PMA questions

[Mike29]

I was wondering if someone can help me with some questions here on PMA my first EMG suggests that spectrum of lower motor neuron only. I did have a second EMG that shows no changes in since prior 6 months ago. I am now at 16 months with no real diagnosis. I cant seem to find much info on progressive muscular atrophy and was wondering about if my first EMG was correct with my symptoms. I have no one to ask but my doctor who tells me to keep waiting till my next EMG that will be 2 more months from now.

I hope I am in the right section so this post, if not maybe someone can direct me.
Thank you for your time.

 

[lgelb]

PMA is a "real diagnosis" that signifies ALS-like progression with no upper neuron sx as yet. I say as yet, because most people end up with upper motor neuron dysfunction as well and progression seems similar to ALS. For that reason, PMA is not a widely-used label any more; most docs would say probable or possible ALS depending on the rest of the clinical picture.

EMGs wouldn't reveal upper motor neuron problems anyway; that is done w/ physical exam and symptoms and is a bit kludgey. Many people start with LMN dysfunction only, so you are not unusual. However, to get Medicare/SSDI/private disability/other benefits, you may "want" an ALS code so you can ask about that.

More significant to your prognosis would be if you had the flail arm or flail leg ALS variants. This would be where your arm(s) or leg(s) look like match sticks, essentially, and were the starting point for the disease. These variants generally have a longer course than "regular" ALS (median survival 5y for flail arm).

If your first and second EMGs were consistent and you have continued, not-improving, progressive weakness, the overall "motor neuron disease" label wouldn't normally change, but you should always get a second opinion at a neuromuscular dz center. In NYC, there are plenty but let us know if you need a suggestion for a center.

Best,
Laurie

 

[LifeEnthusiast]

Sorry for the late reply. As it stands now, I too have a PMA diagnosis. Laurie explained PMA better than I ever could. She's spot on.

 

[Dusty7]

This is from an older post of mine, but is still relevant...

The ALS, PLS, PMA Name Debate

Gowers, a founder of British neurology, stated in 1899 that he had never encountered a single case of PMA in which the pyramidal tracts (UMN) were unaffected. He did not think the introduction of the term “ALS” by Charcot (founder of Neurology) to be very helpful because it implied that the primary lesion was degeneration of the pyramidal tracts and that atrophy of anterior horn cells was secondary. He felt that Charcot's distinction in effect gave a new name to an old disease. He concluded that PMA, PBP, and ALS were "essentially one disease."

Brain in 1933 introduced the term "motor neuron disease" so that apparently different conditions could be brought together in a single general category and used the terms “motor neuron disease” and “ALS” interchangeably.

A series of studies have supported the opinion that PMA, PLS, and PBP were subsets of ALS.

Swank and Putnam in 1943 analyzed 197 patients. They considered PLS and PMA to be ALS that had yet to develop fully.

Lawyer and Netsky in 1953 did an analysis of 53 patients with ALS and concluded that motor neuron diseases form a group that includes PMA, PLS, ALS, and PBP.

Mackay in 1963 reviewed 126 patients with ALS and concluded that regardless of the onset, the spastic forms nearly always become atrophic, the atrophic also spastic, while the spinal forms nearly always become bulbar, and the bulbar forms, if the patients live long enough, become also spinal. The entire group is, therefore, best regarded as a single degenerative disease, ALS, which constitutes a spectrum of atrophic process at one end, spastic at the other, and both in the center. With time, cases at each end of the spectrum move towards the center.

This is where most neurologists appear to be today—with PLS (UMN signs only) on one end of the spectrum, PMA (LMN signs only) on the opposite end of the spectrum and ALS (UMN and LMN signs) in the middle, to which both ends are drawn.

Problems
1) MND, since Brain introduced the term has been used interchangeably with ALS which makes the term confusing at best.
2) The billing codes do not agree with current neurologic thinking. Instead
Amyotrophic lateral sclerosis is 335.20
Progressive muscular atrophy is 335.21
Primary lateral sclerosis is 335.24
And Motor Neuron disease is coded 335.2, which is different from ALS’s code, though the man who coined the term didn’t differentiate between the two.

The Real Problem
The real problem is that, while all the above diseases should be called ALS, the bureaucrats don’t understand that. ALS carries major benefits, while sometimes the terms, PLS, PMA and even MND may not. BUT THEY SHOULD. It is up to you to make sure your billing is coded correctly. Talk to your neuro about the coding problem and if he won’t agree with most other neuros that PLS, PMA and ALS are all the same thing (ALS) then keep fighting with him or find another neuro.

