Multifocal Motor Neuropathy

[juliesmile]

I'm hoping for clarification on MMN. My husband has muscle weakness, atrophy, cramping and fasiculation in legs. Beginning in left then right. I have read about MMN and was just wondering if in the absence of any loss of strength in hands or arms could it possibly be MMN? Just working on eliminating things.

[Peg B]

Hi,

I am currently Dx with either "MMN" or "Atypical ALS" (slow onset) I am positive on the EMG/NVC (3/4 limbs) for ALS and have one "borderline conduction block" in right elbow (possible MMN). Most of my functioning problems are in my right hand/arm, but in a short time, I get very tired and sore walking . (DX 12/2007 - sympton onset 12/2002) So that being said I check a MMN site like this everyday. It is barely active in comparison to this forum, but reading over the threads may be of help to you. I do think that MMN also has leg onset, as that is lower neuron, but Ican't remember at this point. There are many there who have DX of MMN for 12 and more years. The site is noted below. Please let us know if you find it helpful. Best Wishes, Peg

http://www.ninds.nih.gov/disorders/multifocal_neuropathy/multifocal_neuropathy.htm

[juliesmile]

O.K. now I'm even more confused. Can you tell me what the Nerve conduction study tests for. We've had 3 EMG and all show nerve damage in both legs, more on left less so on right. Plus arms were considered inconclusive but not normal. No loss of strength in right leg or arms. Fasiculation in legs, arms back, chest. But I was hoping maybe he had MMN as it's more treatable, right? Anyway, I'm just so lost.

[Peg B]

Hi,

This may help or make it more confusing. I took the following from this website:

http://www.emedicine.com/neuro/TOPIC724.HTM


Clinical and electrodiagnostic criteria for the diagnosis of MMN include the following:


Definite MMN

Weakness without objective sensory loss in the distribution of 2 or more nerves is present.
Definite conduction block is present in 2 or more motor nerves outside of common entrapment sites.
Sensory nerve conduction velocity is normal across the segments with demonstrated motor conduction block.
Results are normal for sensory nerve conduction studies on all tested nerves, with a minimum of 3 nerves tested.
Upper motor neuron signs, including spasticity, clonus, extensor plantar response, and pseudobulbar palsy are absent.
Probable MMN

Weakness without objective sensory loss in the distribution of 2 or more nerves.
The presence of either (1) probable conduction block in 2 or more motor nerve segments that are not common entrapment sites or (2) definite conduction block in one motor nerve and probable conduction block in a different motor nerve segment (uncommon entrapment sites).
Sensory nerve conduction velocity is normal across the segments with demonstrated motor conduction block.
Results are normal for sensory nerve conduction studies on all tested nerves, with a minimum of 3 nerves tested.
Upper motor neuron signs, such as spasticity, clonus, extensor plantar response, and pseudobulbar palsy are absent.

[Arthur Candell]

I have all the symptoms indicating MMN. I have no apparent upper motor involvement, but am getting mixed diagnosis from neurologists. Some say motor neuropathy, some say ALS. A lack of positive diagnosis is depressing. I am in an assisted living facility, going downhill very slowly day by day and discouraged at lack of empathy and diagnostic ability by neurological professionals.

[hopingforthebest]

Go to a ALS Clinic at a University Hospital for an evaluation. Nothing against local neurologist but how many ALS cases do they see? Go to ALS Clinic where they diagnosis many many cases.

Best of luck to you!

[Geo]

If you have MMN They use IVIG ,i had them ,they do 5 visits and 5 injections they come to the house and the nurse puts a IVIG drip on you for apprx. 5 hrs. If this helps you have to have these at regular intervals
BE Prepared for the Cost ,mine was $30,000 . Insurance said yes we cover ,then sent bill for $30,000
I had to call the State Insurance board and they made them Pay Up . beware Geo

[Peg B]

Hi,

I did not realize until today, that this forum had an MMN section. The stuff I wrote before was moved here I guess. Anyway, Today I had another test for the MMN/ALS. The NCS showed improvement and resopnse to IVIG, so they did not even do the EMG. The treatments have helped and it looks much in favor of being Multifocal Motor Neuropathy as opposed to ALS. I am going down to one IVIG treatment a month instead of two a month. So great news. Take Care, Peg

[crystalkk]

Peg,

That is great news....I am very happy for you it's nice to hear positve things.

