Multiple System Atrophy News - October 2002

[Guest guest]

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Multiple System Atrophy News - October 2002

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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

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

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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.

on 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 Lane

Austin TX

USA 78728

Toll free number:

E-mail: Don.Summers@...

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

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 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 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 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. on (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. on 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. on 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

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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, 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, EMSA-SG will facilitate future research into

ecogenetics and molecular pathology of MSA by virtue of

decentralized DNA and brain tissue banking led by Gasser

and 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 or

Dr.Thyagarajan Subramanian at .

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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:

Email: bdickrn@...

Laurel or Greg

Diabetes & Glandular Disease Research Associates, PA

Located in:

San , TX

Telephone: ext. 1454 or ext. 1077

Donna Regula

Dr. Harry Pepe & Associates, Inc.

6248 Miramar Parkway

Miramar, FL 33023

Telephone:

Fax:

Connie S. Bolyard, RN

West Virginia University

Department of Medicine

Section of Endocrinology and Metabolism

C. Bryd Health Science Center

PO Box 9159

town, WV 26506

Telephone:

Janice Stack

s Hopkins Hospital

Located in:

Baltimore, MD

Telephone:

Clinton Corder, PhD, MD

COR Clinical Research, LLC

1211 N. Shartel, Suite 802

Oklahoma City,, OK 73103

Telephone:

Fax:

Email: ccorder@...

Suzanne Lash

Suncoast Neuroscience Associates, Inc.

Located in:

St. sburg, FL

Telephone:

Vendt, RN

Monarch Medical Research

6161 Kempsville Circle, Suite 315

Norfolk, VA 23502

Telephone:

Study Coordinator

Michigan Pain and Neurological Institute

Located in:

Ann Arbor, MI

Telephone: ext. 4

Email: MHNIINFO@...

Norma Skillings, RN, CRC, Carol Clayton, RN, CCRC or

Dr. Sauter

Westmoreland Neurology Associates Inc.

327 West Pittsburgh St.

Greensburg, PA 15601

Telephone: or

Fax:

Email: sauter@...

C. , M.D., Ph.D.

North Alabama Neuroscience Research

1104 Monroe Street

Huntsville, AL 35801

Telephone:

Economou & Associates, LTD

1725 W. on Street, Suite 774

Chicago, IL 60612

Telephone:

Dyan Serna

Medical College of Ohio

3000 Arlington Ave.

Toledo, OH 43614

Telephone:

Fax:

Email: ccorder@...

Dr. Bloomfield, Director of the Syncope Center

NY Presbyterian Hospital

630 W. 168th St.

New York, NY 10032

Telephone:

Fax:

Email: palumbo@...

ine LeBlanc

Dartmouth Hitchcock Medical Center

Department of Neurology

1 Medical Center Drive

Lebanon, NH 03756

Telephone:

Email: ine.R.LeBlanc@...

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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

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To subscribe to the MSA Online Support Group

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

To view past issues of Multiple System Atrophy News please go to:

http://www.mc.vanderbilt.edu/gcrc/aas/indx.htm and click on

" Patient Resources " then on " Multiple System Atrophy News "

Did you know? Much of the research news about MSA comes from leads

from you, our readers. Please, if you know of any research studies

accepting MSA patients kindly share this information by contacting

the MSA News editor at pbower@...

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