TeleEMG Patient Education Series
Series Editors: JF Jabre, MD and OS Shams, MD

Amyotrophic Lateral Sclerosis (ALS) / Motor Neuron Disease (MND) - Part II

Question

Can Fasciculations result from overexertion & how to exclude ALS? Top

Question

I am a 49 year old, that works out all of the time and for the last 5 months have had fasciculations especially in my calfs. I had a normal Nerve Conduction and now they want to do an EMG. The only physical change is that I gained about 15 lbs after beginning to lift weights in February.

I never noticed the Fasics until my Flight Surgeon looked for them at my last flight physical.

My question is do you find that over exertion causes fasciculations, and is there something else I can try before the test? I'm also finding that I get cramps in my calfs and some soreness, (probably from to much exercise). According to my trainer my strength has increased substantially.

These are questions that are hard to ask and I am quite tentative about a " lose - lose " diagnosis. If its BFS there is nothing that can be done about it, and if it isn't there isn’t.

Any Suggestions?

Answer

Too much exertion, or stopping exertion after having exercised for a long period, can cause fasciculations. Usually calf fasciculations and cramps tend to be benign. An EMG however would be a very good idea because it may explore whether or not you have fasciculations (that you are not aware of) in other muscles.

In the work-up for ALS there are many other signs and symptoms than fasciculations that lead to a diagnosis; so a good EMG and and a good neurological work-up are of the utmost importance before making a diagnosis.

Comment

I had the NCS it was negative, and a EMG and the Dr. stated " I needled the tibialis anterior, peroneus longus, gastrocnemius or soleus on either side, quadriceps, hamstrings, deltoid, biceps, triceps, and first dorsal interosseous on the left side. Most of the muscles showed small fasciculation potentials. No runs of fibrillation potentials or positive waves. No bizarre high frequencies discharges. No Myotonic potentials. The motor units that I saw were of normal amplitude and duration."

He then suggested I stay with Acupuncture for a few weeks and see what happens. He also did a lower back MRI which was normal and a spinal X-ray and the only statement was” Small anterior Osteophytes are seen at T12 -L1 and L2-L3.

So my question is what do suggest I do about the Fasciculations / twitches and the leg stiffness that goes with it. I have been taking creatine 5 mg a day, and many non-synthetic vitamins prescribed by my nutritionist. Also I am working out with weights and seeing definite gains in strength, other than my legs.

What are your thoughts on acupuncture for this problem.

I am a pilot and taking certain medications is not an option, I would be glad to pay for your time for a phone consultation, if that is an option...

Answer 1

I would suggest continuing the acupuncture. In general the presence of fasciculations without other EMG findings by itself is not significant. I do not suggest any specific therapy in your case for fasciculations.

Answer 2

Findings such as the ones you describe could well be benign fasciculations. Usually, you would like to see large fascics and some other signs of denervation such as fibs and positive waves for this to be significant. A normal MRI and spine x-rays just indicate that the symptoms are most likely not coming from your back.

Is the EMGer that did the test on you your neurologist? Per my previous reply to your post, I had indicated that EMG is only a part of the work-up, it takes an experienced neurologist to put all of these findings together (and exclude other possibilities) to rule out ALS.

I usually advise patient with symptoms and findings such as yours to seek the advice of an experienced neurologist to look at the whole picture.

I can't tell you what to do with the fascics. I usually recommend decreasing (not stopping) the weight training for a couple of weeks to see if the fascics change. That would be a way to find out.

Normal nerve conduction studies Rules Out MS and ALS? Top

Question

My primary care physician recently ordered a conductive nerve study for me since I have had hand and feet numbness, twitching and weakness in the thighs.

The conductive nerve study was negative as all readings for all 4 extremities were within normal ranges. On the follow up visit when we discussed the test results he said that a normal (negative) conductive nerve study rules out central nervous illnesses such as MS and ALS. Is this the case or should I pursue the opinion of a neurologist?

Answer

Nerve Conduction Studies do not evaluate MS at all, and they are only part of the equation in ALS. Certainly, a normal study rules out neither. When the information you get from a primary care physician (regarding a neurological problem) is unsatisfactory, it is best to seek a neurological opinion.

