ALS or MMN?

[Nolesac]

Hello everyone,

Been reading these forums for a month now ever since MND was brought up by my Neurologist. I wasn't sure which topic to post under i have had one DR here at Jax MAYO diagnose ALS, and the Neurologist i first started going to was leaning towards MMN ( i see him again on tuesday to review results of a lumbar puncture).

I am 28 years old first symptoms i noticed around 7-8 months ago was my right dominant hand weakness and muscle cramping in the thumb muscle would lock up along with my pointer finger. Now my symptoms include my wrist is pretty much useless, muscle atrophy in right arm, fascinations, and weakness in the entire arm. All symptoms pretty much exclusive to that arm although the twitching has seemed to spread as soon as the anxiety and panic set in. Other symptoms unknown if related or not include, ive had a cold atleast 3 times since this started, joint pain in the shoulder, my right hand seems to get very cold which is not unusual for me i have had Hidrosis in my hands my whole life but it gets colder than my left hand, and fatigue. I can still play golf, video games, and Hockey. Although from what i have read i should prolly cut out the Hockey.

I have had two EMGs less than a month apart, xrays of the joints, MRI of brain, spine, and the brachial plexus.

The first EMG was done by a neuro muscular specialist who stated it was unlikely to be ALS due to lack of upper neuron presentation. The 2nd by Mayo who thinks it is ALS. The DR at Mayo called it ALS immediately without even really reviewing all i had brought regarding other visits and last EMG that werent done by his people. My original neurologist was ready to start me on IVIG but wanted to do lumbar puncture first to rule out the mimicers first. What signs of upper neuron involvement could the DR have seen that the others had not? Now I am concerned the Dr at Mayo is head of the ALS dept there, and also worked with my current Neurologist before. Will his diagnosis sway my Neurologist? What if it is MMN?

Any and all responses are greatly appreciated.

 

[Nikki J]

Your age and sex are more typical of MMN certainly. Did you have a conduction delay on your ncs? What were the results of your antigm 1 AB?
If those things were negative then maybe that is why the Mayo doctor called ALS? Did you not say to him why can't this be MMN?

When you see Neuro 1 express all your concerns. If there is any chance of it being MMN most neuros try IVIG and with your demographic I expect that will be doubly so. Hoping it is MMN. It is not a great thing to have until ALS is the alternative then it is wonderful news

 

[Nolesac]

Thank you,


Mayo said it would be very unlikely to be MMN without finding the conduction block also that my reflexes were a little brisk, but the first guy expressed how difficult the block is to find and it may not always be there. I am not sure what antigm 1 AB is? The MAYO guy has poor bedside manner immediately referred me to his Research coordinator to go over possible drug testing.

 

[Nolesac]

O sorry and also i have no family history of MND

 

[Nikki J]

Antigm1 ab is a blood panel. It is not definitive. People with ALS sometimes have abnormal readings though MMN is usually higher / a different pattern. It is not always found in MMN either but given your scenario I would think they would do it. It takes several weeks (3 I think) to get results. Maybe you had it taken and the results are not ready

 

[Nolesac]

Thank you i wrote that down to ask on Tuesday, The one thinking MMN has taken loads of blood and did the lumbar puncture which he had stated all blood work wasnt back yet. I am just worried that he will throw out the MMN diagnosis based on Mayo's diagnosis. I understand that its hard to get insurance to cover IVIg without an MMN diagnosis. At the same time i do not want to get my hopes up.

 

[laurel]

Back in 2007 my husband went through the hoops obtaining diagnosis. We paid $1500 to Athena Labs looking for certain autoimmune disorders etc. All the Athena tests were negative. Hubby had query of ALS, MMN, and CIDP. After they did two rounds of IVIG, in which he had a moderately good response to, they diagnosed him with CIDP. He has stayed on IVIG every 3 weeks, 120 Grams since 2007. Goes to hospital day care 2 days every 3 weeks to get it--he has a port in place for the IVIG as his veins gave out. He was misdiagnosed for about 2 years before getting the CIDP diagnosis so he has an atrophied right hand and drop foot in the rt. foot. The IVIG did not diminish those issues, but it maintains him so he has no further major losses. IVIG is very costly (hubby's treatment cost to the Canadian gov't is $10,000 every 3 weeks) and we had to fight to keep him on it. Right now he has a very supportive neurologist who battles the gov't for us. Good luck with your diagnosing process. Ours was the most traumatic time of our lives. Basically hubby was in diagnostic limbo until he had positive response to IVIG and it took two rounds to show any response. It is worth fighting for a trial of IVIG and make sure that appropriate loading dose is given. Do some reading about IVIG and sound informed when you talk with the doctors.

 

[Nolesac]

Thank you very much. I will do some research.

 

[lgelb]

MMN w/o evidence of conduction block, esp. in a limited study, is a known entity and you can find literature to support that.
Likewise, ALS is not uncommonly diagnosed without seeing UMN signs [yet].
There are several distal LMN syndromes that are neither, some of which may respond to immunosuppressive therapy.
But given your age/gender and sx only in one arm, did anyone mention Hirayama disease?

 

[Nolesac]

Yes actually the first Doctor mentioned it as a possibility but called it monomelic amyotrophy. When i asked the Dr at Mayo he just rejected the idea without much reason as to why it couldnt be

 

[Nolesac]

There is another ALS center here in North Florida at UF Shands i believe, i have been thinking about possibly getting a third look.

 

[lgelb]

Yes, that is another term for it. Same thing. Whenever something is ruled out, you have the absolute right to ask what test, history or exam finding(s) do so.

I think some PALS here have been to Shands so hopefully they will have thoughts.

 

[laurel]

Are you of South East Asian ancestry? If not, maybe that is why the discounted Hirayama Disease/Monomelic Myotrophy.

 

[lgelb]

It is primarily males of SE Asian origin, but not exclusively.

 

[Nolesac]

Nope. I did read it would be really rare. But all of this is rare right?