EMG Results?

[curtis]

Hello Doctor:

I am a 38 year old male who has been having some disturbing symptoms over the last nine months.  I began having fasciculations over most of my body in May of 1999 but they seemed to mostly be in my calves and feet.  In June my neurologist and I noticed some mild atrophy of my right foot.  An EMG was normal except for a slightly slowed velocity of my right tibial nerve (40.6 vs a normal of >41).  All amplitudes were normal.  In October I began having a strange "dull" sensation in my left arm and things also began feeling heavier in that arm.  In November I had another EMG on that arm which was normal.  The sensation soon went away but the "weakness" and fasiculations  remained.  In January, the sensation returned along with some mild transiant dexterity problems in my left hand.  I just recently had another EMG which unfortunatlly showed a rather dramatic drop of amplitude of my left median nerve (13 down to 4). The signal was also "dispersed".  The needle exam was normal (including no fibs or fasics) except for some mild "myopathic changes" in my left deltoid and flexor carpi ulnaris muscles (decreased size, duration; increased frequency). By the way, my right tibial nerve was normal.

I am somewhat concerned about ALS. My grandfather died of it while he was in his late fifties.  Two seperate neurologist do not think I have ALS but cannot give me an explanation for my problems.

My questions are as follows:

1. What can cause a quick and dramatic drop of amplitude and can you experience fasiculations and weakness before anything bad shows up on an EMG (such as a drop in amplitude)?

2.  Can you have atrophy and still have a normal EMG?

3.  Can famial ALS miss one generation and then hit the next?

4.  If you have a low amplitude that is causing weakness and fasiculations, shouldn't something be seen on the needle exam?

5.  If you are having weakness and fasiculations due to ALS, shouldn't you also be seeing fibulations or do they tend to appear later?

Thank you very much for this great service and I look forward to your response.

Curtis

[CCF Neuro[P] MD, RPS]

Dear Curtis:

There are certain criteria for ALS using EMG and ones needs three different muscle groups to show fasciculations and fibrillations.  Clearly, you do not fit the standard criteria.  Second, you did not mention the presents of fibrillations, which must be present for a diagnosis.  Third, your neurological exam, although you feel weakness, did not show objective weakness, another indication that you do not have ALS.  Fourth, you do not have both upper and lower motor neuron signs, also suggesting that you do not have ALS.  After 9 months of symptoms, you should likely show more signs of ALS if you had ALS.  Based on these things, I would agree with you neurologist that you likely do not have ALS.  The hereditary form of ALS would not skip a generation, it is not a dominant trait and this form is so rare, (only 10% of ALS is hereditary) that it would not skip a generation and find you.  Base on this you do not have the hereditary form of ALS.  

The answer to your first question is yes, but for different reasons.  If you had Guillian Barre, the EMG would initially be normal with profound weakness.  However, the weakness is clearly evident on neurological exam, which you definitely did not have.  Usually, with fasciculations and fibrillations, the EMG is clearly positive.  

No, you cannot have atrophy due to a peripheral cause and have a normal EMG (at least that I am aware of).

If you have muscle weakness that is clearly evident on neurological exam, you should have a positive needle exam.  Low amplitude means that the electricity or conduction flowing down the nerve is not correct or there is demyelination occuring.  Here, the needle exam would be normal, but the conduction velocity would be normal.  This is what you would see in early carpal tunnel.  

The answer to your fifth question is yes, if you had ALS you should be seeing fibrillation with your fasciculations.

I hope that I have helped you some.

CCF Neuro MD

[Dana]

What's the difference between fibrillations and fasciculations?

[curtis]

Thank you for your help, but I am just a little confused about one of your answers.  With regard to low amplitude, in your second to last paragraph you say: "Here, the needle exam would be normal, but the conduction velocity would be normal." Should they both be normal or just one of them?  Thanks.
Curtis

[rk]

You can infer the meaning from the previous sentence, "Low amplitude means that the electricity or conduction flowing down the nerve is not correct or there is demyelination occuring."  If the conduction flowing down the nerver is not correct you can infer he meant the needle exam would be normal, but the conduction velocity would be abnormal (as it is not flowing down the nerve correctly).

