PMA Muscle Behaviour

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Neutrino

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Hey All,

Well, 6 months and I'm back :) I won't take up too much time....

Myotonic dystrophy was a red herring unfortunately. So, back to neuro, appointment is still another 2 months away.

In the interim though, things have become more focused. Main symptoms now are just the progressive weakness, atrophy, cramps and fasiculations. I'm now starting not to be able to do things/certain actions/range of movements etc.

Uro-neurologist recently brought up PMA/SMA again on a follow up, so im waiting on genetics for SMA, and kennedys, as unfortuantly these where missed out on the last genetic panel I had done. They are organising another EMG to see if it is not so borderline now for neurogenic changes.

Anyway, reason for popping back in, is the weird muscle behaviour they thought was myotonia, turns out it isn't. I have linked a short video below, hoping to see if anyone has seen anything like this before? It was a smaller part of the hand before, but as the wider atrophy has worsened, so has this. Its now starting on the right hand and feet.

Originally it would just tense the muscle and take about 5-10 seconds for muscle to return to normal. Now it is pretty random how the muscle behaves. Somebody did mention denervation can sometimes look like this....

But thought I'd see if anyone with primarily lower neuron signs (to date i only have clonus as a upper sign) have experienced this.

Thanks all.

 

Nikki J

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Previous thread Atypical Parkinson's like ALS

I am very much lower motor dominant. I have not had that happen nor have I seen it in family members. Were I to flex my wrist as you did my fingers do curl but it is from weakness and they can never straighten until I return my wrist to neutral
 

Bestfriends14

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My husband is mostly lower motor dominant and I have never seen this in him. For what it is worth, I do not see atrophy. I see a hand and fingers that move normally.
 

Neutrino

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Thanks both. It's a weird one I know. I'll see what the neuro thinks of it when I see them. But its good to know its not something obvious.

Yes, on both sides my fingers can't straighten now when wrist flexed, and they are naturally bent over like I'm trying to grab something when resting now.

The atrophy on the hands is mainly the muscles around the pincer grip side, weirdly opposite side of the hand, the muscle seems fine, bulk wise, even with that weird behaviour. Also, suspect its why you all say pictures are rubbish for showing it! 🙂
 

Nikki J

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Confused about your statement fingers can’t straighten when your wrist is bent as they appear to do so in the video at about 20 seconds but as we said before your case is complicated hope you get an answer soon
 

Neutrino

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Sorry, your right, should have read "all fingers fully", as opposed to not at all. As with all my effort, like in the video I can get them most of the way there. Except the little finger, but maybe that has to do with whatever is transpiring with those side muscles.

Thanks again.
 

Neutrino

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Just a update. Had 6 month follow up today (got a call this morning for a cancelation space opening up) and seems unfortunately it is motor neurone disease.

As well as the fasiculations/cramps/atrophy. There is also now brisk reflexes, spasticity, and more widespread clinical weakness. So based on the clinical exam he stated we are more clear cut looking at MND.

Have been booked in for a follow up EMG at oxford next Wednesday. The comment was he would be surprised if its normal now based on clinical exam.

Have also been referred to the MMD centre at Oxford, hopefully should see someone there in next couple of weeks.

One saving grace is he thinks it's slowly progressive. But can't comment on the exact flavour of MMD, as I have central nervous involvement, he thinks its a fringe atypical, possibly genetic version. No idea what that means in terms of progression., but that Oxford will likely have a good idea.

Hopefully being under 40, and for the first 18 months being predominantly LMN, it could be a more favourable prognosis..

I'll wait to change my status until I have been seen at Oxford.
 

lgelb

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I'm sorry to hear this, but "slowly progressive" is a good thing to hear.

Best,
Laurie
 
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