[Al]

David created this forum about a week ago as he had noticed a lot of activity about MMN.
AL.

[Peg B]

Hi Arthur,

I am sorry I did not see your post before. I am so sorry you are not getting a better response from the medical community. There is another forum devoted to MMN you may find some comfort in reading through those posts.

As far as DX, I first had symptoms in 2002, and was told it was probably a pinched nerve. I was DX for ALS Oct 31, 2006 and possible MMN Dec 7 2006 at the University of Michigan ALS/neurology clinic. It is very difficult to DX. Are you getting IVIG? If so - does it help?

It sounds like you are going through this alone and it is very difficult. Please write back and let us know how you are. Sincerely, Peg

[Peg B]

Crystal,


Thanks for your reply and good wishes. Sincerely, Peg

[sisgldnhr]

Peg,

I am so glad to hear your good news!! Lets hope that the meds keep doing their job!!! Its got to be such a relief knowing that its not ALS.

Doing the Snoopy 'happy dance' for you...........Sis

[CindyM]

Hi Peg! FInally some good news around here! GLad to hear it! :-D

[Peg B]

Thanks Sis and Cindy,

I am so grateful. God Bless you both, Peg

[brooksea]

Peg,

You have been a help to so many on this forum. I'm so glad to hear your good news!

May you continue to receive benefit from the treatments!

[hopingforthebest]

Dear Peg:

So happy for your good news! MMN is the treatable disease!!

We knew when IVIG for one year was not helping at all that sooner or later it would have to stop and they gave us the news of PMA classification of ALS.

That is the big indicator that it is MMN that the IVIG helps! Good for you!

Hope you are going out to celebrate this weekend. I will have a glass of wine tonight and toast your good news!

God Bless you,
Patty :-D

[Peg B]

Thank you Jimmercat,

Your words are very kind and mean a great deal to me. Thanks again, Peg

[tmasters]

Peg,

Glad to hear your good news! I've seen your postings here and on the other forum, and I was waiting anxiously to see how this came out for you. I wonder, how long have you been on IVIg, and how much improvement did you see? I thought you were on the fence??

My situation is that I was given an initial dx of MND by my neuro back in January, but the ALS specialist stopped short of an ALS dx because I only have LMN signs. He recommended IVIg to rule out or treat for MMN.

I start the first of 9 IVIg treatments this coming Monday the 10th. I've read that, if there is a return of muscle strength, that it will occur anywhere from a few hours to 3 weeks after the IV. Does this seem consistent with what you've experienced?

Thanks,
Tom

[Peg B]

Hi Tom,

I have had symptoms since 2002. I was also told I have ALS by a neuro Oct 31, 2006. Dec 7, 2006 the ALS specialist wanted to see if MMN, by giving me IVIG. So my story is the same as your in parts. I have no anti bodies in my blood, and only LMN. I may have one conduction block, but not two. I have been on IVIG twice a month since Jan 2007. Overall there is no subjective change either worse or better, but the NCS does show some improvement, which it would't if I had ALS. So that is the story. I am not worse or much better functionally, so it seems the IVIG is keeping me steady. This is very good.

I hope you have great results with the IVIG. If you do have side effects, there are many things they can give you, so make sure you ask. Let us know how it goes.

To all others, Patty etc. thank you so much for your kind words and good wishes. Sincerely, Peg

[tmasters]

Today marks one month since my first IVIg infusion (May 12) and so it's time for an update.

So far, I've had 6 infusions of 35g each. The first 3 were all in the first week, the other 3 were spaced one week apart. I have 3 more to scheduled.

Unfortunately, I haven't noticed any improvement of any of my symptoms. :( It's still very difficult to stand on my toes; walking is slow especially the farther I walk. Right arm is heavy. Actually, if anything, I would say things are just a little bit worse.

I go back to the neuro on July 1 to see if he wants me to continue IVIg or stop or try something else.

-Tom

[JAKE]

I took 2 series of IVIG treatments back in Nov. of 2006 and seen little, if any improvement. A local internal medicine Dr. here where I live wants me to take another treatment for MMN called Cytoxin. I have got to see another nerve/muscle specialist in Jackson, Mississippi on July 3rd to get him to recommend it first because that is the closest place to get it done.

