DX from PLS to ALS

[olly]

hi bill.
my comments were of no offence to you.
i was posting to others who are un-diagnosed,ed not to think they could have als with clean emg,s.
if you look on the "is this als" forum you will understand.
my progression has been slow like yours with the same symptoms starting in the limbs,the bulbar symptoms started 2yrs ago with the having to swallow several times to get food down.
sorry to hear about your dad,i often think it must be worse for those who have had a relative with als,they know whats coming.

 

[Mr. Bill]

Olly,

I didn't take what you said as an offense. I just don't want to worry anyone needlessly. I guess I have to think before I write. That's a novel idea for me.

 

[lydia]

So Bill...I can't help it I have to know...Caroline (olly) warned you...what happened at that last visit to cause your neuro to change his mind...how can you wait till March to find out? Isn't that driving your wife crazy? I would be dragging you to that office. Well maybe a better way to put it is I would be nagging you to that office.

Can you describe the stiffness in your leg(s)? Is it always there, do certain types of activity make it worse, can your doctor tell the stiffness is there (or is it clinical stiffness...haha...joke from the clinical weakness thread)?

Love your icon by the way,

Lydia

 

[Mr. Bill]

So Bill...I can't help it I have to know...Caroline (olly) warned you...what happened at that last visit to cause your neuro to change his mind...how can you wait till March to find out? Isn't that driving your wife crazy? I would be dragging you to that office. Well maybe a better way to put it is I would be nagging you to that office.

Can you describe the stiffness in your leg(s)? Is it always there, do certain types of activity make it worse, can your doctor tell the stiffness is there (or is it clinical stiffness...haha...joke from the clinical weakness thread)?

Love your icon by the way,

Lydia

Lydia,

I lose track of what thread I said what on, but I didn't know he changed the diagnosis until a couple of months after my visit. It was while speaking with the ALS nurse at the clinic that she said "oh, I see your diagnosis changed." Really?

Me going to the Neuro early isn't going to change anything. Maybe it's just that ignorance is bliss. In my case it's everything. )

My wife has suggested that I go in now, but she hasn't pushed it. Maybe she doesn't want to know either. Don't forget we have been dealing with this for four years now.

My legs literally get stiff. I walk like I'm stiff. It's just that the muscles are tight and not real flexible. When I first went to the Neuro he would have me sit down and relax my leg. He would then grab it and whip it around in a circle and back and forth. Back then it was flexible and would pretty move where he put it. It was kind of like whipping a noodle around. Now it's more like whipping a stick around....really stiff.

I know I have quite a way with words, but I try. BTW I can't take any credit for the icon other than swiping it. )

 

[lydia]

Hi Bill,

Thanks for describing your experience. I am not really clear on the difference between PLS and UMN dominant ALS...is that the latter has LMN stuff starting, even if it is barely perceptible and with PLS there are absolutely no LMN signs? And if so, then it looks like what is happening with you...that something showed on that last exam that suggested LMN things were starting up? You didn't have an EMG at that last visit did you?

shoot, I am suddenly so tired I have to go to sleep. Rough day :grin:

Take care-

Lydia

 

[olly]

hi lydia.
with pure pls there should be no lmn involvement on diagnosed,then watched for 3-5yrs.
umn dominant als there is mainly umn symptoms/signs but also some small amount of lmn involvement.
lmn involvement could have come after umn or been too slight and overlooked.
my lmn involvement was found after 7yrs by accident but it has probably been there longer.
around 50% of pls,ers will go on to develop lmn involvement as seen in studies.
there is always lmn involvement on autopsy of those with diagnosed of pls,that is why pls is a mnd .
umn dominant survival rate is less than pls but can be alot more than als.
slow progression is the key.

 

[lydia]

So Caroline, if signs of LMN involvement are always there, it is just a matter if they make themselves known, if they become noticeable enough? So it is like a continuum, and everything on it has both LMN and UMN whether or not they are necessarily visible or measurable, with their positions on the continuum determined by the degree to which the always present LMN makes it self visible (or the always present UMN makes it self visible) with PLS on the one far end (say the right end) with UMN Dominant ALS to the left of that, and then ALS smack in the middle. On the far left end would be ...whatever it is called if only LMN appears (but UMN is there, and not noticeable yet), and then to the right of that would be something like a LMN Dominant ALS (where there is some UMN involvement), and further to the right is ALS, smack in the middle. Well I can picture the above quite nicely, if it is actually a valid/accurate interpretation of events is another story altogether!

Hope your new place is starting to feel like home -

Lydia

 

[lydia]

Caroline, a thought just occurred to me...if LMN signs are always present on autopsy for those diagnosed with PLS....what it is that constitutes a sign on autopsy that wouldn't have been considered a sign or able to be measured when they are alive? What do they look at/examine/measure to decide, yep there's LMN involvement here?

Lydia

 

[Danijela]

My understanding is that one of lower motor neurone involvement sings (in limbs) is muscle atrophy. But may be totally wrong. I am not sure what they will look for in autopsy.

 

[olly]

ok,i have been searching the archives.
i have so many university/research studies and articles on file,here are a few.
basically involvement of other systems may not be obviously present in the desease life course but found on autopsy.
mnd is a umberella term for als/pls/pma/sma/pbp
but only a clinical diagnosed can be given and may be reclassified on autopsy with a definate pathalogical diagnosed with the case of only umn or lmn life involvement
i really hope the info helps,we had a big debate on this earlier on in the year.





