Old 04-19-2017, 02:37 PM #1 (permalink)
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Default Diagnosed Lower Motor Neuron Disease syndrome - is this ALS

Welcome,

I've been on the forum here some time ago asking about my case. Until now I'm pretty much confident I have Benign Fasciculation Syndrome, Idiopathic Fasciculations, you name it. (I never self-diagnosed ALS though, it was a concern of my neurologist, as I had spontaneous activity, namely PSW and fibrillations on EMG) This post is not about me. I'm completely in peace and not looking for any ALS symptoms, but all the research sticked to back of my head and I've met a person with clear Motor Neuron Disease but pure accident.
I've moved back to my home country - Poland. Poland doesn't have dedicated ALS centers, only few neuromuscular neurologist with ALS speciality.
The person I'm talking about first developed weakness in arms 4 years ago, EMG is pretty conclusive in that area with typical pattern of active and chronic denervation and polyphasic MUAP. All other areas however were very inconclusive, bulbar is clean from a start to current date, both emg and symptomatic wise. Weakness in arms have progressed, there is clear atrophy, walking is impaired but proximally, there is not foot drop. One of the best Polish ALS neurologist confirmed lack of UMN involvement and sustain MND diagnosis. He said it may as well be progressive over decades and advised to check for SMBA. He rejects MMN on basis that there is no conduction block and he won't recommend trying IGIV, even where there is clear evidence on NCV on all motor nerves conduction change
Clinically weakness and atrophy is obvious, but progression rate is amazingly slow and lack of UPM puzzles as all, as LMN syndromes are described as rather rapidly progressing and going into full blaze ALS sooner or later
If you have any suggestions that can help my friend please advice, I'm writing in his name as he doesn't speak English

Best regards

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Old 04-19-2017, 03:22 PM #2 (permalink)
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Default Re: Diagnosed Lower Motor Neuron Disease syndrome - is this ALS

Hi, Banjanti, not sure where you read that LMN-only disease progresses more rapidly. For example, flail arm/leg subtypes progress more slowly, bulbar involvement (even on EMG) is usually later than sooner, and both typically present only with LMN signs.

SBMA is more likely to present with bulbar signs but it is another slow form of LMN-only disease, to be sure. I would think the specific signs would be examined for, such as testicular atrophy, breast enlargement, but only a genetic test is certain for that.

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Old 04-19-2017, 03:35 PM #3 (permalink)
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Default Re: Diagnosed Lower Motor Neuron Disease syndrome - is this ALS

Thank you for your opinion, I always value it. I've must made the mistake thinking of disorders like PMA that is said to have 33% survival rate in 5 years, flail arm would fit perfectly besides I though UMN involvement is seen and that differents it from pure LMN syndromes
SMBA is easy genetic test and they are doing it right now, but i agree no bulbar involvement is not consistent
Do you have empirical knowledge when bulbar in this type of progression can be seen or this is purely individualistic?
Gentleman is doing very well for 4 years into it, still fully functional, cannot walk too far, and do anything required dexterity due thenar weaknes and atrophy, his arms are affected as well but other than that he still enjoys fair quality of life
As progression rate puzzles everybody they are trying to determine what to expect in those circumstances
New throughout emg is coming soon and i guess we can deduct something from it

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Old 04-19-2017, 05:17 PM #4 (permalink)
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Default Re: Diagnosed Lower Motor Neuron Disease syndrome - is this ALS

PMA is a hotly-disputed entity and not universally defined, but even 33% survival over 5y would be better than what we think we see for ALS (~20%), so my statement was not inconsistent with that.
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Old 04-19-2017, 05:56 PM #5 (permalink)
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Default Re: Diagnosed Lower Motor Neuron Disease syndrome - is this ALS

My husband remains very Lower Motor dominant, to the extent that even almost ten months since diagnosis it seems to be in question that he has any upper Motor signs. MNDs with this pattern usually progress more slowly, not less per our Doctor. My husband is still walking talking driving and eating with only a cane for assistance and a special knife to accommodate some hand weakness (which is made worse by some non ALS issues with his hands). His first symptom was drop foot.
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Old 04-20-2017, 12:57 AM #6 (permalink)
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Default Re: Diagnosed Lower Motor Neuron Disease syndrome - is this ALS

Laurie - obviously you're right, it has better survival rate in 5 years window, my point was he's at his 4 years with very little progression so he's luckily looking for more then that and this seems pretty rare
Lenore - thank you for your comment. This is very comforting, it seem that experience wise lack of umn involvement really means better prognosis. There are no umn signs in that case 4 years into disease, looks remarkable and seems good news indeed
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Old 04-20-2017, 01:08 AM #7 (permalink)
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Default Re: Diagnosed Lower Motor Neuron Disease syndrome - is this ALS

None of us can see the future. UMN signs can develop at any time, as can bulbar signs, just as longstanding UMN-only disease (PLS) can progress to ALS.

But yes, we do know that some people with LMN-dominant disease do not develop bulbar signs in their lifetime.

As with all MND, he should hope for the best, plan for something less.

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Old 04-20-2017, 02:45 AM #8 (permalink)
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Default Re: Diagnosed Lower Motor Neuron Disease syndrome - is this ALS

This makes a lot of sense, thank you again for your opinion.
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