Living with Neuromuscular Disease: My Story

ranpaumme

Member
Hello everyone, here is my story--everyone seems to be a little different. I am a 49 y o male who happens to be a physician (radiologist) but my knowledge of neuromuscular disease is really no more than any interested layperson doing diligent research on their own condition. I have been told by one of Canada's top ALS specialists that I do not have that disease, rather I have BFS. However I must confess I am still very worried, and can' t get over it. Here's why: My symptoms are 99% from the right elbow down, they started in Oct 04 with a funny aching in the right thumb pad, followed shortly after by a fluttery feeling there. It didn't register on my consciousness for a couple of weeks, but I finally realized I was fasciculating (in one or two distinct spots on the thumb pad). Then I actually looked carefully at my hands, and to my Horror!--my dominant right hand had a smaller pad of muscle than my left. Not much but enough to notice if you really looked. Oh-oh, fascics, query cramps and possible atrophy--early ALS what else? So began an oddessy of panic, tapering to anxiety and resignation of doom, still not completely resolved, although maybe it should be if I wasn't such a worrier and pessimist. My local neuro didn't think much of my story, found no weakness or other finding on exam in Nov 04, her NCV showed mild to moderate bilateral carpal tunnel, possibly a red herring since I have no sensory symptoms. Her EMG exam was completely clean. She said no evidence of anterior horn disease (ALS). Two months later nothing much changed ( the first dorsal interosseous started twitching and aching), I forgot to mention that my forearm ached and tired easily, still does, but never twitched. In Jan 05 I went to the university centre, saw the ALS specialist, no abnormality on physical exam, although he, like my local neuro, also did not notice the asymmetry of my hands until it was pointed out. He said my right hand looks like a normal hand, only when placed beside the left could any difference be seen and you really had to look. I would say the right thumb pad is 20% smaller, the right first dorsal interosseous is 5% smaller and the muscles on the small finger side of the hand are also about5% smaller than on the left. No weakness on objective testing by him. Their NCV also showed mild bilateral carpal tunnel syndrome, but again the absence of any sensory symptoms makes you skeptical of the significance of this and the fascics are not just in the median nerve distribution. Their EMG was done by a neurologist who was the head of the EMG lab, he was training a resident so they were muttering away to each other while doing the EMG. At one point with the needle in the right thumb they got a distinct "hum" lasting about a second, this turned out to be a CRD (complex repetitive discharge). I didn't like that one bit. There were occ. fascics in several muscles, and he said there was an occ. "poly" in the thoracic paraspinals which he said was normal for those muscles. At the end he said you have fasiciculations, but no evidence of ALS, he kept saying it's not the fasics but the company they keep, and there was no bad company. A week later I returned for a summary/review with the ALS specialist who said I had fasciculations and nothing else. So what about the CRD? I asked. Oh, the repetitive discharges he said, just some membrane excitability, nothing to worry about. I needed more reassurance so I said: we sometimes say things to cover our butts, like: "I find no evidence of disease X",(implying you could still have it, I just don't see it), versus saying "You do not have disease X, period".
OK, he said "I am an ALS specialist, I see this disease all the time, and you do not have it. You have BFS. Now get over it and get back to work"
I said why just in my right hand and forearm (like limb onset ***)? He shrugged and said it was a common place for BFS too. By the way, it was a somewhat tongue in cheek conversation, I think I was getting amusingly (to him) obsessional. One more thing, I did see him 5 years earlier for what to me seems now to be more typical BFS, ie sudden onset of fascics in the calves for several weeks to months, which disappeared and were forgotten. This time seemed different, weird and alarming.
Now it's about two months later, and about five full months since I noticed the asymmetry in my hands. Nothing has really changed, the fasics are somewhat less (Cal-Mag and clonazepam help), the funny "cramping which is not really cramping" is unchanged and the achy soreness and easy fatigue is unchanged and every day seems much the same, there is still no weakness ( nor should there be if it is BFS). I just keep worrying, is it atrophy, if so why no weakness, is it really slow ALS after all, if not atrophy why the difference and why is the dominant hand smaller and the one with the symptoms....blah blah blah-stop already! It drives you crazy.
Maybe my left hand has bulked up since I changed my work habits. In the days when we looked at X-ray films, I used my left hand to put up the films on viewscreens and used my right hand to clench the dictaphone. For the last 3 years, I use my left hand to literally clench a dictaphone for hours every day, while my right hand just pushes &clicks a mouse on my imaging workstation. Who knows? Sometimes it's just too much. Is there anyone out there who can relate to any of my symptoms, and is there anyone whose dominant hand is a bit smaller for any (non-pathologic) reason? My forearms are symmetric and my right upper arm is a bit more developed as I would expect, and I think always has been. I have no idea about my hand, obviously if it hasn't changed in five months, it must have been like that for a while beforehand.
 
Welcome to the board. I can relate to your story extremely well. My right hand is also visibly smaller in muscle bulk than my left - and I too am right handed. I noticed this 15 months ago right after I started twitching at random in various places. It really scared me and I've wondered all this time about how my right hand could be smaller than my left. I had a clean EMG like you and I was told repeatedly by my neuro that I don't have ALS. But I just couldn't let go of the asymmetry thing. I can do more reps with the hand grip I have with my right hand, but it's just not as bulky as my left. So I've learned to accept that having "atrophy" - brought on by a progressive disorder like ALS - in my right hand for 15 months without weakness would be exceedingly, exceptionally rare.

I hope you get some good support here.

Johnny
 
Aremm,

I have heard others worry about their dominant size having less muscle mass, so you're not alone. I don't recall any of them turning out to have xxx.

Have you read the Mayo paper on long-term follow up on their BFS patients? Years on, none of them have developed any neuro disease after a BFS dx (sample of 120 some patients). It's the gold-standard and I've yet to see any paper contradict it. Basically it indicates--if you pass a physical exam and EMG (when presenting for twitching), you're essentially in the clear. I heard one of my neuros tell one of his students about the Mayo study, saying it was a very good paper, and that if someone has a clean exam and EMG, it's benign--but he said it with the intonation that is meant to put an end to debate.

I had repeated EMGs (for anxiety really, my request) by a neuromuscular specialist at a teaching university. On one of them, he also picked up occasional polyfasic potentials but said they were within normal limits. That was almost five years ago now I think and I had another clean EMG after that. Heck if you think that's scary some people with BFS have had sharp waves and fibs but still no xxx! That would be scary.

I've also read many times that if you have noticeable atrophy, you would have to have real weakness in that muscle.

Another neuro who has had bfs himself told me you can expect a little weakness and fatigue with it. I definitely get that, especially after tongue twitching, making my speech feel clumsy (but not overtly--I don't slur, though I sometimes feel like I'm right on the edge).

The thing that really matters is whether you have true weakness. Consider this--twitching before weakness is pretty rare--only 3%-7% of ALS patients (depending on the study) present with twitching before weakness (which usually follows quickly). Twitching is evidence of established nerve damage that should show up on EMG. It is exceedingly rare for someone presenting with twitching to develop ALS after a clean neuro exam and EMG, so rare that it's anecdotal if at all, well within the neighborhood of coincidence (getting xxx unrelated to the BFS). It's more like neuromuscular folklore. I've only heard of (but haven't read) one German study on one patient who did that. My neuro said when that happens it's EMG error.

Nobody here is qualified to dx you of course, but the odds do seem to be overwhelmingly in your favor. As my neuro said during one of my panic attacks-- be strong, be strong.
 
You use different muscles in each hand depending on the tasks you do which would make your non dom hand bigger in certain places. I can carry my kids around with my non-dom arm much better.
 

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