Comment

You said that the NCS is only part of the equation for ALS. What are the other parts of the diagnostic equation for ALS?

Answer

The other parts of that equation are the Neurological and Physical Exams and abnormal EMG needle electrode examination.

Relative occurrence of fibrillations and positive sharp waves in ALS Top

Question

I read in one of the articles which dealt with ALS that in ALS, the EMG show mainly sharp positive waves and fasciculations, and only sometimes fibrillations (which are less common in ALS than PSW AND FASICS) ACCORDING TO YOUR EXPERIENCE, IS it true? AND IF SO why PSW are seen in ALS much more than fibrillations)?

Answer

This is not true; the fibrillations are similar in occurrence to that of positive sharp waves in ALS.

ANA and Sedimentation Rate in MS or ALS Top

Question

My primary care physician ordered an antinuclear antibody and sedimentation rate tests as part of a screening for the symptoms I presented. Both tests were normal as was my conductive nerve study (conduction on 4 extremities but not the inserted needle test).

Will a SED rate or ANA be elevated for either ALS or MS?

I have an appointment with a neurologist in 6 weeks (I was not able to get an appointment any sooner).

Any other advice? My chief complaint is weakness in the front of my thighs and muscle twitching primarily in the lower legs but shows up in the thighs when they are tired. Twitching has shown up on my upper arms and occasionally in my hands, back and face. Sometimes I might go a day or two without a noticeable any twitching but when I am fatigued twitching is more common.

I should mention that the twitching started after I had a reaction to Septra about 2 months ago. I don't know if that was just a coincidence or not but the reaction was significant with burning gums, insomnia, loss of appetite and numbness in my legs from the knees down. Prior to that my main complaint was fatigue and numbness in my hands and feets. The numbness, which started about 4 months ago, seems to have gone away.

I should also mention that I have had what I would call long-term tiredeness, achiness, unrefreshing sleep and such for about the last 10 years. I had come to terms with those long-term symptoms but theses new issues are frightening.

I am a 43-year-old male with a desk job.

Thanks for your input. It is hard to wait so long to see the neurologist. I made the appointment over a month ago and still have 6 weeks to wait.

Answer

The sedimentation rate or ANA have no relation to either diagnosis. They are not part of tests done for those diseases. From your symptoms, it is a good idea to see neurologist. He would hopefully sort out your symptoms. He may also request an EMG, which should help to explain at least some of your symptoms.

Acute denervation in ALS Top

Question

I know that according to most neurology books, in ALS both ACUTE AND CHRONIC DENERVATION ARE seen. However, I am not sure which one is most prominent in early ALS?

Actually, according to your own clinical experience How prominent is the acute denervation in early ALS? (PSW, fibs)

Have you seen any patients with MND (ALS, SMA, progressive muscular atrophy) who showed only chronic denervation (giant units) and no acute denervation at all in any of the limbs?

Is it possible with motor neuron diseases, according to your own experience? (Because It is not quite clear from the books)

Answer

It depends on the time of presentation. Generally speaking the denervation activity are seen at all times; either acute or chronic stages of MND. The changes of the MUP can tell you about chronicity. If they are high amplitude (giant MUPs), then indicate a chronic stage. Therefore, in early stages, mainly the denervation activity without much of MUP amplitude changes. While later on denervation activity still there in addition to high amplitude MUPs.

Comment

Thank you again for your reply!

My neurologist has told me that some MND's are very slow (for example SMA) and there for the chronic changes there are prominent.

1. Does it mean you NEVER see acute Denervation in SMA (fibs, PSW) but only chronic changes, because they are very slow diseases?

2. And about the chronic changes in MND (ALS, SMA..) you mentioned huge amplitudes... but what about highly polyphasic, long duration units? Why aren’t they ever mentioned with ALS or SMA in most textbooks?

3.what would be abnormal amplitude in ALS/SMA?

