relief.

In another study conducted on the effect of volume and concentration on the Diffusion of Botulinum Exotoxin A. The prospective randomized controlled study involved 10 patients for cosmetic purposes with dynamic forehead lines. The patients received a single injection 2.5 cm above the orbital rim on both sides with half receiving a low concentration (2 u/0.1 ml) vs high concentration (2 u/0.01 ml). For two patients who

Our Studies These are some preliminary results over the past year with using double concentration treatment for our BEB patients. A retrospective cohort study with 14 patients with BEB treated with double concentration botulinum toxin. This study considered regular concentration as 5 u/0.1 ml and double concentration as 10 u/0.1 ml.

Demographics: The average age of the patient was 70 (range 55-82); 10 female (71%) and 4 male (29%).

BEB diagnosis average of 16 years (range 2-32 years).

Many of the patients had additional diagnoses, Meige syndrome (6/14, 43%), Apraxia of lid opening (3/14, 21%) and Torticollis (2/14, 14%). Many of the patients also had prior treatments including Neurotoxin (100%), Lorazepam, Clonazepam (50%), Muscle relaxants -orphenadrine, baclofen, tizanidine (71%) and Surgery myectomy (14%).

The follow-up and duration of treatment with the patients’ initial findings is that both the regular and double concentration groups average treatment dose (units) have been relatively similar (regular – 74.6 (36.7 u/side) vs double -78.1 (38.7 u/side). The follow up interval (weeks) has been similar as well (regular – CONTINUED ON NEXT PAGE 9

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10.5 (range 6-12) vs double – 11.9 (range 6-15). The average duration of effect (weeks) for regular – (7.4 (range 3-10) vs double – 8.3 (range 4-11) one week longer.

In terms of complications, ptosis and diplopia yielded similar results for both regular and double concentration. ptosis: 5/14 (36%) – regular and 4/14 (29%) - double concentration; diplopia: 1/14 (7%) for both regular and double concentration. Dry Eye yielded 7/14 (50%) regular and 4/14 (29%) double concentration. The difference is thought that double concentration you are not injecting as much fluid overall and the injection remains more focal in the muscle and shouldn’t travel as much to cause more side effects.

Overall, 8 of 14 patients (57%) reported improvement in dry eye; 7 of 14 patients (50%) had no side effects or complications; and 2 of 14 patients (14%) switched back to regular concentration due to trouble closing their eyes and tearing.

In summary, the findings yielded these results: 1. Double concentration botulinum toxin injections (100u/ml) resulted in less patient-reported dry eye symptoms.

2. Higher concentration, lower volume injections may cause less side effects due to less diffusion of toxin.

3. Similar efficacy and duration of symptom relief between regular and double concentration.

References: 1. Boyle MH, McGwin G, Flanagan CE, Vicinanzo MG, Long JA. High versus low concentration botulinum toxin A for benign essential blepharospasm: does dilution make a difference?

Ophthalmic Plast Reconstr Surg. 2009 MarApr;25(2):81-4 2. Hsu JTS, Dover JS, Arndt KA. Effect of volume and concentration on the diffusion of botulinum exotoxin A. Arch Dermatol. 2004 Nov;140(11):1351-4.

Question & Answer Q: Is this something that we ask our injectors about with what they have been injected with already and what options do you have?

A: Yes, obviously in every practice it’s going to vary probably in what concentration is being used but most commonly 50 units per millileter is a pretty standard concentration that is being used, but ask your injector if there is a different concentration that would be an option for you. If you are using 100 per ml it is slightly more challenging to give injections because it’s a very low volume that you are injecting. You have to be very careful with the injection, certain physicians may not feel comfortable doing that small of an injection so it may depend on the experience as well.

SURGERY FOR ASSOCIATED EYELID MALPOSITIONS PRESENTED BY ROBI N. MAAMARI, MD, ASSISTANT PROFESSOR OF OPHTHALMOLOGY AT THE JOHN F. HARDESTY MD, DEPARTMENT OF OPHTHALMOLOGY AND VISUAL SCIENCES • REPORTED BY BRYAN RENEHAN Dr. Maamari’s objective was to introduce common eyelid surgeries that address symptoms associated with blepharospasm and to discuss the approaches and benefits of these surgeries.

