Questions on ALS symptoms

[lgelb]

We're doing new posters a disservice if we overemphasize the UMN + LMN signs criterion. This is where the cluster of MNDs come in -- PLS, PMA, ALS. And then there are the flail arm and flail leg ALS variants, both of which typically present w/ LMN signs only (as does PMA). This is all semantics and manuscript fodder. It does not change the fact that if you have a MND and thus far only LMN or UMN signs have presented, you still have an incurable and generally fatal (in PLS or Kennedy disease, you might live long enough to die of something else) disease. If people want the diagnostic criteria for clinical trials, there's Google for that. But most clinics will still code "ALS" for, say, "PMA" for social program eligibility and such. And UMN signs not infrequently present a couple of years after LMN, as confirmed in numerous papers.

 

[Dusty7]

I agree we must be careful not to overemphasize the UMN + LMN signs criterion, although El Escorial and Al Awaji strongly emphasize those criteria. And, yes, MNDs cover a broad spectrum and anybody with PLS or PMA may slide into the ALS category at any time. As my neuro and I have discussed, only about 5-7% of people with MND can be classified as having PMA. So having PMA is like being at the top of a steep slope that leads down to ALS. Few manage to stay there for very long. And in a study of PMA the researchers would only allow in patients who had had PMA for 4 or more years. Even then, some of the subjects developed UMN and became PALS during the study period. And, yes, the debate goes on among the professional neuro community as to whether these are even separate diseases or simply different manifestations of the same disease.

The problem as I see it is that posters to the forum want to know which they have because the prognoses are radically different.

ALS life expectancy averages about two to five years from the time of diagnosis. (ALSA)
PLS is not fatal. (NINDS)
PMA life expectancy is generally longer than the average for all cases of MND, averaging 5 – 10 years. (MND Association)

I don’t think it’s realistic to tell a DIHALSer that, yes, it looks like you have an MND and your prognosis is 2 years to not fatal. We know more about MND than that and I think DIHALSers want to know what their prognosis is. If we don’t tell them what we know, they will just go googling and may really get some bad information.

Yes, we have an obligation not to tell people, you have no LMN, you’re out of the woods. Or without UMN, it’s no big deal (as I pointed out to the OP above--the lack of UMN, “would point away from you having ALS, though a lower motor neuron predominant form of MND called PMA is still a possibility.”) And, yes, most clinics will code PMA as ALS, which is where the person will probably end up, but the person with PMA probably won’t qualify for ALS drug trials, even if coded for ALS.

 

[chuckstr]

Hello all again,

I had my ALS "Center of Excellence" appointment last week. I'm not quite sure what to make of it. The Dr I saw was not a neuromuscular specialist but is apparently the Dr that all of the patients not referred for dirty EMGs that are screened for MND see. She did a pretty thorough neuro exam and looked at the ridge on my tongue that I showed her and said she saw it but that it wasn't atrophy. She also looked at the MRIs I've had done and said she suspected a C6 radiculopathy without myelopathy and that I didn't have an MND. I asked how she could be sure and she mentioned the pain and sensory symptoms as well as the quickness with which my whole left arm became weak. She pointed out that tons of people have fasiculations and that mine seemed to be very sporadic which is not indicative of ALS. I asked about an EMG, and she said I didn't need one but to call her if the weakness progressed. Should I take this at face value as a specialist opinion that I don't have ALS?

I have also had some trouble with excess saliva (or not being able to swallow it, hard to tell) for the last week or so in addition to the other symptoms. No trouble with regular liquids or solids (although I seem to hiccup more). Does this sound familiar as an ALS symptom relatively early on?

I also have my regular neurologist EMG scheduled tomorrow. If that EMG turns out ok, does it seem that I should move on from an ALS/MND or is it worth going back to the specialty center or to the UW where they have accessible neuromuscular specialists?

Either way I'm going to see someone about the health anxiety that's been building up for me. It's screwing up my life and marriage.

Thanks for listening, and for all of your help,

Chuck

 

[chuckstr]

I had the EMG from the local neuro. He tested my weak left arm and both my legs. He was in a bit of a hurry since we got started late but he said no ALS. He said my legs were normal but there were definite denervation signs along the muscles from the c6 nerve root only. He tested many muscles in my arm and found those not from c6 to be fine. I've read that it can be difficult to distinguish MND from radiculopathy on EMG. He seemed very sure about this, but for you EMG knowledgeable folks, is there something else I should ask or follow up with or does this sound definitive.

Thanks again everyone for your help,

Chuck

 

[MaxEidswick]

>t started late but he said no ALS

That is what you want to hear; however my first neuro said the same thing, our ALS clinic did my diagnosis three months later ...

 

[chuckstr]

Wow, I'm very sorry Max. What diagnosis did the previous neuro give you based on the emg? Was there something they missed?

Thank you for answering,

Chuck

 

[MaxEidswick]

>What diagnosis did the previous neuro give you based on the emg? Was there something they missed?

the quack prescribed anti-anxiety drug and sent me to a cardiologist -- also to a hand surgeon to do a nerve release -- proof idiots do exist as I listened to her ...

 

[Nikki J]

Max I am curious. Did the "quack" do an EMG? If so did she EMG the wrong muscles? It seems so unlikely that anyone would miss the fibs etc if they were testing the affected area but can totally see that if they tested the wrong areas they could miss something. It is so different than my diagnosis. The EMG neuro said" show me what happens when you fall" i said " my leg does this" and she said " maybe it is a problem with this muscle" inserted her needle and that was it. Think it shows how important it is to get a neuro who listens. If she had randomly EMGd my leg she could have missed the affected muscles

 

[MaxEidswick]

>Max I am curious. Did the "quack" do an EMG?

yes, although I suspect the EMG equip was faulty ...

