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Lola-77

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Hi everyone - just wanting some advice and support and hoping for some help...

My father has recently been diagnosed with MND after 7 years of asymmetric muscle weakness and atrophy just in his right hand and arm. He was originally diagnosed (7years ago) with multfocal motor neuropathy but as the IVIG treatment hasnt stopped the progression of the weakness, his diagnosis was changed this year. Its come as a huge shock....

3-4 years ago Dad also developed some shortness of breath which was originally thought to be unrelated to the weakness elsewhere. Since the change of diagnosis, Dad has developed some further weakness with his breathing...his muscle power and strength is good (normal) in his left arm and both legs are strong and normal

He continues to have no upper motor signs just lower motor signs. He has developed some back weakness in the past 3 months which have made it really hard for him to walk up stairs...he is still ambulant but chooses to use a wheelchair as he fears falling (he has had two falls this year when his back gave way). He has started using Bipap in the last week as he has an increase in his CO2 in his blood, Oxygen sats are normal.

I really need some help in understanding why there has been a change in diagnosis after 7-8 years since the onset of symptoms I need help understanding why the new diagnosis has been made. Of interest, i have just read some of Dad's tests and the neurologist said that there was a suspicion of mildly prolonged F waves....i understand this might be more indicative of a neuropathy than motor neurone disease. He also had chronic denervation with little reinnervation....what does this indicate? Does this happen in MND?

I have tried talking to people about all of my doubts (the neurologist) but everyone has been really dismissive. I just wanted to know whether other people have had similar progression and whether it is right for me to be doubtful of the change in diagnosis. Our contact with health professionals has been patchy and sporadic and i just feel like they dont know my Dad.

Any advice would be so helpful

Thank you
Lola
 

laurel

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Hi Lola,

I don't have any good responses to your questions, but I have copied your post and sent it to Wright. He is our in house expert on these tough cases. If you had 10 posts, you could send him a private message, but since you don't have that many I will send it over to him.

Laurel
 

Lola-77

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Thanks Laurel, thats really kind of you - hoping to get my head around things :)

Lola
 

wright

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Hello Lola

Laurel did PM me, so you have her to thank for my opinion. She is such a sweetheart.

The prolonged F-wave was likely the reason he got an intial diagnosis of multi-focal motor neuropath. It could be that in a nerve or two that were tested during his EMG, that such a finding could turn-up but it not be related to the condition. Couple that with the failure of IVIg not stopping the progression of his symptoms and you get a neuro that changes his diagnosis to MND . . . in this case . . . PMA (PMA is an MND that affects solely the lower motor neurons. ALS is an MND that affects both lower and upper motor neurons).

Having said all of that: you have shared that his initial symptoms started in his right limb and have not travelled to his other limbs (the other three are still strong). That is not how PMA typically works. What does work like that is a condition called monomelic amyotrophy, which typically affects a single upper limb and then stops.

Of course there is also the issue of his breathing problems. You said that his neuro thought it was not related to his arm problem and it still might not be. Have they definitively proven that his breathing issues are due to problems with his respiratory muscles?

Bottom line: I don't believe they can give him a diagnosis of PMA unless he gets another EMG. Furthermore, they can't lump his breathing issues into the PMA blanket unless they've proven that his breathing issues are due to muscle weakness of his respiratory muscles. Have you thought of getting another opinion or at the very least, requested that another EMG be done?
 

Lola-77

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Thank you for getting back to me Wright - really value your thoughts,

Yes he has normal muscle power in his legs and left arm, he does have does left shoulder weakness and a weak lower back but it's nothing compared to his right arm and hand. He has fasticulations everywhere - does that occur in monomelic amyotrophy? Also regarding the lower back weakness - would this be a manifestation of lower motor issues?

Also I just wanted to check out his EMG findings...what does chronic denervation with little reinnervation mean (I think i get the denervation bit but what does "little reinnervation" mean?

They dont seem to have any other explanation for his breathing problems - they initially thought he had a left raised hemidiaphragm (not really sure what this means) and apparently there was a "poor response over the phrenic nerve on stimulation" - does this indicate a similar disease process as in his arm? Dad has difficult lying on his back - and has breathing issues for around 3-4 years...

Yes - I would like a second opinion but it would mean a lot of travelling for Dad which he really isnt keen on. Since diagnosis, he's really anxious all the time and his confidence has plummeted.

Thank you very much for taking the time to respond and i look forward to hearing your thoughts

Lola
 

wright

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Hello again Lola, I'm glad I can help

Given that he has no weakness in his other limbs, certainly points in the monomelic amyotrophy direction. This could be confirmed if they would simply do a four limb EMG to see if those limbs are clean. To answer your question about twitching: twitching can happen for any number of reasons and those body-wide twitches could be completely unrelated. Again, the EMG would shed light on that.

His left shoulder weakness and lower back problems can certainly stem from postural issues that he most likely has, given he is probably compensating for the weakness in his right arm and does a lot of sitting. Therefore, these other issues might be a consequence of his real condition. I'm certainly not saying definitively that that's what's going on but it's a possibility. That is why a second opinion or further testing by his current neuro is in order.

