UMN Dominant ALS

[bilphi]

Turns out that I'm not sure I should be in the PLS forum or the ALS forum. According to my neuro now I have UMN dominant ALS. At the last check up he must have noted some LMN symptoms.

ALS is rare, PLS is rarer, what's the chances of UMN dominant ALS?

Is their anyone else out there with the same diagnosed? He said that it's slowly progressing so that's a good thing.

 

[sukilou]

Hi Bill,

Yesterday I received a diagnosed of UMN Dominant ALS from my ALS specialist doctor at an ALS clinic. I don't know whether the chances of receiving this diagnosed are any different from receiving any other diagnosed of ALS. In my case my first symptoms were UMN, but I did not go to the doctor for over a year because I thought it was arthritis related. You can look up my earlier posts for my story. Also my doctor told me that this variant could be slower progressing, but that it will progress. My LMN involvement is not as pronounced as my UMN at this time, but I do have clinical weakness in right foot (with atrophy), right and left legs, left hand (with atrophy), and right and left arms. My first EMG was done in Sept, with abnormalities showing, and then confirmed by a second EMG at the clinic last week.

My first appt at the multidisciplinary clinic with the ALS team is this coming Mon, and I was asked to participate in a study to determine any bio markers for a possible blood test to help in diagnoseding ALS. I said yes, and I'm excited about participating. It's only one small way in which I can maybe help. Got to keep the faith and keep on trucking!

 

[olly]

hi bill and lonna

welcome to the "in between club"
both me and hopingforcure are in the same club.
i have mainly umn involvement but lmn weakness in my left leg,with some atrophy of left foot,shoulders ,arms and a little bit in my right hand.
my neuro diagnosed mnd,no definate pls/als.
he said he did not know were i fit in,but with my slow progression it does not really matter.
i am supprised they diagnosed umn dominant als as i have only known that term used in studies and not given as a diagnosed.
some think pls is a different desease to als and not a sub type..................if that was the case why do pls patients show lmn involvement on autopsy?
like variants in ms i think this is the case for mnd,s.

 

[bilphi]

Caroline and Lonna,

Aren't we special? :-D For some reason I don't feel special, I feel very, very tired.

Just as of late my arms feel very weak. I don't even want to use them. My legs have been that way for quite some time and I'm used to that. Every progression seems to be more and more tramatic......maybe I'm getting weary of the whole thing.

Anyway, this diagnosed is much preferrable than some we could have. I hope both of you stay in touch and let me kinow how you're doing.

 

[hopingforcure]

Hi Girls and Guys
You know I think what Caroline said is so true, they do not seem to have a UMN ALS as a real diagnosed. but it seems as many people with PLS actually have that. I think that is what a lot of the slow progressors actually have. I talked to a PHD ALS specialist, and he felt that UMN symptoms could in fact show LMN problems from damage. Olly called it correct some of us just fall into that gray area, we have ALS/PLS but fall into a more of a middle ground. But then again some feel PLS is a different disease all together, and the people who change to ALS from PLS were just misdiagnosed. from the start. My what a tangled web we live in.. Keep your chin up ladies and gents, we are in this together....8):-D

 

[sral]

Bill / Lonna,

How long after first symptom did your lower motor neuron symptoms appear?

They say that as time passes that one has only UMN symptoms that the odds are lower for it turning into ALS.

Caroline I believe only showed lmn symptoms many years into it.

 

[sukilou]

Hi SRAL, I think my first LMN symptoms were the twitches at about 1 1/2 yrs from when I started falling. The falling and loss of balance were in Jan 06, cramping about June 07, then twitching randomly shortly after that. That's when I started with the doctors in June 07. The crazy thing (or maybe good thing) is that I didn't realize that the fasics were anything significant and didn't report it to my doctor at that time. I kept thinking arthritis or fibro, and the doctor didn't discover anything but hyper reflexes in legs. I went to the neuro for the first time in Aug 07, and he also thought muscular skeletal problem. It has been since then that the spasticity and rigidity increased, many falls, spasms, cramps, all over twitching, weakness, atrophy. What I question now is how long had the UMN degeneration been going on before I noticed it because I have had stiffness and muscle aching for over 15 yrs. More questions than answers! But the bottom line is that I meet the El Escorial criteria for Definite ALS, according to Dr. Goslin, so I go on from here. I call it "the new me."

