from MMN to ALS

[deedee8611]

Hello everyone,

I am new to this ALS fourm but have been on MMN foumn..Was just told that I have ALS instead of MMN..I would like to know if you will share with me the test they have done on you, the symptoms you have and how long you have had this..A few drs. are going back and forth because I get some benefit to the IVIG infusions every 2 weeks and the time that I have had this..It has been 5yrs. and mostly in right hand now just spreading to left hand and feeling it in legs and EMG test shows it in back muscles..They have done very little testing and just curious what everyone else have had done and what they feel..
Thanks so much.
DeeDee

[lisag]

Deedee..sorry to hear about your diagnosis,,I don't have ALS but my husband has had itfor over 7 years now..
IVIG was shown to be ineffective in ALS in several clinical trials many years ago..
So if you and a few of your docs feel it is helping..my guess is there is a "chance
' you do not have ALS..there is no one definitive test for ALS..but ALS destroys both upper and lower motor neurons..
At any rate ..anything you feel is helping you I'd personally recommend continuing as there are just not many proven treatments for ALS right now..Lisa

[lisag]

One more thing to add..also keep in mind the progression rate of ALS is highly variable...some people remain functional for many years..so try not to worry too much about the later stages of this disease...even if you do end up having it.. Lisa

[deedee8611]

Hi Lisa,

Thank you for responding...I sorry that your husband or any of us have this disease..How is your husband doing..Any advice would be appreciated..

The reason they changed their minds from MMN to ALS is they find no condution block and I'm still losing muscle and nerve even with the infusions..I'm not sure who to go to after the MIND Institute if they decide ALS..I learned alot about MMN in the last 2 yrs. and I have no clue where to start with ALS..What to expect..Any input would help..I'm just feel lost here..

Thanks again Lisa,

DeeDee

[lisag]

Hi Deedee...
My husband Gary unfortunately was a fast progreesor..but ALS from individual to individual can be very variable..that is why it is so hard sometimes for researchers to try to figure out what helps and what doesn't..
The upside of that though..is that you could be one of the lucky slow progressors..and if it has taken them this long to figure out it could be ALS ...that is most likely a very good sign..
Advice......in 7 years I've made so many mistakes it's tough to start..but I'll try..
I wish my husband and I would have taken things more day by day and lived in the moment not focusing so much on worrying about later stages..which honestly beleve it or not turn out to be easier in someways to deal with..but they are alot to swallow for the newly diagnosed in one big chunk...
The other advice I have is to stay on top of your general health..get an oximeter as soon as you develop breathing capacity problems at the slightest ...
Ironically... the regrets we do not have are experimental treatments..he tried..although none panned out the way we hoped.. after years I have come to the conclusion there is no magic bullet to be seen in the near future..beware of charlatans...they do exist in huge number.so be careful ..talk to your docs before you jump into anything..
ITGG...the same stuff you are taking now except intrathecally delivered in the spine... was experimented on with people with ALS in 2001 in San Juan ...although IVGG had never shown any positive effect in peole with ALS in clinical trial..
My husband is one who felt like ITGG benefited him temporarily...but at the high cost, level of pain,reluctance of the medical community as no "provable" benefit it was dropped..
My advice here in your particular scenario is ..if IVIG is still covered by your insurance..or if you and your docs feel it is beneficial at any rate and it is reasonably affordable... continue it..because the only FdA approved drug out there is Rilutek and it has very limited positive data...this is especially true if right now ALS is only a "possible" diagnosis..
Keep in miind ...I'm not a doc or even a medical professional...so any advice I give is just my opinion.. it should be discussed with your own docs...
I am sure others here have some advice...there is a wealth of knowledge ..and a host of experienes here to draw from...I hope this helps some in the meantime...Lisa

[deedee8611]

Hi Lisa,

Thank you so much for all the advice and info..I know we aren't drs. and we are just people helping each other out..Talking to each other we can help learn what questions to ask and where to go to get help..I really appreciate it..

Sorry to hear the experiments did not work out..Bless your husband for trying..It is people like him who help find the cures..

What are charlatans...

I do take one day at a time and try not to think about dying but instead I think about living and each day is a blessed day..

