MMN Diagnosis Q

[Chels]

Hi everyone, I've been researching MMN and find it a little hard to comprehend all of it as far as testing and everything goes. From what I understand, you typically have a conduction block/s and/or test positive for certain antibodies. I'm confused about the antibody part because I've also read that only about 50% of people with MMN will have a positive GM1 IgM but if you do, the specificity for MMN is almost 90%? I have quite a long story to my symptoms and testing like a lot of other people so to keep it pretty short, I've been having problems for about a year now. Testing for everything to find absolutely nothing. Two muscle biopsies show mild denervation with a 'supposedly' normal EMG 6 months ago. Fast track to now, I'm seeing a new DR and just got another EMG and repetitive nerve stimulation I believe they called it. I don't know the results of that yet but during the test the Dr said I do have abnormalities and was asking if I get cramping a lot, focusing a bit on my hand/arm because it was worse than my leg I guess. Anyway, I also just received some lab results from my PCP that the ALS Dr ran on me right before the EMG for "Ganglio Monosial Antibodies." It says 29 IV or less: Negative, 30 to 50 IV: Equivocal, 50 to 100: Positive. For GM1, Ab IgM I have 65 IV. I guess that's obviously positive but when I try looking up ranges or numbers from other people just to compare, everyone else talks about titers like 1:1000 (Not sure the correct numbers). I don't really have any sensory type issues and the symptoms of this do sound like mine a lot. I'm hoping I'm reading my results right and that MMN is what is most likely wrong with me. I have an appt. next week to discuss my results but I'm feeling very anxious and very relieved at the same time so I'm trying to make some more sense of it all. I have a lot of questions I'll be asking him of course too but I like to go in somewhat pre-informed :grin:

 

[Northern Dancer]

Chels,
Consider this. After reading many posts here, I think that concentrating on being pre-informed down to 'titers like 1:1000' is a waste of time and often interferes with informed professional care. I have never asked to read my lab or EMG results even though my job before I retired required me to be very familiar with results like those. Has this affected the course of my disease? Absolutely not.

Seems to me if we left the interpreting to experienced professionals that are familiar with our personal and unique situations, there would be a hell of a lot less nervous, obsessed posters and a lot less angst from PALS and CALS who get upset with repeated questions.

ND

 

[Chels]

Okay, well considering my results did not have titers, I can't say that I'm actually concentrating on them. Which is what my actual question was about. I wasn't asking anyone to interpret my results for me, but when you receive them in the mail, naturally you're going to look them up. Mine are positive, as the form says, however, the way mine was "measured" I guess you could call it, is different from others I've spoken with. So considering most people with MMN have most likely gotten this test before, I thought perhaps one of them might know why. And you're absolutely right about personal and unique situations. Mine happens to be a horrid mess with misdiagnosis, misread reports, "lost tests" ignored tests, and unprofessional people screwing with my life. The only reason that is now known, is because I decided one day to get pre-informed when blatantly obvious things to any average person, didn't make sense. So in my situation, thank god I did ask for all my results. I've now been referred to a new Dr who has to clean up after everyone else. I'm not going to dive into my complete history for the sake of having my questions/concerns validated, however, I went from a healthy young mother, to crazy, to having a very rare muscle disease, and then surprise! The ALS clinic. So if I bothered anyone with my question about a blood test, I'm sorry. I'm just a little excited about what I believe to be unexpected good news. If my question was "repeated" I would love to read the others and be more than happy to close my thread. I have looked already though and couldn't find anything. Perhaps I'm mistaken. And if I'm considered somebody nervous or obsessed, then maybe I am. But I have every right to be nervous and obsessed with, what's killing me.

 

[Northern Dancer]

Chels,

I wholeheartedly agree with you that you have every right to be upset with your situation. Who wouldn't feel 'crazy' and obsessed when faced with this? You are not alone. Criticising you was not my intention. I was just giving you my personal opinion.