 

[LifeEnthusiast]

Wow, Dusty. Thanks for the information. It's helped me grasp things in leaps and bounds and cleared up a ton of confusion I had. Again, thanks for responding.

 

[van1]

Dusty you are very informative. Thank You.

 

[Mike29]

I still at this moment do not know for sure if it is PMA or something that is a mimic of MND seems this is my third neuromuscular I have seen and she will not yet say what I have for sure. She has performed a battery of blood work that all shows nothing and EMG and NCS to see if any change has occurred since my first EMG/NCS in Sept. 2014. She then said no changes and no further denervation was seen, my clinical exam she said was good.

The first test was done by a hospital EMG facility and that test was never discussed with me by my first Neuo who said not to worry about the EMG So I requested copies of all tests and his notes on me. I read the test but dont understand them completely as to why it was put into PMA, On my second test my neuro said no changes but said it was atypical of MND and possible some spectrum of peripheral nerve hyperexcitability.

I was hoping to see if someone would know what to look for in the EMG test and what I should ask my neuro on my next visit in 1 month. I was hoping to talk with someone who has this to see if my symptoms where similar, I feel like I am losing my mind past 17 months, I have read so much and these topics and still lost, my last hope is to talk to someone who may have the dreaded disease, it amazes me how little doctors know on neurological issues.

My symptoms started last May 2014 just with cramping in my hands and stomach, then I noticed my right biceps where twitching, went to see my GP doctor who referred me to a neuro who then sent me for the EMG . Seems a few months go by and now my shoulder and right leg are doing the same thing and very painfull muscles all over. Now 17 months later there is not a muscle in my body seems not to shake and twitch, some are jerks of the muscle and some cramping at time very painful. I have a hard time functioning these days with so much pain, jerking and tremors.
Is this the sign of MND, I have seen like I said a regular neurologist and 2 neuromuscular. I tried going to Dr Mark Sivak in Mount Sinai NYC who was supposed to be a specialist with this but after 1-1/2 hr exam and questions just told me come back in 3 months, I moved on to my last doctor who is now in Westchester Medical Center Dr. Thomas an Assistant Professor of Neurology. I sorry to give such small bit of my story. I am hoping that this is not MND but I feel it might be for me.

I thank you all for your time and information you have provided.

 

[Atsugi]

Mark, to be sure, I'm not a doctor. I know ALS fairly well because my wife (a doctor) died of ALS, I discuss ALS with the members of this forum, and I read a lot about ALS. Still, I'm no expert.

The symptoms you describe seem to be all Lower Motor Neuron stuff, which is consistent with PMA, not MND/ALS. The fact that your symptoms are more widespread now is certainly concerning, but they haven't spread to the UMN spectrum, so that's good.

Some of the literature says PMA is a version of ALS that eventually leads to ALS. However, some of my reading says that since the prognosis is better in PMA, it's important to distinguish the two. They have different ICD codes. I've read that some doctors will write up PMA as "ALS: Lower Motor Dominant."

One important consideration is Social Security benefits. ALS claims are expedited as a TERI (terminal) case and automatically given contingent approval. PMA is not. Medicare eligibility is immediate for ALS (although it often takes 4-5 months to actually start), but MC eligibility for PMA has to wait 24 months like most disabilities.

To answer your questions, in my humble opinion, you don't have ALS.

 

[Dusty7]

The easiest way to start is for you to tell us what the summaries in your EMG reports said.

If you want to do it the hard way, then you need to look at your EMG reports and see which muscles were tested and what the results of each muscle test were. Check for insertional activity, fibrillations and sharp waves. These will tell where you have acute (current) denervation. Then look at the MUPS section to see if you have any abnormal readings there--duration, poyphasicity, etc. This will indicate chronic (old) deneravtion.

ALS is a disease of widespread, acute and chronic denervation that affects the upper motor neurons and the lower motor neurons. (The EMG will only spot LMN problems. UMN problems are determined by clinical examination.) If your EMG does not show both acute and chronic denervation, it's not ALS. If it shows both acute and chonic denervation, it still may not be ALS, as it could be an entrapment neuropathy, a peripheral neuropathy, or something else.

So, what do your EMGs say?

 

[Mike29]

There are 10 pages to my EMG study from the first one is there a way I can post it here as a pdf or something and cut out my personal info?
My second EMG was given by my neuromuscular and i have no reading but a summary of what she tested I can copy the info and post it that way Dusty

 

[Mike29]

I figured a way to get the info in here from word this is my last visit has my EMG info and what was tested and I still have my first one that I will copy over after you see this most recent.
I dont understand most of it only what she explains to me but I feel short on questions to my neuro.