Thanks again,
DeeDee

[lisag]

Hi Deedee,
That a great attitude...it is hard not to worry about things that may come down the road..but you just can't...living in the moment is the way to go..
Charlatans..are snake oil salesman..promising cures that don't exist..watch out..there are many that exist for this disease..it is best to discuss any treat plans with your docs..
My husband and I always did..they didn't always agree with him..but felt it was his right to try what he wished..and warned him at times of possible complications...
In your case your docs seem to think the IVIG is helping somewhat ...at least some of your docs do from what you posted ...and your diagnosis isn't definite..so if you and your docs feel it helps..although research for ALS shows its ineffective..unless it is a cost factor..I wouldn't see discontinuing it..again just my opinion ...and something I'd discuss with a doc..
Truly though don't feel too sorry for my husband ..with his intial rate of progression he had a 21 month life expectancy..7 years ago. its tough I won't lie in late stages..but that constant fear is gone..and you do learn to make the best of what you have...Lisa

[deedee8611]

Hi Lisa,

Pretty much all the drs. think it is ALS even the one who ordered the IVIG infusions up for 3 months..My family dr. and my nurse are the ones who were shocked..Family dr. admitted not knowing much about it..We will find out next month what the dr. who ordered the IVIG will say next..The only way I can continue the IVIG if this dr. lets me otherwise I'm done with it..If he thinks for sure it is ALS then the insurace won't pay so I have to stop..

I try to have a good attitude but not a day goes by I don't think about the end results and it scares the **** out of me..In time I will learn to accept it but it's all so new to me yet..

The drs. won't even tell me how long I have..I guess it is hard to predict isn't it...Thank you for taking time to talk to me..I know you must be busy with your husband and I thank you again..

DeeDee

[lisag]

DeeDee your welcome..I know your scared and frustrated...and I "unfortunately " understand the insurance thing..yeah IVIG will be too expensive once your "medically labeled" with ALS...if it is what you have..in a way its good they are not rushing a diagnosis...if you and your docs feel it has been benefical at all...
Deedee...there are no set rules as how fast this disease will progress.keep in mind that it took them a long time to diagnos you..that is a very good sign for slow progression..even now it appears you still do not have an official diagnosis yet...
What I'm trying to say..is yeah ALS can move so fast some people may only have months..but there are several people who live functional lives years ...and yes even decades later...
There maybe alot of decisions you'll need to make down the road regarding invasive procedures/life support etc....but they will be "your" choice...and today is not the day to dwell on them..that road is miles away...
Do you have any problems at all walking, talking ,eating etc..?What symptoms do you have? Right now staying healthy overall..keeping a posiitive attitude and avoiding injuries from falls etc..is paramount..
Also please note..that ALS falls under the category of MND...motor nueron diseases..there are variants of ALS called PLS and PMA...with much longer survial rates and much slower decline of disability ...Lisa

[SteveS]

DeeDee, hi. I am in a similar situation. My 1st DX was MMN as of last May. My symptoms were weakness in my left hand and slowness of speech and have progressed very slowly. There are some others (fasciculations, emotional liability, swallowing problems, excess saliva) but they were so minor that my doctor didn't see them as consequential. The 1st EMG showed conduction block in my left arm and leg. I never have have had any problems with my legs. I did a week of IVIg in August and got zero results. So I went for a second opinion and this doc says it's ALS. Even the first doc is starting to think so. He now tells me that my first EMG was done so early in the progression of the disease that the denervation was not yet measurable. Anyway. I am going in for a third opinion October 17th. I have accepted that it is probably ALS but it would be nice to know something for sure. I am encouraged by reading these forums to see that there is hope that this thing can go very slowly. I hope for you and me that the docs can determine that it is something else but in the meantime I enjoy life every day; I hope the same for you.

Let me know if you get any updates or if the IVIg starts working better. I think I have one more week to be scheduled before I am cut off so I hope to finally see some benefit too.

Take Care,
Steve

[isic]

What is termed "ALS"? Non MMN neuron death? Neuron death with no KNOWN cause? What is termed ALS?

[lisag]

Hope this helps answer your questions...ALS falls under a larger umbrella called Motor Neuron Disease...ALS is a motor neuron disease..but not all motor neuron diseases are ALS..PLS is typically a non fatal form of MND...as it attacks the upper motor neurons but not lower..Lisa

http://www.wfnals.org/guidelines/1998elescorial/elescorial1998criteria.htm

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

[isic]

Dear lisaq,
Thank you for responding, but your response does not address my question. You, and the website, only cite symptoms. Is ALS a neuron death disease with no known cause?

