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Lola-77

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Joined
May 28, 2012
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Loved one DX
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UK
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C.I
Hello everyone, I'm very new to all this so forgive me if I get any of the terminology wrong. I've been reading your posts for a little while and would really value and advice or support you may have... :) I'm finding things very hard at the moment....

My dad was diagnosed with multifocal motor neuropathy just under 7 years ago, beginning with weakness in his hand. He had extensive tests by Consultant Neurologists...there was no evidence of conduction block or GM antibodies but a slight prolongation of F waves (I have no idea what this means…..is it suggestive of ALS?). His EMG showed chronic nerve degeneration, plantar reflex was normal.

Anyway, he was commenced on IVIG around 18 months after his initial symptoms and his treatment has been very sporadic due to organizational changes in the hospital dept. A few years ago, his neuro believes he has had some response to IVIG and that it is arresting any further decline BUT more recently (over the past year or so) his breathing has been affected (shortness of breath, inability to lie flat) as well as some weakness in his legs. Dad has seen a respiratory dr who diagnosed dad with sleep apnoea and Type 2 respiratory failure… he gets breathless during activities (walking) and describes a pressing down feeling on his diaphragm.

In our last appt with the neuro he surprised us by saying that Dad most probably had motor neurone disease/ALS. The neuro was pretty flippant about delivering the diagnosis…we had previously been told it was not ALS.

This came as a massive shock and I, more that anyone have struggled to cope with the news. I actually wrote a letter to the neuro to seek further clarification but have heard nothing yet. So far, his symptoms are weakness and atrophy in his right arm, some weakness (no atrophy) in his legs and shortness of breath and fasticulations. No issues with swallowing After seeing our reaction during the consultation with him, the neuro suggested we try a more regular course of IVIG and then review in 6 months to see how things progress.

We were left feeling completely shattered and unprepared and very much in limbo. He didn’t really give us any further info and only recently has organised a meeting with a physio and OT. From looking through other people's experiences, it seems like motor neurone disease is often given as a differential diagnosis. Dad's recently had a course of IVIG and feels stronger and his breathing is a little better. He has however, (since the motor neurone probable diagnosis had two falls). Does IVIG usually improve ALS symptoms? Or could this be a subjective improvement? How quickly does MMN progress? Can it progress with IVIG?

We're still very much in limbo about all of this and dont know what to think. How closely can MMN mimic motor neurone? Has anyone else had these experiences? Its so difficult to accept ALS when multifocal motor neuropathy has been the primary diagnosis. I just feel scared....maybe at what to expect....i’m praying (regardless of what Dad has, that he’ll be around to see me get married and have kids).

Thank you so much for listening...any thoughts or advice would be welcomed....any thoughts on how i can keep strong for myself and my family would be welcomed xx
 

HelenL

Very helpful member
Joined
Dec 17, 2010
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1,602
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PALS
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MA
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Southborough
Hi Lola-77, wish there were some words of wisdom I could give you, but don't know the answers to your questions. Some of the others here are much more expert than I, but haven't been on for a few days.

In my opinion, the fact that he's had this for 7 years is good news, as whatever it is, it's been progressing slowly. The trick is to find out what it really is, and to get to a neuro that sticks around long enough to answer your questions. I can't believe some of the horror stories I hear!

If he does already have the ALS diagnosis, contact your local ALSA and MDA agencies (not sure if they're the same in the UK or not) and see what they can do to help. Remember to reach out and make yourself known to them!

Wishing you good luck, and having a positive attitude does help.
 

glupavomomiche

Distinguished member
Joined
Sep 25, 2011
Messages
282
Reason
PALS
Diagnosis
09/2011
Country
US
State
Texas
City
Podunk
Hi Lola,

So sorry your dad is going through this! I'm not a doctor or medical professional, but I do have some understanding of MMN vs. ALS.

The arm onset with weakness and some atrophy you describe is indeed characteristic of MMN. Fasciculations are also common in MMN, not just ALS. They can look very similar electrodiagnostically, so the main way the doctor diferentiates between the two is by upper motor neuron (UMN) signs. This would be the Babinski sign, brisk deep tendon reflexes, Hoffman sign, jaw jerk, etc. Did the neurologist mention any of these signs were present now? (I know you said there was no Babinski on the original exam)

IVIG does not help in ALS. IVIG works in motor neuropathies because those are immune-mediated and that is precisely what IVIG treats. ALS is at this time not identified as auntoimmune and PALS do not show any favorable response to IVIG. Sometimes where the doctor still can't distinguish between a motor neuropathy and ALS, it's common for the doctor to prescribe a round of IVIG to see if symptoms improve. If they do not, then that result helps point the doctor towards ALS. I'm assuming that is why the neurologist has recommended this course of action for your dad. I know there are quite a few folks on the forum who went through this type of testing, so maybe they will speak up with their experiences.