[KMH]

Curtis,
Your case sound quite similiar to mine.  I have not expereinced any feelings of weakness more traveling cramps.  My fascics have similiar pattern.  I had clean EMG and Nerve conduction.  Did your Neuro suggest any follow up?  From what I have gathered from my Doc and the net, if it were something more serious it would most likely show up on your EMG.  I don't think we will ever know why this happens.  But we are fortunate enough to check out OK.  To Dana the diff between fibs are that they are movements in the muscles not usually visible from the surface also a sign of denervation of the muscle commonly found in Neuro muscular diseases like ALS.  Fasciculations are visible twitches of individual muscle fibers usually visible to the naked eye.  Fascics are common in the general population and by themselves are harmless (meaning in absence of fibrilations, weakness etc.)

[CCF Neuro[P] MD, RPS]

Dear Curtis:

Sorry for the typo:  It should read: Here the needle exam would be normal but the conduction velocity would be abnormal.

CCF Neuro MD

[jeffco]

With long history of numbness,burning,tingling in both legs and recurring nerve impingement causing both arms to be numb (especially upon awakening in A.M., I recently underwent extemsive testing. EMG normal, all nerve tests normal. Found: spike IgM (monoclonal), deficiency "C" S" proteins, abnormality chromosome #1. Bone marroe biopsy ruled out amylois=dosis, Multiple Myeloma ( maybe MGUS). Most interesting finding: Factor V Leiden -positive.

Anyone have similar experience? Any idea if all "problems" related?

[CCF Neuro[P] MD, RPS]

Dear Jeffco:

The problems that you have are not unusual in themselves, but that you have them all in combination is alittle unusual.  

CCF Neuro MD

[curtis]

Dear Doctor:

With regard to my question on low amplitude, should I also have a low velocity reading?  If this is true, could the test have been done in error since my velocity was normal?  In addition, what can a "dispersed" signal mean?  Should I be concerned?

Thanks,
Curtis

[dave]

I'm curious about Fasiculations and other symptoms and a 'quiet' EMG. After EMG the Neurologist said that's a good sign, but a month later concurred with opinion of referred Neurologist that I have 'what appears to be Amyotrophic Lateral Sclerosis'.
So for no visible atrophy (I have always been very muscular), but super fatigue, clonus in both ankles, very hyper reflexes everywhere, and lately muscles exhaust very quickly. My '?' is how much can ALS vary in progression. Thanks. I'll whine no more...

[CCF Neuro[P] MD, RPS]

Dear Dave:

I am very, very sorry to hear that you have ALS.  Although ALS can vary in the time of onset to the conclusion of the disease, usually it is somewhere between 3-5 years.  Many times it is diagnosed late and the time is shorten.  If you have what we call bulbar signs (difficulty swallowing, incr salvia, difficulty chewing food) the time is even shorter.  This is a disease that one has to think about family. How far do you want to take medical management and what you think how much can you tolerate.  The issues of mechanical ventillation, living wills, measures that you will except and won't except must be dealt with, by you, your physician, and your family.  

Again, I am really sorry about your disease.

Sincerely,

CCF Neuro MD

[dave]

Thank you very much. I apologize for intruding on the Q&A's of the others. Just a note, I'm enjoy my life more than ever now, and I guess my main confusion over progression is that other than extreme fatigue and stiffness, I'm OK...other symptoms are subtle and slowly progressing. I don't have the Bulbar signs yet.I go back to my boxing days...as long as I'm in the ring I'm going to fight. I usually lost to knock outs though. Thanks again. You guys are great.

[lavon]

I'm curious as to what signs would indicate Dave has ALS?  What is a "quiet EMG"?  I also have hyperactive reflexes, fatigue, clonus in both left ankle and wrist, and stiffness but understand these are upper motor signs and ALS must be both upper and lower.  The diagnosis I was given was Primary Lateral Sclerosis which I understand is considered a separate process by most and certainly more hopeful than ALS.  Oh, I also have fasciculations in many muscles but normal EMG and a normal muscle biopsy.  The ALS specialist I saw in Houston indicated the EMG and/or biopsy would have been different had I had ALS.

[CCF Neuro[P] MD, RPS]

Dear Lavon:

ALS is both upper and lower motor neuron disease, as you describe.  Dave had upper motor neuron symptoms such as clonus, increase DTRs and lower motor neurons symptoms of easy muscle fatigue that is visable on neurological exam.

Sincerely,

CCF Neuro MD

[david]

What exactly is clonus? I hear so many different definitions of this...What exactly is the foot or hand or bodypart doing that indicates this?

Thank You.

[dave]

In my case,David, the clonus I have refers to a type of bouncing down reflex at my feet that is somehow activated at the ankles. 2 doctors out of 7 that tested me didn't find it while the other 5 found it quite easily.They said it's an upper motor sign. (I should have asked for an oil change) I think sometimes clonus can refer to continuous bouncing with no control. I fortunately don't have that type. But I'm just learning about this stuff.

