Official poll results

[olly]

Here are the official poll results taken from 22 pls/umn patients that took part.
Thank you for your contribution .
Please give your thoughts and comments on the results.
Feel free to print out to show your neuro if it may be of advantage.

 

[olly]

I added old dog and added what others missed,i had to exclude pals but thank you for taking part.
I did not realise we had so many pls'ers so it was nice to see you all taking part.

To me the muscle twitching result is very suprising and don't think its a chance thing.
I never had fasic's until I started with pls .

Also just over half do have plateaus ,I think that could be higher as it can be difficult for some to differentiate what is a plateau.

 

[Nikki J]

Really interesting Olly. Good work! I do not know if you can tease out from the bladder issues sex and age? Bladder spasms are actually pretty common in women approaching and in menopause. Obviously PLS would make this much worse if a person was predisposed to the problem, a kind of synergistic effect.

 

[olly]

Good point nikki,majority were women with only 2 men who voted bladder issues.
Bladder issues are documented as a symptom in pls/hsp on the hsp website.

I started with pls 15yrs ago aged 30/31 yrs old and had bladder issues ...going through menopause now I do contribute some of the bladder weakness to this and age.
However I personally can tell when bladder spasticity is due to the pls as it is usually when spasticity is really bad from waist down.

 

[olly]

billbell,did you vote?
Don't think you did,would you like me to add you?
Please tell me what you would vote

 

[Nikki J]

Were there any women in your group without bladder issues? If so are they early in their disease process? Can you tell? It would be interesting to see what is different in the people who do not have the symptoms that seem pretty common. And if symptoms seem to cluster. Are those who twitch also weak etc?
So many questions to ponder!

 

[affected]

thanks for taking the time on this Olly, that is really interesting data!

Like Nikki it just makes me think of more and more sub-questions, but then I love data and analysing it...

 

[olly]

BREAKDOWN OF PERCENTAGES

Muscle twitching 17 out of 22.....8/11 were 0-5yr progression,5/6 were 5-10yrs and 4/5 were 10+yrs

Pain 16 out of 22.....7/11 were 0-5yr progression,4/6 were 5-10yrs and 5/5were 10+yrs

Bladder issues 9 out of 22.......5/11 were 0-5yrs progression,1/6 were 5-10yrs and 3/5 were 10+yrs

Bulbar issues 15 out of 22.....7/11 were 0-5yr progression, 5/6 were 5-10yrs and 3/5 were 10+yrs(one is hsp)

Clinical weakness 16 out of 22....8/11 were 0-5yr progression,4/6 were 5-10yrs and 4/5 were 10+yrs

Use mechanical aids 15 out of 22...7/11 were 0-5yr progression,4/6 were 5-10yrs and 4/5 were 10+yrs

Plateaus 11 out of 22.....3/11 were 0-5 yr progression,4/6 were 5-10yrs and 4/5 were 10+yrs

 

[olly]

Intresting to me is that progression and disability seems to be worse on onset and that plateaus develop after 5+yrs.
I found this to be so,the first 5 or so years saw a downward decline for me but after that plateaus became longer and progression slowed right down.

 

[affected]

I hope no one minds me jumping in here as I am a CALS and dealing with a rapid progression bulbar onset but ...

Those stats really are very interesting Olly, especially what you are seeing about progression being faster in the first 5 years, then slowing with longer plateaus. I guess that is one of the big things about pls and why it is so possible to live so long with the disease?

Something that occurred to me is to wonder whether there was an initial long period of very small symptoms that were possibly ignored, that may show a period of slow progression til a point where things pick up for some years.

I only ask this because my PALS and I know that ALS was slowly working on his body for 3 - 4 years before there were enough symptoms that it became obvious something was wrong and not getting better. Once that point was reached, for him, the progression started to pick up the pace and after the first 6 months (when it was mostly bulbar) it just started to move through his body and the pace has only seemed to increase since.

The kinds of small symptoms my PALS experienced were so small at the time that I am always dubious to print them here as the DIHALS readers may see them and really freak out. If he had never developed ALS they would still be little things we wouldn't really think about. Like I have lots of 'things' about my body being over 50 that I just shrug and accept and are really nothing to worry about. It was only the hindsight of being diagnosed with ALS and our searching for what was going on during that process that allowed us to even piece all that together.

 

[dalvin]

Tillie, can't say anything bout others but I had bulbar issues almost 10 years ago. The docs at that time and place denied that they were even possible. It wasn't till I started having problems with falling that I was taken seriously.(bout 2 1/2 years ago). After that progression speeded up rapidly. Now one neuro is saying pls and another is saying als. Go figure, they can't even agree now

 

[affected]

I've heard it said, but not sure really about this one - that some people get diagnosed pls and then years later progression gets faster and the diagnosis is changed to als?

No wonder the research on this disease is so difficult, it has far too many variations of how it presents and progresses.

 

[Dusty7]

Tillie, according to als dot ca --
"Some ALS specialists believe that PLS is on the ALS continuum and may not be a separate disease, but a very slow-progress-
ing type of ALS. ALS may begin with signs of only upper or lower motor neuron involvement, so a process that begins with
upper motor neuron degeneration and that initially is considered to be PLS has the potential to be reclassified as ALS if suffi-
cient signs of both upper and lower motor neuron involvement develop over time."

Also, PMA, which is at the opposite end of the spectrum from PLS (LMN signs with no UMN signs) can be reclssified and I believe I have read that 50% of people diagnosed with PMA, in fact, have UMN lesions at autopsy. And since PMA is only 5% as common as ALS, it's kind of like, once you hit the ALS spectrum, it's kind of hard to stop before sliding into ALS.

 

[olly]

Tillie...following on what Dusty posted.

A Diagnosis of PLS is given between 5-7yrs after symptom onset to rule out ALS and LMN signs ...you are correct in that symptoms/signs may have been present before but the person did not recognise them. Like me,people noticed I dropped things too much to be a coincidence .

As Dusty said on autopsy PLS patients have been found to have some LMN involvement that may not have been physically obvious in life.
However as PLS patients mean age of onset is 50+yrs and the fact that PLS progression is slow I suspect those autopsy patients were 60's 70's or 80's and they died of unrelated MND causes.
Dusty mentioned PMA but also on ALS patient autopsy they found other areas of the nervous system had disease involvement leading to some neurology researchers thinking it could actually be a multi system disease and not just confined to motor neurons.

As I was only 30 and now 15yrs into this I do worry about my future....I ofton wonder if I will reach pension age of 60,i seriously don't think so.

All the PLS studies I have read tend to be from the 1990's ,they are seriously lagging behind that is why majority of neurologists don't know anything about PLS.
I stand by what I have said before....A PLS patient is the expert in PLS ...at least till they get there backsides into gear.

 

[olly]

Dalvin I would say you were Bulbar onset PLS given your initial 10yrs of bulbar symptoms before limb onset.
Given that bulbar onset ALS has the lowest survival rate and even a person with slow bulbar onset would have probably died within that time.
Have they done a bulbar emg?