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Multiple System Atrophy News - October 2002

Table of Contents

1. SUPPORT GROUP EVENTS
    a. SDS/MSA Regional Support Group Meeting A Huge Success!
    b. SPECIAL REPORT: Synopsis of the Chicago SDS/MSA Meeting
        by Zac Carter

2. MULTIPLE SYSTEM ATROPHY RESEARCH NEWS
    a. European Multiple System Atrophy (EMSA-SG) Study Group Program

3. CLINICAL TRIALS RECRUITING MSA PATIENTS
    a. Drug Study - Hytrin (Terazosin)
    b. Drug Study - Midodrine Hydrochloride (ProAmatine)

4. ODDS AND ENDS
    a. Dr. Cliff Shults Appears on MDTV ______________________________________________________

1. SUPPORT GROUP EVENTS
a. SDS/MSA Support Group Regional Meeting A Huge Success!

SDS/MSA Support Group Regional Meeting
Chicago Hilton
September 13-15, 2002

We had the most wonderful and uplifting meeting we have ever had. The meeting started off with a social hour on Friday night. 35 people showed up for snacks, beverages and lots of conversation.

Saturday we opened the meeting day with a breakfast buffet. Don Summers opened the meeting with greetings and introductions. We counted 80 noses in our meeting. These noses belonged to Physicians, patients, caregivers, family members, other professional staff and SDS/MSA Support Group Board members. They came from CA, CO, OK, MO, MN, IN; WI, OH, GA, PA, KY, TX, FL and of course IL. We even had a Doctor who was passing by in the hallway who had never heard of SDS/MSA and asked if she could sit in on the meeting. She and the 79 other noses were educated by Dr. Janice Gilden, our Host; who spoke on Treatment of Orthostatic Hypotension; Dr. Tom Chelimsky who spoke on MSA and Movement Disorders; Dr. David Robertson who spoke on What's New in MSA and Research; Dr. Fetnat Fouad-Tarazi who spoke on Cardiac Considerations; Dr. Nalinaskha Joshi who spoke on Sleep Disorders; Barbara Fox M.S.W. a social worker; Lori Hedges from Horizon Hospice and Randee Sable from Resurrection Home Health.

Saturday afternoon we had a Round table discussion where the audience asked the doctors questions. We also had breakout sessions for the patients with Don Summers as the mediator and Lyn Wood had the caregivers and family members. The meeting ended at 4:30 p.m.

Sunday there were 25 for breakfast and informal meeting. There were lots of friendships made and information sharing.

A big Thank you to all Doctors, patients, caregivers and family members for making the meeting a huge success.

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The SDS/MSA Support Group is a Non Profit corporation devoted to reaching and assisting the Patients, Caregivers, Family Members and Physicians who are dealing with Shy-Drager Syndrome (Also known as Multiple System Atrophy).

Our mission is to educate and support these people by establishing a never-ending circle of information among all involved. This has become known as the " Circle of Hope".

Your financial assistance is always needed and greatly appreciated.

Contributions may be mailed to:
The SDS/MSA Support Group
2004 Howard Lane
Austin TX
USA 78728
Toll free number: 866-737-4999
E-mail: Don.Summers@shy-drager.com
Website: http://www.shy-drager.com

All contributions will be acknowledged and are tax deductible.

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b. SPECIAL REPORT: Synopsis of the Chicago SDS/MSA Meeting

Synopsis of Chicago SDS/MSA Conference 2002
By Zac Carter

14 September 2002

Don Summers, full of energy and love for MSA patients, families and doctors, welcomed us all and officially opened the SDS/MSA Support Regional Meeting in Chicago. Eighty people were in attendance with representatives from California to New York and many states in between, including a large turnout from the Chicagoland area. Dr. Janice Gilden, our host, gathered together an expert panel of doctors to discuss Multiple System Atrophy. From the start, she set a tone of openness about the variety of symptoms and experiences.

