atrophy in pls

[olly]

as pls progresses and weakness increases dissuse atrophy can develop.
dissuse atrophy does not mean its in a muscle that is no longer in use but it can occur in weakened muscles that are not able to contract to there ability.
i have atrophy in my weakest areas though unlike in als it is not very progressive.

i came accross an article while looking for something else.....its from the merck medical site.
i found this peice very enlightening and thought it was very important to show you,especially akmom who is concerned about her atrophy.



Primary Lateral Sclerosis and Progressive Pseudobulbar Palsy: These disorders are rare, slowly progressive variants of amyotrophic lateral sclerosis. Primary lateral sclerosis affects mainly the arms and legs, and progressive pseudobulbar palsy affects mainly the muscles of the face, jaw, and throat. In both disorders, severe stiffness accompanies muscle weakness. Emotions may be changeable: People with progressive pseudobulbar palsy may switch from happiness to sadness quickly and without reason. Inappropriate emotional outbursts are common. Many years later, muscles begin to twitch and waste away.

 

[abbas child]

Caroline, that's very interesting. I have neck weakness as well as trunk weakness and am incredibly stiff. Your quoted paragraph actually makes me feel better about it, as I've been wondering if I've caused the stiffness by lack of enough stretching. What can't be helped is much easier to handle.

Hope AKmom sees your thread.

Ann XO

 

[AKmom]

well then I guess I am at the twitch and waste stage of the disease process. Lately the twitch has been at the back of my left leg just below the knee...very intense twitch I might add...annoying. This unfortunately is my stronger leg and the one I use to go up and down a step...like getting into the truck. The twitching in my hands have all but disappeared, but now my hands are looking "bony" as the atrophy seems to still be happening. My feet do not twitch anymore either, but my feet and ankles are very skinny compared to the rest of my legs. So not sure if that is wasting or not. Either way...they will give me answers at Mayo I am sure. Monday is the day!

Thanks Caroline for the info!

 

[olly]

joyce,i have been twitching for years so i am not too bothered about that.
intrestingly my weakest side is my left were you can see more of the atrophy in places.
my left foot is now smaller in the width and i find it hard to keep slip on shoes on,my pt said my arch had collapsed due to weakness but i can't tell.
my left ankle has very slowly progressed,it has small scoops of atrophy around it.
i did have photos on my albums some time back,may take another to show you.
i have a constant twitch in my left palm that never goes away,my palm has sunk in and that hand looks very much different to my right meaty hand.
i am right handed so use it more,dont know if that means anything.

was reading up on weakness for another member and thread and i came accross some intresting info.
i have the usual trouble with dexterity,fine motor movements and stiffness/spasms with the hand.
what bugs me is i have trouble doing certain things,like using remotes (pressing down the buttons) and for instance clicking with the mouse.
just silly things but then its these things that get to you most frustrated.
i was reading about fatigue/percived weakness and clinical weakness.
it mentioned among other things clinical weakness could be determined by thumb pinching!?,this confused me as to what it meant.
while watching tv and pressing the remote button with my thumb but nothing happening i had a little investigation.............bingo.....i realised what it meant.
pressing my thumb to my finger as hard as i could i realised i could not feel the strength there that is the pressure .
i know this pressure /strength test as it is how i can feel the clinical weakness in my left lower leg.........if i push down as hard as i can on my left leg i can not feel the pressure of my foot against the floor compared to my right.........can you understand that?
even though i have had trouble from the start with my left leg and told on my first neuro exam it had clinical weakness it was not untill stubbling upon this that i realised i could determine what the neuro could feel.

some say pls is a completely different desease to als ,others say its a slower progressive subtype..............after many years studying up i believe its the latter.
on autopsy pls patients show signs (that may have not manifested into visiable signs/symptoms during life)of lmn involvement.
it is said in time these may have years later presented more visiable signs to that of als.
given old text books.....avarage age of onset is 50yrs (seems a lot younger now and for als)and progression is 20-25yrs (but als was 3-5yrs but that is no longer valid)then a pls patient would be 70-75 plus at the time of death and so there would be other causes other than mnd related.


godbless you ann,yes with als you could be experiencing the umn stiffness.
if its too troublesome you could maybe ask to try some kind of muscle relaxant like baclofen to relieve it.

 

[abbas child]

Caroline, thank you. Such good information. So far, it isn't nearly so troublesome that I'd risk the possible strength loss that can be part of baclofen use. I do have spasms in my hands when they're tired, but the UMN signs I'm experiencing are late into the party, so new to me. I wish your PLS would slow down and halt for a few decades and stop messing with you.

Finally, I can say that it's true I don't have my head screwed on right! It's official!

 

[olly]

ann, i did actually think the same thing.
you do probably need a bit of umn stiffness to counteract the lmn weakness.
oh boy,its just a constant struggle of keeping the body going.

 

[olly]

ps.....my head screwed off and flew away years ago lol.
sorry,my typing is getting worse and keep having to edit mistakes.