The surgeries discussed consisted of both upper eyelid and lower eyelid surgeries.

Robi N. Maamari, MD The most common upper eyelid surgeries are blepharoplasty to address hooding due to excess tissue in the eyelids and ptosis repair to correct the vision blockage caused by drooping upper eyelids.

Right Column: Before top pictures and after surgery bottom pictures.

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The upper blepharoplasty results in the removal of upper eyelid tissue consisting of excess skin, orbicularis oculi muscle, and/or orbital fat. This surgery may be performed if necessary along with a myectomy surgery.

Ptosis repair consists of three types of surgeries.

The levator advancement surgery consists of the shortening and reattachment of the levator muscle to lift eyelid. The Muller muscle resection for mild/moderate ptosis repair shortens the Muller muscle (the superior tarsal muscle is a structural muscle which functions to maintain the elevation of the upper eyelid. It is unique in that it consists of thin fibers of the smooth muscle).

The Frontalis Sling lifts the lids with a sling to brows by coupling eyelid muscles to the forehead muscle (frontalis muscle) with a small inserted sling.

Botulinum toxin treatments after surgery shows that the patients generally needed smaller doses of botulinum toxin and the interval of treatment was extended: Preop: 80.4 U (32-110; SD 33.2) – 9.6-week intervals Postop: 61.4 U (24-110; SD 34.7) – 9.8-week intervals Upper eyelid surgical expectation postop included swelling and bruising which was generally improved 50% in two weeks but can be up to 2-3 months for remaining swelling to improve. In some cases, there was an incomplete closure (lagophthalmos). With the frontalis sling levator surgery and Muller resection required

The most common upper eyelid surgeries are blepharoplasty to address hooding due to excess tissue in the eyelids and ptosis repair to correct the vision blockage caused by drooping upper eyelids. frequent lubrication with ointment, gel, or drops.

The risks of upper eyelid surgery were persistent lagophthalmos; corneal abrasions or ulcers; forehead numbness (with frontalis sling); and contour asymmetry.

Lower eyelid surgeries address the problem when the lower eyelid either turns out (Ectropion) resulting in horizonal laxity, irritation, and watering or the eyelid turning in (Entropion) resulting in the eyelashes irritating the eye. Both problems are caused by a muscle imbalance called horizontal laxity.

The surgical goal to treat an ectropion lower lid is the horizontal tightening/improved apposition. The surgical techniques consist of lateral tarsal strip – which anchors the lid at the outer rim; full-thickness wedge – which tightens by shortening the lower lid; and a medial spindle – which turns the tear drain (punctum) inward.

In the case of entropion lower lid, the surgical goal is to re-establish lower eyelid anchor to stabilize the lower lid and prevent inward rotation. The surgical techniques include: horizontal muscle tightening; reanchoring the lower eyelid retractors; and removing the overriding orbicularis muscle. One benefit to this last surgery is the possible “extended myectomy” through lower lid incision at the same time.

In summary, surgical intervention may be indicated to address eyelid malposition associated with blepharospasm. Upper and lower eyelid surgical techniques can safely correct various symptoms including peripheral vision obstruction and eyelid irritation.

PLEASE REMEMBER BEBRF IN YOUR WILL 11

MAKING SENSE OF ALL THOSE BOTULINUM NEUROTOXINS PRESENTED BY STEPHEN M. COUCH, MD, FACS, PROFESSOR AND RESEARCHER - ORBITAL AND OCULAR FACIAL PLASTIC SURGERY AT WASHINGTON UNIVERSITY IN ST. LOUIS, MISSOURI • REPORTED BY TISHANA CUNDIFF Dr. Couch began his presentation by pointing out the frequent generic mis-use of the name Botox. He reminded the audience that much like tissues often being called Kleenex (a brand-name), or a cotton-tipped applicator being routinely referred to as a Q-tip, again a brand-name, Botox, although it, “is actually a brandStephen M. Couch, MD name for a specific medication,” it is frequently mis-used to refer to various botulinum neurotoxins.