Answer

First I would like to make myself clearer and try to explain my understanding to these interesting disorders. There are spectrum or range of diseases due to motor neuron affection (anterior horn cell diseases). This spectrum includes SMA, MND and ALS. The MND term is frequently used to mean ALS. SMA although is anterior horn cell disease but has only signs of lower motor neuron affection, absent reflexes and very slow progression. From the EMG point of view, MND or SMA all show denervation activity with neurogenic MUPs. The EMG is not enough to differentiate between MND and SMA, although generally the MUPs tend to be more complex and higher in SMA (slow progression). Clinical examination is important in this. Now, regarding the MUP, when it is chronic it becomes not only high but gets polyphasic and increased in duration. It is not mentioned in standard textbook may be because they are not specialized books. But this is known in EMG textbooks.

Comment

One of the things which also is not very clear from most text books is polyphasic units: I know that polyphasic units by definition are those with more than 5 phases. However, Are units with 3 or 4 phases are as completely normal as the mono and diphasic units?

I will put it this way: if you Do motor unit analysis to a patient and you find out that most of his units are of 3 and 4 phases (rather than 2 or 1) would you be more concerned?

Here is a quote from a manual that made me think that 3 and 4 phases are not as normal as 2 or 1:

"Fasciculation potentials can be monophasic or diphasic, looking like normal motor units..." - Does it mean that normal motor units are only these of 1 or 2 phases?

Answer

This is a good point. Let me just make a correction, potentials with more than 4 phases are called polyphasic. The units with 3 or 4 phases are normal. I will not be concerned if I see units of 3 or 4 phases rather than 1 or 2 phases.

Comment

Yet about the polyphasic units:

I read that some polyphasic units are also present in healthy people:

1. Is it true according to your clinical experience?

2. (Would it be 10%, 20%. of all MUPs in a muscle that would be considered normal), yet is it totally normal?

3. Does it mean that there is a constant reinnervation-taking place in each one of us? Does it happen with tensed muscles

4.Are the "normal" polyphasic units you see highly polyphasic or just 5,6 phases (board line) or both?

Thank you again; I just could not find the answers anywhere!

Answer

1. Yes, this is true.

2. This is also generally true.

3. This process is not usually a normal phenomenon. Reinnervation has to do with denervated muscle not with "tensed" muscle. It is the process which takes place by the surviving motor units after nerve damage to restore muscle function. It is a reaction of the healthy fibers to the loss of nearby fibers.

4. I do not know, although I did not see a "normal" highly polyphasic MUP, however, I think practically, it does not matter whether the MUP has 5, 6 or more phases. What matters is how many (%) are recorded out of 20/study.

Definite ALS and EMG/NCV Top

Question

I was diagnosed definite ALS after one EMG/NCV exam and one clinical exam. I am 37 yr. old male. I show upper and lower MN involvement, hyperreflexia in affected limbs, bulbar signs. My EMG/ncv of affected areas showed: nerve conduction studies and latencies normal. Quiet insertion throughout, interference patterns decreased, some fibrillations and positive waves detected.

The tests were interpreted thusly:

Fairly widespread changes in tongue, proximal upper limb particularly as well as lower limb consisting of relatively mild denervation and decreased motor unit output. May very well be MND.

Does this one EMG rule out other syndromes entirely? Blood serum shows elevated anti gm1 antibodies. What about axonal motor neuropathies, or immune mediated demyelinating neuropathies?

Answer

MND is a diagnosis of exclusion, that means one must rule out ALL OTHER possible causes of these symptoms such as Thyroid/Parathyroid disease, Toxic Exposure, Motor Neuropathies with conduction blocks, Tumors, Acrylamide toxicity etc.. etc.. and is best done by a qualified Neurologist, after which I always advise my patients to seek a totally independent second opinion. So even though the EMG may be suggestive it is by no means diagnostic, until and unless all of the above has been ruled out.

The elevated AntiGM1 antibodies can point to multifocal neuropathies, conduction blocks and the immune mediated neuropathies, all of which are treatable in one way or another.

By the way, I saw no mention of fasciculations in the symptoms or EMG findings you describe. WHAT about real weakness (like foot drop...) WAS it detected by your neuros? And what about fasciculation (can you see, feel them)

Comment

Yes, fasciculations are abundant. There is muscle wasting in the left hand, arm and left leg. Foot drop is becoming apparent.

