rmt
Distinguished member
- Joined
- Sep 18, 2019
- Messages
- 379
- Reason
- Lost a loved one
- Diagnosis
- 07/2019
- Country
- US
- State
- WA
- City
- Seattle
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!
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!