Is dysarthria an upper motor neuron sign?

rmt

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Is spastic dysarthia an upper motor neuron sign? Or do you have to have actual muscle spasticity or other upper motor neuron signs (pseudobulbar affect, Hoffman, Babinski, etc)?

The reason I ask is I'm trying to figure out what upper motor neuron signs (if any) that my husband has. He has a diagnosis of Probable PLS from a major ALS clinic in July 2019 with speech issues since May 2018.

He had a second opinion on Monday at a different ALS clinic with another ALS specialist. The Fellow did a very thorough exam and seemed very thoughtful and involved in the specifics of my husbands symptoms/timeline. We are at 21 months and his only symptom is spastic/ataxic dysarthria. No muscle spasticity or weakness anywhere. No brisk reflexes (the fellow said his jaw jerk was "maybe a little brisk", but he didn't actually seem convinced). No pseudobulbar affect.

The senior ALS doctor came in and flat out said it is definitely ALS (he says he doesn't diagnose PLS). Nothing else could possibly present this way since we've ruled out all the mimics. He didn't care that it has been 21 months with nothing but the dysarthria and that he had a clean EMG last year. He didn't care that at times his speech is better (not perfect but significantly better and not just better in the morning). He spent a few minutes checking him over and then it was all about how if they could find some lower motor neuron signs they could try to get him into a trial.

So they did a repeat NCS and EMG and both were totally normal. Even his tongue. The fellow even brought in an attending because he wanted somebody to verify everything was normal. We were so relieved because the doctor made it sound like it was a sure thing that the EMG would be abnormal.

So he has no lower motor neuron signs. Is the dysarthria by itself an upper motor neuron sign? Even if there are no signs of actual muscle spasticity?

Has anybody heard of either ALS or PLS presenting like this with absolutely no other symptoms than dysarthria for so long?

Maybe I'm just in denial, but from everything I've read, this seems very atypical. The Fellow pulled us aside as we were leaving and said he had never seen anybody progress this slowly.

I've always wondered if there is a functional speech disorder component to his dysarthria. But then I think that if I'm wrong then I'm a total jerk for even thinking that!
 

KarenNWendyn

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I don’t see how they could diagnose ALS with the clean EMG, especially with the bulbar region being clean. PLS maybe. I think spastic dysarthria is an upper motor neuron sign. Is his speech problem progressing?
 

lgelb

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Spastic dysarthria, if that's really what it is (dysarthria subtypes can be confused, and ataxic is distinct from spastic, so you can't mush them together) is most often due to a stroke [could be silent], brain injury (any falls or other trauma?) or tumor [the most likely non-MND explanation if there has been significant progression]. Then there is a whole 'nother list for the ataxic subtype, and each has different symptoms apart from speech, that you haven't mentioned.

Has his brain been MRI'd at an academic medical center? Even if it were PLS, the waxing/waning seemingly doesn't fit. The nerves die or they don't.

I'd go to the general neuro/stroke service at the institutions connected with whichever ALS clinic(s) you haven't been to: UW, Virginia Mason or Swedish, and try for a high-end MRI if they agree it's indicated and he hasn't had one (different units use some different post-processing) with a distinct lesion as the primary differential. Some potentially dire differentials should serve, with the right justification, as sufficient impetus for your plan to reimburse it, if there is no recent study. If there is, you might want to have a neuroradiologist re-analyze it, just to cut the cards.

There's another testable hypothesis here, that he could benefit from speech therapy, as in the silent stroke scenario. If he did, that would obviously argue against an MND (and some other dx) as well as providing benefit. So I'd try to get an order for that, too, unless already tried -- each of these centers have such a department, and an evaluation comes along with it, from an SLP who may have more experience than the neuros you've seen with what speech deficits are associated with varied dx.

Best,
Laurie
 

rmt

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I was shocked on Monday when the doctor was so nonchalant about diagnosing him with ALS! He said my husband has no weakness, atrophy, hyper-reflexes, etc and then jumped right into how it was definitely ALS! My jaw hit the floor! And then when the EMG was clean, nope, still definitely ALS. I was blown away.

