Probable PLS Diagnosis

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rmt

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My husband received a "probably PLS" diagnosis in July 2019 (from an ALS clinic in a major hospital). I've been lurking around the forum for a while but couldn't bring myself to post anything. But you are all so supportive and helpful, so I figured it was time.

Anyway, back to my husband. He is 70. His only symptoms are his speech and swalllowing. Late spring/early summer of 2018, we noticed he was speaking more slowly. By November 2018, he was slurring some, but not all the time. I'd say he speaks slowly and slurs occasionally. And his voice sounds different than before. He sounds a little nasal and hoarse. He had some mild swallowing issues mostly with liquids, but that seem to be much better now (or is he just more careful?).

Since November 2018, his symptoms haven't changed or gotten worse. He has no other symptoms besides his speech and the mild swallowing issue. He lifts weights almost every day and we walk a lot. He plays guitar. So his arms and legs seem fine. Oddly, he can chew gum for hours and never gets tired. He can whistle. So his jaw and face muscles seem fine.

He sounds the best in the morning and generally sounds a little worse by the end of the day. In a long conversation, he gets more nasal and hoarse and sometimes slurs more at the end. Then it recovers if he doesn't talk for a while. It always gets better over night, so each morning he starts at about the same speech quality.

When he was working, his speech would get SO bad by the end of the work day. Then it would recover over night. Each day of the work week, it would get a little worse. And then he would recover over the weekend. He is retired now, and generally doesn't have the giant swings in his speech that he had while working. Interestingly, after he works out, his speech is worse for a while and then recovers. And when he is stressed or excited, his speech is definitely worse. He sounds terrible at every doctor appointment and when we walk out the door he starts sounding better almost immediately. Not perfect, but back to his baseline.

He has had so many tests and everything came back normal. He had an EMG in April 2019 and it was totally normal. He had a laryngeal EMG in August 2019 and it was normal. MRI last fall was normal for age. Repeat MRI with contrast in October 2019 was normal for age. He tested negative for myasthenia gravis antibodies (AChR and MuSK) and Kennedy's disease. His FVC was 107% in July. No clinical weakness anywhere, including his face and tongue. One speech pathologist though his dysarthria sounded flaccid. Another thinks it sounds more ataxic. And the ALS specialist thinks it sounds spastic.

Since the EMG was normal, and the myasthenia antibodies were negative, the neuromuscular specialist thinks it is probably PLS. Yes, we know the statistics about the probability of progression to ALS. I spend a lot of time in a state of panic that it is going to progress to ALS.

Do any of you have such a variability in your symptoms throughout the day? Do you have any suggestions for things to do or avoid to keep things more consistent?

Thanks so much for any input you can give. As you know, this is all very overwhelming but I'm happy to have a place like this to come for support and insight.
 

lgelb

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I'm very sorry to hear of your husband's diagnosis, R. We will support you however possible.

It sounds like you've been through a few stops, but with what you describe, I'd make sure one of them has been another neuromuscular center, just to make doubly sure everything treatable has been ruled out.

Stable symptoms over a year would certainly make me hopeful that even if it is PLS, and even if it progresses to ALS, that there are some reasonably good years left. But since we say hope for the best and prepare for the worst, I would follow the usual paperwork processes/safeguards for terminal illness, including an advance directive that speaks to the possibility of the loss of mobility.

And though I could say a thousand things to point out the futility of worrying about progression, if you keep living your lives with enthusiasm, I think you will come to see that for yourself.

Best,
Laurie
 

rmt

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Thanks for your thoughts Laurie. You are definitely right about living with enthusiasm. I'm working really hard on doing that. My husband it actually better at not worrying than I am!

We have most of the legal stuff in place, but I hadn't thought about the advanced directive for loss of mobility. We are meeting with our Will/Living Will/Trust guy next week and I'll make sure to get that added. Thanks for that advice!

We have been to two neuromuscular clinics, both at major universities. There was some thought it could be a weird myasthenia presentation, but with no eye symptoms or antibodies, they ruled it out. And apparently his speech doesn't "sound" like myasthenia gravis patients. But we are hoping to try the myasthenia treatment (Pyridostigmine) on the slim chance it is that. The side effects seem to be not too bad. At least if we try it and it doesn't work, then we can stop wondering if we are missing something that might help.
 

lgelb

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I take it his brain was imaged/GI/respiratory tract examined? A small stroke, localized mass or aneurysm would be thoughts, likewise atypical Parkinson's.
 

rmt

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The MRI was of his brain and neck. They never suggested checking his GI or respiratory tract. They did the blood test for paraneoplastic and it came back negative.
 

