progression and severity of PLS

Status
Not open for further replies.

larryz

New member
Joined
Oct 4, 2007
Messages
6
Reason
DX UMND/PLS
Diagnosis
01/2006
Country
US
State
New York
City
Albany
Hi folks-- I'd like to hear from some of you about the following. I have been diagnosed about 2 years with pls. I began noticing the symptoms about 10-12 years ago-- in my legs and feet. They were contracting or spasming and were annoying-- my feet, when I started walking after any period of rest were painful until I'd walked for a while. The spasms in my legs (mostly left) continued for years and increased in severity. I was diagnosed with rheumatoid arthritis but that turned out to be wrong. Various other diagnosis were tried until finally PLS. I take about 70 mg of Baclofen over the course of a day and it has really helped the spasms but now my legs get tired easily and if I foregt the Baclofen, the spasm are REALLY uncomfortable. BUT-- I don't have the severity of some of the symptoms I read in some of your posts.. If PLS is progressive, is it slower in some than others. Is it possible to have a "slight" case of PLS?-- I doubt it. Any thoughts--Larryz
 

Geo

Senior member
Joined
Aug 24, 2007
Messages
500
Reason
DX UMND/PLS
Diagnosis
12/2001
Country
US
State
Florida
City
Ruskin
Larry ,you might Look at HSP Heredity Spastic Paralgia . Im 8 ish years into PLS mine started as Bulbar then the legs . Geo
 

Jennifer51

Distinguished member
Joined
Jul 7, 2008
Messages
441
Reason
PALS
Diagnosis
03/2008
Country
UK
State
England
City
London
Larry ,you might Look at HSP Heredity Spastic Paralgia . Im 8 ish years into PLS mine started as Bulbar then the legs . Geo
Hello Geo....(out of interest we live in london but have had 3 homes in florida (Kissimmee for almost 20 years)
I was interested to see that you were diagnosed many years ago with PLS. did they diagnose it straight away as PLS or did they say it was ALS to start with. I have a DX of Bulbar onset als...started with slurred speach Jan 08 but now my legs and arms are going...all in the space of less than 6 months. I am getting some pain in hips, arms and neck and I am sure I am loosing a little bit of balance...so I wanted to try to get someone to look into PLS. My neuro is so full of himself as being brilliant at diagnosis, so I dare not suggest it to him, but I am going to a new ALS clinic next week so would like to ask there. I know I am clutching at straws, but being told to expect 6-36 months, you have to try to look for something else. Jennifer 51
 

sral

Distinguished member
Joined
Sep 15, 2007
Messages
289
Reason
Learn about ALS
Diagnosis
n/a
Country
CA
State
ontario
City
toronto
Jennifer,

I'm so sorry to hear of your diagnosis. My mom too started with bulbar symptoms about 2.7 months ago. When diagnosed last year, they did a ton of blood work and an EMG. The result of the two combined made them conclude last summer that it was not ALS. My mom's EMG was negative, i.e. she had no nerve damage at all (per the neuro). At the point of diagnosis because her symptoms had only started about 21 months prior they diagnosed her with upper motor neuron disease. Her neuro said that the 36 month point (3 yrs) if her EMG was still negative then it could be called PLS.

Have you had an EMG done?
 
Joined
Jun 2, 2008
Messages
12
Reason
Learn about ALS
Country
US
State
iowa
City
ankeny
Hi Larry,

The common theme I have heard from the several neurologists that I have seen, is that neurological disorders don't come with a road map. Yet, since joining this group, I am finding so many people like us are out there, experiencing similar health problems and frustrations. I am currently on 80MG of Baclofen daily and I understand what happens when you miss taking them. In my case the symptoms began in the legs, then the hands, arms, shoulders, torso, neck and now in my facial muscles. This has happened over the couse of four years. I was missdiagnosed with Parkinson's Disease and to this day they still won't put a name on my condition for reasons I understand. From what I have read, PLS can affect a persons life for years or even decades and is a progressive disease. What I find the most frustrating about all of this, is that everyone on this site has to deal with a disease that gets so little attention. The good news is there are a whole host of good people on this site that understand and have a great deal of information for you. - Mitch
 

olly

Extremely helpful member
Joined
Jan 10, 2008
Messages
2,732
Reason
PALS
Diagnosis
11/2007
Country
uk
State
uk
City
uk
Hello

LARRY
your neuro needs to watch you for 5yrs to see the progression.
do you have any trouble with arms,upper body or bulbar?
if it stays mostly in the legs after a number of years it is probably hsp.
everyone is different but the first few years are the hardest.

