UMN signs in diagnosis of ALS, please read.

[marywest45]

Hello,
I haven't posted here for a while, was on a trip to Mexico (forfilling my bucket list).
Today, I had a follow up appt with my neuro at Sinai; he was out sick and I ended up seeing his partner... This doctor told me that I don't have any signs of UMN based on his exam.
So I asked him: How was I diagnosed with ALS without signs of UMN? He replied that I need to check with Drs. at Hopkins who diagnosed me. I proceeded on asking him again: Do you think I dont have ALS? and he goes:" I didn't say that".
I'm not a medical person, but sure read a lot about ALS lately, and was under impression that UMN and LMN signs must be present for the diagnosis.
Do any of you have a diagnosis of ALS without presence of UMN signs on clinical exam?
I'm loosing muscles in upper back, chest and my arms; can't raise my hands to wash my hair, trouble breathing, twitching. Could I have something else?
Hopkins doctor said that I have denervation along my spine and in all limbs.

God bless, Mary

[joelc]

You have to have both upper and lower, so you are correct in this.

[Danijela]

If you only have lower motor neuron damage symptoms such as fasciculations, muscle weakness and muscle aprophy, and you happen to be in the uk, you would be given a diagnosis of PMA - progressive muscular atrophy, considered to be one of the entities in the spectrum that is mnd. In addition, a warning that umn signs can appear at any time plus a promise of a slightly better life expectancy than with the classic form of mnd (ALS) may be given.

[John1]

I have noticed that diagnosticians in Canada, and perhaps the US, seem to lump both classic ALS and PMA into the catch-all, ALS.

[Zaphoon]

Well, if the doctors are willing to call it ALS with just LMN involvement, why not do the same for folks with just UMN involvment?

I really think there needs to be a distinction, though, because the differences between PLS, ALS and PMA are pretty dramatic.

It is my personal, unprofessional opinion that if you have only UMN signs, you do not have ALS. I'm just a piano tuner; what do I know?

PZ

[joelc]

I was just at an ALS symposium yesterday and that is what the neurologists there said, it has to contain both for an ALS diagnosis.

[shelleynshaggy]

Our doc at the ALS Clinic states it has to be both upper & lower involvement. Jim has UMN - they are waiting to see if it progresses to include muscle atrophy. Confused me because his sx were at the time mostly lower extremity. Per neuro - the upper controls the reflex/nerve part of the neuron and the lower the muscular part of the neuron - best laymans terms he could give me. Made a little more sense. (Not fully though)

Therefore, he has MND/ALS syndrome.

[hopingforcure]

You know it is funny how the nerves control areas. I am very UMN dominant. although I do have atrophy, fasics, and such, I mostly have very heavy hyper reflexes, stiffness, clonus, startle reflex, and spasicity. Hope that helps.

[Danijela]

Having said all of the above, most specialists in the UK would simply term it as MND, avoiding more specific labels unless pushed to specify. I guess they are more willing to attach PLS label as this gives a greater sense of hope to the patient, knowing that the outcomes are much better with PLS (e.g. normal life span). When Laurence was first diagnosed I was hoping for PMA (and better outcome), as the atrophy was in his left hand only, and no visible UMN signs (apart from brisk relfexes) were present.
He was, however, correctly given the diagnosis of MND/ALS - classic type as he fitted El Escorial criteria. I longingly said something about PMA (you know you have reached the bottom when prepared to take PMA, but hey!) to which he replied that the only thing that matters is the rate of progression, and that patients with PMA can have the same or even quicker progression than the classic type (ALS) if their breathing muscles are affected soon upon onset of the disease.

Since then, I have given up on those labels, and focused on the rate of progression, his ability to perform daily tasks and our time together/what we can do together.

[Big Mike]

I'm in the same boat as hopingforcure. Very UMN dominant. Although both umn and lmn symptoms must be present for ALS dx, I get the impression that the umn aspect is not very pronounced in most als patients upon first clinical presentation.