 

[olly]

hello everyone.
i started with umn symptoms in may 1999(odd symptoms before like falling over and dropping things but may the painfull spasms started)
i was first sent to a rheumatologist who said that all the tests were fine,by this time i started with weakness and was sent to neurology.
my neuro noticed clinical weakness in my left lower leg that at that time had footdrop.
after several years of continuous tests,mri,s to rule out ms them multi system/cerebeller atrophy it only left mnd.
it was a couple of years ago a younger neuro i saw as mine was away did a test no one else has done,he positioned while layed on the clinic bed my legs in a certain position with knees slightly bent.
then he stroked down the inside foot toe to heel,my right leg was ok but my left lower leg just went beserk quivering.
it was so funny really as the neuro was in awe and said to the nurse stood behind me to look,he kept doing it and both of the stood there ooing and aaring.
i was used to being prodded and poked so thought nothing of it and did not ask what was the matter.
then a few months later at the rehab centre the doctor said i had hypotonia/loss of contraction in my left lower leg,i did not react to the statement as i did not have a clue.
he got agitated and explained it to me,even then i was like ok.
just over a year ago when i was finally diagnosed with mnd i asked my neuro about this but he was reluctant to answer.
so how long has the lmn been there? thats anyone guess.
at my last visit my neuro said my progression has been slow and that is all that matters.
i have had fassics in certain places months after i got ill starting in my left lower leg,now they are in the right.
certain areas like my left foot,upper arms/shoulders there is muscle loss ,i had fassics there too.
just stiffness/weakness in my right hand with some muscle loss around the thumb/hand part,i have had trouble with things like tv remotes,buttons for a while before noticing the muscle loss.
i have weakness but not to the point i can not move a certain part at all.
possibility of mnd was mentioned to me some years ago but i dismissed it thinking it could not possibly be that.
it was only after diagnosed and joining this forum i began to truly understand this desease.
i have alot to be thankfull for despite this, so i just concentrate on daily life .
sorry for going on lol,explaining ones medical history takes time.

 

[sukilou]

Hi Caroline,

"Ignorance is Bliss," as they say. I think that if I had realized the significance of my symptoms 3 yrs ago, I would have been a basket case long before any diagnosed was given! Because a small frontal lobe meningioma was an "incidental finding" on my first brain MRI, July 07, I became so focused on that, that I didn't think about anything else. I had joined a forum called Meningioma Mommas to learn what I could, but realized from them and my neuro that it was not causing my balance and walking problems. I did ask my ALS neuro if the meningioma could contribute to my developing Frontal Temporal Dementia. She said no, so for now I have crossed that off my list. Ha!

I've been following your posts since I joined this forum. I, too, was an avid gardener, live on 3 acres, with 2 dogs, alpacas and parakeet, and my husband. We are looking into making our bathroom into a walkin shower. From your description of your new housing, it sounds as though your living situation will be much better for you.:-D

 

[Big Mike]

UMN predominant als uncommon

I too was diagnosed with UMN dominant als. I guess it falls somewhere in-between classical als and pls. I wouldn't be surprised if pls is just another variant of als as well. I read a scientific article where out of 661 als patients first appearance at an als clinic in Canada, only 27 (4%) presented with spasticity, which is consistent with the UMN dominant variant. So, just like pls, apparently it's pretty rare as well.

 

[mydog]

My husband has UMN dominant mnd, we think he has pls, but the neurologist is not willing to give the illness that label. Chris's symptoms started way back in 2001 and he has just been given his diagnosis od mnd recently. His main problems are with spacticity of the legs which is severe, and talking problems. He falls frequently, has problems with balance. The main thing is that progress is very slow, which is good. Take care

 

[Geo]

Now im really confused

I always thaught that with ALS the nerves die ,henceforth the EMG shows this . I see no mentions of abnormal or Bad EMGs . In PLS EMGs are normal ,spinal taps Normal , Blood tests Normal . And whatever else . This is why Im really confused . Someone saying they have been given a diagnosis of UPPER , LOWER ALS . Without mentioning anything about an Abnormal EMG .

 

[sukilou]

I'll jump into this discussion--I was diagnoseded with UMN dominant ALS. Yes, my 2 EMG showed LMN involvement, hence the ALS diagnosed. Because I presented with UMN symptoms first and they are more prevalent than loss of muscle strength is why the UMN dominant diagnosed. In fact the first neuro thought for sure that I had PLS until the EMG. And my ALS neuro said that my LMN symptoms were subtle, but they were there and showed up on the second EMG also.

 

[GlenBrittle]

Hi Geo,

For ALS, its not the nerve that dies as I understand it. Its the cells that transfer the commands to the muscles that die. The result is the same though. As far as the other MND variants , it might be true about the nerves. I have not researched the others.

Glen

 

[Big Mike]

UMN damage is not detected on the emg machine. It can only be identified by clinical signs such as spasticity, clonus, and brisk reflexes. Only LMN signs show up on the emg, which are indicative of chronic innervation due to denervation.

As far as the neurons and nerves go, I think Glenn is right--only the neurons are directly affected, although recent evidence also implicates the neuromuscular junctions as well.