[Peg B]

HI,

I wish you both well. I hope Cytoxin works better Jake and that you Tom maybe get some more time on IVIG, to see if it helps. I don't see much of a difference on or off. Today I got my first IVIG after waiting a month instead of every two weeks. At the end of the month it did not feel any different overall. Some days are better than others. But the objective test showed some improvement after a year and a half.

Please keep writing on this site and give those updates. NO ONE that I have run into besides medical personnel has even heard of MMN. Thanks, Peg

[Dave G]

Hi all. I live in England and I was diagnosed with MMN at the end of last year. The first noticable symptoms were observed by me back in 2002 when my left hand started to 'sag'. The fingers wouldn't work and I had difficulty doing fine work like fastening buttons and zips.

At that time my doctor diagnosed a mild stroke and though this was not confirmed by the hospital I was treated with aspirin until last year.

Around August last year my mobility started to go and progressed rapidly to a point where I could only walk with the aid of a stick and had to retire on medical grounds. Following my MMN diagnosis last November I began a course of treatment with an Immunoglobulin infusion. So far I have had four courses of treatment, the last one ending yesterday (31st July). In spite of this treatment there has been a slow but steady deterioration in my mobility to the point where I now find it difficult to walk more than a couple of hundred yards.

I would be interested to hear of other peoples experience with IGG. Does it usually work? Do people find temporary respite from the symptoms following the infusion? Is it actually worth taking?

I look forward to your comments.

[Peg B]

Hi Dave,

Welcome. I am sorry you have to be here too, but it is helpful to have a place to talk about this. My symptoms also started in 2002.

There are others on that forum who have MMN and the infusion does not work for them. We (US/Canada) refer to it as IVIG. you called it IGG. I assume it is the same thing where you get a 4 to 6 hour infusion of blood products. I have a differentiated diagnosis of ALS or MMG. But it sounds the same as I have only lower motor neuron signs. (The doc says I am positive for ALS in three limbs according to my EMG. Does your bloodwork show any problems? Do you tire easily? I am right handed and my right hand and legs are affected. Are you left handed? Where and how do you get your infusion?

To answer your question is IVIG worth it? I don't know. I am now on montly infusions. I was on every two weeks from Jan 06 to June 08. I can't tell that much difference on or off except I may be more tired. However, it is not an even decline to infusion day. Some days I can move my baby finger and don't feel as week. Other days I cannot. I think the not knowing for sure gets to me sometimes, but my progression is very slow and it sounds like yours is also. Please write back and try to answer my questions to you and tell us more. I fell a lot before I stopped working did you? Again welcome. Sincerely, Peg

[Dave G]

Hi Peg.

Thanks for your welcome and your comments. it's good to be able to talk to people with MMN rather than to professionals who only know about it in a theoretical way.

I will try to answer your questions.

Although my left hand was the first limb to be affected I am in fact right handed. My right leg and foot are the most badly affected lower limbs though the left side is deteriorating too. I haven't had any real feed-back on my bloods yet though God knows they've taken enough of it. I do tire easily and I now need someone with me if I am going more than a couple of hundred yards. Luckily I live in the town centre so I am still able to access the shops and bars though with increasing difficulty.

My reference to IGG was a mistype. I was referring to IVIG. I was tired at the time. So far I have had 4 courses of IVIG. The first course was in January of this year following my diagnosis. The other 3 courses were on a monthly basis in May, June and July.

Treatment consisted of a 160m IV infusion of IG in a solution of 1600m over a period of 4/5 days. The first 2 courses were administered as an in-patient in the Neurology ward of a local hospital. I was then given the choice of remaining as an in-patient for the remaining 2 courses or attending a day clinic in a different but still local hospital. I chose the day clinic and for some reason the same course of treatment takes a day less on average than the in-patient ward does. I can also get home every tea time.

I do have falls and trips but not as many as might be thought. Most of the falls are caused by the fact that my right foot sags downwards from the ankle and I catch it on any small protuberance on the ground. I have learned to compensate by lifting my feet higher when I walk. It looks ungainly but at least I don't fall over as much.

Like you I have good days and bad days. On a good day I can still do quite a lot for myself, albeit a little bit at a time and very slowly. On a bad day I tend to sit at the computer and doze.

