Is this PMA?

[StillSteve]

This is my first post to this forum, and I first want to express my thanks to and admiration for all of the PALS and caregivers here. That you find the time and energy to contribute to this forum is awe-inspiring and humbling. You have my deepest respect.

I know from reading posts to this forum that there is no set pattern in ALS or upper motor neuron diseases. But I am curious to know how many others have histories similar to mine. My neuro has told me that "unfortunately, ALS is a possibility," but I have no diagnosis. My symptoms seem to match PMA pretty well, though I'm hoping that multifocal motor neuropathy (MMN) is ultimately found. I will (as briefly as possible) give an outline of my history, and then ask a couple of questions.

Here is where I am at: I first noticed cramping in my left hand about a year ago (perhaps May of 2009, but I'm fuzzy on the exact timing; it could be 10-14 months now). At least six months ago I noticed loss of strength in my left hand (couldn't use fingernail clippers; trembling). I saw a doctor (routine checkup) three months ago (Feb 2010) who noticed significant atrophy in my left hand and arm; at around this same time I first noticed fasciculations. These fascics are occurring from my shoulders to my upper legs and continue to this day. Most are in my left arm, but they've occurred everywhere. I'm not sure, but I think I have felt very slight fascics (fibrulations?) in my face four or five times.

I've had two abnormal EMGs (mid Feb and two weeks ago). Most of the abnormalities found in left arm. Had one "long duration" VMUAP in my right arm (first EMG), and one fascic found in upper back (second EMG). I was told after second EMG that left arm is getting worse and a fascic in back (she could have found others if she tried to, I'm sure), but nothing "significant" in other extremities. I would say that the "needle part" of my EMGs has been thorough in my left arm, and very limited in my right arm (both times). There was some limited testing of my lower legs (both the first time, just the left the second time). I've had thorough NCVs of the left arm both times, limited on the right arm and both legs the first time. Thorough on the left leg the second time (none on the right arm).

MRIs (very thorough) showed nothing very unusual; slight compression but not in the right place or bad enough to explain everything in arm (let alone widespread fascics). Blood testing all normal, except for an initial test which showed slightly elevated Lyme antibodies (but follow-up Western Blot test was negative).

To date, I haven't noticed any bulbar symptoms (no trouble swallowing, no fasics in tongue, no pseudobulbar, etc). No upper motor neuron signs (according to my neuro and as far as I can tell). No balance issues.

The atrophy in my left arm is definitely getting worse. My left pectoral muscle is clearly atrophied as well (from lack of use?). I continue to touch type (for example) effectively with my left hand; coordination is OK, but because of weakness I have trouble using the shift key consistently. No atrophy in my right arm or elsewhere that I can tell.

Subjectively, I feel a generalized weakness. Tendonitis (which I've always had) seems to be getting worse, particularly in my right arm. I have subjective feelings of weakness in left leg greater than right leg. Feeling of "deadness" and, occasionally, numbness/"pins-and-needles" in my left leg greater than right. I get exhausted easily after physical exertion, though I am continuing to play some softball and volleyball.

I realize that stress (depression?) may be contributing a little or a lot to my feelings of weakness and physical exhaustion. Though I continue to be active, I'm not as active as I was six months ago or so. I will turn 50 this summer.

At the best of times, I feel pretty good except for feeling tired and weak (subjective), and except for my left arm.

My neurologist is in the process of getting me a referral to a specialist at the University of Minnesota. I was disappointed to hear yesterday that the doctor she had mentioned to me last week is "not taking new patients" (he has a worldwide reputation and his work, particularly on MMN, and is widely published). She (my current neuro) has told me that she can't diagnose me with ALS, since I am presenting symptoms in only one arm (she said--incorrectly if I understand the literature--that involvment of three of my four limbs is required). But she clearly believes I have ALS, and told my me and my wife that MMN is a possibility, but that I do not have motor conduction block. She said that the only other possibility is monomelic amyotrophy (which is absurd).

I apologize for the length of that summary. Now for my questions:

- How typical is my history, given that one year after I first noticed anything, my symptoms seem to be limited to my left arm (except for fascics)?

