uncertain DX

[BLPhill]

Has anybody ever heard of the diagnosed of a pls form of als? If so, please explain it to me. My first diagnosed was pls. The second was a pls form of als. I go back to U of W for third opinion on Feb 20th. The more I go to the dr the more confused I get. I have just decided that, it is what it is, and its gonna do what its gonna do, regardless of what they call it. Does anybody want to lay any bets as to what the third opinion will be?

 

[olly]

the only thing i can think of that he may mean is umn dominant als.
this would mean mainly umn involvement/symptoms with minimal slow progressing lmn involvement/symptoms.
in pure pls there is no lmn symptoms according to the criteria.
i have mainly umn but a little lmn.
this area can be a bit shadey and hard to understand.

 

[Danijela]

Here is my undestanding:

In the UK the umbrella term that used for the three main types (ALS, PLS and PMA) is MOTOR NEURON DISEASE or MND.

In the States (and other places) the umbrella term that is used for the main types and denoting all types of motor neuron disease is ALS (incorporating 'actual' ALS, PLS and PMA).

PLS form of ALS (umbrella term) is just that - PLS, involvement of upper motor neurons, PLS type of motor neuron disease.

MND is a preferred term where there is a belief that there is likely to be an overlap between different types (danger of further inolvement such as progressing from having only lower motor neuron damage to having both lower and upper, and the other way round).

 

[Zaphoon]

Betty,

Let me further muddy the waters here... ALS involves both upper and lower motor neuron death. If only your upper motor neurons are dying, the call it PLS and give you a window of 4 years to check for the start of lower motor neuron death.

If they detect an amount of lower motor neuron involvement but not enough to call it definite ALS, they will tag it upper motor neuron dominate ALS which typically has a slower rate of progression.

If they detect a lot of lower motor neuron death, they will change the diagnosed to ALS.

This is my understanding. Often times, ALS will start with upper motor neuron involvement only and it is classified as PLS until lower motor neurons become involved.
Sometimes it can take up to 4 years for this to occur.

Zaphoon

 

[rose]

Betty,

I have no answers for you, just wanted to say "good luck" with your appointment tomorrow. I'm sure you want clarity ~ black and white answers~ but sometimes they are a long time coming.

 

[BLPhill]

When I went to the last neuromuscular specialist he asked "what have the other drs been telling you'. I told him I had been given the diagnosed of PLS.

After a brief exam by this dr, he said he thought ALS, due to weakening bulbar muscles and increased left side weakness. Now this is the 2nd dr 2nd opinion. He also put the note into pcp, I have a pls form of als. these are his words.

I have also been in the process of a 3rd opinion, by another neuromuscular specialist at the university of washington in Seattle. He scheduled me for a 2nd EMG/NCV which I have already done on Jan 30th. Also he ordered a full set of MRI which I also did on Jan 30th. I go tomorrow on friday to get his opinion and test results. I was told the MRI would only take one hour, well that turned into three hours. Probably not a good sign.

On 2nd EMG/NCV I was told by the dr performing this, I do not have any of the 4 required areas of denerveation to diagnosed als. He also said not that I wouldnt in the future, but currently I do not.

Do you see where my confusion comes in? Currently I cannot talk. I can walk with a walker and thank god for the walker or I would have done many a face plant.

I am not even stressed out about the whole thing anymore, because I do know it is one or the other and eventually they both do the same thing. Thru all this I have learned, if you are not satisfied with one opinion get a second and a third, how ever many it takes, since having MND I have plenty of free time.

 

[BLPhill]

If it is umn dominate als, why dont they keep it simple and call it that.

 

[sukilou]

Hi Betty,

Here's another answer for you. My ALS neuro has diagnoseded me as having UMN Dominant ALS even though I meet all the criteria for a definite ALS diagnosed. She has told me that my upper motor symptoms are much more prominent and advanced, with my lower motor neuron symptoms being more subtle. I have had 2 emgs, both showing chronic denervation and reinervation in 3 areas. I am having slight bulbar symptoms, but much difficulty walking. I must use a walker at all times now, and will be getting a power chair shortly. I have had many falls due to my unsteadiness.

I hope that your 3rd opinion will clarify the diagnosed for you.

Good luck!