Dr. Tom Chelimsky (Case Western Reserve University) presented the first talk. Billed as a neuroscience talk - 'What is SDS/MSA and Autonomic Dysfunction' - it turned out to be much more. Dr. Chelimsky explained that while there was unity in the disease process itself from person to person with MSA, a wide variety of symptoms are seen in the disease. Observations of MSA patients with common symptoms have given way to descriptions of three separate types of MSA called Striatonigral Degeneration, sporadic Olivopontocerebellar Atrophy and Shy-Drager Syndrome.

In Parkinson's patients, the substantia nigra is the main area of the brain affected. 80% of dopamine making cells die before patients present with neurologically noticeable symptoms. On diagnosis, Parkinson's patients are then given the drug L-dopa. In that disease, the way L-dopa works is somewhat understood. More (multiple) areas of the brain, I should add, are damaged in MSA than in Parkinson's Disease, hence the name Multiple System Atrophy. Important to his talk, a primary area of the brain damaged in MSA is the striatum. There is as yet no known drug to repair damage to cells in this area. The two areas mentioned (the striatum and the substantia nigra) do work so closely together that if one is damaged, the other is damaged as well, as seen on autopsy.

This intimate relationship brings us to the first form of MSA, striatonigral degeneration (SND). Dr. Chelimsky described this form as many things getting smaller, i.e. slower and smaller movements, shorter gait, fine tremor, smaller/softer voice, smaller handwriting. Parkinson's medications may help somewhat, but patients will not see the same relief that Parkinson's patients experience. In contrast to SND, the next form of MSA, sporadic Olivopontocerebellar atrophy (OPCA), is marked by damage to the cerebellum; movements are, thus, often bigger, i.e. lack of coordination, loss of balance, irregular handwriting, slurred speech, and changes in volume up and down. In the last form, Shy-Drager Syndrome, autonomic symptoms predominate including orthostatic hypotension and perhaps abnormal body sweating, as opposed to the parkinsonian or cerebellar symptoms. There are a host of other symptoms from bladder problems to sexual dysfunction which MSA patients often have. And of course, patients get a smattering of symptoms from two or even all three forms of MSA - hence the now common Neapolitan Ice Cream analogy. (See footnote for an explanation of this analogy.) And as you might now surmise, L-dopa does even less good, if any, in OPCA and Shy-Drager forms of MSA. There is as yet no equivalent to L-dopa for MSA patients.

Dr. Fetnat Fouad-Terazi (Cleveland Clinic) gave the second talk, 'Cardiac Considerations'. Simply put, she pointed out the importance of differentiating the various causes of syncope (fainting) and postural tachycardia (fast heart rate upon standing). Such careful attention to causes can help determine the appropriate course of medication as well as when a treatment may be inadvisable.

Dr. Nalinaskha Joshi (Saint Mary of Nazareth Hospital Center) followed with 'Sleep Disorders'. He kindly provided an outline handout and gave a lucid presentation on these common MSA problems. After discussing the physiology of sleep disturbances in MSA patients, he went over treatment regimes including mechanical options (CPAP and BIPAP) and surgical options. Other helpful reminders were sleep hygiene (no TV, radio and phone in the bedroom), avoidance of alcohol a few hours before bed, and regular sleep hours.

Next up, Dr. Janice Gilden (Chicago Medical School and Saint Mary Of Nazareth Hospital Center), a Midodrine expert I should add, presented 'Treatment of Orthostatic Hypotension'. OH sufferers were proud to hear such eloquent treatment options and plans available to new patients now having to battle drops in blood pressure. Two of the most favored medications are Florinef and Midodrine (marketed as ProAmatine in the United States and Gutron overseas), although she did review less commonly prescribed treatments which may be more appropriate in individual cases. Florinef increases blood volume in order to help prevent drops in blood pressure. Midodrine acts by constricting blood vessels and, thus, raising the patient's blood pressure. Of the two medications, Midodrine has proven more beneficial. Doctors, now, often add one of these medications to the other in order to attain the desired result, adjusting dosages accordingly. Also to be taken into account, Dr. Gilden noted that Midodrine is most effective within one hour and may last from two to six hours depending on the person. As for supplementary therapies, salt remains a significant volume building agent. Moreover, recent studies have shown water to be on average the most important and effective agent to raise blood pressure. We must not forget to make use of this vital tool. Other tricks Dr. Gilden mentioned were elevating the head of the bed at night, consuming smaller meals because of resultant drops in blood pressure after large meals, and timing medicine and water intake with mealtimes in order to minimize drops in blood pressure that can occur after eating. Lastly, she highly recommended that patients be seen by autonomic specialists due to their specific knowledge and experience in the complexities of blood pressure regulation.

