UMN dominant ALS

[Alyoop]

Hi does anyone out there have UMN dominant ALS? If so how fast does it progress? I realise everyone is different, but some personal information would be great. I have read some interesting articles about it, with the stats on survival ( hate talking about that) but unlike info on MS I cannot find any studies that report the rate of deterioration during that time. Ie average time to being in a wheelchair etc.
Thanks
Aly

 

[John1]

Interesting question Alyoop. Do you have reason to suspect that UMN dominant ALS tends to a slower/faster progression rate than LMN dominant or combined UMN/LMN? I am strongly UMN dominant. No fasics and little atrophy. I bought my first scooter five years after diagnosis. I still use a scooter, not a chair, breathe without assistance (but breathing is impaired), and my speech is usually fairly intelligible. I have lost most leg strength, especially balance, and am losing dexterity in my hands after nearly 10 years.

How is your progression? How long before diagnosis did you have symptoms? I think this provides a good indication of your expected progression rate.

 

[Alyoop]

UMN -dominant ALS has a longer average lifespan. The average is 10 -12 tears as opposed to regular common ALS that has 3 -4 year average life span.
This of course is average, so heaps live much longer (and sadly less).

The genetic variant is similar, and can have dominant UMN signs for many years until it changes to both UMN and LMN. The genetic one is very interesting as it can have a clean EMG for years. So all the people trying to be helpful saying that the EMG is the key, are not actually correct. I am lucky as my husband is a neurologist so has been busy discovering varients that he did not know about, and sending the literature to my neurologist (who may or may not appreciate his input)! ha ha

They want me to have the genetic test that is available, but to their horror I sad no. We suffers have got to keep a bit of control, dont you think?
Thanks for the information re your progression. It was really helpful
Cheers Aly:lol:

 

[sadiemae]

What exactly is UMN dominant ALS? Yesterday at clinic doctor said that my husband was very slow progressing and that the main part of his ALS is in the brain as opposed to the spine, which I take to be UMN dominant. The doctor did not use the phrase UMN dominant, but by researching I think this is what he meant.

 

[John1]

UMN -dominant ALS has a longer average lifespan. The average is 10 -12 tears ....Thanks for the information re your progression. It was really helpful
Cheers Aly:lol:

Hi Aly,

I think I've shed more than that already.

But seriously that was a really interesting post and it's the first time I've heard that since my diagnosis. After a few years of expecting the worst in a relatively short time, I began to rethink how I was going to live my remaining time and began to take a longer view of it. I wish my neurologist had mentioned this to me. I find neurologists here very ignorant of ALS. After spending 6 years trying to find out what was wrong by making the rounds of most of the neurologists here, I took off to the Montreal Neurological Hospital where I finally received my diagnosis.

I hope your progression is slow too and you may well be around for a real therapy.

John

 

[olly]

Abstract
Abstract A new amyotrophic lateral sclerosis (ALS) category named 'UMN-dominant ALS' and defined as 'due predominantly to UMN signs but with minor electromyogram (EMG) denervation or LMN signs on examination' has been proposed. The clinical and laboratory features of 20 patients with UMN-dominant ALS are described here, their disease course is analysed longitudinally according to their disability progression, and all these parameters are compared with those of typical ALS patients. Ten women and 10 men diagnosed with UMN-dominant ALS were evaluated. Their mean age at disease onset was 58.6 years. At the most recent evaluation, after a mean disease duration of 7.7 years, all patients progressed to a tetrapyramidal syndrome with pseudobulbar features of varying degree. No patient had respiratory problems. Cognitive impairment was observed in eight patients. The differences in disease progression between the UMN-dominant ALS and typical ALS patients proved significant (p <0.02) both with regard to the total ALSFRS-R score at six months and to each single region subscore at 12 months. Our findings suggest that there is both a different pattern of disability and longer survival in UMN-dominant ALS compared to classic ALS patients.

i found this recent study dated nov09.

 

[Alyoop]

Hi Sadiemae.
UMN dominant ALS, effects mainly the upper motor neurones, so yes to make it simple the nerves originate in the brain and feed into the spinal cord. The symptoms are spasticity (stiffness) and the reflexes are brisk. Weakness can occur as well as some atrophy, but the tyope of atrophy in umn is mainly lack of use as opposed to dying nerves feeding the muscles. EG I have a spatic gait that causes me to uinder use my quad muscles especially on one side, I therefore have a bit of muscle atrophy in those muscles that I am not using enough. Hope that makes sense.
So in umn dom als. It often starts in the UMN and sometimes stays that way for many years, eventually encompassing the LMN as well, so that causes the fasciculations and muscle wasting, breathing problems etc.
Bulbar symptoms can occur early or late.

John
The neurologists really do not all know about the different sorts, ALS specialists would of course no, but neurologists have there own subspecialities that they are very good at. My husband is the best around here in epilepsy, another is a movement disorder specialist(parkinsons etc) some are guns at peripheral nerves etc. So its not that they know everything in great detail about all neurological illnesses. Don't get me wrong they no heaps about lots, but the really out there rare things cause them to hit the medical journals and find out. So I suppose its important that where possible people get a second opinion from a specialist in the field in which there disorder fits.

Blah Blah. I hope I have made sense. I like you probably have UMN dom ALS, but time will tell. Maybe its just something else. I am going to have yearly EMG which I read in a medical journal today is good practise when someone had ?pls?als. It just keeps an eye on things. That is why I have just told my neurologist that I will not be seeing him in Feb like he wants, but July, cause I know he will stick heaps of needles in me and I have already had 4 too many.

Stay well my friends and hit this great world while you are still up and running. The Docs are often wrong

 

[pudge44]

My husband started with a long period ( years ) of severe leg cramping then just like overnight ( approx a year ago ) speech difficulty and weakness in his arms, his speech is now very difficult, a real struggle, and he now has much more feeling of weakness in his hands & upper arms ( though still working ) and he has heavy feeling when he walks ( his walk looks much different now) , from what I can pick up on this thread he has upper and lower MN weakness but I am not a doctor LOL, anyway if someone with the same symptoms who is has a difinitive diagnoses could shed some light to their symptoms this would be helpful PEACE LIGHT AND LOVE TO YOU ALL XO

 

[Alyoop]

Pudge, it's interesting that often in retrospect we can look back at symptoms that at the time we just coped with, thought nothing of. The years of cramps is a very common theme. I had 2 years of cramp in my lower legs (not calfves) that drove me crazy, but I did not seek any answers. It went away for a year and I forgot about it. My next trouble was not being able to climb even 1 flight of stairs without difficulty. I was going to the gymn and running 4 km most day, so the weakness was strange. Suddenly the cramp came back in the same place and running was not possible. Then I got fasciculations which I now know are benign, thank goodness. Anyway a spastic type gait rapidly followed and foot drop on one side. Clinical weakness is mainly in my hips and mild in my foot, hence my inability to go up slopes. The cramp stopped last year, as abruptly as it had started, but I got it again yesterday . Anyway now everything has slowed down and I have gotten no worse in 9 months.
I really believe that there are a huge amount of variations to MND and sometimes that's why you get cases that move at a tremendously slow rate , like Stephen hawking. Your husbands cramp, slurred speech, weakening limbs is fairly classical MND symptoms. He has been given an ALS diagnosis so he must have evidence of lmn and umn signs in several different places.