The addition of respiratory symptoms does not automatically mean ALS. I would think it would be a good idea for him to see a pulmonologist regarding the respiratory symptoms. It could be very likely that he does indeed have MMN as you have been told all along and has now developed COPD on top of it. A good pulmonogist should be able to help you tell the difference.

It's a tricky situation with no easy answers. While it is extremely unfortunate that the neurologist was so blase about telling you of the possible ALS, I do think he is being appropriately concerned and taking a reasonable course of action. But really, the most defining factor here are the UMN signs, so that is what you need to question him about. If the reflexes were brisk with Babinski and Hoffman signs, then I would also think ALS should be considered. However, if the areas affected by MMN have decreased reflexes while the unaffected areas have normal reflexes, with no Babinski or Hoffman signs, this definitely points away from ALS.

I hope this is helpful, and remember, I'm not a doctor... this is just my understanding of the relationship between MMN and ALS.

Good luck to you and your dad and try to stay calm during this uncertain time. What will be will be. There is nothing you or anyone can do to change it and acceptance of what will be and doing the best you can with the hand you have been dealt is all that anyone can do. I believe everything happens for a reason and we must face our adversity with as much strength and grace we can muster!
 

Lola-77

Member
Joined
May 28, 2012
Messages
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Reason
Loved one DX
Country
UK
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C.I
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C.I
Sarah and Helen,

Thank you so much for your responses, I cant tell you how much reading this forum has helped me in managing some of the feelings I've encountered in response to my Dad's illness.

I'm trying hard to be strong and on my good days feel that this journey is making my family stronger and showing us what's important in life.... I really do believe that "our strength grows out of our weakness".

We havent been given a definite ALS diagnosis, just a highly probable diagnosis....the neurologist seems pretty stumped about my Dad - apparently there's no knowing either way and the not knowing is the scary bit. It feels hard to accept something that may not be.

I think i understand a bit about Upper motor signs but would appreciate any further info you might have on what this is. From the list you gave Sarah, I do know that the Babinski and Hoffman signs were normal (Babinski is the same as plantar?!) and there are no issues with jaw jerk and no swallowing issues. I think the neuro did say something about dad having brisk reflexes....I'm not sure though...what do brisk reflexes mean?

Thank you so much for your help,

Take good care xx
 

Lola-77

Member
Joined
May 28, 2012
Messages
11
Reason
Loved one DX
Country
UK
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C.I
City
C.I
Sorry I left something out....he did see a breathing specialist who was the first one who queried the diagnosis of Multifocal motor neuropathy as he said that breathing was not involved with this condition although from my reading, I know that although it is rare....MMn can effect breathing muscles....Dad is now on a CPAP machine at night for the sleep apnoea....
 

Peg B

Distinguished member
Joined
Nov 5, 2006
Messages
414
Reason
PALS
Diagnosis
10/2006
Country
US
State
Michigan
City
Flint
Hi Lola-77,

I am sorry to hear about this turn with your dad. Sarah was exactly right and very helpful. Let me repeat that ALS DOES NOT RESPOND to IVIG. I was first Dx with ALS then possible MMN and was put on IVIG to see if I responded. I did and have MMN. I would like to add a few things that stuck out for me.

You said your Dad's IVIG has been inconsistant. That is a problem. I started IVIG in Jan of 2006. My first year and 1/2 was every 2 weeks. We tried every 4 weeks for 2 months and I was SOOOOO TIRED. We went to every 3 weeks and I have been on that ever since with one exception. This Jan and Feb 2012 I was unable to get treatment due to insurance reasons. My baby finger went right back to being atrohphied I got tired and could no longer type with 2 hands or hold a pen in a normal way. So after 5 years of not thinking IVIG helped all that much I could see it helps much. I started treatment again on March 1 and things are great.

I do not have the antibodies problem and after a couple of EMGs they found a partial conduction block. When I was first DX with ALS we told our childdren (adults) it was "Not time to worry." We would wait. Well the reality is it is never time to worry as worry adds nothing to the mix. I hope the 6 months of consistent IVIG continues to improve your Dad's condidtion. I also am not a Dr. but I do know that one can have MMN and other diseases too like COPD. Hang in there and do fun things with your dad when you can. Best Wishes, Peg
 

notme

Extremely helpful member
Joined
Apr 3, 2011
Messages
2,605
Reason
PALS
Diagnosis
08/2011
Country
US
State
Fl
City
Orlando
I'd see an ALS specia list if I were you. Did they ord er a bi-pap for breathing? It sounds like one is in order for him! IF you have a bad vibe about the doctor--seek a second opinion!

It seems like so many of the specia lists lose their bedside manner! You'll find lots of support here, whatever the outcome.
 
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