[KMH]

Dave your sense of humor an attitude towards this challenge is to be commended.  Did you come to the neuro you spoke of with weakness?  Why did your first neuro refer you elsewhere?  Just curious. Will send up a word to the big guy in your name.  Keep fighting, never quit.

[dave]

It's a long story, but I was being evaluated by Othopedic Surgeon for neck and back injuries on the job for a Workers Comp thing when after treating me for about a year, he did some extra detailed reflex checks because of hyper reflexes in knees and such. That's when he found the clonus and suggested I follow up with my regular insurance Doc. When the company and all the lawyers heard about possible neuro problems, everybody scrambled to cover their Butts. So needless to say I got a lot of 2nd and 5th opinions and referrals and great care. The weakness (mild, but very bothersome) was mainly on my left side, and really didn't start until 47. My wife found the fasiculations in my calves and feet which put the docs in the direction of ALS. All of my other symptoms are mild, but I do notice changes. The one that really pisses me off is constantly tripping over my left foot and scuffing my 'NuBucks' on the Santa Monica Pier.
When I got the diagnosis, I went out and got the sports car I've wanted for 10 years, and decided then if I'm going out, I'm going out with the top down and the CD's loud.
I've been around children with Cerebal Palsey for many years. They are my inspiration in any tough time, which I really don't feel I'm having yet. I'm a coward with pain, though.
And the Big Guy rides with me. Thanks

[KMH]

Thanks for the reply.. Should have known your fron SO CAL...  I lived in HB for three years and used to sell in Santa Monica.  I'll tell you even on your worst sales day life was still good in Santa Monica.  Good Luck Friend.

[CCF Neuro[P] MD, RPS]

Thanks for the comments.  I lived on 7th Street in Santa Monica when I was at UCLA for 5 years.  I do miss the sun and mexican food.  

CCF Neuro MD

[dave]

Hey Doc,(I mean no disrespect) my fasiculations are constant and seem to be slowly spreading up the backs of my legs and some are getting a lot more 'bounce' to them.Now noticing them in my left hand near the thumb. As things progress is this the usual? Do they increase, decrease or what? As I said I have no visable atrophy, so I'm not sure what to expect.
Has anyone else tried 'Strip Twitching' with their partners? The one who doesn't guess the closest to the next twitch has to...well you get the drift. My wife loses a lot now; I've been watching for some patterns. It's actually fun. Maybe Milton-Bradley could fund it....
Yes...Santa Monica. It was beautiful there today. UCLA Med Center is where I go for ALSA support group. Come back and visit; Loretta (the organizer) brings good donuts and coffee. Fantastic and caring people. I go to those and realize blessings.
By the way...Thank You for this 'forum.'

[CCF Neuro[P] MD, RPS]

Dear Dave:

Hang-in there and just avoid the knock out punch.  By the way, go to Stans donut shop by UCLA, if I remember correctly they are excellent.  Stick you toes in the Pacific for me.

CCF Neuro MD

[dave]

Thanks. I know Stans Donuts well. Any word on the progression or Digression of fasicultions as ALS does it's thing?
I learned to have a good 'cut-man' in my corner to be able to see the knockout punch before it lands. Just make sure he's not a rabbi....

[CCF Neuro[P] MD, RPS]

Dear Dave:

Hang in there, and avoid the left hook.

CCF Neuro MD

[dave]

Quick question: got blood test results showing very elevated levels of Creatine. Is that significant with ALS and would that explain my muscles not showing atrophy up to now? And about the left hook...I was watching for the left hooker, which may explain my losses...Thanks. I'll go to the beach now.

[CCF Neuro[P] MD, RPS]

Dear Dave:

This is a muscle protein that when phosphorylated can allow the muscle to work, independent of ATP.  Many ALS patients take creatine supplements, and if you do, this would explain the high level in your bood. If the creatine is in the form of the enzyme that phosphorylated creatine, creatine kinase or CPK then this would indicate muscle breakdown.

Sincerely,

CCF Neuro MD

[dave]

Thank you. I'm not familiar with the terms 'ATP' and 'phosphorylated'. I'll look them up. My creatine-serum level was '1.5'. I haven't taken creatine, so I assume my bod is producing it. I guess it's the enzyme variety.Thanks again for the quick response.

[CCF Neuro[P] MD, RPS]

your welcome.

CCF Neuro MD