Before the final talk of the day, we were greeted by a vibrant and incredible social worker, Barbara Fox (Saint Mary of Nazareth Hospital Center). She discussed 'Emotions, Stress, Counseling and Coping', a topic that held everyone's attention and offered more than we could realize. Once the emotional door was open, two short but important talks were given by Horizon Hospice and Resurrection Home Health Care -- services of which we should all be aware as options.

Before lunch, Dr. David Robertson (Vanderbilt University) shared his expertise on some research advances. To begin with, some 13 years ago only 4 physicians attended the American Autonomic Society meeting. Last year, eighty doctors were in attendance. Quite an improvement! Of research note, he presented initial findings of a recent paper he read which suggests doses of CoQ10 at 1200mg/day could be of benefit in slowing Parkinson's. Then of special mention, we received a short genetics seminar. The DNA in MSA patients, he said, was of sound, proper structure. However, there is an improper folding of the polypeptides throughout the DNA. The protein from this faulty mechanism then builds up in certain cells forming inclusions known as glial cytoplasmic inclusions. This genetic finding could be important in advancing the understanding of MSA. (Note: Dr. Robertson does not use PET scans as a diagnostic tool for MSA, only as a research tool.)

A delightful lunch was followed by a panel discussion with the aforementioned specialists. From the topic of support stockings to double vision, the doctors tried to help. For instance, if you live up North where stockings can also keep you warm, a man may actually wear them. In cases where patients do wear support hose, thigh-highs with a tight girdle were suggested for ease of getting on and off as well as going to the bathroom. On this same topic, be sure check your prescription in order to purchase stockings with sufficient tightness. And, do not forget to take the stockings off when lying down; without such diligence, they will not be effective when the patient returns to a standing position.

As for double vision, it may be caused by orthostatic hypotension if it occurs only when standing. If it occurs when standing and laying down, then it may well be a symptom of MSA. And finally let me mention the topic of memory. Dementia, Dr. Robertson said, is usually not associated with MSA. Indeed, it may indicate a related disorder called Diffuse Lewy Body Disease. (Note: MSA patients may have cognitive impairment. Memory remains intact but there is a retrieval of memory problem. Additionally, executive functioning can be impaired. These are primarily frontal lobe problems.) Neuropsychologists are specifically trained to diagnosis cognitive difficulties. As for research elsewhere in the US and abroad, discussion among the panel was not forthcoming. Dr. Chelimsky did though, mention that the Multiple System Atrophy Newsletter prepared by Pam Bower was a good source for current research study updates.

The day was brought to a close by two separate open dialogue patient and caregiver sessions with Don Summers and Lyn Wood respectively. And, Sunday afforded a meeting of the minds wherein the business side of things was discussed.

In conclusion, what does this conference mean for MSA patients?

- The word about orthostatic hypotension medications is getting out. Midodrine use is becoming more prevalent, often in conjunction with Florinef. Doctors are becoming more flexible about the timing and dosage of Midodrine taking into account each patient's individual reaction to the medication as well as climate, elevations in blood pressure and other factors.

- On the sleep front, polysomnography tests (PSG) are important due to the prevalence of sleep disorders in MSA. Appropriate prescribed devices and/or surgery may be helpful in alleviating symptoms of sleep apnea, etc. But also, it is important to know that we can work on training our brains with good sleep hygiene so that the disease does not have as great an effect on disturbing our rest.