I hope this answers your questions. I look forward to talking again.

[Peg B]

Hi Dave,

The distribution of your IVIG is very different from mine. I get it once a month now at home. I am so excited as tomorrow is the day. I feel like a vampire who needs her blood fix. Yesterday we - my dog and I then walked to the park. Between my pug (3/4 pug and 1/4 Haveneese) It took us an hour to walk a mile. We sat at the park then my husband came and we watched a ballgame. I was out about 5 hours. Today, however, I went to mass and then spent the rest of the day on the couch. My legs ache and I feel very week. I could not figure out why I was so tired, but now that I wrote this I guess yesterday affected today a lot.

The way you discribed your foot sounds like what people refer to as "foot drop." I don't have that, I just fall by loosing my balance if I do not pay attention. I am going to attend a rally for an ALS walk in August. I may try to walk and just take my cane with me. I usually only use it in the airport or when we are walking somewhere and want to move faster. We went to London last year and I used it everywhere I went so I did not hold my family up. It worked well.

I still have a differential diagnosis of ALS or MMN. Do you or are they saying MMN for sure?

I think I am going to try to get my IVIG schedule to every 3 weeks. I will see the Doc in Sept. Take Good Care and thanks for writing back. Peg

[Dave G]

The only diagnosis I have been given is MMN. My neurologist seems convinced that this is the problem and to be honest I hadn't even thought of questioning this.

Taking into account that the IVIG seems to have had no effect on my condition I might talk to him about possible alternative diagnoses' at my next appointment.

[tmasters]

I would be interested to hear of other peoples experience with IGG. Does it usually work? Do people find temporary respite from the symptoms following the infusion? Is it actually worth taking?

I look forward to your comments.

Dave,

Here's a good site from Johns Hopkins which explains MMN treatment options. According to this, IVIg is effective in 80% of MMN cases. There's also some dosage information:
http://www.neuro.jhmi.edu/MMN/treatment.html

Here's another one, from Washington University, which supports the 80%:
http://neuromuscular.wustl.edu/antibody/pnimdem.html#mmn

Also, Peg is spot on with the "foot drop". That's what it's called.

My symptoms are like yours and Peg's, but mine started in my foot/leg and moved to my arm. Typically, 80% of the time, MMN will start in the hand/arm and move to the leg. So I'm still MMN or ALS, waiting and wondering...

I don't know why the doctor could be convinced you have MMN unless you respond very favorably to IVIg. ALS doesn't. One indicator is to look for GM1 antibodies in your blood. Did you have this test? It's not conclusive, but an indicator.

-Tom

[Peg B]

Hi Dave,

Just want to wish you all well. I hope that treatment is working. Dave please let me know what the Dr. says. Tom and Arthur and Jake. How are you all doing?

I will call my Dr. tomorrow and ask for every three weeks for IVIG treatment as I got too tired this last month. I had treatment today and my nurse said, my "lung capacity is diminished." It sounds horible to me, but she assured me I just need to do deep breathing to exercise my lungs. So I pass this on take deep breaths.

Take good care, Peg

[JAKE]

Peg,

I went to see Dr. Vedanarayanan, professor of neurology at the University of Mississippi Medical center on July 3rd. Upon completion of his physical examination of me he said what I had didn't appear to be ALS, due to not having any stiffness in my body and the lack of spascisity in my body. He said the problems lies within the miscommunication of the nerve cells sending signals to the muscles but said he felt like it was confined to the spinal region, and that there was not a problem with signals being sent from the brain. They did quite a bit of bloodwork and I went back on July 28th for more bloodwork and my 4th EMG. He referred to my problem being in the area of Spinal Muscular Atrophy. He wants to wait for the results of the last bloodwork which is genetic testing to determine if he is going to recommend IVIG treatments again for me. He is also wanting to do a muscle biopsy and a spinal tap within the next 2 months.

[tmasters]

Hi Peg,

I'm doing about the same I guess. I only really notice progressive weakness when I think back a month or two and compare to what I'm doing now. So it's pretty slow and gradual, just like I like it. Still no DX for me; either MMN or ALS.

I have my final IVIg infusion scheduled for Friday. My neuro won't continue it unless Dr. Graves at UCLA recommends continuing. So I see Dr. Graves next week.

