"Most likely" ALS

[EricInLA]

Hello all,

I posted this past October, worried about symptoms I have been having over the past 18 months that might suggest ALS.

My symptoms were nonstop fasciclations, concentrated mostly in the arms, but in other places too, plus some odd weaknesses in hands and fingers that seemed to arrive in the last few months only. The consensus on this forum was that it didn't sound like ALS. The post is no longer open, so if someone can bring it back here, I'd appreciate it.

As I reported in that post, I left off in October, with my neurologist here in LA telling me it was doubtful that this was ALS because of long duration with minimal progression, strong clinical exam, and nonfocal fasciculations.

To bring you up to date. . . I went back for a follow-up visit in November, and after my clinical exam he expressed concerned that my flexor finger muscles had deteriorated just a little bit, enough to warrant: 1) a repeat EMG test (my original one in May, 2018 hadn't been "clean" but suggested ulnar neuropathy and/or radiculopathy, but left the door open to MND if things got worse), and 2) that I go get a second-opinion from a neuro-muscular specialist in San Francisco. So I did the EMG test again, and it was simlar to the first, but showed that I have chronic denervation in my right hand/arm that is not limited to the ulnar nerve. The other areas of the body were clean.

The visit to the neuro-muscular specialist happened yesterday, and did not go well. He believes it is likely that I have ALS, because he couldn't make an argument for any other cause for what's going on with me. He says radiculopathy is probably out because my MRI, though it showed narrowing/stenosis and disc protrusions, indicated NO cord compression. Also I don't have pain or numbness, which would typically go with radiculopathy. He says the only outside shot of this not being ALS is to re-do the neck MRI to see if it's got worse over the past 20 months, but again, no pain, no numbness, so it's doubtful.

Needles to say, his diagnosis was a sucker punch to me and my wife. We just didn't see it coming, based on my local neurologist feeling relatively good about me. The specialist admits it's not a slam dunk case because my progression is so slow (20 months in, and no real functional failures, though I do have a little weakness in some fingers that I notice, and some mild hyperreflexivity, of course the incessant fascics), but he says that does happen in some cases, and he didn't want to leave me with hope that it's something else. And he's the expert. He thinks it's a strong enough case to warrant the immediate commencement of the two 'R' drugs for ALS. Said something about maybe a new oral way to take the Radicava. Also said my slow progression indicates it will continue to progress slow (which is good news), and suggests a longer lifespan that usual for this disease.

I don't know want else to say, or what help to ask for, we are just in a fog. I don't even know if this is the right area to post - does this go to "Newly Diagnosed"? He did say he is going to write down "ALS" on my report so I guess that's a diagnosis. I will go check in with my local ALS group at Cedars Sinai, and I guess look at clinical trials. Any advice is welcomed and appreciated from this helpful community. As you all know, when someone gives you this news, it's nothing less than devastating. Couldn't sleep even WITH my friend Adivan.

 

[Nikki J]

Probably on the eve of diagnosis. . .

Hello folks, I am a 49-year old male in Southern California, living with the fear that I have ALS. I know this is a familiar trope, because I have spent many months reading posts on this support forum. This is the first time I am posting. Let me say first that I'm so impressed with the level...

www.alsforums.com

I am sorry you were told this. I am confused because your emg doesn’t sound diagnostic. ALS isn’t diagnosed by saying well it isn’t this or that so it must be ALS without having an emg and other things to back it up. I would seek another neuromuscular opinion.

around here the insurances are extremely strict about radicava. They require, among other things, El Escorial criteria diagnosis which you are not describing.

may I ask what institution this doctor is at? His name?

i don’t know. Maybe you do have ALS but from what you have said the evidence isn’t there yet and I wouldn’t be comfortable accepting this without consulting another neuromuscular specialist

 

[KarenNWendyn]

I’m also sorry to hear this. It’s never easy to get that kind of news. But I agree with Nikki that it doesn’t sound like a slam dunk diagnosis.

What I said to another poster here also applies in your situation —All that we on this forum can do is render our opinion. It’s hard when we don’t have the privilege of seeing you, having access to all your data, or being able to have a conversation with your neurologist. I wish you the best and hope things work out for you as best they can.

In any event, make the most of what you can still do, and it sounds like you are nearly fully functional. I hope it remains that way for a long, long time. Sleep will get easier.

 

[EricInLA]

Thank you both for your quick replies. I saw Dr. Jonathan Katz, at Farber-Norris/Sutter Health in SF. I did inquire about El Escorial, and he admits it doesn't meet the "definite" standard, but instead would be something like "suspected" or "possible" ALS, but for practical purposes he believes it's by far the most likely scenario. He did also suggest I should go see the local ALS experts at Cedars-Sinai, which is close to my home, so of course I will do that. I hope Blue Shield in CA will allow Radicava, but I haven't quite gotten there yet. There is all fresh and raw and sad. Thanks for the good thoughts and the word on sleep.