[lisag]

Hi..I am sorry..I thought you were asking what was ALS by clinical definition?
But, to answer your question..yes...ALS causes both upper and lower motor neuron death..there is no definite cause..in some heridity cases it is linked to a defective SOD1 gene..but in 90% of cases the cause is unknown and the term used to describe it is sporadic.. most research today points to genetic components and environmental factors combined....Lisa

[deedee8611]

Hi Steve,

Thank you too for your input..I have been with the MMN fourm for almost 2 years..One round of IVIG might not have been enough to give you any positive results..I started out getting them every 2 weeks and it took several of them before I started seeing results..On the MMN fourm everyone was different..some felt results right away and others it took several before they felt the results..I would see about getting them for awhile before giving up on them..One reason they changed their minds on me is because I have no conduction block..I wish you could have read the MMN fourms before this went down..I would really question the ALS..See several drs..if you have too..I get my next one tomorrow..This dr. is allowing 3 months more cuz the question about it helping me..I go back to him Nov. 14 and will have him talk to my nurse who has been giving me the IVIG for the last 2 yrs..It helps me but am still losing..Not sure why but that is another reason they changed it to ALS..Well be glad to answer any questions I can...thank you...

Lisa,

My symptoms started out with cramping in the right fingers in july of 2002..That is when I first went to the dr. so it was before then..For a long time it was just the cramping of fingers..then I started getting fasciculations and muscle wasting on the top of my hand..In Aug. of 2004 was told I had MMN and started getting IVIG infusions every 2 weeks..After several months I was almost back to normal when they dropped me down to every 3 weeks..In 3 months time I went down hill fast and lost alot..went back to every 2 weeks and gained some back..then in June of 2006 after another EMG test they changed it to ALS and stopped the infusions..took me 3 months to get into the MIND Institute and in that 3 months I lost almost complete use of the hand..this dr. thinks it is ALS too but ordered up the infusion for 3 months to see what happens..After the first one noticed some improvement..I had a claw hand and had trouble opening up my and and couldn't get the fingers straight..Now I can almost get them straight again..I have had twiching of the face and tongue and I have noticed some slow speech at times..It comes and goes..Once in a great while have trouble swallowing but that has happened only a few times..when they stopped the infusions the end of 2nd month started feeling it in the legs..fluttering and cramping..since starting the infusions up the cramping is very little and the fluttering is calming down..the last EMG shows it is started in left arm and both legs and back muscles..I have felt fluttering in all this places so I know it is traveling..I still have full use of left hand and both legs..
I did not know there was variants of ALS..will read up on that..
Thanks again..I seem to learn more each time we write..

thank you steve and lisa..

DeeDee

[lisag]

Deedee..
I am sorry to hear of your situation..
My advice to you..is to see if one of your docs who feels IVIG has helped somewhat will hold off on trying to diagnos ALS too quickly....
If they can't do that... because you have met "definite" criteria for ALS ...possibly look into discussing trying to get off label usage for your particular case covered by insurance ...( although this last scenario will be tough as gamma globulin is very expensive)
As far as ALS goes..I wish I could say Rilutek ( the only FDA approved drug ) or "current " clinical trials that are out there would be better...I'm sorry to state I just don't feel that way right now..
Steve,
I too agree with Deedee..eventually people do have to accept they have ALS if they do...but I'd make darn sure it is what you have and get second opinions before accepting it..
In both your cases if your progression is slow..it is a very good sign.. I hope it continues to be that way.,Lisa

I also hope both of you get in touch with the research org called ALSTDF...they are one I highly recommend to all PALS ,,they also have a researcher there who can help with many of your questions better than I...the website is www.als.net

[deedee8611]

Hi,

Can any one tell me if they experience numbness in the forehead...This seems to come and go...Not sure if it is a symptom of ALS or not..

Steve,

I was on the MMN fourm for 2 yrs and still am..I have read a few times that there are people with MMN who have twiching of the tongue, swallowing problems and hoarsness..Not a lot but a few have so I would check into that..

Thanks,
DeeDee

[SteveS]

I've never had numbness anywhere. The only seemingly weird symptom I've ever noticed is a constant nasal congestion...

Unfortunately, a had another EMG on Friday and this time my doc found no trace of the conduction block that led him to diagnose the MMN last May. Coupled with the lack of any response to the IVIg and he has changed his dx to ALS like my 2nd opinion. I still have a 3rd coming on Tuesday.