He explained that clostridium botulinum is a bacteria that forms spores commonly found in soil and can be deadly in the form of botulism as it causes muscle paralysis. This same clostridium botulinum has been found to produce seven neurotoxins, labeled by scientists as A through G. In the 1980s, Toxins A and B were found to have medical applications in low controlled doses as it causes a temporary muscle paralysis. Toxins C and D have not been shown to work in humans. Nothing was noted about the remaining three.

In examining the history of the development of these neurotoxins, Dr. Couch took us back to the 1920s. At this time, Dr. Herman Sommer purified BoNT type A. In the 1950s, it was shown to reduce muscle contraction.

As research continued over the decades, in 1989, the use of Botox brand for blepharospasm, strabismus, and hemifacial spasm was first approved by the FDA. The following decade, Dr. Jean Carruthers pioneered the cosmetic use of this same brand Botox, finding that when injected into the two vertical lines between the brows, frequently referred to as, “the 11s,” it smoothed the lines in the middle of your forhead.

Dr. Couch explained there are several subtypes of botulinum toxins that are categorized by, “where exactly in the neuron they work.”

Subtype A includes Botox, Dysport, Xeomin, Jeuveau, and Daxi.

Subtype B includes the medically approved Myoblock.

Of these different toxins, Botox and Xeomin are most frequently used for patients diagnosed with Blepharospasm.

Dr. Couch said he frequently refers to the fact that the effects are not permanent as the, “GOOD and BAD of it is that it goes away.” He explained, “If you don’t like it, it goes away, or if we need to modify the dose, it goes away. It’s BAD in the sense that if you like it [the results] and it works really well, you’ve got to keep getting injected.” This is

due to the nerve sprouting and the muscle reenervation.

The protein in its medical formula is either refrigerated or vacuum dried. It has to be reconstituted by fluid. It is important that it not be shaken, but rather swirled, to attempt to reduce the formation of bubbles prior to injecting, thus providing better concentration.

Concentration can be varied based upon reconstitution/ dilution. The injection sites, just under the skin into the muscles, are precise around the eye. Many injectors use topical anesthetics prior to administering.

Contraindicators were shared, including not administering to pregnant or nursing. Patients with some neurological conditions are also not candidates. He also reminded that therapy should not be repeated within 30 days.

Complications were also shared. Double vision is noted if the fluid enters the eye socket.

Of these medications, Dr. Couch noted:

TYPE A • Botox is vacuum sealed and requires proper refrigeration. It is approved for Blepharospasm and has several other approved uses.

•D ysport comes frozen and requires refrigeration. It is NOT approved for Blepharospasm.

•X eomin - does NOT require refrigeration and can be used in the same ration as BOTOX. • J euveau AKA “Newtox” is currently only used cosmetically.

•D axi (newest on the market) is being found to stick better in the cells and may last up to 6 months. It is noted by many patients to be more painful in administration. It has recently been approved for certain dystonias.

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TYPE B INCLUDES MYOBLOC - IS THE ONLY SUBTYPE B CURRENTLY, “FDA APPROVED FOR CERVICAL DYSTONIA.” Since “all botulinum toxins are a zinc based protein” it has been found to bind better if there are higher levels of Zync in the body. Dr. Soparkar recently developed a medication called ZYTAZE, a combination of Zync and Phytase. Although it may increase efficacy, it was not particularly endorsed.

Research continues in the potential uses of these neurotoxins. One of potential interest to the audience is a current study related to a toxin with a topical application.

DYSTONIA ADVOCACY DAY 2024 REPORTED BY HEIDI COGGESHALL The Benign Essential Blepharospasm Research Foundation through the Dystonia Advocacy Network (DAN) recently met with members of Congress to advocate for their support. Dystonia Advocacy Day was March 19, 2024. During the pandemic we advocated virtually but with the offices back open, we went back to the Hill. It is always a productive and interesting day for the volunteers. Meetings are set up with Senators and House Representatives in their various offices with the volunteers typically meeting the official’s health care aide. Volunteers are grouped by region and usually represent a variety of dystonias.

This year we asked each Senator to be a cosponsor and advance the bipartisan Safe Step Act and each Representative the same for the HELP Copays Act. Below is a photo with Senator Lisa Murkowski of Alaska who is the sponsor of the Safe Step Act. Also pictured is Senator Ron Wyden of Oregon on the right.