KellyR30

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My husband received a "probably PLS" diagnosis in July 2019 (from an ALS clinic in a major hospital). I've been lurking around the forum for a while but couldn't bring myself to post anything. But you are all so supportive and helpful, so I figured it was time.

Anyway, back to my husband. He is 70. His only symptoms are his speech and swalllowing. Late spring/early summer of 2018, we noticed he was speaking more slowly. By November 2018, he was slurring some, but not all the time. I'd say he speaks slowly and slurs occasionally. And his voice sounds different than before. He sounds a little nasal and hoarse. He had some mild swallowing issues mostly with liquids, but that seem to be much better now (or is he just more careful?).

Since November 2018, his symptoms haven't changed or gotten worse. He has no other symptoms besides his speech and the mild swallowing issue. He lifts weights almost every day and we walk a lot. He plays guitar. So his arms and legs seem fine. Oddly, he can chew gum for hours and never gets tired. He can whistle. So his jaw and face muscles seem fine.

He sounds the best in the morning and generally sounds a little worse by the end of the day. In a long conversation, he gets more nasal and hoarse and sometimes slurs more at the end. Then it recovers if he doesn't talk for a while. It always gets better over night, so each morning he starts at about the same speech quality.

When he was working, his speech would get SO bad by the end of the work day. Then it would recover over night. Each day of the work week, it would get a little worse. And then he would recover over the weekend. He is retired now, and generally doesn't have the giant swings in his speech that he had while working. Interestingly, after he works out, his speech is worse for a while and then recovers. And when he is stressed or excited, his speech is definitely worse. He sounds terrible at every doctor appointment and when we walk out the door he starts sounding better almost immediately. Not perfect, but back to his baseline.

He has had so many tests and everything came back normal. He had an EMG in April 2019 and it was totally normal. He had a laryngeal EMG in August 2019 and it was normal. MRI last fall was normal for age. Repeat MRI with contrast in October 2019 was normal for age. He tested negative for myasthenia gravis antibodies (AChR and MuSK) and Kennedy's disease. His FVC was 107% in July. No clinical weakness anywhere, including his face and tongue. One speech pathologist though his dysarthria sounded flaccid. Another thinks it sounds more ataxic. And the ALS specialist thinks it sounds spastic.

Since the EMG was normal, and the myasthenia antibodies were negative, the neuromuscular specialist thinks it is probably PLS. Yes, we know the statistics about the probability of progression to ALS. I spend a lot of time in a state of panic that it is going to progress to ALS.

Do any of you have such a variability in your symptoms throughout the day? Do you have any suggestions for things to do or avoid to keep things more consistent?

Thanks so much for any input you can give. As you know, this is all very overwhelming but I'm happy to have a place like this to come for support and insight.


There is serronegative myasthenia (meaning no positive antibodies show up).
I recommend you join a Facebook group called “myasthenia gravis” (photo attached) and ask questions there. They are amazing and can help you! Speech that improves with rest is typical with mg. They can help you in finding a mg specialist . A trial of the drug mestinon wouldn’t harm you. You would know quickly if its going to help. Good luck!
 

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rmt

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CALS
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07/2019
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Seattle
Thanks so much for the idea of looking for myasthenia group on Facebook. I'm definitely going to do that! And we are trying to contact his doctor to try the mestinon. Seems like it is worth a shot! I'll let you know how it goes!
 

rmt

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Well, he tried mestinon and it didn't help his speech at all but it did give him bladder spasms and a few other uncomfortable side effects. Needless to say, that experiment is now over. It was a long shot that he had MG, but still disappointing to not have something that could possibly treated by a few pills a day.

We feel like we have exhausted all the treatable things that could be causing his speech issue. And somehow I'm finding peace in that. Like after chasing a fix for the last year and a half, now I can stop looking for the magical thing that will make it all better and we can just start enjoying life again.
 

lgelb

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I'm sorry the mestinon trial didn't pan out. As you say, life is short for anyone and we can only live to what is fullest for us.
 
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