New-comer - Observations and Questions

[Guest guest]

Hi Fred,

It sounds to me like you have hit the main sites for information on

cerebellar atrophy or ataxia. Make sure you check out the E-NAF Bulletin

Board accessible from the NAF website and also their newsletter

Generations... many articles are available online.

Have you seen this site yet? http://www.geneclinics.org/ You have to

register and then click on GeneReviews (button at the top of the page) and

search for " ataxia " . You'll find descriptions of all the known hereditary

types of SCA and other lesser known ataxias. This site is updated

frequently.

To stay informed of the latest research I search Pubmed regularly:

http://www.ncbi.nlm.nih.gov/entrez

I'm curious what genetic tests you've had done to this point. Have they

checked you for Friedreich's? It's rare as a late onset but not unheard of.

Multiple System Atrophy presents first with cerebellar atrophy/ataxia

symptoms in about 20% of cases, in the other 80% it presents first with

parkinson-like symptoms. According to Dr. Sid Gilman's (U of Mich.)

research 25% of people who first present with sporadic cerebellar atrophy

tend to progress further to develop more symptoms characteristic of MSA

within 5 years. Although we do know of patients who have lived with a

diagnosis of MSA for 20 years or more, this would not be the average. You

may have gotten that figure from a site that was discussing hereditary

ataxia which tends to have a slower course.

Here are two articles you may not have seen:

Multiple System Atrophy

http://www.emedicine.com/neuro/topic671.htm

Olivopontocerebellar Atrophy

http://www.emedicine.com/neuro/topic282.htm

And here's something your doctors should attempt to rule out in your case:

Paraneoplastic Cerebellar Degeneration

http://www.emedicine.com/neuro/topic299.htm

My mother-in-law was diagnosed with OPCA/ataxia in 1994 with no history of a

similar disorder in her family. I contacted NAF at that time and began

researching the various forms of ataxia, both hereditary & sporadic. If

genetic forms of ataxia are ruled out some people never find out their true

diagnosis. In my mother-in-law's case we only suspected MSA because of her

other symptoms which included: Sleep Apnea & loud snoring, low blood

pressure & urinary incontinence These symptoms are consistent with

" possible MSA " .

Regards,

Pam

[Guest guest]

Fred,

Welcome to our family, sorry you had to find us. We have a couple

of other people from New Zealand, but haven't heard from them in a while.

We have a bunch of Aussies on the list.

Sources - I can't come up with any more sites than you have found.

This group is good at finding new info as all of us use different search

engines. ) Of course that often means that you hear about the same

research from Reuters, AP, Yahoo, MSN news, and many other sources, but

that doesn't hurt.

High arches - sorry I've never heard a word about that associated with

MSA, my wife did not have them.

Nutritional supplements - We have heard a lot, we have yet to hear any

definitive positive results from known MSA patients. The only semi-authoritive

thing I have seen is that 400-800 IU of vitamin E, 60 mg of vitamin C and

some (probably 200 mg or less) CoQ10 per day MIGHT slow the progression

slightly. But the study concluded ONLY that "it might be worthwhile

to continue the study". It was a very low sampling and was not published

(reviewers tore it apart). It was done on rats with unspecified ataxia

problems and I do not know how they extrapolated it to human dosage, but

those amounts fall within the U.S. government guidelines for those antioxidents.

Unfortunately, adding all three has never been tested for toxicity either

(according to one reviewer) and since the reviews, the abstract disappeared

from the web. I can not remember the name of the grad student and

the link now reads, "Not found". I have not seen anything on NAC

on MSA.

I have not seen Dr. Tuft's work either, do you have a website where

it is published? Several people on the list say that blueberries

are good for constipation. Often extracts do not work as well as

the real thing as some nutrients are lost in the extraction process.

I am sure that eating two cups of blueberries a day would not harm anyone

(except their pocketbook maybe). We do believe that cranberries are

helpful for preventing urinary tract infections (UTI's).

We strongly believe in drinking a full two litres of liquids per day

(caffinated drinks do not count). This helps maintain your BP and

it helps prevent UTI's by preventing dehydration which can be a serious

problem with MSA.

EXERCISE - This

is good for maintaining movement - our motto is "Use it or lose it".

Range of motion exerciseis mandatory, aerobic is okay if you can do it,

however if it causes falls or dizziness - stop. My recommendation

is a minimum od 20 minutes twice a day every day for range of motion.

More exercise than that is up to what you can do, do NOT exercise to the

point that you are in pain.

DIET - A

balanced diet is best. If you are having any problems with swallowing

or speech, a mechanically soft diet is best. Liquids and solid food

may become a swallowing problem at some point.

ALTERNATIVE/COMPLIMENTARY TREATMENTS

- We have heard

much, we have seen little. Accupuncture has helped a few with temporary

pain relief, but so have muscle relaxers (which are cheaper). Massage

seems to work for pain and stiffness somewhat also, but the cost benefit

here again is questionable.