His lower back problem can also be due to lower motor neuron problems, as can his breathing problems. The statement "poor response of the phrenic nerve" can be due to lots and lots of different things and the term "response" in that context can indicate a number of things as well.

The term "little reinnervation" is something I can't fully comment on unless I can see his EMG and read the impression of his neuro. That kind of rhetoric can be stated for a number of different reasons.

Bottom line: There are just too many unanswered questions in my opinion, that beg for more tests to be done.
 

Famdamily

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Hello Lola-77,

I'm glad to see you are getting help already from some amazing members here. This place can be such a blessing in our quest to understand what is happening to us or someone we love.

I am sending prayers for your dad, you and your family. I also have been diagnosed with monomelic amyotrophy. It might help your dad and you to know, that in my case, I have had it for 20+ years...and am going strong although I am extremely gimpy (highly technical medical term) in my left arm. I also, like your father, have battled lower back problems and pain over the years. My lower back has been much better during the past two years after I left a fun job,but one that required lifting 50+ pounds repeatedly.

Monomelic is very rare from my understanding and can certainly be a confusing and scary journey because there is so much to learn. I truly hope your dad and you can join us here and find the information and support so many on here kindly give and receive. The members who have joined you here already in this thread can offer the best advice.

Your father is fortunate to have people like you in his life who care so much. Bless you and tell your dad our thoughts and prayers are with him too. I hope as the answers come in this journey that you have had to embark on, that those answers offer as much good news as mine have.

Peace and hope to you :)
 

Lola-77

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Thank you Famdamily for your kind wishes - in your experience, have you ever had any difficulties with breathing? Have your lower back issues had any impact on walking (Dad can walk, he just feels that when his back "goes", he might fall)

I am so fortunate that whatever dad has, it is a slow progression but with so many unanswered questions, i find myself questioning MND, particularly when it continues to be isolated to his right arm and hand. The MND diagnosis has had a huge impact on my mum and dad, he's a big believer is "what the Dr says is right and shouldnt be questioned" - whereas I am a registered nurse and know that Drs, only being human, do get it wrong sometimes.

Warm wishes to you and thank you for taking time to respond :)
 

Lola-77

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Thank you to again Wright,

I will ask Dad's neurologist for a second opinion or further EMGs. It's helpful knowing that others feel there might be further tests that need to be done before an official PMA or MND/ALS diagnosis is given.

Just out of interest, I have been reading about post polio syndrome - although Dad isnt aware whether he contracted polio as a youngster - others in his family did. Dad grew up in southern italy in quite a poor region (Drs visits were expensive) - would it be possible for Dad to have contracted polio and for him not to know whether he had it? Maybe i'm clutching at staws here....

Lola
 

Famdamily

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Hello again Lola,

Yes absolutely the lower back problems can impact walking. When my back goes I too have found myself on the floor although the last time was a few years ago thankfully. Having the single limb mnd doesn't make us immune to really bad backs that many people have to live with. Sounds like your dad could have similar problems. There are excercises that can help build stronger ab muscles and better ways to lift (less weight) that improves the back problems, in my personal experience.

Not to dismiss the breathing problems at all, but honestly the diagnosis of any mnd can cause anxiety that makes us hyper focus on every twinge, breath, strain etc., and maybe through time, with a better understanding that if indeed it remains in one limb, your dad can have every expectation of living a full life, he will be relieved. I found I truly hadn't exhaled and relaxed until after recent emg findings confirmed it was staying single limb, even though that weakness gets slowly worse. What a relief. The gimpy limb becomes no big deal and you learn to adjust around it to the point that no one else even notices you aren't really using that arm much.

Like others have suggested, getting the emg and nerve conduction study as well as a cervical mri may help your family as they seek a solid diagnosis.

I am happy to offer whatever personal experience I can. 20 years ago, once my diagnosis settled in, i truly began to see it as a blessing. I learned to value being alive every single day and seeking the joy in each day. There are more than enough sorrows, so focusing on the joy in each day has been a gift. I am so lucky to have this extremely slow, single limb progression that serves as my reminder to not take life for granted.

Keep us posted and wright and others helped me a great deal in understanding the emg results and so much more.

Keep smiling.
 
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Famdamily

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Lola,

Just to add, my neuro suggested I read up on post polio syndrome as the symptoms for my monomelic diagnosis are similar, so yes it can be helpful information apparently. While they aren't the same, the symptoms are very similar in someways, according to my doctor.

Take care! :)
 

Lola-77

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Thanks Famdamily - is it possible to have had polio without having had a prior paralytic attack? I mean can you have had an intial bout of polio but without paralysis, just a fever-like illness...? Just wandering as Dads symptoms are more like post polio but he cannot recall having had any childhood paralysis from polio....

Lola
 

Famdamily

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Hello Lola,

That is a good question, that I don't know the answer to either. I'm sorry I can't be of more help on that. Other members on here may be able to offer more insight. Keep us updated and please don't hesitate to ask anything. There are very knowledgable folks on the many forums within this site.

Welcome and prayers for you and your family.

Famdamily
 

Lola-77

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Thank u Mulino - I too pray that he makes a full recovery - he truly is an amazing person :)
 
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