No one should ever feel that they are bothering forum members. If I made you feel that way I am truly sorry.

Lorna

 

[Chels]

Thank you. Rereading my first post combined with my lack of other posts, I realize I came off as a self diagnoser or maybe "new" if that's a good word. I have been reading on this forum for quite awhile as well as others and I'm quite aware of all the people without any significant problem, trying to find one, if that makes any sense. I assure you I'm not one of them even though you don't know me from Adam lol Like I kinda said before, this past year with all these Dr's and different tests have been a complete nightmare and a lot of it is SO bizarre, no one would ever believe any of it had I posted anything before lol But actually, a lot of the information I received here is the main reason I knew something wasn't right. I'm one of those people that never go to the Dr and just kinda let everything run it's course until it's better. I never got better this time, obviously, but whenever they ran tests and whatever else, I never questioned them and didn't really even think about it being an option to see them, kwim? Anyway, I've gotten a lot of this crap where I see someone and they know exactly what's wrong with me and what to do. When it turned out to be wrong, they would pretty much tell me, tough shit and get lost. Then when I would see someone new, they would question me about my positive test for this and that or how come I never got this done, etc. Just a total mess I can't even begin to describe without labeling myself insane lol I never really thought that I had something completely horrible even though they were suggesting it. When I was diagnosed with the muscle disease, it made complete sense to me and I did like my Rheumo a lot. But after some time, lack of response to treatment, new symptoms, and the two muscle biopsies showing denervation, it was quite obvious I belonged in a Neuro type category. When I finally seen one, she was more than thrilled with my story and actually had my appt. bumped up over a 6 month wait because she found me so interesting. However, she had me diagnosed before I even walked in the door. With the same muscle disease in fact, only juvenile form, basing everything off reports by other Drs, that were incorrect, not to mention irrelevent because my symptoms were quite different at this point, opposed to 6 months before this. So they did more stainings on the biopsies, she was wrong, and then I wasn't so interesting anymore. My PCP couldn't believe it all and sent me where I am now. He said he thinks this is a motor nerve disorder but my pattern of progression and such is a little odd so he said he wanted to run some test for an autoimmune type of problem I haven't been tested for yet. I just got my results in the mail that say I'm positive for the IgM. I imagine, or so I'm hoping, that even if he doesn't think it's MMN specifically, it at least points in the direction of something else and I can just do whatever needs to be done and try to get my life back in order. I am still quite upset over all this as a whole, but I've already accepted the fact that something is wrong and my life is going to be different. I just want to know why now. I'm also just feeling kinda angry because if it really was as simple as a blood test for me, this could've been possibly taken care of a long time ago. But I guess it doesn't really matter and I just have to wait and see what happens Wednesday. Anyway, sorry that was so long and turned into a woe is me story. I just wanted to explain myself a little bit and I do appreciate your responses, so thank you and I'm sorry I'm being overly sensitive.

 

[Peg B]

Hi Chels,

I am sorry this has all been so upsetting. I asked a neurologist in 2002 if I had a systemic disease like ALS or MS and she said "NO." I kept falling and asked another one to "check me out." His DX was I needed to exercise more. LOL and "don't we all."

In 2006 I was DX by a different neurologist (local Detroit Metro area) ie across the street from St Mary's in Livonia. He said ALS for sure, but go to U of M for a second opinion. They said, maybe MMN. So then I began IVIG treatments and responded to them ie almost no more progression but enough damage had been done to my hand (R) along with super tired cramps etc that I was officially disabled. I am older than you and had already done 36 years in education. So that's my story. Conclusion to this part is it takes a long time, as you already know, to get diagnosed.

I however do not have the antibody blood thing. They did find one partial block in my right arm and I am positive in 3 limbs by the EMG for lower motor neuron issues. So my symptoms are not real clear but I do respond to IVIG so if I respond I must have MMN. Kind of a back door DX but U of M is great.