AFO's - Sorry,

I am not sure what you mean here, it sounds like what we in the USA call

a "soft cast" and I know of no one who wears one. My wife wore a

foot brace/splint to help stretch her tendons and prevent "foot drop" at

times. It may have helped.

LIFE-SPAN - This

is a very anectdotal subject. Because of how long it took to diagnose,

questionable diagnoses, and other factors - we still GUESS the range is

on the order of 2-25 years. One study back in 1995 suggested that

the average was 9.5 years with a range of 2-20 years. We DO know

more about how to care for patients today than then. My wife showed

her first signs of MSA (fatigue and stiffness) in April 1986, but was not

diagnosed with any movement disorder until they said Parkinson's in 1990,

then she was changed to MSA in 1995. Was completely in a wheelchair by

early 1996. She died in late 2001. Watching for signs of infection,

aspiration and fighting dehydration can save the patient's life.

Exercise can help keep movement. A good attitude on the patients

part can help a lot. Then we have Aussie Anne - all the doctor has

to do is tell her she is going to die, then she goes out and gets another

university degree. They told her (23 years now Anne?) that she had

a year to live, she got a college degree in 1999. They told her 18

months ago that she had a few months to live, she got a stomach pacemaker

and is back working on another degree (taking a statistics course now).

SPONTANEOUS REMISSION -

This does not apply -

Cancer is cells growing wild, you can not stop them and the remission is

when they stop growing wild for a time. MSA is the slow death of

brain cells and at present there is no way to stop them from dying.

We are hopeful they will find a way to slow the death, or grow new cells

with stem cell research, but that is still in the future at this point.

Keep exercising!

Take care, Bill Werre, Herndon,

Virginia, USA (I hope to be in NSW and Queensland, Australia in May

for about two weeks)

[Guest guest]

To the shydrager group at large,

I assume that some have already responded to this posting as far other sites of interest, but

I was intrigued by the "high foot arch" question. My brother (who has MSA) has a very high foot

arch. He was never able to wear any type of shoe but a lace-up do to this issue. Does anyone

have any other information on this issue?

Jerry Cash

-----Original Message-----From: Fred Seeker Sent: Friday, March 29, 2002 12:52 AMTo: shydrager Subject: New-comer - Observations and Questions

I have subscribed to this forum since last month. This is my first posting, but I have perused the emails with interest and the site looks well-run and helpful.

I was diagnosed last year with spino-cerebellar atrophy (SCA) - not otherwise specified. Late on-set (50'ies). Possibly sporadic (non-hereditary) as no known family history. The neurologists think it started about 6-7 years ago. They had previously diagnosed me with something else, otherwise I would have looked at SCA earlier.

My primary symptoms are 1) ataxia. I walk with difficulty using a walking stick. I wear a brace (AFO) on my left leg.

2) fatigue coming late in the day (I still work running a business from home).

My wife and I have spent a lot of time searching the Internet for information on SCA, and we still have a lot of web sites on our list that we have not yet had time to cover. Nevertheless, I hope you, who read this, will be able to help me with answers to the following questions:

SOURCES OF INFORMATION

I am looking for web sites that can be consulted regularly for new information, incl. email sites, like this one, newsletters etc.

I have already located the following sites:

Internaf Yahoo Group

National Ataxia Foundation

MGH Braintalk communities

Euro-ataxia newsletter

Friedrich's Ataxia Research Alliance

HIGH FOOT ARCHES

I have high arches. Is this associated with any SCA-like disorders other than Friedrich's Ataxia (FA)? I believe I was born with high arches.

NUTRITIONAL SUPPLEMENTS

Rather than drugs/medication, which I have not had to resort to (yet). I have been taking a batch of various vitamins and minerals for years.

A) There are a number of reports about the use of NAC (N-Acetyl-Cysteine) in conjunction with various vitamins/minerals.

Has anybody on this list tried this; if so, for which SCA and has it worked? It is supposed, among other things, to improve motor

skills and stabilize or slow down the disorder.

Dr. A. ph of Tuft's University has published information about improved motor skills in rats after blueberry consumption, and more recently human trials apparently have shown a 5% improvement in motor skills after the consumption of one cup of blueberries and 10% after eating 2 cups. Has anybody tried it? Would blueberry extract tablets work? They are available.

C) Do you know of anything else that helps?

EXERCISE

I do full range-of-motion and stretches daily, set up partly by a physiotherapist and partly based on exercises for MS and PD patients with a few from the National Ataxia Foundation web site thrown in. In addition, I use an exercycle for my legs and a grinder for my arms, for aerobic exercise.

However, I am very interested in any exercises to specifically help strengthen the legs and improve walking.