Our men and women in uniform are at an increased risk of developing dystonia as a result of a brain injury sustained during military service. Thus, we asked that ‘dystonia’ be continually included as a condition eligible for study through the Department of Defense PeerReviewed Medical Research Program. A few years ago, the condition was omitted from the list, and we lobbied to have it included again. We continue to advocate for it to remain. Fortunately for 2024, it was included.

We also asked Congress provide the National Institute of Health (NIH) with at least $51.3 billion to fund research

into dystonia and other disorders. The NIH supports research into dystonia, primarily through the National Institute of Neurological Disorders and Stroke (NINDS).

Without continued funding, the medical research community is concerned that development of treatment options will be delayed, and we may lose the next generation of talented young investigators to foreign competition and other fields. Finally, we asked that the CDC is provided the funding for the Chronic Diseases Education and Awareness Program.

We hope we made an impact educating members of Congress and their aides on what dystonia is and the impact they can make on our patient community. Feel free to volunteer to attend next year if you are in the area and/or also when the DAN does their advocacy virtually.

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SUPPORT GROUP MEETINGS To get your support group meeting in the next issue of the newsletter, please notify the Foundation office, before July 1, 2024, 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

NATIONAL SUPPORT

Dallas/Fort Worth SGM

National Support Group Meeting Date: Monday, May 20, 2024 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

Date: Tuesday, June 18, 2024 Time: Noon, Central Time Location: Zoom (Please contact Ena Wilmot for invitation instructions to meeting.)

Contact Person: Ena Wilmot Phone: 817-488-0445 Email: enamwa@hotmail.com Tarrant/Parker Counties, TX SGM Date: Tuesday, August 13, 2024 Time: Noon, Central Time Location: Zoom (Please contact Sharon West for invitation instructions to meeting.)

Contact Person: Sharon West Phone: 817-297-4389 Email: swest124@swbell.com Dallas/Fort Worth SGM Date: Tuesday, October 15, 2024 Time: Noon, Central Time Location: Zoom (Please contact Ena Wilmot for invitation instructions to meeting.)

Contact Person: Ena Wilmot Phone: 817-488-0445 Email: enamwa@hotmail.com North East Texas SGM Date: Tuesday, December 10, 2024 Time: Noon, Central Time Location: Zoom (Please contact Linda Trahan for invitation instructions to meeting.)

Contact Person: Linda Trahan Phone: 214-563-7937 Email: linda.trahan@sbcglobal.net 14

National Support Group Meeting Date: Wednesday, June 12, 2024 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: Thursday, September 19, 2024 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: Thursday, October 10, 2024 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 INTERNATIONAL SUPPORT

National Support Group Meeting Date: Friday, July 12, 2024 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

UK Support Group Meeting Date: Thursday, June 20, 2024 Time: 1pm – 3pm, U.S. 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, August 16, 2024 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

SUPPORT GROUP MEETING PHOTOS 04/12/24 HOUSTON SUPPORT GROUP MEETING

03/14/24 NATIONAL SUPPORT GROUP MEETING

02/23/24 NATIONAL SUPPORT GROUP MEETING

04/09/24 PARKER/TARRANT SUPPORT GROUP MEETING

02/23/24 NATIONAL SUPPORT

GROUP MEETING

TEXAS SUPPORT GROUP LEADERS MEET REPORTED BY ENA WILMOT Here is a picture from our working session where we got together to discuss ways of improving support and service to our patients in our respective areas. By supporting each other we can better support our patients. This includes the resumption of in-person meetings, especially for those patients who have expressed that they are not a fan of ZOOM. We extended a hearty welcome to Mark Freeman for joining our group. With the addition of Mark Freeman as an Area Rep, finally Texas is covered.

From left to right is Mark Freeman, Central Texas Area Rep, Sharon West, West Dallas and Fort Worth Texas Area Rep, Linda Trahan, Rockwall, and East Texas Area Rep, and Ena Wilmot, DFW Area Rep and State Coordinator. Not pictured, Carlas Powell, Houston Area Rep. As you can see, your key people are at work in the Southwest.