GENNIFER
very few neuro's have seen a case of pls here in the uk and lable you as als/mnd.
have you had a emg? was it ok or not?
i have to force any info from my neuro,but as he has not had a case only hsp which is common they dont have much to go on only medical texts that are ofton misleading or wrong ,these are outdated as we need new research.

both of you take good care.
caroline:-D
 

Jennifer51

Distinguished member
Joined
Jul 7, 2008
Messages
441
Reason
PALS
Diagnosis
03/2008
Country
UK
State
England
City
London
Jennifer,

I'm so sorry to hear of your diagnosis. My mom too started with bulbar symptoms about 2.7 months ago. When diagnosed last year, they did a ton of blood work and an EMG. The result of the two combined made them conclude last summer that it was not ALS. My mom's EMG was negative, i.e. she had no nerve damage at all (per the neuro). At the point of diagnosis because her symptoms had only started about 21 months prior they diagnosed her with upper motor neuron disease. Her neuro said that the 36 month point (3 yrs) if her EMG was still negative then it could be called PLS.

Have you had an EMG done?

yes and the neuro said it was ...cast iron evidence ....of psuedo bulbar als...but I still want to prove them all wrong.... Can they be SO sure of results with the EMG. I was told the guy who did it was one of the best in the field, in London. They say there is NO test to say for certain that you have als. So why hold so much store with the EMG....can it prove any other disease?
sorry, new to this, have not got to grips with all the technical stuff...learning fast though.
 

sral

Distinguished member
Joined
Sep 15, 2007
Messages
289
Reason
Learn about ALS
Diagnosis
n/a
Country
CA
State
ontario
City
toronto
Jennifer,

It could definitely be something else. If they are trying to understand whether it is only upper motor neuron damage (PLS) or both upper and lower (ALS) they use the EMG test. The EMG is testing the lower motor neuron damage. If there is any nerve damage detected with the EMG it is a sign that there is damage to the lower motor neurons. This said, ALS is not the only disease that would bring on lower motor neuron damage. There was a case I had read about a member who did have some abnormality visible on the EMG and was misdiagnosed by two neuros as having ALS and then later re diagnosed with thyroid cancer. This is a rare case, but it highlights that it isn't always that straight forward and easy.

If you are uncomfortable, search out another well recognized neurologist who specializes or sees ALS patients and get a second opinion.

My heart goes out to you. I'm happy to see that you're not giving up and are fighting.

All the best.

By the way, I had made a typo, my mom's symptoms started 2 years and 7 months ago with bulbar onset. I do not know if it has progressed into ALS today as she has not had an EMG in a year now. However, she can still do everything in slower motion and with some effort (talking, walking, stairs). Upper motor neuron damage usually causes balance issues as well.
 

olly

Extremely helpful member
Joined
Jan 10, 2008
Messages
2,732
Reason
PALS
Diagnosis
11/2007
Country
uk
State
uk
City
uk
jennifer

as sral said a emg will show lmn involvement in bulbar onset als.
if there is none it could be bulbar onset pls,mine as many others started in my legs but some start with bulbar onset.
do you have any real weakness or atrophy anywhere?
i would ask for a emg,it could give you a more definate diagnosed and may even rule out als.
take good care.
caroline:)
 

Jennifer51

Distinguished member
Joined
Jul 7, 2008
Messages
441
Reason
PALS
Diagnosis
03/2008
Country
UK
State
England
City
London
Jennifer,

It could definitely be something else. If they are trying to understand whether it is only upper motor neuron damage (PLS) or both upper and lower (ALS) they use the EMG test. The EMG is testing the lower motor neuron damage. If there is any nerve damage detected with the EMG it is a sign that there is damage to the lower motor neurons. This said, ALS is not the only disease that would bring on lower motor neuron damage. There was a case I had read about a member who did have some abnormality visible on the EMG and was misdiagnosed by two neuros as having ALS and then later re diagnosed with thyroid cancer. This is a rare case, but it highlights that it isn't always that straight forward and easy.

