Questions

[konagirl]

Hi there, I hope my questions are not too silly, I am just trying to wrap my brain around some things. To be fair, and honest, we do not have a diagnosis yet so maybe I should not even be posting here, please tell me if I need to leave. Our neuro has told us he is confident my husband is facing a motor neuron disease and he mentioned PLS. We go back 12/2 for another EMG and some other test results and we are expecting a firm diagnosis on that day.

Here is what I understand: PLS effects upper motor neurons only. ALS effects both upper and lower. LMN will show on an EMG, UMP will not. So with a clean EMG, that means no LMN involvement and the diagnosis will remain PLS. I also understand that a true PLS diagnosis needs about 5 years to be firm because many times ALS will present as PLS in the early stages. Please tell me if I am understanding any of this incorrectly.

Here is where I get very confused: UMN symptoms are what produce spasticity? LMN are what produce clinical weakness and paralysis? My husband has no foot drop or the loss of usage for any muscle yet, so this makes sense to me, I think. But....I see that many people with PLS are in a wheelchair, or have lost the ability to speak or use their hands. I cant seem to comprehend HOW someone has lost use of their legs but nothing shows on an EMG? At that point, wouldn't it be muscle degeneration causing the need for a wheelchair? But if still nothing is picking up on an EMG, then it couldn't be muscle degeneration, right? So what is it that causes the loss of functionality in a limb for a person with PLS?

I really hope all that came out ok and I did not confuse too badly. I just beginning to learn all of this and it is all very overwhelming and confusing. Other than PLS progressing slower...is there any other real difference in that and ALS?

Thank you in advance for your help.

 

[Nikki J]

There is weakness of UMN origin this is a very basic summary of the differences
Medic Guide: Upper motor neuron vs lower motor neuron weakness

 

[billbell52]

The link Nikki provided is pretty succinct, however, I do have one correction. I have small fasciculations mostly in my calf muscles. So rather than 'Absent' I would say 'Absent or Small' and for ALS I would say 'Pronounced'.

The other major difference is about 5600 people get ALS each year and about 60 people get PLS. PLS is very rare.

 

[gooseberry]

konagirl, Especially in the beginning it is very confusing. I would suggest you right down all your questions in a notebook to take to your hubby's appt on 12/2. That way you can get everything answered and write it down to refer back to. I would also suggest you find out how you can get a copy of the office notes and test results from the visit. If they are doing an emg you want the interpretation portion also. They don't always give this voluntarily.

Always feel free to ask your questions. We help and learn from each other. Best of luck. Step

 

[Green Queen]

Kelly I agree with Steph, we learn so much more from each other than reading all the techno mumbo jumbo on the Internet.
I supposedly have PLS, and there's so many variables it does make it very hard to understand.
I haven't read the link yet, I will, but one reason why I prefer my scooter (Henry) to walking is that I get so tired.

All the best.

God bless, Janelle x

 

[konagirl]

Thank you. Really.

You know when you sit in a chair and bounce your leg? If that is happening involuntarily, is that what clonus is? Chads leg bounces and he can't get it to stop sometimes. He does have fasciculation's too and they are VERY pronounced. I can't remember the day we first noticed them, but they have been constant in both arms, from his neck to his elbows, 24/7 for at least 2.5 months that I can remember back to and since then, there has never been a moment where they were gone. Their intensity changes but they do not go away.

I realize our appointment is tomorrow and I can just wait to ask the Dr all of this, but I am going crazy waiting. I am still looking for signs that this could be something else. He is only 36 and we have two small children that worship the ground that man walks on. I want to wake up tomorrow and find out all of this has been a bad dream.

 

[Nikki J]

In spite of the link I don't think the pronounced twitching is definitive for LMN disease. Sorry. My point in posting it was really to document that Weakness can be part of a purely UMN syndrome. sounds maybe like clonus but the doctor will tell you right away if it is. Good luck tomorrow

 

[gooseberry]

I know its hard, so very hard to wait. But only your husbands doctor can see the whole picture and make an accurate diagnosis. We are speculating symptom by symptom. I dont say this to be mean but rather because no one wants to misguide you. Thinking of you both and hoping your appointment gives you some answers. Steph