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GARDENING – MY RESPITE FROM BEB PATIENT STORY – ANDREA FISHER

I was diagnosed in late 2007 at the age of 47. I was very fortunate to be diagnosed by the first eye doctor I saw, and also that this was in the age of Google, which brought me here to the BEBRF. The Bulletin Board was my life saver and the cyber-friendships I have made here are near and dear to my heart. This article is written in memory of Denise, whom I celebrate each day in my gardens with the mermaid I placed there in her honor.

In the beginning, like many of you here, I struggled with how I could possibly survive this blow life had dealt me. I had no idea what my life would look like as it became increasingly harder to work, to drive, and to function. What I remember from those early days, was going to sit in my gardens. As I sat in the swing with my eyes closed, I would focus on the feeling of the breeze, the sound of the birds, the warmth of the sun. As I fought back the tears and emotions that came with this life change, I tried very hard to use my other senses – sound, smell, and feelings – even taste, to continue to enjoy the gardens I had just started working at our new home.

Somedays now when I step outside, be it the wind or the sun or whatever the mysterious cause that results in my eyes clamping shut, I make my way to the chair or bench and sit down to enjoy the sounds of the water flowing, to just listen to life around me. I take deep breaths and am grateful for the time to sit and enjoy, giving myself permission to Just Be. I think Acceptance is the part of the journey that has really helped me to live my best life. In the beginning, I had to work through the stages of grief. Denial, Anger, Bargaining, Depression, Acceptance … but when I finally reached acceptance my life resumed – accepting the change and doing what I CAN do, without focusing on what I can no longer do. There are moments that it hits me – when I must reach out for a ride or admit to someone that I cannot drive or when lagging behind on a hike as I try to maneuver the pathway with my eyes closed. Having to ask for help and acknowledge the loss of independence can bring on the sudden tears from being dependent on others, or that sudden feeling in a Box Store that I have to leave NOW – I cannot be in here one more minute, be it the lighting or the

dogs on long leashes that I can easily trip over while their mindless owners pay no attention … those moments I have to Just Breath and return to my garden.

In my garden, I feel safe and creative and stress free.

I am free to just sit if my eyes choose not to function, or I can design between the blinks – there is no rush. I can do as much or as little as I want to do. Some days I get overwhelmed – living in the south my gardens never stop growing and sometimes I feel I will never have enough good eye days to do it all. But then I remember I don’t have to do it all – I can garden as an activity, it doesn’t ever have to be done, and never will be. I can listen to the bird song and feel the breeze. I can cloud watch in between blinks as I feel the warmth of the sun. BEB has taught me to slow down, to let go of perfection and the need to do it all. It has forced me to listen to my body because if I don’t, the inevitable shutdown will force me to pay attention.

I have gained fabulous friendships by being forced to ask for help. I have limited my life to what is really important to me. I created a new path in my career that allows me flexibility and now work 100% remotely from home.

But always, I look to the gardens for my peace – the reminder that beauty surrounds me whether my eyes are open or closed. That I do not have to do everything perfectly and I can still live a life of joy and creativity, that I CAN do hard things and sometimes, even find that things aren’t so hard when I choose to do the right things for myself.

I have gardened in the south, as well as in the Rocky Mountains. There, the limited season had me surrounded by my plants indoors most of the year as I watched the snow fall and felt the warmth of the fire, watching the everchanging mountain sky – always in between the blinks, but it was always there waiting when my eyes reopened again. I found joy in exploring the city on foot and by bus when I went for injections. Do you realize the multitude of garden styles that you are able to observe when you wander and are not sitting in traffic or trying to park? I came to enjoy those days of exploration as a gift that BEB gave to me, experiences I would have never had if I weren’t dependent on public transportation and my own two feet. I learned that the joy of gardening is shared by many – wherever you go.

So, even now as I struggle to read the gardening book for my Book Club at the Botanical Gardens nearby, I know it’s OK. I don’t have to read perfectly to be surrounded by new friends who share my passion, and that just doing what I can is enough. When my eyes close, I can stop to smell the roses.

Andrea Fisher lives in Vero Beach, FL