One short story. Last week I called the ALS association because I needed some help with insurance. I told the woman that my Dx is really MMN and she knew what is was. I burst into tears because I did not have to explain myself AGAIN. So please know you are validated in your frustration and have lots of prayers you get a clear DX soon. Take Good Care, Peg

 

[rulimping]

Hi Chels,

I've just been diagnosed with MMN, after an inconclusive investigation with another neuro about 1.5 yrs ago (and he advised me it was stress ...:evil

It's a very stressful time and sounds like it's been a tough road for you so far.

I'm also a youngish Mum; never been ill, so this is all rather confronting! I have found searching on the internet useful to gather more info, although I leave diagnosis & clinical advice up to my new neuro! I've downloaded lots of articles if you would like me to send them to you - just let me know your email.

Anyway, this (with my small non medical brain is what I understand so far on MMN):

• it's restricted to motor nerves, altho some minor sensory involvement has been seen in a few cases
• it typically is worse in hands / feet (distal), and of these hands are usually worse
• it's chronic - always there
• it's progressive - gradual decline or stepwise - and is asymmetrical
• some neuro reflexes may be weak or absent
• EMG shows multi focal (many areas) and persistent partial conduction block
• anti GM1 can be seen but not always, and they are not 100% sure on what the autoimmune mechanism is, but the outcome is inflammation which causes demyelination
• it usually (70-90%) of cases responds to IVIG
• if it is MMN, the sooner IVIG is started the better the likelihood of recovery of muscle strength,
• altho it won't be 100%
• there are other things that if absent assist with differential diagnosis (eg bulbar involvement

[*]untreated it will result in partial paralysis of affected areas
[*]there is no cure yet; it can go into remission
[*]if IVIG doesn't work, or efficacy fades over time, stronger immunosuppressants can be tried, but more research is needed here
[*]steroids may make it worse
[*]symptoms may include muscle weakness / tremor / twitchy muscles (fasciculations) & cramps & the pattern described above.

My neuro diagnosed on EMG, hx, physical exam and bloods, but we haven't done anti GM1 yet - he advised that was more for validation than diagnosed.

I've just started IVIG 0.4g/kg, daily induction for one week, then maintenance 1ce mthly. Only a bit tired and headaches from today (day 4). No physical change seen yet - crossing fingers!

I found getting the diagnosed and working with a great neuro to be really helpful for getting my anxiety under control.

Feel free to reach out here anytime or to me privately - I assume there's a mechanism via the board?

All the best in your journey,
from a hot and summer-is-here Australia
Jacquie


I've copied abstract from a recent o'view on MMN which I found helpful:

Multifocal Motor Neuropathy: Update on Clinical
Characteristics, Pathophysiological Concepts and
Therapeutic Options
Sven G. Meuth Christoph Kleinschnitz
Department of Neurology, University of Wuerzburg, Wuerzburg , Germany

Eur Neurol 2010;63:193–204
DOI: 10.1159/000282734

Abstract
Multifocal motor neuropathy (MMN) is an acquired immunemediated
neuropathy characterized by chronic or stepwise
progressive asymmetrical limb weakness without sensory
deficits. The upper extremities are more often affected than
the lower extremities with distal paresis dominating over
proximal paresis. Important diagnostic features are persistent
multifocal partial conduction blocks (CBs) and the presence
of high-titer anti-GM1 serum antibodies. Motor neuron
disease, other chronic dysimmune neuropathies, such as
chronic inflammatory demyelinating polyneuropathy and
the Lewis-Sumner syndrome (MADSAM neuropathy), are
important differential diagnoses. While corticosteroids and
plasma exchange are largely ineffective, high-dose intravenous
immunoglobulins are regarded as first-line treatment.
In spite of significant success in elucidating the underlying
disease mechanisms in MMN during the past few years, important
pathophysiological issues and the optimum longterm
therapy remain to be clarified. The present review summarizes
the clinical picture and current pathophysiological
concepts of MMN with a special focus on the molecular and
electrophysiological basis of CBs and highlights established
therapies as well as possible novel treatment options.
Copyright © 2010 S. Karger AG, Basel