MRI brain 4/27/15: There are a few scattered small T2 and FLAIR hyperintense foci predominantly involving the subcortical white matter of the bialteral frontal lobes. There are nonspecific.
CT Chest: no tumor. Emphysematous changes.

MRIC-spine 9/4/14: C3-C4: central disc osteophyte. C4-C5: broad disc osteophyte, eccentric to the right with mild canal and right»left NF narrowing. C5-C6: Disc osteophyte, left greater than right foraminal narrowing. C6-C7: moderate b/l NF narrowin. C7-T1: Mild canal and foraminal narrowing.

MRIT spine 3/23/15: At C6-C7 and C7-T1 there are shalow disc bulges with mild to moderate bilatearl neural canal stenosis. T2-T3 and T3-T4: shallow left parasagittal disc herniation. T6-T7: Small left parasagittal disc herniation.

MRI lumbar spine 3/16/15: L3-L4: mild diffuse disc bulge with mild bilateral NF narrowing, right greater than left. L4-L5: Mild bilateral NF narrowing.

MRI of the abdomen 2/9/15: Hepatic hemangioma. Left renal cyst. Evaluation/EMG at

Montefiore 9/16/14: decreased left superficial peroneal. Minimal slowing of left peroneal motor. EMG: fasciculations in almost all muscles tested, other than tongue. Neurogenic units in teh right triceps, deltoid and FDI. Left deltoid and FDI. Progressive muscular atrophy?

Patient with diffuse muscle twitches, including tongue.
EMG 3/10/15: In summary, the fasciculations seen in the thoracic paraspinal muscles, arms, legs and tongue in addition to mild neurogenic changes in the arm and leg are suspicious, but not diagnostic for motor neuron disease. The lack of significant change from the prior study done 6 months ago is atypical for motor neuron disease, particularly the lack of active denervation on EMG. Superimposed radiculopathy was not full excluded. The minimal sensory changes in the arms are of unclear etiology in isolation; this may be technical or related to mononeuropathies that are not fully characterized on this study. The presence of an underlying sensory neuropathy is less likely with preserved sensory responses in the legs. F/u study is recommended in 3 to 6 months.

Bloodwork 3/16/15: Normal HgbA1c, zinc, IFE, cryoglobulins, lead, b1, GAD, Hu, GM-1, Sulfatide, c-Anca, panca, gliadin, vitamin E, voltage gated potassium channel antibody. B6 high 69 (21)

I thank you all for your help.

 

[Atsugi]

I'm sorry to read that you are in pain. I hope you can find someone with PMA who can understand and give advice.

As to ALS/MND, I'm not a neurologist, but the statement "lack of active denervation" and the fact that you don't have any paralysis pretty much say you don't have ALS.

 

[Dusty7]

The most important parts of the EMG summaries are:
9/16/14--...are "not diagnostic for motor neuron disease..."
His final words, "Progressive muscular atrophy?" seem more like a guess than a diagnosis, especially with no findings of acute denervation, but I'm not a doctor, so...

3/10/15--"...the fasciculations seen in the thoracic paraspinal muscles, arms, legs and tongue in addition to mild neurogenic changes in the arm and leg are suspicious, but not diagnostic for motor neuron disease."

And "The lack of significant change from the prior study done 6 months ago is atypical for motor neuron disease, particularly the lack of active denervation on EMG."

So, I'm not seeing ALS, or even the LMN predominant version (PMA) in the summaries you posted--not without active denervation"--but I would agree you need follow-up EMGs.

 

[Mike29]

Thank you all for you responses, I guess I will have to wait and see what comes of next follow up's. I would imagine that since every ones symptoms are different it will not mater to try to compare to narrow down whats going on. I feel so lost and my family is suffering with me. I pray for all of us to find peace.
I am sorry to take your time away on my problem.

 

[Nikki J]

I agree with Dusty. I understand it is frustrating not to have a solid answer but often in neurology it is wait for things to develop ( or not) and in many ways not having an answer quickly is good in that whatever it is is not progressing significantly. Some people ( including Mike's wife) go from fine to dead in less than a year. Try to enjoy the next couple of months if you can. To me it sounds hopeful as things have been stable and are not currently MND but it really seems as if your doctors have been very straight with you and are not giving a diagnosis because there is not one to give