I am not pessimistic but try to prepare for the worst while I hope for the best. Thanks for your tips and advice.

Cheers,
Steve

[deedee8611]

Hi Steve,

How can they find conduction block one time and then have it disappear..They never found conduction block on me...I have problems with nasal congestion too..That started when I started the IVIG infusions..
How long did you get infusions and how often..As I said before, everyone responds to the IVIG at different rates..It took me several before I started to notice that it helped..That is why I question the ALS is the infusion do help me even though I am still loosing..
Please let us know what you find out..Good luck..

DeeDee

[lisag]

Deedee and Steve....please keep us updated on your situations...
DeeDee....I certainly know very little about MMN...just a little as it was first investigated in my husbands case...but ruled out for many reasons in his case we know he has ALS ....however...I thought I read somewhere that there are some people who do not have conduction blocks and still have MMN...am I incorrect on this ?
At any rate, I do know that numbness is not a symptom of ALS ...I would tell your doc if your experiencing this at your next appointment..as such symptoms tend to point away from the disease not towards it..however stating that..I do not wish to get your hopes up too high...as it is quite possible you could have other things going on and still have ALS...Lisa

PS...Found a reference...came from a thread I posted earlier on clinical defintion of ALS ...
http://www.wfnals.org/guidelines/1998elescorial/elescorial1998criteria.htm
Checkout this paragraph...from the above link...
ALS-Mimic Syndromes - These syndromes occur as a consequence of other, non-ALS pathogenic processes, and do not represent other forms of ALS. ALS-Mimic Syndromes include the post-poliomyelitis syndrome, multifocal motor neuropathy with or without conduction block; endocrinopathies, especially hyperparathyroid or hyperthyroid states; lead intoxication; infections; and paraneoplastic syndromes.

Also to the issue of insurance coverage and gamma globulin..personally I'd try to use this arguement..ALS is very rarely diagnosed as "clinically definite" at first...there are two categories almost all PALS are put into before a "definite" diagnosis can be made.."clinically probable" "or clincally "possible"...if you did intially have a diagnosis of MMN...I'd try ask the physician if there were any tests that showed ALS was definite..if only clinically possible or probable ..then I'd ask why can not the IVGG be continued.and covered by insurance..especially DeeDee in your case where you feel it has helped some...to me it would be worth a shot to present this type of arguement ...

[SteveS]

Good morning DeeDee and Lisa,

Well, I've had doctor #3 tells me it is ALS. No real surprise there as I have all of the sx even if they rank only 1 or 2 on a scale of 10 in severity. The best guess about why the conduction block was measured in May was a misread coming from the cervical stimulation??? In other words, the block was measured not in the extremity near the hand but nearer to the shoulder and was measured not when they shock from locations on the arm but from when they put that shocker on the back of the neck. Coupled with the fact that I was so early catching this that the sx had not manifested themselves so strongly as to give a really good reading, the May EMG was likely incorrectly interpreted.

I only did one week of the IVIg and my doc now won't order more. I don't necessarily disagree now that I've had 3 independent docs tell me the same thing. My family has been really strong so far as I have just told them this past weekend. I am planning to go to some of the local support group meetings because I have soooooo many questions. But all and all, life is still good.

Lisa, how long has your husband been dx'd? Is his progression slow or fast? DeeDee, yours is slow isn't it? Feel free to e-mail me at smoothsaling at yahoo dot com (note, 1 "i", not 2).

Talk to you later,
Steve

[lisag]

Hi Steve,
I'm sorry to hear about your diagnosis..but I know several PALS who remain fiunctional years and a few even decades after diagnosis..So all hope is not lost...though I feel planning ahead in regards to equipment needs is a smart move on your part...
My husband Gary was disgnosed with bulbar ALS in 1999..He is currently on a vent as of June 2004 only comes off the vent for showers 30 minutes to an hour ...He was considered a moderate progresser at the time. for bulbar ALS a typically more aggressive onset...coincidently or not downgrade to a slow progressor after experimental ITGG ( intrathecal gamma globulin) in 2001...although at that time ALS had already left him unable to walk, talk or swallow anything but pureed foods...ITGG never went to clinical trial as the results were not strong enough..only a pilot study was done..which showed no "significant " results..
IVGG ( intravaneous gamma globbulin (is something I could never recommend a PALS to continue out of pocket)..As it is expensive and failed in clinical trials...however in Deedee case I do feel it is worth pursuing " insurance coverage" if she and her docs feel she had slight improvements..
Again so very sorry to hear of confirmation of your diagnosis....but please know ALS goes at an individual pace..there are no definite timelines with this disease..staying ahead of equipment needs is smart..but dont dwell too much about what may happen in the future..take it a day at a time..Lisa

