Status
Not open for further replies.

KANSASTOM

Distinguished member
Joined
Jul 2, 2008
Messages
165
Reason
Learn about ALS
Country
US
State
ks
City
wichita
If you have only lower motor neuron involvement, then you have Spinal Muscular Atrophy, a pure motor neuropathy or some sort of other lmn disease. From what I have been reading these are disabling but not fatal. If you have pure upper motor neuron involvement then you have HSP or PLS and both of these are disabling but not fatal. Now if you have both then it is ALS and it is fatal. My question is why? Why is the sum of two non-fatal diseases fatal? I understand that the decline of respitatory function is what makes ALS fatal, but why wouldn't you have the same result with a pure lmn?
 

PDaddy

Distinguished member
Joined
Jan 8, 2008
Messages
264
Diagnosis
04/2008
Country
US
State
CA
City
Claremont
Wright?

That's a damn good question, if your assumptions are correct. Maybe Wright (does he have a first name, or is it Wright?) will shed some light...
 

wright

Very helpful member
Joined
Jan 12, 2008
Messages
1,160
Country
US
State
xx
City
xxxx
Hello Tom

Spinal muscular atrophy (SMA) is different than progressive muscular atrophy (PMA).

SMA is a condition found in infants, children and young adults. The time in the life that it is contracted dictates what type it is. They are categorized from Type I all the way to Type IV, with Type I being found in infants, Type II in children, Type III in teenagers and people in their twenties and Type IV is an adult form found in people older than 30. It is very, very, very slow in its progression and is typically not fatal.

PMA on the other hand, is fatal. This is a variant of ALS where there is solely lower motor neuron involvement. It's progression isn't typically as fast as ALS but the way it progresses is the same. It will march up and down limbs, have bulbar involvement and will make it's way to the respiratory muscles eventually.

There are certainly other conditions that cause lower motor neuron problems (peripheral neuropathy for example) that are not typically fatal, because they are not progressive (for the most part). They more or less hit you then stop. Flare-ups can certainly occur (and these flare-ups can be acute or chronic) but the progression isn't like ALS or PMA, which is relentless; once ALS or PMA start, they simply do not stop.
 

KANSASTOM

Distinguished member
Joined
Jul 2, 2008
Messages
165
Reason
Learn about ALS
Country
US
State
ks
City
wichita
So it looks like the difference between an Adult form of Spinal Muscular Atrophy and PMA is the rate of progression.
In the early stages of the adult form of Spinal Muscular Atrophy or a pure motor neuropathy does the emg results look the same as ALS or PMA?
 

joelc

Moderator emeritus
Joined
Jul 15, 2006
Messages
2,786
Reason
PALS
Diagnosis
09/2005
Country
CA
State
BC
City
Abbotsford
I sincerely believe ALS does not have to be fatal!

With the technology we have today we can live, breath and communicate just fine for a long long time! We just have to be willing to accept these helpful items!

I for one have accepted these and I plan on being around for at least another 20 years.

GOD BLESS YOU ALL!
 

Al

Moderator emeritus
Joined
May 25, 2004
Messages
7,960
Reason
PALS
Diagnosis
10/2003
Country
CA
State
On
City
NW of Toronto
I'm sure Quadbliss would agree with you Joel.

AL.
 

KANSASTOM

Distinguished member
Joined
Jul 2, 2008
Messages
165
Reason
Learn about ALS
Country
US
State
ks
City
wichita
I was very surprised tah there are alot of people that have had Als for a very long time. When I ask my neurologists how long did I have, he told me that he didn't have a crystal ball but the mean time was three years, I think he is way out of touch with reality.
 
Last edited by a moderator:

wright

Very helpful member
Joined
Jan 12, 2008
Messages
1,160
Country
US
State
xx
City
xxxx
Joel, I agree fully. What I wrote about progression and what one sees written on progression, is when there are no medical interventions taken.

Quadbliss is a perfect example of someone who has lived a long time with ALS because he has chosen to use technology. I applaud him, you and anyone else who chooses to do so.

Having said that, I respect anyone's decision as to how they handle this disease. It is certainly a personal choice.
 

Al

Moderator emeritus
Joined
May 25, 2004
Messages
7,960
Reason
PALS
Diagnosis
10/2003
Country
CA
State
On
City
NW of Toronto
Not really. The stats still say 80% of diagnosed people will be dead in 2 to 5 years. Three as a mean would not be far off sorry to say. Personally I'm coming up on 5 years. So I'm well past the mean. Still fighting.

AL.
 

joelc

Moderator emeritus
Joined
Jul 15, 2006
Messages
2,786
Reason
PALS
Diagnosis
09/2005
Country
CA
State
BC
City
Abbotsford
Al, with all due respect those numbers would change significantly if more PALS chose to live by accepting either a PEG or tracheostomy and vent or both. Unfortunately it is still a very small number who choose these life saving items.
 

Al

Moderator emeritus
Joined
May 25, 2004
Messages
7,960
Reason
PALS
Diagnosis
10/2003
Country
CA
State
On
City
NW of Toronto
For sure Joel but we have to try to educate the PALS and CALS here and elsewhere. There have been countless people here say that their Doctor said that it is not possible to keep a PALS at home without specialised care and help and they discourage them from it. Totally false. It can be done. It's not easy but it can be done. We now have a few trach patients and quite a few PEG's. A lot more than 4 years ago. People telling the good results here help. Thanks for sharing yours.

AL.
 

CindyM

Moderator emeritus
Joined
Sep 17, 2006
Messages
3,543
Reason
Learn about ALS
Country
US
State
New England
City
Anytown
So I'm well past the mean. Still fighting.

Amen, Al. And I am fighting for you and Lee in spirit! Cindy
 
Status
Not open for further replies.
Top