 

[hopingforcure]

Thanks for the always helpful info pretty Olly.. You are never in need of an edit around here. You are a big help, and a great friend..

 

[olly]

oh golly gosh h4c.......i feel like the teachers pet:lol:
never be in dougbt of how much you mean to me:grin:

 

[AKmom]

thank you for the info Caroline. I know I have had clinical weakness long before any of the atrophy. For at least the past 5 years there has been clinical weakness in my hands. First starting on the right then eventually within the past 2 or 3 years, in the left. The docs would have me squeeze their hands and I would squeeze as hard as I can and they would tell me to squeeze harder! Then I was always told "wow, your right is really weak!" They would then have me squeeze some metal device that measures the strength in numbers. On the right I could not registar anything and my arm would start to shake the harder I tried. My last neuro appointment at the end of '09, I could not pull my feet upward against the docs resistance. And only the left could go up slightly without resistance but it could not remain up. I also could not lift my legs from the thigh for very high when laying down or for very long...same with my lower legs when dangling them off the table and trying to lift them.

I am thinking my first symptom was back when I was thirty, but not 100% sure. Back then it was that I was constantly falling when I was trying to run. And I could no longer kick a soccer ball straight on the right. I was a soccer coach and it was a daily thing for me to do both of these activities. I always thought that maybe I injured my leg somehow. Never really had it looked at...but I did mention to the doc once (a year or so later after I noticed it was happening all the time). Nothing was ever done. Then I was in a car accident that caused a brain injury. Shortly after that I started limping for no reason. I didn't even notice I was doing it till my hubby asked about it. There wasnt any pain at that time other than the migraines I was having after the accident. Years went by and I mentioned to the docs several times these symptoms and they would do neuro exams and then send me to a new neuro who would say its not ms after expensive tests....this went on till the stroke. So now it comes down to back to basics of the progressions of my symptoms.

I have made a symptom/diagnosis list. On the left is all the un-diagnosises of symtoms with dates of onsets and on the right is the various diagnosises that have been given, even the ones that were later changed and their corresponding date. This should hopefully make it easier for this new doc at Mayo have an overall picture of this complex history. Luckily a wise person years ago told me to keep a symptom diary. So its rather accurate. I cannot help but feel a lump in my throat as the day gets closer to leaving. I know that I will be fine, just kinda nerve wracking thinking that one of two things will happen. Either I get an answer to my problems or I don't get an answer but the usual "I don't know what it is, lets wait and see". Its the latter that really makes this difficult to do.

 

[olly]

i started about the age of 32yrs with persistant dropping things,clumsiness and bad balance issues.
the drooping and clumsiness was noticed more by others,i did not think anything of it at the time.
balance issues were bad,if i sat on the stool at work i would fall off to the side..................always my right side which i find kind of strange.
started with muscle spasms several months later about may,crippled was sent to see rehumi who did all the tests and was thinking of putting me on methatroxate till i told him of a neuro symptom i was having and he said i needed a neuro not a rehumi that was august.
september i was bedridden and got emergency neuro appointment a few weeks later in october.
on my first exam they found clinical weakness in my left leg,later i realised this was why i had been dragging it about..............ignorance is bliss.

i did keep a symptom diary during my early years also on my doctors advice, at an appointment i showed my neuro and he said your quite poorly are'nt you..............doh!

i think i read it was the 28th your appointment...............please let us know before you go.
pretty sure you will be ok,well not ok but things are not quite as bad as you think they are.............you know what i mean.

 

[Bad Balance]

Although I have only been diagnosed since this last December, I also now realize that I have exhibited symptoms fo several years.

I know that each of us present a bit differently and we are all on our linear progressions at different points. That said, I cannot believe how my body still responds positively to use...whether it is actual exercise or just pushing for continued use.

IMHO it is critical to keep on trying.

 

[olly]

Upper motor neuron. Upper motor neuron weakness presents in a rather characteristic pattern. First of all, it tends to affect broad areas of the body. This is because axons are quite tightly packed when they are in the brain stem and spinal cord. Typically, weakness is accompanied by an increase in deep tendon reflexes such as the biceps, triceps, patellar or ankle jerks. There is usually a decrease in superficial reflexes such as the abdominal, cremasteric reflex or the normal plantar response. There may well be the appearance of pathological responses, such as the Babinski response. Finally, upper motor neuron lesions are characterized by relatively minimal atrophy, and do not lead to the fasciculations. It is noteworthy that some atrophy may take place of disuse nature, since the affected body part is unlikely to be exercised regularly. There may be increased tone of a spastic nature associated with heightened muscles stretch reflexes. This increased tone tends to appear in a "clasp knife" pattern. One caveat in the evaluation of upper motor neuron problems is that very large and acute injuries may produce a period of generally diminished tone and responses that has been termed "spinal cord shock" since this is usually seen with acute traumatic spinal cord injuries.



this is from a neurological website.
once again it explains disuse atrophy in pls.
the hypotonia/loss of contraction in my left lower leg was caused by an acute umn lesion ,here described as spinal cord shock.