[deedee8611]

Hi Steve,

I'm really sorry to hear that the drs. say ALS..I have had 6 drs. say I have ALS but one is questioning the IVIG infusions cuz I say they are helping me..So I haven't given up yet that it might be something else..My symptoms started about 5 yrs ago..so far it is done most of the damage in the right arm..I do feel it in the left arm and when they stopped the infusions for 3 months by the end of 2nd month started feeling it in the legs..They have all said that it is the slow kind..They said it could be several years before the other arm is like the right one..Or they have said that it could just stop and not go anywhere for awhile..So many different opinions that I'm not sure any more..I do know that MMN and ALS are very close in symtoms..I also know that it took several infusions before I felt the effects of it..Some people might get relief from the first one others take several..Might be worth checking out the MMN site..Multifocal Motor Neuropathy...there are alot of people on there that were told they had ALS and then told MMN or CIPD..If you still question the dx please keep seeing drs..If you want to e-mail me with any questions please do at deedee8611@aol.com..I would be glad to talk with you...
Good luck Steve...

Hi Lisa,

I will keep you up dated..I go back to the MIND Institute on Nov. 14th and I'm going to have him talk to my nurse of 2 yrs. on my condition..I get some relief from the IVIG so that is why I am questioning the dx..I learned on the MMN forum that there are several people who don't have condution block and also a couple of them have had twiching of the face and tongue and slow speech..Most people don't have that but it can happen and there have been cases of it reported..I know that there is a good chance I do have ALS...I will be having a good talk with the dr. on Nov. 14th..
As you told steve I take one day at a time and enjoy everyday instead of worring about what time I don't have..It makes you apperciate every day more..keep in touch..

DeeDee

[lisag]

Deedee,
The fact that you were diagnosed with MMN for 5 years and are still highly functional is what I find intriguing..it is quite possible your progression was a very slow one...and that IVGG was merely a placebo effect..
What I wonder though..is even if you do have ALS could it be possible it is helping slow your progression..the clinal trials that were done were very small on IVGG in which it failed...as it is very expensive..it is certainly something I could never recommend continuing out of pocket..as your finances would be depleted very quickly...
However, the slower they diagnos ALS "officially " in my opinion..the better off you are..if you truly do have ALS..as otherwise it would probably be very difficult to get an IRB for IVGG continued insurance coverage..
I do hope it is MMN that you have..but with 6 docs thinking it is ALS..it is quite possible it could be ALS ..however the question to me is then no matter what it is..is the IVGG helping slow your progression?...
I do not wish to give you false hope..but on the otherhand if it is possible it is helping I do hope you can continue IVGG as long as possible..and I'd try to fight the "official" diagnosis of ALS for insurance coverage of IVGG as long as possible,,as the other route for a IRB would be much harder..if not next to impossible..
Please keep in touch and keep us updated..hang in there and take it day by day...Lisa

[lisag]

DeeDee,
I'm double posting..but want you to see these links..as this refers to what you are taking just a different method.for ALS...again look at the findings it was dropped as the expense and lack of significant evidence ..but it could have helped slow progression for some..my husband was one of those 39 patients tracked and coincidentally or not was down-graded to a slow progressor..it by no means stopped the progression of his ALS..but I will always wonder did it really help or was it coincidence...

http://www.als.net/treatments/reviews/reviewsarticle.asp?articleid=1653

Here is a link to a study showing why IV gamma globulin ( the method you are using )was not further invsetigated for ALS...

http://www.als.net/research/studies/publishedClinicalTrialDetail.asp?studyID=6

Notice neither study rules out the "possibilty" it can help slow the disease..however the reason stated for dropping it is due to the expense outweighing the benefit....who is to say if given early and for long periods of time as you have had it..the benefits could not be more substantial in slowing progresssion ... which is my theory on this even if turns out you have really had ALS for all these years instead of MMN......Lisa