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Emanol

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Hello,

I am writing to you to ask for help understanding the last report from my father's neurologist. He is 60 year-old men who was previously diagnosed with PLS (three years of symptoms). I am interested in knowing if the new report suggests to you that the diagnosis might change to ALS. I will be translating from Spanish:

"Clinical signs are of Primary Lateral Sclerosis but recently he has clinical signs of Lower Motor Neuron involvement on three levels: bulbar (espastic dysphagia) cervical (very mild skeletal muscle atrophy on upper limbs) and lumbosacral (weakness flexing the muscle). No tongue atrophy or fasciculations."

What do people think? Does this indicate lower motor neron involvement? I thought that espastic dysphagia was a sign of Upper rather than Lower involvement...

Also, in case this is useful: FVC (force vital capacity)102%
 
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KimT

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It would be helpful if he had an EMG and we could see the report.
 

lgelb

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Assuming you mean spastic dysphagia, that is not really a specific term and the issues in swallowing may arise from the throat or esophagus. In the case of difficulty flexing the muscle, the issue for UMN vs. LMN dysfunction would come down to tone (high vs. low) and other characteristics. Without seeing your dad, therefore, it will be hard for us to say. As you know, UMN weakness can present with mild atrophy so that is not telling, either.

If he is anxious to know, he could have another EMG to see if signs of LMN dysfunction are present, but at this point since any LMN involvement is evidently mild, it is hard to see what he would do with this information. If he has ALS, the LMN involvement will become evident over time. Until then, why not proceed as if he has PLS?

Best,
Laurie
 

Emanol

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Assuming you mean spastic dysphagia, that is not really a specific term and the issues in swallowing may arise from the throat or esophagus. In the case of difficulty flexing the muscle, the issue for UMN vs. LMN dysfunction would come down to tone (high vs. low) and other characteristics. Without seeing your dad, therefore, it will be hard for us to say. As you know, UMN weakness can present with mild atrophy so that is not telling, either.

If he is anxious to know, he could have another EMG to see if signs of LMN dysfunction are present, but at this point since any LMN involvement is evidently mild, it is hard to see what he would do with this information. If he has ALS, the LMN involvement will become evident over time. Until then, why not proceed as if he has PLS?

Best,
Laurie
Thank you so so much. That was so extremely helpful.

For me, one of the issues is that I am not sure our neurologist has much experience with PLS. For instance, she proscribed Riluzole and took out Amantadine, and since then my father's spasticity has worsened considerably. I feel that she is treating him as a "general MND case," without giving him specific meds that could be useful for his spasticity, such as Baclofen.

I have asked my father to see someone else but he is reluctant and things in Spain move at a glacial pace... I hope I can convince him to see someone after Christmas.

Again, thank you a lot

E
 

lgelb

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Amantadine is less often used for UMN spasticity than baclofen and tizanidine, but I agree he deserves to be treated for it. Tizanidine is better tolerated for some, apart from potential caffeine interaction.
 

Emanol

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Thank you. The reason my father was taking Amantadine is that he was previously misdiagnosed with atypical Parkinsonism. However, since the diagnosis changed our neuro took out Amantadine with nothing to replace it with, and my father has suffered the consequences in the form of a much more pronounced spasticity.
 

ShiftKicker

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Oh, yikes. Your poor dad. Baclofen seems to be very effective for lots of people. Tizanidine/Zanaflex is also an excellent option if Baclofen doesn't suit. Those aren't the only options, but they are the most common. If his neuro is not willing to treat your dad's biggest symptom, another doctor might be in order. Ridiculous to withdraw meds for a wrong diagnosis and not consider comfort or symptom mitigation for his new diagnosis.

Your dad's doctor needs to be proactive and work through your dad's symptoms to relieve them as best as possible. Reducing spasticity can improve quality of life- allow for better mobility, maybe even allow for some exercise and stretching, improve quality of sleep and reduce fatigue. Spasticity, even when mild, is insidious and can create serious fatigue because he has to fight every movement, even chewing, if his jaw is spastic.

From what you reported in your original post in this thread, it sounds like a clinical exam has turned up possible lower motor signs. Will they be conducting another emg to confirm this? Some slower progressing forms of ALS do appear as PLS for the first few years. While the standard seems to be a PLS diagnosis after 4 or 5 years of UMN only signs, sometimes lower motor signs do develop outside of this time frame to make ALS a more appropriate diagnosis. As Laurie says though, it doesn't change the treatment- which the doctor seems a bit behind on dealing with no matter what the eventual diagnosis. But sometimes people find comfort in knowing for sure- so if your dad might find not knowing stressful, an emg is important.
 

