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Justme111

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HI,

Thanks for allowing me to post. I think its great to have a site like this for those in need.

I understand that fasciculations in motor neuron disease are attributed to problems with lower motor neurons. I have clear upper motor neuron disease as diagnosed by two major clinics (brisk knee & arm reflexes, hoffmans & tremnor, jaw jerk, spasticity). My muscle biopsy from Dec 10 showed prior dennervation/reinnervation & fibre type grouping. I have weakness & fatigue with activity, slight clinical weakness in my left hand, otherwise 5/5 in all extremities. I understand that I have upper motor neuron disease. What I'm wondering about is how can I have fasciculations everywhere without this showing on the EMG? Fasciculations are supposedly indicative of dennervation.

I've noticed some slight atrophy in my left pectoral muscle and possibly some in my left leg, but could just be due to weight loss in my left leg. otherwise no real obvious atrophy.




That said, my most recent very thorough EMG was normal. How is it that I can have motor neuron disease with widespread fasciculations (everywhere on my body - 20 to 50 an hour), and still have a NORMAL EMG? Someone that understands EMGs in detail please explain this to me. Thanks so much!


Emg report Said:

"Extensive nerve conduction studies and needle EMG were carried out in the left upper and lower limbs, as well as needle EMG in the lumbar and thoracic paraspinal muscles. The study is normal. Other than a couple of fasciculation potentials (of normal configuration) in the left abductor pollicis brevis, no spontaneous discharges were seen and the motor unit potentials were all normal in configuration and firing pattern"



I think it's hightly unlikely that I would develop motor neuron disease AND fasciculations that are BENIGN at the same time.

This is what puzzles me and is basically my question -- Why aren't my fasciculations showing up as abnormalities on the EMGs nor my weakness? Everything I've read says fasciculations and weakness= lower motor neuron disease. Any help would be greatly appreciated.
 
How did your neurologist explain the test results and what diagnosis has he/she given you?
 
hi justme.
i have pls/umn disease(10+yrs) and have had twitching from about several months after first symptoms.
they say no fassics in pls but many do get twitching,this is probably due to nerve irritation from the umn problems .
in ms that also effects the grey matter/umn's twitching is common.

i too have atrophy now but disuse atrophy due to weakened muscles.
also i have clinical weakness/hypotonia in my left leg.

you sound pretty much like me.
how long have you had pls?or the symptoms?
normally its 3-5yrs watching for a diagnosed.

a clean emg is good news,as long as your progression is steady you will be ok(well,as ok as you can be with pls)
 
Hi Justme,

There is another active member (Zaphoon) who has a PLS diagnosed and has fasciculations. With the holiday weekend upon us, it may take him a while to see this post though.

My neuro has said that even the medical community does not really understand fascis, that they can happen for a number of different reasons, and nerve irritation is one of them.

Good luck to you.
 
Justme,

I can understand your concern. To clearly have UMN issues as you do, it is a no-brainer that you would be concerned with the potential development of LMN problems. The appearance of fasciculations in the presence of UMN would understandably frighten anyone. It seems odd to me too that you would suffer from UMN and that your twitches would be considered "benign." I suppose since they are not accompanied by other LMN signs they merit that designation. I frequently hear that one should judge fasciculations by the company they keep, and I suppose I wasn't making the distinction that by "company" it is probably meant "LMN company" not "UMN company".

With that said, I have found that many people with neurological issues twitch as well, despite twitching not being part of their particular disorder's symptom list. One of my differentials is Parkinsons's, and I was surprised to read that many with PD suffer from fasciculations as well. It sounds like you are another PLSer that twitches. Are you officially diagnosed with PLS or are the doctors just calling it UMN syndrome or something like that?

I plan on pming you after the weekend regarding your muscle biopsy, if you don't mind.

Have a nice weekend, it is gorgeous here.

Lydia
 
Justme,

Join the crowd! Fasciculations can be caused by a variety of issues outside of lower motor nueron death. My last EMG was almost 2 years ago and I have had non-stop fascics in my legs the entire time with random twitches elsewhere.

I've been told it is not likely that the EMG is going to change as benign fascics don't suddenly become pathological. I have spasticity, bilateral babinksi and cramps out the wazoo. No Hoffman or jaw-jerk though.

I'm thinking my PLS could be compounded with BFS or some other peripheral nerve disorder.
 
