Re: Ultrasound in ALS diagnosis of ALS
I read abstracts of the Awaji criteria a couple of years ago now. At the time I came across them, this forum was flooded with scared twitchy people, and I thought if the Awaji standard was discussed here at all, there could easily be widespread panic among those already so inclined to panic, and not listen to reason or their neuro (or even get themselves to a doctor in the first place).
If ultrasound and Awaji become frequent topics of discussion here, hopefully, from the very first its talked about (i.e. NOW) and every time thereafter, those responding are able to steer those who are scared back to considering the entire picture of what is involved with considering MND, and what the manifestations of MND are, other than fasciculations.
That said, the concept of using ultrasound is of much interest to me in my own situation. I don't talk publicly much anymore about it, but, for the sake of what is being discussed here I will.
I originally was diagnosed with bulbar onset of ALS. Even back then I was not ‘typical,’ but, I met the El Escorial criteria. I'd had two EMGs of my bulbar region that were diagnostic for MND, I had abnormal reflexes throughout part of my body, but not all of it, and I had clinical weakness of the bulbar region. Spinal tap, MRI of brain stem, blood labs, and all other diagnostic testing turned up nothing abnormal. I had undergone increasingly aggressive immune-modulation therapy with no results at all; hence, inflammatory ALS mimics were ruled out.
However, when the weakness made its way into my limbs, and subsequent EMGs still were normal in those areas, it became more complicated. Eventually I had a single fiber EMG, which was abnormal too. Myasthenia Gravis had already been ruled out, but we tried Mestinon, just to be able to say that we did. It did not help, and made me worse.
At this point, my neurologist explained to me that even though I technically met diagnostic criteria for ALS, that it was her opinion that I had an unnamed MND variant.
My progression has been very slow. Early on I needed help with breathing and am reliant on bipap when I lay down, or sleeping (even sitting up asleep) I need it. I do have a PEG and use it for between 25% and 85% of my nutrition depending on the day. Testing revealed my swallow was impaired in the late winter/early spring of 2008(don't remember exactly); However, it was more than 3 years after that before the PEG was placed.
I believe that I am one of the people who would benefit greatly from the ultrasound guidance. I've not had an EMG in over two years. At this point, I cannot lift my right arm much due to atrophy of my scapula. It is 'winged' and gets moved out of place easily. Of course I have other issues more areas than just the shoulder and bulbar region, but the scapula weakness and atrophy is the most clear- cut example.
When I saw my neurologist last month we discussed perhaps having another EMG focusing on that shoulder blade. This differs from how many of us have EMGs performed. Oftentimes it’s the areas that do not seem to be affected which are tested first, because (as we all know if we've been reading here) the EMG picks up abnormality before the person senses a problem.
Like Helen, and Barry, I have never been very twitchy. Just am not. I notice more fasciculations when I'm tired. I was exhausted yesterday, caught sight of myself in the mirror, and the area between my eyebrows over the bridge of my nose was having a dance party! (But I couldn't feel it) so, who really knows when they're experiencing them if they can't actually feel them, and don't happen to see them.
In my completely non-professional opinion, the lack of EMG findings in my limbs up to this point (albeit its been 2+ years since one was performed) is related to the slowness of my progression. It goes hand in hand with the common acceptance that the longer it takes to diagnose ALS, the slower the progression will be, and the inverse, the clearer the picture, and quicker solid diagnosis; the faster the progression.