Emanol

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Thank you very much. We will contact the neuro today and see how things go. I very much hope she can give him something for the spasticity, although I am also concerned about the side effects since my dad is very sensitive to medication.
 

Emanol

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Also, I would appreciate if you help me evaluate the results of his breathing test (again, I'm using the Spanish version but I hope it will be understandable to those who are used to seeing these things)

Sat. basal 98%
FVC 102%
PIM -102cm of water (86%)
Basal oximetries: medium basal around 93.5%, CT90 3-4%,
IDA 16-17/H

Thanks,
E.
 

Emanol

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Sorry, I meant spastic dysarthria, not dysphagia.

Something I just found out looking over my dad's medical history: the doctor said that "very mild skeletal muscle atrophy on upper limbs" was a new symptom. However, the same symptom was already noticed in December 2017, the same month he did an EMG that cleared him of LMN damage... so still hope for lack of LMN symptoms I think.

I called the doctor and she basically told me that it did not make sense to prescribe Baclofen because the dizziness would offset the advantages. She said that Baclofen and Tizanidine barely do anything to help and can make walking more difficult because of increased dizziness and weakness. We will be seeing another doctor on January 4th.

Also, I apologize if it is a bit annoying to hear me debate out loud with myself. I am very thankful that this group provides with a supportive space during these times of uncertainty.

Best,
E.
 
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Nikki J

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I am glad you are seeing another doctor for an opinion. Many find those meds useful.

Weakness from them is really if improving spasticity unmasks weakness A little spasticity can actually be useful. This was the case with my sister who had ALS and both significant spasticity and emg confirmed lower motor neuron disease and weakness. She found no baclofen during the day and full dose at bedtime worked for her while she walked then she started taking baclofen in the day. She definitely found it helped
 

ShiftKicker

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Please don't hesitate to work things out via the forum like this. It's exactly what this forum is for.

I have to admit, I am frustrated with your dad's doctor- I can only imagine how you feel. As Nikki says above, yes, these drugs can sometimes unmask LMN weakness once the spasticity is reduced, but that's no reason not to give them a try. Your dad's doctor is incredibly conservative. Your dad's comfort is very important. In fact, it's paramount.

Spasticity could be causing all manner of subtle issues- poor sleep, fatigue, incoordination, etc. Even a mild reduction in spasticity could help him maintain better fitness and cardio health- which has profound health benefits all across the board. A reduction in spasticity at night could benefit his sleep and help him better manage his days. A decent sleep improves resilience and allows for better stress management. Muscles strained by constant spasticity can get sore, reduce mobility, are more fatigued, less responsive- a whole night of muscle rest could profoundly improve your dad's ability to move around the following day and definitely prolong mobility. And so on. As Nikki says, just taking Baclofen at night can help even if he doesn't take it during the day.

It may be hard, given your medical system (I empathize- I come from Canada, and we have a similar medical "culture"), but I urge you to push his doctor to actively treat his spasticity, or maybe find another doctor. Discarding a treatment because it might cause weakness, when there is a more likely larger benefit to improving his quality of life seems inappropriate. However, perhaps your dad's doctor has more information about your dad than what I am reading about here.
 

Emanol

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Hello everyone. I have some updates:

Out of my father's request, we did a third EMG. There is no acute denervation but there are some abnormalities. There were no fib/psw and amplitude/duration/PP were within the bounds of normality, but there were fasciculations in different regions. Given my father's symptoms, the doctor suggested that it could indicate mild LMN damage. I was reassured that it did not look too serious for now, though, especially since some landmark clinical symptoms such as weight loss have not appeared in 3 years we've been dealing with this.

For what I read, it's as if he would not fit El Escorial criteria but might fit the Awaji criteria, where fasciculations can be used as evidence of active denervation instead of fib/psw.

I also consulted with another neurologist. He similarly counseled against Baclofen for similar reasons: he is afraid that Baclofen might increase weakness and reduce spasticity. We will seek another opinion and attend a specialized MND center rather than individual neurologists next time. For now, my dad seems to have less discomfort at night but his walk is still very rigid.
 
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lgelb

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There is always a tradeoff between spasticity and weakness, since drugs of the baclofen ilk relax muscles and reduce tone, making them harder to mobilize. Your dad is the one best equipped to make that tradeoff.

Sometimes drugs that are more generally sedating are used in low doses, e.g. benzodiazepines, as they can be more easily titrated as you go. CBD is also an option to consider.
 
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