I would describe his progression as, his worst isn't any worse than it ever was, but his best isn't as good as it once was. Does that make sense? He isn't any worse than his "bad days" in 2018, but other than a few isolated incidents recently over the holidays (when he was really happy and relaxed), his best days now aren't as good as his best days in 2018. Since this all started, his speech quality has always fluctuated greatly.

He had a non-contrast MRI in December 2018 and a contrast MRI in October 2019 at an academic medical center. Both were normal. He hasn't had any falls. He had afib that started just before his speech started slowing down in May 2018. And he had a successful afib ablation in October 2018, about 2 weeks before the slurring started suddenly. The slurring came on during a road trip, so I remember it vividly because I thought he was having a stoke! Which definitely could happen after the afib ablation! They didn't do the MRI until about 6 weeks later (he refused to go to the hospital right away, and I'm so mad at myself for not making him go immediately!). He had another "episode" in January 2019 where suddenly after a coughing spell he couldn't speak at all for a few hours. I think it is certainly possible there was a small stroke then too. But the second MRI (9 months after the episode) didn't show anything.

He has done some speech therapy, and we both think it helps. It certainly has helped his palate strength, which was the one thing the ENT found last summer (mild palate weakness as she called it). When he started, it was like he couldn't remember how to stop air from coming out his nose when he whistled, etc. But within a few days, he could do all the exercises and can whistle again. That doesn't sound very PLS/ALS-like, right?

I was thinking it was probably PLS after that diagnosis last summer. But after this latest visit, clean EMG and some of the other atypical symptom waxing/waning, I'm not so sure. I will say that neither of the ALS doctors cared at all about the sudden onset of the actual slurring or the episode in January 2019 where he couldn't talk at all for several hours. I almost wish we hadn't mentioned that his speech was a little slower for months before the slurring started because that is all they cared about.
 

KarenNWendyn

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I think your concerns are justified. The sudden onset after an A fib procedure definitely suggests a vascular event. And the course isn’t at all suggestive of a motor neuron disease. Definitely seek another opinion.
 

rmt

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Thank you so much for taking the time to give me your advice. ALS never seemed to fit for me but I always worried I was just in denial. But the longer this goes on, the less crazy I feel for thinking something else is going on. I thought the ALS specialist on Monday was going to help figure it out, but apparently not. We will keep digging...
 

Jleblanc

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Hi. I am in a similar situation. I have some mild tongue and facial weakness and dysphasia with moderate dysarthria. This started 10 months ago, got worse this January and has stayed the same since. My EMG on March 9th was also normal. And I was diagnosed with ALS. I don’t know if I’m in denial either. Maybe I do have ALS but, with the exception of talking, I can still do everything I could do before this started. I hope you get answers. I intend to keep looking as well.
 

Nikki J

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Jleblanc sorry you had to find us. I hope that you have had or are having 2 neuromuscular opinions. With your location they should be UMass and Mass General ALS clinics . I realize it is difficult right now to get appointments but those would be the places to be seen. If you have not gone to either I would set up appointments with both ( if possible)
 

Jleblanc

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Jleblanc sorry you had to find us. I hope that you have had or are having 2 neuromuscular opinions. With your location they should be UMass and Mass General ALS clinics . I realize it is difficult right now to get appointments but those would be the places to be seen. If you have not gone to either I would set up appointments with both ( if possible)
Hi Nikki,
Thank you. I am a current patient at MGH’s Healey Center. I have only seen one specialist. But I will be seeking a second opinion. My regular neurologist was encouraged by the results of the EMG. The specialist believes I do have it. I want to be sure. And I know it can be tricky to diagnose.

Hopefully, we will soon have COVID-19 under control so all of us can get back with our physicians. I have a “virtual visit” next week. Should be interesting!
 

Nikki J

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I am glad you are at mgh. I go there too. I would still go to UMass for another opinion
 
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