 

[Chels]

Peg - You just made me laugh :lol: Before anyone started to sort of take me seriously, my Dr said I needed to exercise more and "what do you expect when you go from doing nothing all the time to something." Referring to my arm becoming immobilized after briefly experimenting with my new Wii :roll: The thing is, the summer before this I was still overweight from my pregnancy and also in a program for law enforcement. So by the time I got "sick" I had already lost a good 30 lbs dieting and exercising. I felt great. I met the height/weight standards for the police academy and just felt really good overall. U of M is actually who diagnosed me with Myositis. I liked my Rheum's a lot, but the Neuro's were nothing but problems :-| Regardless, I didn't just get "bored" one day and decide to screw up all my plans and start acting crazy, ya know? I don't have to be dying, or have something completely horrible, incurable, etc. But I do know SOMETHING is wrong with me and it's ridiculous that the hardest part of being diagnosed, is just getting someone to listen to you. I was amazed reading other people's stories about how common that is too.

rulimping - Thank you so much for the very thorough and informative response. I guess you could say I'm pretty technical minded, so I like to at least partially understand some of this stuff so I know what they're talking about. I always ask questions when I see them, but I do always have a lot that don't necessarily 'matter' so if I kind of already get it, I won't waste time asking that stuff opposed to things that actually do matter. If that makes sense. I can't help it, it's just the way I am lol I seen my Dr the other day and I don't know the specifics of my EMG and stuff, but I guess it appears that I didn't show any conduction block. I guess it did show that I have the tendency to cramp easily and some other stuff I'm not too sure about, but actual cramping isn't necessarily one of my main issues and contradicts the antibodies I have so he isn't too sure what it is. He did bring up the IVIG as an option but doesn't feel comfortable starting me on that without the conduction block. I guess my antibodies could also be irrelevent to my problem but he's just not sure so he's sending me to the Mayo Clinic. I just got the call from them yesterday and I have to be there on January 3rd. Have any of you been there before? I think it will go well and everything seems to at least be pointing in the right direction of something now. If anything, I'm having a problem with my hand now and it's really starting to bother me so I hope something can be done about that. Thank you guys for your responses and it makes me feel better to know I'm not the only one in this boat. Even if MMN isn't my answer I appreciate the information and kind words you've given me.

 

[Peg B]

Hi,

Best wishes with the Mayo Clinic. I have read that on another MMN forum (which I can no longer find) that a lot of the people go to the Mayo clinic and it is very helpful. I hope you get some concrete answers. Let us know how it goes. In the meantime Happy Holidays. Best Wishes, Peg

 

[finallymmn]

Can not believe after all my searching that I found a forum about mmn. I have been suffering with this for at least 8-10 years. I have gone to dr after dr with no luck, the last one told me that I had nerve damage for no reason. My mmn started in my left foot and I have a severe foot drop. After noticing some weakness in my left hand I decided to try one more dr., someone was on my side because she was the one who diagnosed me. I had the EMG which she told me was "very" telling and the Anti Gm1 test which was negative but she said that it didn't matter. Working to start IVIG in a few weeks, she would just like me to go to physical therapy to get a better idea of where I am at and if the IVIG helps and I gain strength. It feels good to have a diagnosis and know that there is something that is "really" wrong with me. Can anyone tell me if this makes you really tired. I seem to be tired all the time, but am wondering if it's the mmn or just the fact that i am a mom and working full time. Thanks for your help!