 

[Dusty7]

"I've read that it can be difficult to distinguish MND from radiculopathy on EMG."

I am not a doctor, obviously, but I would say that the big difference between MND and a radic is that MND is widespread while a radic is pretty well focused along one nerve root. A radic can be eliminated by a clean MRI of the implicated nerve root area. You did not have that; your MRI showed problems in the C6 nerve root area, so I would say it is pretty definitive. All you could do at this point if you don't want to believe the good news is ask for an EMG on your good arm, other muscles in the legs and a sampling of paraspinals. If they still can't find widespread enervation, I think you need to accept hat it's a 6 radic.

 

[chuckstr]

Thanks so much Dusty. The difference you pointed out is exactly what my neuro said. I'm not sure what other leg muscles (he did 20 all-together) he could do but I can definitely ask about the paraspinals and other arm when I see him next week. I know it sounds a bit wacky and probably is but I want to be as sure as I can be.

I got a copy of all of the EMG results today. One weird thing is I believe they normally ask you to contract the muscles after they check for spontaneous activity and he didn't do that. My understanding (which may be incorrect) is this allows measurement of things like MUAP and recruitment. If I understand this has to do with how the muscles are reinervating if they are which I would very much like to know. Other than that it looks like a text book for denervation of the muscles along the c6 nerve root (psws, fibs in biceps, wrist extensors, brach radialis, pronator teres).

Thanks for all your help and support,

Chuck

 

[lgelb]

Chuck,
Agree w/ Dusty, if all points to C6 only not a MND distribution of neuropathy. Since you're in Seattle, you can get a 2nd opinion as to the completeness of the EMG via UW, if that's not where you went.

Dusty,
The PMA distinction, though still cited in some associations' copy blocks (that are seldom updated based on literature), has not been well-supported in more recent studies. There is a growing school of thought that all LMN-only disorders, if actually MNDs, apart from Kennedy dz, are on the ALS continuum, as you mentioned, with many ultimately developing UMN signs and the operational definition of PMA often boiling down to slow-progression LMN-dominant ALS. So just would not want someone diagnosed with "PMA" as an increasingly outmoded freestanding diagnosed to think they have twice as long ipso facto and plan accordingly. And PLS can indeed be fatal (33% mortality in one long-term study), nor is an initial diagnosed necessarily correct, given that an MND that is UMN-dominant "converts" to PLS after 4y and no wasting).

As you say, the ALS trials are more exclusive but apart from stem cells (as they grow up, pun intended), no rx has shown definitive delayed progression in more than a subgroup so no one should as yet mourn ineligibility, instead should focus, IMHO, on interventions with more evidence of effectiveness across larger groups, e.g. diet, exercise (active/passive depending on dz stage), etc.

So as supported by the available evidence, provisional MND diagnosed early in dz may well place a pt at the ultimately "wrong" part of the spectrum, and so the take-home is more likely "you're dying w/ in a few yrs" than any other prognosis. That said, clearly the more yrs you show no LMN signs, esp. atrophy, the better your prognosis. The converse is less true.

 

[chuckstr]

Thanks for the response. The EMG wasn't done at UW, I have an appointment there for 6/27. It was done by a local neurologist on the eastside, who (supposedly) has some experience with neuromuscular disorders. I'm also waiting for a call from the head of the ALS clinic at VM (which I am glad I did not hold my breath for ). Do you have any experience with those folks (Dr Elliot specifically)?

Thank you,

Chuck

 

[Dusty7]

lgelb, I don't think we have any real disagreement. If I were in charge, I think I would call our diseases UMN/ALS, LMN/ALS and T/ALS (for typical ALS). Since the diseases are all in the same spectrum, this would more clearly label them as a common disease while still being able to distinguish the different types. I don't think we can just call them all ALS, since they have different symptoms and prognoses--and yes, they may all end up in the same place, but not at the same time or at the same pace. But I think the differences are significant to the patient. Technically, I have been diagnosed as LMN predominant ALS (traditionally PMA). I know I will undoubtedly slide into typical ALS some day, but for right now I'd rather be a slowly progressive form of PMA than to be pure ALS. And I am sure there are others on the forum who would agree. So if people with T/ALS would rather have LMN/ALS, the two forms of the disease need different names.

 

[texastracy]

Please keep that appointment in June.

My first EMG was done by a doctor lots of sticks in arms and one leg. No contraction of the muscles. It was so easy. Result: you have carpal tunnel, with signs of some neurological problem of unknown origin.

Next one was done by the neuro that I see for seizures. He did a very thorough EMG of arms, legs, and neck. This one was not easy. I bruise easy plus I take aspirin daily. Plus I also had an MRI and spinal tap, and countless blood tests. Result: ALS in the differential diagnosis.

Then to the clinic in Dallas. That doc did an extremely thorough EMG with an extensive neuro eval. We spent 4 hours with Dr. Heitzman. He does all his patients EMGs and he is good at it. Final diagnosis. ALS.

The whole process started in July and ended in September. My symptoms started in my hands about two years ago. I can still walk, write legibly, and speak. I use a BiPap at night and am getting my power chair because I've had 10 falls in the last 9 months.