- My neuro told me that I don't have motor conduction block (MCB), but I've read that this can be difficult to detect and I have very little confidence that the NCVs were thorough enough to be definitive. Does anyone have any experience with MCB and if so, was it easily determined?

- Should I just (try to) be patient, understanding that if I do have PMA or ALS, it will become clear in time and that there is nothing I can do to change that, or should I agressively try to get a definitive answer because if this is something else that is treatable (MMN?) I am losing valuable time without treatments?

Thanks for reading, and thanks for any help you can provide.
B

 

[sadiemae]

I would push for a DX if you are a Veteran, or are no longer working and need disability. HUGS Lori

 

[halfin]

Welcome to the forum, Bachster. I like your name and photo.

If you could type in the results of your EMG test, we have a member here named Wright who is very knowledgable in interpreting them.

Unfortunately I don't know much about MMN and nothing about MCB, sorry. I thought they did blood tests to rule out MMN in my case.

Your progression is slow for ALS or PMA but not unheard of. If, worst case, you should turn out to have one of these, the slow progression would be a good sign.

Some doctors follow what are called the El Escorial criteria for diagnosis. This divides the body into 4 regions: legs, chest, arms and head. They want to see symptoms in 2 or 3 regions for a probable or definite diagnosis of ALS. Not all doctors follow these rules, though.

I would press for a referral to a neurologist who specializes in MND. It's too bad that one doctor isn't available, but probably others could be found. That seems like the best way to move forward. Good luck -

 

[KANSASTOM]

Why would Monomelic Amyotrophy be absurd?

 

[StillSteve]

Thanks, all of you, for the welcome.

I am a veteran, but not a war veteran or retiree, so I don't think I have anything coming from the VA. I am still employed and lucky to be insured. My sense of urgency about a diagnosis is two-pronged: 1) I don't want to continue to experience degeneration if a treatment exists (as in MMN), and 2) I just want to *know* so that I can (I hope) get past the anxiety that comes from being stuck "in limbo."

I do have the results of my first EMG/NCV study, and will ask for a copy of the second one soon. I could post these somewhere, but I assume that the forum is not the place for this. Maybe somewhere in my profile area; I haven't explored that much at this point.

My calling monomelic amyotrophy an "absurd" suggestion may just be a reflection of my ignorance, but what I have read about it indicates that it is endemic to parts of Asia (India and China?), strikes 20-25 year-old males almost exclusively, and would not present with fasciculations in unaffected areas of my body. I'm 49, from the U.S., and have widespread fascics. I'd love to be proven wrong, but it this seems an unlikely diagnosis to me.

B

 

[laurel]

Hi Bachster. Your story is almost identical to my husband's story. His first symptoms were weakened pincher grip in rt. hand. Had EMG and NCS three times over 3 years with a local neurologist--each time neuro. said there was nothing significant. His right hand got weaker, and then he was diagnosed with carpal tunnel and had nerve entrapment surgery. No improvement and things were worse and he returned to the surgeon. Surgeon said it just takes time for nerves to re-grow. Finally we paid for a second opinion as his right hand and forearm were showing major atrophy. This doctor said hubby had PMA and needed more testing. Returned to the original neuro. for more tests i.e. NCS , EMG, MRI's. By this time hubby couldn't use eating utensils or turn a key in a lock. His thenar plane was atrophied as well as his forearm of the right arm. He was fatigued all the time and had very poor concentration. Neuro. scratches head and says he isn't sure i.e. ALS, MMN, CIDP. Hubby was referred to a neurologist at a neuromuscular disease unit. All tests repeated(but the comprehensiveness of the EMG and NCS were like night and day), lumbar puncture added, and blood work through Athena Labs. (sensori-motor panel). He was given a trial loading dose of IVIG before diagnosis, but had ALS as a differential diagnosis with this specialist as well. Just prior to getting the IVIG, he developed foot drop of the right foot as well. He responded fairly well to the loading dose and subsequent doses. He has battled fatigue, fasciculations in the affected limbs i.e. rt. arm and rt. leg, some sensory pins and needles in feet--especially after increased activity. All this started when he was around 55 years of age. He wound up taking early retirement from his job as an airline pilot due to his symptoms.