 

[Nikki J]

I am glad you are going to Cedars and hope you get there soon. They actually rediagnosed ( apparently correctly) someone I know who had received an ALS diagnosis from another ALS center ( not the one you attended)

good luck keep us posted

 

[EricInLA]

Hi folks - I went for another consultation today with the ALS specialist at UCLA. The doctor did a full clinical exam and reviewed all my test reports. Then she began using the word "unfortunately" a lot which was not a good sign. Basically, she is concurring with Dr. Katz in San Francisco; namely, this is "most likely" ALS even though I don't meet the El Escorial criteria yet. She based her decision on roughly the same grounds as Dr. Katz - evidence from my EMG combined with hyperreflexivity that can't be explained any other way. I thought that because my hyperreflexivity was symmetrical, it wasn't clinically significant, but she says that isn't the case and that ALS often presents with symmetrical hyperreflexes. I also received the results of my second cervical spine MRI yesterday and sure enough it's not showing any interruption in spinal cord signal so that can't explain my symptoms. I quizzed her on the mimics that I had hoped for and she explained why those aren't presenting. I pinned her down on whether she was actually giving me a firm diagnosis, and she says it is a "tentative diagnosis" but she was clear that I shouldn't have false hope. I have to say, however, she did so in a very compassionate and caring manner, which was nice. She did say it was a "diagnosis" for purposes of applying for benefits, etc... My wife and I had already mentally digested this with the last doctor, so we weren't falling to pieces like last time. Very tough nonetheless. I'm still going to go for a (3rd) opinion at Cedars as that is where my care will be, but I'm trying to be sober and realistic about this. She said it was good that I was already using Riluzole -- she was less enthusiastic about Radicava.

Well, I didn't want to be a member of this club, but here I am. From all my browsing I know how empathetic and helpful (and funny) the people on this forum are, which I am already grateful for. I'm sure I will seek your guidance many times on this journey, and will try to help others in any way I can.

Eric

 

[KarenNWendyn]

I’m sorry about the news. What’s confusing to me is that a “tentative” ALS diagnosis might qualify you for SSD. When I was diagnosed in 2017, I was told that only a definite diagnosis qualifies for SSD and Medicare in terms of ALS.

In any event, I realize those particulars are less important than whether or not your motor neurons are dying. Assuming that’s the case, I wish you a very slow course with function preserved for a long time.

I definitely recommend that 3rd opinion at Cedars. Hoping for the best.

 

[Clearwater AL]

To receive SSDI benefits the Neurologist must submit the Diagnostic Code for ALS
and his/her records/summary/conclusion for review/verification.

Fortunately, ALS (with the Diagnostic Code) moves through SS much faster than other
disability applications.

 

[lgelb]

Eric, can you post your latest EMG with identifiers removed? If it is being used as Exhibit A, given the atypical onset/progression you describe, it would be helpful to those of us who remain bemused at your second "probable" dx. I don't want to offer any false hope, of course.

I will say, for general reference, that radiculopathy, essentially pinched nerve roots, is a different condition than cord compression, which is myelopathy. So if someone said, "You can't have radiculopathy because there's no cord compression," that would not make sense. Maybe he was conflating the two for simplicity.

Neither normal signal on MRI nor your lack of pain rule out nerve root damage (radiculopathy). The normal signal rules out severe cord compression. It is the EMG that would speak to a possible neuronopathy as ALS is.

I take it your PCP or someone did lab work/examined you to eliminate metabolic/endocrine disorders and kidney issues?

Best,
Laurie

 

[EricInLA]

Thanks for your responses. I did ask the Neurologist today specifically if her "tentative" diagnosis would be sufficient to apply for benefits, and she said yes, which maybe means she's going to leave off the word "tentative"? Anyway, it's good to know about that diagnostic code. (Before today I had no idea that ALS would qualify me for Medicare. I'm 49 so Medicare was the furthest thing from my mind.)

Laurie - I'm happy to post the EMG - I will redact the identifiers and post tomorrow. I'm curious to hear feedback. You make a good point about the cord compression and pinched nerve roots. I was wondering about that today and asked the Doctor about that exactly. She felt the pinched nerve root theory was unlikely because of my hyperreflexiveness (even though it's symmetrical), and because fasciculations are much more common with ALS than pinched nerves. I did have labs taken more than once - offhand I don't know about the items you mention but I will inquire. I gotta imagine they checked for everything but I will make sure.

 

[Nikki J]

Eric I am sorry about all of this. However it turns out this is obviously a horrible time.

you do know, even with a diagnosis ssdi/ medicare is only available after you stop working? maybe you have already stopped? There is a thread in resources in the general section about the process

 

[Kristina1]

Sorry to hear about your diagnosis, Eric. The wider forum is great for support and questions you might have along the way. Welcome <3

 

[Clearwater AL]

Eric, some Neurologists are not familiar with the ridge rules SS has for granting SSDI benefits.
They (SS) very much employ "CYA" (in this circumstance replace the
"Y" with "T" being the word "their" A$$es. Disability benefit eligible applications
are under attack now by political forces. Lawyers get involved ... well, 'll leave it at that.

If you get a chance politely ask your Neuro if he/she will be using the designated
code for confirmed ALS. If not be patient.... it may take longer.

Wishing the best for you.

PS. Are you a veteran.... ever served? If so, contact the PVA (Paralyzed Veterans of
America). They helped my case sail through. They specialize in ALS.

Sorry for all the edits,,, I'm having difficulty composing now. Change the word "case"
above to "benefits" Maybe I should just quit.

 

[EricInLA]

Thank you all for your responses. Al - Do not quit! Edits are fine. It is interesting, though not surprising, that the disability stuff is getting political. I am a "recovering lawyer" myself, very happy not to be practicing any more. I am not a veteran, so that avenue is not open to me. Also, I am still working, so I'll have to consider my options re Medicare. I have my own business these days, so maybe I'll have to become "semi-retired".

 

[KarenNWendyn]

Eric, if you are able to work and enjoy it, I suggest you continue to do it as long as you can. You might make more money than you would on SSD. That assumes you also have health insurance because ALS is one helluva preexisting condition.