- As for promise in research, an understanding of the pharmacology of the striatum (for those familiar with the term basal ganglia, it is part of the striatum) could lead to an L-dopa of sorts for MSA. In that case, a medication could help alleviate many MSA symptoms; but as in Parkinson's disease, it would not be a cure.

- A bigger possible breakthrough was described on the level of DNA wherein the prevention or correction of the folding problem could avert the formation of glial cytoplasmic inclusions in the multiple areas of the brain.

- Where research falls short, we continue to live our lives. Social workers can be a great help in coping with illness for the patient and the caregiver. When the patient is homebound, home health services can play a vital role in maintaining dignity and care. Hospice provides a remarkable service to those who wish to remain home during the final stages of the disease.

- Laughter, friendships, questions, discussions, and even some answers marked this conference as another rewarding gathering led by Don Summers.

Thank you Don!

- Zac Carter

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Footnote: MSA and the Neapolitan Ice Cream Analogy
by Pam Bower

Think of MSA as the 3 flavoured ice cream called Neapolitan which has vanilla, chocolate and strawberry all mixed together. Imagine if you took one scoop of that ice cream and put it in a dish, then took another scoop and put it in another dish and compared the two dishes. You would notice that there is not the exact same amount of vanilla, chocolate and strawberry in both of the dishes.

Multiple System Atrophy is the same as the Neapolitan ice cream. There are three flavours included in MSA:

1. Shy-Drager Syndrome (SDS) - Think of it as the strawberry ice cream
2. Olivopontocerebellar atrophy (OPCA) - Think of it as the vanilla ice cream
3. Striatonigral Degeneration (SND) - Think of it as the chocolate ice cream

Whether someone is told they have SDS or OPCA or SND they all have one scoop of Neapolitan ice cream in their dish. They all have Multiple System Atrophy.

If their symptoms are mainly orthostatic hypotension or urinary incontinence they have mostly strawberry ice cream in their dish (SDS). If their symptoms are mainly cerebellar ataxia they have mostly vanilla ice cream in their dish (OPCA). If their symptoms are mainly tremors they have mostly chocolate ice cream in their dish (SND). If they have all of the above symptoms then they might have nearly equal amounts of chocolate, strawberry and vanilla in their dish.

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2. MULTIPLE SYSTEM ATROPHY RESEARCH NEWS

a. European Multiple System Atrophy (EMSA-SG) Study Group Program
http://www.movementdisorders.org/pdf/MovingAlong_summer_02.pdf

European Multiple System Atrophy (EMSA-SG) Study Group Program
-- Gregor K. Wenning, MD, PhD, Universitats Klinik fur Neurogie, Innsbruck, Austria

Recognizing a growing need for therapeutic intervention in MSA, the EMSA-SG was formed in 1999 by 20 research groups in eleven European countries (Germany, Austria, France, United Kingdom, Portugal, Spain, Italy, Sweden, Denmark, Slovenia and Israel). EMSA-SG is coordinated by Werner Poewe and Gregor Wenning at the University of Innsbruck. In March 2001, EMSA-SG received EC support for a three- year project with the 5th framework program. The project aims to establish a European MSA Registry (EMSA-R), a unified MSA rating scale (UMSARS) as well as a "Core Assessment Program for Interventional Therapy" (CAPIT) in MSA (CAPIT-MSA). CAPIT-MSA will be designed similar to previous EC sponsored concerted efforts in Parkinson's disease (CAPIT-PD, Defer 1999) and Huntington's Disease (CAPIT-HD, Quinn 1996). CAPIT-MSA will comprise a novel set of EMSA-SG diagnostic criteria, a novel Unified Rating Scale (UMSARS) and additional investigations including autonomic function and urodynamic tests as well as structural and functional brain imaging. Task forces have been set up to promote development of the CAPIT components. The CAPIT-MSA trial protocol will be designed and validated through the first ever prospective natural history study of European patients. During the natural history study, EMSA-SG will facilitate future research into ecogenetics and molecular pathology of MSA by virtue of decentralized DNA and brain tissue banking led by Thomas Gasser and Andrew Lees.