If you are uncomfortable, search out another well recognized neurologist who specializes or sees ALS patients and get a second opinion.

My heart goes out to you. I'm happy to see that you're not giving up and are fighting.

All the best.

By the way, I had made a typo, my mom's symptoms started 2 years and 7 months ago with bulbar onset. I do not know if it has progressed into ALS today as she has not had an EMG in a year now. However, she can still do everything in slower motion and with some effort (talking, walking, stairs). Upper motor neuron damage usually causes balance issues as well.


Thanks for that...it makes things a little clearer...I did have lower neuron damage...legs are now letting me know that for sure...dont like stairs any more...but hey I am still walking...indoors...and only just outdoors, so I am much better than some. I guess we all HAVE to keep hoping that the DX is wrong. Seeing a new nuero next week at a new ALS clinic being set up in London (Barts and Royal London). Hope I might learn something there. The Neuro is Italian so may know more about the lithium trials in Italy.
Jennifer
 

Jennifer51

Distinguished member
Joined
Jul 7, 2008
Messages
441
Reason
PALS
Diagnosis
03/2008
Country
UK
State
England
City
London
as sral said a emg will show lmn involvement in bulbar onset als.
if there is none it could be bulbar onset pls,mine as many others started in my legs but some start with bulbar onset.
do you have any real weakness or atrophy anywhere?
i would ask for a emg,it could give you a more definate diagnosed and may even rule out als.
take good care.
caroline:)

yes had the emg and nerve conductive tests..showed lmn damage, and now have some weakness in legs.. Just cant give up hope of proving somebody wrong with the diagnosed.
jennifer51
 

sral

Distinguished member
Joined
Sep 15, 2007
Messages
289
Reason
Learn about ALS
Diagnosis
n/a
Country
CA
State
ontario
City
toronto
Jennifer,

I'm glad to hear you are staying positive.

By the way, having leg symptoms does not mean lower motor neuron damage. The lower motor neuron part is whether the sigal is being affected from the brain or the spine. People with PLS or upper motor neuron damage also have lower limbs affected.

Take care.
 

olly

Extremely helpful member
Joined
Jan 10, 2008
Messages
2,732
Reason
PALS
Diagnosis
11/2007
Country
uk
State
uk
City
uk
jennifer

i have hypotonia/loss of contration in my left lower leg that would be viewed as lmn involvement. i have slight atrophy in the left ankle/foot also,plus weak right hand.
in pure pls there technically should not be any lmn involvment present,but on postmortem there is always lmn evidence present.
some pls'ers have some none progressive lmn signs/symptoms but not enough to diagnosed als,in clinic trials/texts these are called umn dominant als.
i know some pls'ers like myself who have slight lmn,some have atrophy in there hands but did not progress much.
that is why the docs will wait and see and follow progression as in the early stages many of the mnd's can resemble another one.
hope that helps and keep up the possotive spirit. your doing great.
take good care
caroline:-D
 
Last edited:

sral

Distinguished member
Joined
Sep 15, 2007
Messages
289
Reason
Learn about ALS
Diagnosis
n/a
Country
CA
State
ontario
City
toronto
Caroline,

I admire how wonderfully supportive and positive you are.

Hope you are keeping well.
 

MICHELLE40f

Active member
Joined
Dec 23, 2007
Messages
39
Country
US
State
GEORGIA
City
ST.MARYS
hi Larry

I have a diagnoses of hereditary spastic paraplegia,has any neuro ever mentioned this one to you? You dont have to have an obvious "hereditary pattern" to have this,matter of fact some people are diagnosed with "apparently sporadic spastic paraplegia".....but sometimes i wonder if mine is actually a slower form of pls,because I have some arm symptoms(which "some" people do with hsp) and at times(if im real tired) i also have slurred speech................confusing........Michelle:) ps. glad you are having slow progression:mrgreen:
 
Status
Not open for further replies.
Top