 

[Chels]

finallymmn - I haven't been diagnosed with MMN, but it seems that everything I've been presented with, researched, and heard from others, fatigue seems to run rampid with all these disorders especially ones that are autoimmune. One of my first symptoms was that I felt completely exhausted all the time, no matter what I did/didn't do, or how much I slept. I'm a Mom to a toddler and was in college then so I'm quite sure that didn't help at all. Like I said, I haven't been diagnosed with this or anything else, besides DM which is questionable now, but I couldn't imagine working full time, or actually even part time in my current state, so kudos to you x100 and everyone else that are managing so well. I've read from a couple other people on a different forum that after starting IVIG they felt remarkedly better fairly quickly so I hope you have good results from this very quickly. Let us know how everything continues to go for you.

 

[rulimping]

Hi Finallymmn,

I'm also just diagnosed and had IVIG "induction" last week.

I think I get more muscle fatigue, because muscular weakness / atrophy has started to kick in.... but I also exercise less, as a nerve driving my calf muscle is affected, which makes me limp slightly, so that is probably also a factor.

Also a f/t working Mum so that's enough in itself!

I really wish you the best in your care and treatment.

Regards

 

[Peg B]

Hi Finallymmn,

Well you are correct - tired is so right there. When I first had symptoms back in Dec 2002 (I was very tired and I had trouble moving my right pinky finger and could not hold a pen long enough to write a personal check without changing the pen position.) I had just completed 30 years of teaching/working with emotionally impaired adolescents. I spent 15 years working in a state hospital and another 15 in a public school. My classroom was on the second floor and my legs were tired and I just felt tired. I was also teaching at a university part time and working on my Ph.D part time. OF COURSE I was tired you say. But I had always done such and never felt THAT tired. I thought I finally burned out on the kids

So I began to change jobs (4 new ones - promotions over the next 4 years). Now Zoom to 2006 and I was DX with ALS then later instead MMN. Then I realized how everytime I changed jobs my biggest thought was - a smaller buiding, a smaller parking lot, no stairs. It was not my schedule - it was MMN. I have had treatment - IVIG since 2007. I just had it on Tues of this week. I feel very good. Yesterday I was hyper. In about 2 weeks I will need to slow down a bit and will feel more tired but then with the treatment (every 3 weeks) I will have more energy again.

Keeping as fit as possible is very important. I've lost 25 pounds since last January and exercise 3 to 4 times a week. I really could not do that before IVIG. But even without the exercise and weight loss my tiredness has been a function of MMN. I also have atrophy of some muscles in my right hand and I am permanately disabled but if I had had treatment earlier I might not be. I hope that addresses your concern and that IVIG works well for you. Please keep us posted now that you have found this forum. ps I am also a mom but my husband was the full time home parent. Best Wishes, Peg

 

[finallymmn]

I'm back after being gone for a while and I can't believe it, I did my first round of IVIG over the last week of December and I am seeing a lot of improvement in my left ankle (which has been affected the most by MMN). I can now actually walk without picking up my entire leg. I really didn't have a lot of hope going into the IVIG because I have had MMN for the last 8-10 years but was only recently diagnosed. My only side effect from the IVIG was a headache afterwards even though I was premedicated with tylenol and benadryl. I now have 4 weeks of PT and then an appointment with my neuro on 2/8; I'm curious how we will proceed and what will happen next. I also could not believe the amount billed to my insurance for 4 days of IVIG! The pain that I had from the top of my hip past my tush on my left side is gone and I'm assuming that has to do with the IVIG although not sure if it had to do with the MMN, I will have to ask her that when I meet with her next. and my left hand has gained some strength although now it seems a little shaky rather than weak. Any guesses on where my doc will take me from here?

 

[Peg B]

No not really any guesses of where your Doc will go. I would ask about compozine as a pre med as it is for headaches and nausea (my understanding of it) and using it, I have not had a headache since my first IVIG.

I am glad to hear your good results. IVIG is very expensive and a real blessing to those of us it works for. Again glad to hear your good news. Keep us posted with what your Doc does and your responses. Best Wishes, Peg