These activities will hopefully lead to clinical trial activity within the next few years. A phase II growth hormone intervention trial has already been launched in four EMSA-SG sites. EMSA-SG has established close ties with the Northern American MSA Study Group (NAMSA-SG) chaired by Cliff Shults, San Diego, CA, USA, who are presently waiting for NIH approval of their work program, which includes a natural history study. Although financial support can only be offered to official EC partners, EMSA-SG welcomes new affiliates in the Study Group who will be regularly updated on the work program and upcoming meetings. A homepage has been set up for all those wishing to contact the Study Group (www.emsa-sg.org/).

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3. CLINICAL TRIALS RECRUITING MSA PATIENTS

a. Drug Study - Hytrin (Terazosin)
Opportunity for Patients with Multiple System Atrophy

The Cleveland Clinic Foundation is beginning a study investigating the effects of the drug Hytrin (Terazosin) on improving the symptoms commonly associated with Multiple System Atrophy. Hytrin is a commonly used, FDA-approved medication used in the treatment of Benign Prostatic Hypertrophy (BPH) (to relieve urinary retention) and Hypertension (to lower blood pressure). Patients interested in participating would undergo a neurological and physical evaluation including questionnaires and timed motor tests. Subjects are then randomized, like a flip of a coin, to either Hytrin or a placebo for several weeks and undergo a series of neurological and physical evaluations. Neither you nor your doctor will know if you are on the study medication. Every one or two weeks for 12 weeks (end of study), subjects will undergo further neurological evaluations.

For more information, contact Ruthie Kolb, at (216) 444-4598 or Dr.Thyagarajan Subramanian at (216) 444-4270.

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b. Drug Study - Midodrine Hydrochloride (ProAmatine)
http://www.centerwatch.com/patient/studies/stu37732.html

Summary: Do you often suffer from dizziness, lightheadedness, fainting and weakness in the standing/upright position?

A Multi-Center, Double-Blind, Randomized, Placebo-Controlled, Crossover Study to Assess the Clinical Benefit of Midodrine Hydrochloride (ProAmatine ) in Patients with Neurogenic Orthostatic Hypotension.

We are seeking male and female patients to voluntarily take part in a clinical research study. Patients must be aged 18 or older and diagnosed with symptomatic orthostatic hypotension (low blood pressure while in the upright position) due to Parkinson's disease, multiple system atrophy, pure autonomic failure or autonomic neuropathies (i.e. neurogenic orthostatic hypotension). Symptoms of low blood pressure include dizziness, lightheadedness, changes in vision and generalized weakness upon standing. The main effect of the drug being studied is to increase blood pressure in the upright position so symptoms will decrease.

The purpose of this clinical study is to further assess the clinical benefit of midodrine hydrochloride (ProAmatine ), an approved treatment for orthostatic hypotension. During the course of the study, participants will receive either ProAmatine or a placebo. Assessments will be made using questionnaires that measure symptom and activity levels. Blood pressure in the lying down and standing positions will be measured at each visit.

You should be aware that because ProAmatine can cause marked elevation of blood pressure while in the lying down position, it should be used in patients whose lives are considerably impaired despite standard clinical care. The indication for use of ProAmatine in the treatment of symptomatic orthostatic hypotension is based primarily on a change in a surrogate marker of effectiveness, that is, an increase in systolic blood pressure measured one minute after standing, a surrogate marker considered likely to correspond to a clinical benefit. At present, however, clinical benefits of ProAmatine , principally improved ability to carry out activities of daily living, have not been verified.

ProAmatine is contraindicated in patients with severe organic heart disease, acute renal disease, urinary retention, pheochromocytoma or thyrotoxicisis. ProAmatine should not be used in patients with persistent and excessive supine hypertension.