How long do you exercise for?

DIET

Are there any dietary restrictions or recommendations?

ALTERNATIVE/COMPLIMENTARY TREATMENTS

Some MS and PD sites mention various treatments (acupuncture, Ayurvedic medecine etc), but I have seen nothing on the SCA

sites. Has anybody come across something that works? Not a cure (that would be terrific!), but to alleviate symptoms.

AFO's

I am interested in exchanging information with anybody that wears an AFO; specifically, do you find that as your condition deteriorates, changes need to be made to the AFO for it to hold the leg in the proper position for walking?

LIFE-SPAN

I gather that some of you out there have lived with this condition for 20 years or more. Would you like to share any secrets for your longer life-span with us?

SPONTANEOUS REMISSION

Finally, does anybody know of any cases of spontaneous remission ('induced ' or otherwise) for our affliction?

I will be grateful for responses to my rather lengthy initial submission. If somebody prefers to email me direct, please feel free to do so.

If you do not wish to belong to shydrager, you may unsubscribe by sending a blank email to shydrager-unsubscribe

[Guest guest]

I have FA and due to the high arches, loss of feeling and control, and also

because I live alone in a 3 up 3 down, thus stairs, I can only wear high sided

lace up or tight zipped boots. These limit the ankle collapsing (difficult to

describe) which make stairs hazardous. I have some normal lace ups for days out,

where I expect to be wheelchaired. I have some elastic sided cowboy boots (for

out), but they are very hard to get off, un-aided, but they keep my ankles

straight too.

-- from Brine 49 FA UK

- brian@...>  Written at 21:16:45 on 01-04-2002

I noted that on  Mon, 1 Apr 2002 08:48:40 -0500,

Jerry@...> wrote:

>

>To the  shydrager group at large,

> 

>I  assume that some have already responded to this posting as far other sites

of  interest, but

>I was  intrigued by the " high foot arch " question.  My brother (who has MSA)

has a  very high foot

>arch.  He was never able to wear any type of shoe but a lace-up do  to this

issue.  Does anyone

>have  any other information on this issue?

[Guest guest]

I have FA and due to the high arches, loss of feeling and control, and also

because I live alone in a 3 up 3 down, thus stairs, I can only wear high sided

lace up or tight zipped boots. These limit the ankle collapsing (difficult to

describe) which make stairs hazardous. I have some normal lace ups for days out,

where I expect to be wheelchaired. I have some elastic sided cowboy boots (for

out), but they are very hard to get off, un-aided, but they keep my ankles

straight too.

-- from Brine 49 FA UK

- brian@...>  Written at 21:16:45 on 01-04-2002

I noted that on  Mon, 1 Apr 2002 08:48:40 -0500,

Jerry@...> wrote:

>

>To the  shydrager group at large,

> 

>I  assume that some have already responded to this posting as far other sites

of  interest, but

>I was  intrigued by the " high foot arch " question.  My brother (who has MSA)

has a  very high foot

>arch.  He was never able to wear any type of shoe but a lace-up do  to this

issue.  Does anyone

>have  any other information on this issue?

[Guest guest]

,

Many thanks for all the trouble you took to answer so fully. I am sorry

haven't been able to reply earlier, but I have been away.

Nutritional supplements - I have been taking different supplements, vitamins

and minerals for year, incl. multivitamins, 500 mg vitamin C, 400 IU vitamin

E and 50 mg CoQ10. There are studies on NAC for OPCA, FA and other SCAs. I

will

probably start using it, but will be discussing it with my neurologist

first.

Re blueberries see

http://neuro-mancer.mgh.harvard.edu/cgi-bin/forumdisplay.cgi?action=topics&f

Look for blueberries in the ataxia forum

http://www.alzheimers.org/nianews/nianews23.html

I haven't been able to find out any more about the trials for patients with

ataxia. Perhaps somebody from the

MSA group with a bit of clout could get in touch with Dr. ph and ask

him!

Could you elaborate on aspiration?

I am fascinated to read about Aussie Anne. Has she shared the secret of her

success with the group?

Enjoy your trip to Australia.

In digest 1374 Werre wrote:

> Welcome to our family, sorry you had to find us.

>

> Nutritional supplements - We have heard a lot, we have yet to hear any

> definitive positive results from known MSA patients.

>

> I have not seen Dr. Tuft's work either, do you have a website where it

> is published?

>

> Watching for signs of infection, aspiration and fighting dehydration can

save the

> patient's life.

>

> Then we have Aussie Anne - all the doctor has to

> do is tell her she is going to die, then she goes out and gets another

> university degree. They told her (23 years now Anne?) that she had a

> year to live, she got a college degree in 1999. They told her 18 months

> ago that she had a few months to live, she got a stomach pacemaker and

> is back working on another degree (taking a statistics course now).