PLS can surely cause twitching like my friends have stated. I really do believe the twitchin in nerve and muscle irritation and such. I believe the twitching is really unexplained in it's cause and effect in this thing. I think the worry warts will think well I have twitching she is saying that it could be ALS.. I say to them, twitching means nothing, it is part of the Clinical picture, and the Testing picture..
 
Hi,

Thank you for so many replies! Im glad to know that the fasciculations CAn be due to upper motor nueron disease not just Lower motor neuron. It seems it is common after all but not sure why the clinical weakness in my hand did not cause emg changes?

Thank you for your time along with your care to reply!
 
hi justme.
if the weakness is from umn involvement it may not show up on the emg.
to evaluate umn death and possible weaker areas they use a mr-spectroscopy,i suppose a bit like a mri.
this is only used rarely as not many hospitals have them.
it can not be used to diagnosed hsp/pls as it can only be used as i said to evaluate long term patients..........i suppose more of a study aid.
i have only heard of a handfull of people who have had this done.
 
Hi,

The weakness is from lmn , here is my confusion.
 
Possibly a single fiber EMG could be done on your forearm? I'm just throwing this out there, as you say your weakness is LMN.

My own experience was 2 EMGs of bulbar area diagnostic for MND. When weakness moved to limbs, it was assumed by all, that I had bulbar onset of ALS, instead of PBP (which most people with bulbar in fact do have) BUT, months later, another limb EMG was done, still normal. So, in came the specialist who does the single fiber EMGs. Did that over my eye, and it was abnormal. I BEGGED for him to do it on my affected arm or leg (as I've read it can be done there) but he said the best place was by the eye. The findings with the SFEMG were abnormal.

The single fiber EMG is mainly used to diagnosed Myasthenia Gravis. but, MG has been ruled out for me. In my situation, nothing more in the way of testing is planned for anytime soon. My neurologist said my diagnosed still stands, as bulbar region continues to progress, although somewhat slowly. Before my diagnosed of PBP I'd completed about a year's worth of immune modulation therapy with no effect, so I've been told there's nothing left to try, and nothing left (specifically) to look for. BTW, I have little UMN involvement. Some hyper reflexes, jaw jerk, can't stick out my tongue, etc, but its not like I'm UMN dominant.

However, if, for you, the single fiber test found (or did not find) abnormalities, it could help narrow the search. I don't know enough about myasthenia to be able to say if people ever have UMN involvement. I've never read up on it much, because every specialist says, no, that's not it.
 
How do you know the weakness in your hand is LMN problems and not UMN problems?
 
Hello Justme111

I got your public message and so I'll reply to you here.

I'm a bit confused by your EMG and how it didn't pick-up the reinnervation that your muscle biopsy did . . . although that would tell me that the reinnervation was not wide-spread . . . a good sign.

Your EMG indicates that you do not have any type of lower motor neuron problems that would be attributed to any type of lower motor neuron disease. By the time symptoms are manifested with lower motor neuron diseases, the denervation is fairly wide-spread and would be picked-up by the EMG.

Fasiculations in the absence of active denervation and reinnervation means absolutely nothing (and by the way, wouldn't necessarily mean anything sinister if they were all found together), which is why they said your EMG is normal. You had no spontaneous discharges (spontaneous discharge is an indication of active denervation) and your motor unit potentials were normal as well (an increase in their amplitude and/or duration would indicate reinnervation).

As has been stated in other posts: fasics can happen for a gazillion different reasons and you are the victim of one of them.

I hope that helps.
 
Thank you all VERY much for replying.

Wright?

If the emg did not pick up the denervation and reinnervation but the biopsy showed prior denervation and reinnervation , in your opinion, from what you know, would that mean the weakness in my left hand could not be from als? Dr said it was clinical weakness.

Im trying to hold on to hope & Im really confused as to weather als can be ruled out because of a negative emg.

I guess im just not sure what to make of all of this but does the emg mean anything? Could the Dr have done it too early?

Thanks :?
 
If the EMG was done in the affected area and the EMG was normal, that means that your weakness cannot be caused by lower motor neuron problems.

You did say you have been diagnosed with an upper motor neuron disease (am I to assume it is PLS?). Upper motor neuron dysfunction cannot be picked-up by an EMG; that is diagnosed clinically, as it appears the neuro's have done. Upper motor neuron dysfunction can cause weakness but it is due more to stiffness, spasticity and loss of coordination, which you indicate you have. Is that what the neuro's are attributing your weakness to?

Where was the fiber type grouping found? What muscles were EMG'd? How long have you had your symptoms? How quickly have they progressed?
 
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