It doesn't seem that IVIg is doing me any benefit anyway. I'm about the same as when I started, maybe a little worse.

I'm so sorry to hear your breathing seems to be affected. Do you think it's MMN/ALS related, or maybe just a normal respiratory infection or something? I should improve if it's from an acute illness. Have you ever gotten an FVC score, where they quantify your breathing ability? This is something they would do at an ALS clinic, and I'm sure you've seen the FVC graphs of the PALS on the PLM site. This seems like one more way to track the progress of the disease.

Best of luck to you.

Keep the faith,
-Tom

[Peg B]

Hi Tom,

My nurse does not seem to be concerned, but I realized today that sometimes when I am talking I have to stop and take a breath. However, that is usually when I am upset or excited. I have had bouts of bronchitis and had tests on my breathing capacity, but never a baseline. I requested on Tuesday going back on IVIG every 3 weeks and they said OK. It could be I do have MMN and the less frequent treatment impacted my breathing. When I go to the ALS clinic in September, I will get it checked.

I too do not notice much difference except I am more tired and also more emotional. I cry for happy and sad stuff, even store clerks who are extra nice to me when I am tired can sometimes do it. YIKES! Do you have any of that. I know it is an ALS symptom but is it MMN too? I don't know.

Then again my husband had prostate cancer surgery April 1 and we found out yesterday from an MRI of his back, that something (could be just inflamation) is there and he needs to consult with his surgeon. (PSA count is negligible) But that stuff makes a person more emotional too. I find it difficult to figure out where to put the cause of this or that, so in the end I just read Nora Robert books and take my mind off of what I have no control over. That and/or beer work most of the time.

Keep me posted on what Dr Graves says please. (That was our family doc's name when I was a little kid.) I assume you are still working and not much has changed on that. Take good care Tom. Thanks for your concern, Peg

[Peg B]

Hi Jake,

It sounds like you have the lower neuron problems (from the spinal cord) but not the upper neuron (from the brain). Did the blood work before show GM1 antibodies issues? Did the Dr. mention a conduction block? I agree with Al it sounds like you are in good hands. Best wishes to you.

I also wondered if you and others have checked out the MMN forum. I found it very helpful, especially when I was first diagnosed with possilbe MMN. Take Good Care, Peg

[tmasters]

Hi Tom,
...
I too do not notice much difference except I am more tired and also more emotional. I cry for happy and sad stuff, even store clerks who are extra nice to me when I am tired can sometimes do it. YIKES! Do you have any of that. I know it is an ALS symptom but is it MMN too? I don't know.
...

Peg,

I suppose the tendency to be over-emotional is a symptom of any serious illness as we deal with grief. But I think the ALS symptom you're referring to "emotional lability" which is an UMN (upper motor neuron) sign. MMN only presents LMN (lower motor neuron) signs, so emotional lability wouldn't be present in MMN. Let's hope in your case it's just grief/stress you're feeling.

Yes, I check the MMN forum and find it to be great for MMN-related information, especially IVIg. Not much posting goes on there, though. BTW: I got freaked out one day when I read Chad Bowman's postings there, and then he posted on this forum later as CBowman for a while. Yikes! Well, that's not my story, but shook me up none-the-less.

Yup, I'm still working, walking, and living my life. My first grandchild was born on Sunday and we have something new and exciting to focus on right now!

Keep the faith,
-Tom

[crystalkk]

Tom,

Congrats, on the birth of your first grandchild.

[Peg B]

Thanks Tom,

I did not know the emotional lability was an upper neuron issue. So that answered my question about its relationship to MMN. I appreciate knowing such facts.

I also felt very sad about Chad Bowman. He laughed at my joke the first time I wrote on this forum. He said he was going to use it. I also saw his entry on the MMN site. It does make it such a small world with very many caring people.

Congratulations on your first grandchild. Which is it, a granddaughter or grandson? Our youngest daughter was married last August. She just got a teaching job with insurance, so we are hoping for a grandchild soon. In the meantime my niece lets me take care of her twin baby girls who will be a year old this month. It is such a win win because she has a 6 and 7 year old too. I watched the twins (last school year) in the morning for about 1 hour and 1/2 so she could be with the other two and get them to school and I got my grandma fix. I am so happy for you. Congratulations again. Sincerely, Peg