ALS may present initially with signs of only upper or lower motor neuron involvement. Thus, a process that initially is considered PMA or PLS has the potential to be reclassified as ALS if sufficient signs of both upper and lower motor neuron involvement develop over time. In some cases, such reclassification may occur only at autopsy (eg, pyramidal tract involvement is found in patients who did not have signs of upper motor neuron involvement during life and whose disease was therefore classified on clinical grounds as PMA).

Recent reports have described patients with one of the genes for familial ALS in whom only lower motor neuron involvement was seen during life and at autopsy. Most investigators would classify this disease pattern as ALS, on the basis of the gene's presence (even though its clinical expression was incomplete). This position is supported by the recently revised World Federation of Neurology diagnostic criteria for pathology.

.............................................................................................................................................






Department of Neurology, The Queen Elizabeth Hospital, South Australia.

Motor Neurone Disease (MND) is one of the commonest neurodegenerative disorders of adulthood. MND characteristically presents with a combination of both upper and lower motor neurone features. Primary Lateral Sclerosis (PLS) is thought to be a variant of MND presenting with purely upper motor neurone signs. Debate continues over whether PLS constitutes a distinct pathological entity or whether it is part of the spectrum of motor neurone diseases that present as an upper motor neurone-predominant form of MND. We present a case of MND with purely upper motor neurone features and a prominent pain component. A pre-mortem diagnosis of PLS was made, however autopsy findings demonstrated both upper and lower motor neurone involvement. We believe these findings support the view that PLS is not a discrete pathological entity, but that it is a part of the range of motor neurone diseases that present with predominant but not exclusive upper motor neurone involvement. This case also highlights the feature that pain may be associated with MND even though it is not appreciated to have a sensory pathology.
.......Primary lateral sclerosis (PLS) has been defined as a rare. Non-hereditary disease characterized by progressive spinobulbar spasticity, related to the exclusive involvement of precentral pyramidal neurons, with secondary pyramidal tract degeneration and a preservation of anterior horn motor neurons, the latter allowing PLS to be distinguish from amyotrophic lateral sclerosis (ALS). However, a clear distinction between the two diseases remains a subject of debate. With this in mind, we assessed patients with meeting the previously published criteria for PLS in a prospective, longitudinal study.At regular intervals, we analyzed various clinical and electrophysiological parameters in nine patients with a diagnosis of PLS. We made a deltoid muscle biopsy and PET study.Our results provide evidence that degeneration in PLS is not restricted to the upper motor neurons but also affects the lower motor neurons. The distinction between ALS and PLS is related to the degree and stability of lower motor neuron involvement.In view of the similarities with ALS, we consider that PLS may represent a slowly progressive syndrome closely related to this disease.

PMID: 11311289 [PubMed - indexed for MEDLINE]..........................................................................................................................
However from a pathology point of view, there's little that actually firmly distinguishes PLS or PMA from ALS. Pathologically, people with all these conditions have similar findings, and there's probably actually a spectrum of upper and lower motor neurone involvement in everyone. (quote from dr paul wicks a member here)


here is some more info to look at.
http://books.google.com/books?id=l9...desease&lr=lang_en&num=50&as_brr=0#PPA2226,M1

 

[olly]

here is a great article on classification of mnds
http://wiki.iop.kcl.ac.uk/default.a...Classification of Motor Neurone Diseases.html

 

[Mr. Bill]

Boy I can't get my little mind around all of that. Is the moral of the story that UMN and LMN is involved in both ALS and PLS? It is just the extent of the involvement. I may have missed the gist of all that. HELP!

 

[olly]

sorry bill:lol:
when i reply to some questions i try to show sound proof from many neurological sites.
its hard to try to deleate alot of unnesessary info.
basically i was just showing that a clinical diagnosed is just that,definate pathological diagnosed in some cases of just umn or lmn involvement in life is needed on autopsy.
autopsy usually reveals other areas involved.
..
i think i will just let everyone else figure things out and do there own research.
i just found out today my aunty has terminal lung cancer that has spread to her brain,they can,t do anything and she is having seizures now. they have sent her home to die after taking months to diagnosed her properly.
i am fed up with all this c**p

 

[Mr. Bill]

sorry bill:lol:
when i reply to some questions i try to show sound proof from many neurological sites.
its hard to try to deleate alot of unnesessary info.
basically i was just showing that a clinical diagnosed is just that,definate pathological diagnosed in some cases of just umn or lmn involvement in life is needed on autopsy.
autopsy usually reveals other areas involved.
..
i think i will just let everyone else figure things out and do there own research.
i just found out today my aunty has terminal lung cancer that has spread to her brain,they can,t do anything and she is having seizures now. they have sent her home to die after taking months to diagnosed her properly.
i am fed up with all this c**p

Caroline,

I doubt that anyone else needed an explanation. I do appreciate your efforts, please keep it up.

I'm so sorry to hear about your aunt. I cannot imagine being in a situation like that. My sympathy goes out to you and your aunt. I hope she is not suffering.

Hang in there Caroline.

 

[lydia]

Oh Caroline, I am so sorry to hear about your aunt. You have too much to bear, you really do.

I appreciate the research and the links you posted. It did help me make sense of my original question; someone even used the word spectrum (I said continuum, I think)...I think I finally got it right. I copied it all and saved to my own growing file. THANK YOU. How you manage to keep track of it all I will never know.

Please take care, and again, I am so sorry about your aunt.

Lydia