Please consult with your physician to see whether you might benefit from participation in this study.

The length of this study is approximately 8 weeks with a minimum of 7 required office visits. Additional office visits may be necessary. Individual patient participation could be longer or shorter depending on the number of site visits needed. Patients will receive all study- related procedures at no charge and will be financially compensated for completed site visits.

Contact a study center near you:

Barbara Dick, RN
Neurological Associates of Delaware Valley
One Medical Center Blvd.
Upland, PA 19013
Telephone: 610-876-4800
Email: bdickrn@yahoo.com

Laurel or Greg
Diabetes & Glandular Disease Research Associates, PA
Located in:
San Antonio, TX
Telephone: 210-615-5565 ext. 1454 or ext. 1077

Donna Regula
Dr. Harry Pepe & Associates, Inc.
6248 Miramar Parkway
Miramar, FL 33023
Telephone: 954-981-4811
Fax: 954-981-9295

Connie S. Bolyard, RN
West Virginia University
Department of Medicine
Section of Endocrinology and Metabolism
Robert C. Bryd Health Science Center
PO Box 9159
Morgantown, WV 26506
Telephone: 304-293-4117

Janice Stack
Johns Hopkins Hospital
Located in:
Baltimore, MD
Telephone: 410-614-4576

Clinton Corder, PhD, MD
COR Clinical Research, LLC
1211 N. Shartel, Suite 802
Oklahoma City,, OK 73103
Telephone: 405-272-8481
Fax: 405-272-8491
Email: ccorder@corclinical.com

Suzanne Lash
Suncoast Neuroscience Associates, Inc.
Located in:
St. Petersburg, FL
Telephone: 727-824-7135

David Vendt, RN
Monarch Medical Research
6161 Kempsville Circle, Suite 315
Norfolk, VA 23502
Telephone: 757-466-7263
 

Study Coordinator
Michigan Pain and Neurological Institute
Located in:
Ann Arbor, MI
Telephone: 734-677-6000 ext. 4
Email: MHNIINFO@AOL.com

Norma Skillings, RN, CRC, Carol Clayton, RN, CCRC or
Dr. Michael Sauter
Westmoreland Neurology Associates Inc.
327 West Pittsburgh St.
Greensburg, PA 15601
Telephone: 724-835-1921 or 724-836-7450
Fax: 724-836-7452
Email: sauter@westol.com

George C. Morgan, M.D., Ph.D.
North Alabama Neuroscience Research
1104 Monroe Street
Huntsville, AL 35801
Telephone: 256-533-3552

Economou & Associates, LTD
1725 W. Harrison Street, Suite 774
Chicago, IL 60612
Telephone: 312-829-3532

Dyan Serna
Medical College of Ohio
3000 Arlington Ave.
Toledo, OH 43614
Telephone: 419-383-3697
Fax: 419-383-3041
Email: ccorder@corclinical.com

Dr. Daniel Bloomfield, Director of the Syncope Center
Y Presbyterian Hospital
630 W. 168th St.
New York, NY 10032
Telephone: 212-305-6634
Fax: 212-305-3137
Email: palumbo@nyp.org

Pauline LeBlanc
Dartmouth Hitchcock Medical Center
Department of Neurology
1 Medical Center Drive
Lebanon, NH 03756
Telephone: 603-650-4165
Email: Pauline.R.LeBlanc@Hitchcock.Org

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4. ODDS & ENDS

a. Dr. Cliff Shults Appears on MDTV
MDTV: New Developments In Parkinson's Disease
(#6384; 26 min.)

Dr. Cliff Shults hosts a national panel of experts in genetics and neurology to discuss current research in genetic and environmental causes of Parkinson's Disease.

Watch it now using RealPlayer. http://www.uctv.tv/library-test.asp?showID=6384

 

If you are interested in being a support group leader or local contact send a note to MSA-Contacts-owner@yahoogroups.com

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To subscribe to the MSA Online Support Group

Please visit http://groups.yahoo.com/group/shydrager

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