Now he is diagnosed with CIDP-MADSAM and receives $10,000 worth of IVIG every 3 weeks to maintain and not get worse. And from my reading MMN can be diagnosed without conduction block. I think often it is difficult to distinguish CIDP-MADSAM from MMN--often the person will show deterioration on steroids if it is MMN and that often is the clue to the diagnostic dilemma cases. We went through terrible stress during a lengthy diagnostic process. I won't ever forget walking under the cloud of fear about having the differential diagnosis of ALS. Hubby is 3 years into the IVIG treatment and I still watch him like a hawk--still can't shake the fear totally. Good luck and I will be praying for you that your story has a better ending than ALS.
Laurel

 

[KANSASTOM]

Yes, I kinda felt the same way when I was diagnosed with Monomelic Anyotrophy, but only after being diagnosed with ALS first. I am Kansas born white male 46 years old now and my symptoms have not really changed for almost four years now. I have only meet one other person at the MDA clinic with MMA, he was youger than me and white. I still am unsure of my diagnosis, but I do get twitches everywhere in my body which always concerns me. I pray everyday I have MMA, but I do understand your concerns. I hope you have something else than ALS.

 

[MultifocalMotorNeuropathy]

I'm a 21 year old male with MMN with CB. It wasn't until my 3rd EMG and 2nd NCS 20 months from my chief complaint of weakness that conduction block was found. It helped that I knew which nerve had issues and that I was able to direct to the technician to be extremely meticulous in going centimeter by centimeter on that nerve to find the CB. It probably also helped that my symptoms had progressed with enough time for CB to develop. Don't give up; you'll figure it out. (an electrical engineering background certainly doesn't hurt either when dealing in this space)

 

[Danijela]

- How typical is my history, given that one year after I first noticed anything, my symptoms seem to be limited to my left arm (except for fascics)?


My partner's symptoms remained confined to his left hand/arm for two years. His EMG, did, however, show changes in abdominal area and legs, even though at the time of diagnosis these were not affected (visibly).

What are your reflexes like?

Wishing you all the best. Dani

 

[StillSteve]

Thanks again for all of the responses. Some very interesting information.

Laurel: Yes, I do see a lot of similarities between your husband's story and mine. I see some differences, too, at least if I am understanding correctly. Both of my EMGs have come back "dirty" for one thing. Sounds like your husband had three years of clean EMGs before the more extensive tests by specialists turned up something. But our symptoms are similar. It doesn't sound like a picnic, what he and you are going through, but I'm happy for you that it isn't worse.

KansasTom: I guess I have a lot to learn about monomelic amyotrophy and can't dismiss it out-of-hand. Hey, Kansas isn't that far from Minnesota: maybe something is in the air. I'll take a four-year plateau right now if I can get it!

MMN: Your story of going three years before MCB was found is encouraging to me. When did you begin treatment, and has it helped? (I'm concerned about waiting years for treatment if I'm lucky enough to have MMN.) I have an unexplained, small (less than pea-sized), hard knot near my elbow where I think my ulnar nerve runs. I asked my neuro about it and she just said "you don't have conduction block" and showed no interest in the bump (didn't look at it, let alone touch it). Did you find a bump? I've read that "onion bulb" tumor-like bumps are sometimes manifested and detectable.

Dani: My reflexes are fine. Somewhat diminished, I think, but this brings up the fact that I was diagnosed in 2007 with Adie syndrome by an neuro-ophthamologist. I definitely have Adie pupil (my right pupil does not react to light), but this Dr tested my reflexes, found none (or little), and said I had the syndrome. Probably for years he said (based on the fact that my affected pupil was/is smaller than the unaffected one). This is a neurological condition that is thought to be caused by a virus (I was asked about syphilis). I've mentioned this to my neuro and she didn't give any indication of interest. She says I have normal reflexes, which is puzzling to me. The earlier doctor was a very highly-regarded doctor at the UofM, and he didn't find reflexes. I suppose this means nothing, but seems odd to me.

Thank you to all. You've given me more to think about, some hope, and I appreciate your concern. My best wishes to each of you.

B