Answer

Insist on checking the possibility of multifocal motor neuropathy IT is very possible and less devastating than MND

1.DO you get cramps, especially the ones that wake you up when you sleep at night?? 2.and about your weakness... DO you find it difficult to open a jar use a key to open a door, or even walk- It is important to understand how profound is your weakness

3.can you stand up without using your hands from sitting on the floor?? (Try it)

Comment

Answers to your questions:

1. I have cramps but not intense enough to wake me. 2. My weakness is confined to the left side. Opening a jar, squeezing toothpaste are difficult with that hand. Although my left leg is affected I have no trouble ambulating. 3. I can still get up from a sitting position.

LL weakness & EMG findings. Is it ALS? Top

Question

Hi. I have been having progressive lower extremity weakness over the past three years. I have seen 3 ortho doc's thinking it was my knee (I have a chronic ache in my left knee). Two days ago I underwent EMG and nerve conduction studies (very painful). The nerve studies were normal. The EMG revealed Fibrillations and positive waves in my calves, supraspinous muscles and deltoids. There was very little muscle fiber in my left calf. Are these findings consistent with ALS? How about MD? I am a 29-year-old female. What other testing can I expect?

Answer

On one hand, the EMG findings are incomplete to draw a conclusion i.e. the motor unit potentials (MUP) description. On the other hand, the EMG findings must be taken in context of the clinical picture (history and examination) and not on its own, because those may suggest any of them or other diseases. I would recommend seeing a neurologist (if you have not yet seen one) before proceeding with other investigations.

I have twitching in right leg. Am I considered as having ALS? Top

Question

I am quite nervous about this twitching I have been having in my right calf, along the side-front. You can see it jumping around. It's 24/7. It's been going on for about 2 months and progressively getting worse. I also have some twitching in my right arm and right buttocks, but not as severe. It never lets ups. I have a crampy feeling in the backs of both legs at times. At one time, my right calf felt as though it had a cramp, but it really wasn't a 'cramp'. Almost a burn. Also, my left shoulder seems to want to pop out of it's socket in the morning. It feels as if there is a torn tendon or something. I see no atrophy and it feels as strong as the right shoulder. I have no weakness that I can tell. I went and saw a neurologist and he saw the twitching and said it was nothing to worry about and said; "YOU DO NOT HAVE ALS!", firmly and positively. I completely disagreed about not having an EMG, so he finally gave in. I found this very strange.
I am very scared about ALS and my doctors ability. HE comes highly recommended and has diagnosed about 20 ALS patients in his life.
Should I be concerned?

Answer

Neurologists rely on many symptoms and findings to diagnose or rule out ALS. While twitching in the muscle (facics) can be a sign of the disease, not everybody who has them has ALS. If your doctor is familiar with the disease, the chances are he's confident in what he's telling you and you should feel assured. If not, you should seek a second opinion to allay your fears.

By the way, did your doctor tell you what he/she suspected as the cause of your symptoms?

Tissue Transglutaminase Antibody in ALS Top

Question

Anyone knows of having a high titer of Tissue Transglutaminase Antibody and ALS. Thanks

Answer 2

There was a paper in the Archives of Neurology "Glutamate Transporters in Neurologic Disease by Nicholas J. Maragakis, MD; Jeffrey D. Rothstein, MD, PhD, March issue, 2001. "Glutamate is the primary excitatory amino acid neurotransmitter in the human brain. It is important in synaptic plasticity, learning, and development. Its activity at the synaptic cleft is carefully balanced by receptor inactivation and glutamate reuptake. When this balance is upset, excess glutamate can itself become neurotoxic". Then there was a comment that Glutamate is increased in ALS "Evidence for glutamate contributing to motor neuron degeneration in ALS initially came from several studies that suggested that cerebrospinal fluid glutamate levels may be elevated in patients with sporadic ALS", also "These earlier studies reported that motor cortex and spinal cord tissue glutamate levels were decreased 30% to 45% in patients with ALS" and "In those studies, a significant loss of high-affinity, sodium-dependent glutamate transport was found in ALS" "the loss of glutamate transport is seen both in familial models of ALS and in sporadic disease". It was concluded that "Regardless of the mechanism, the loss of EAAT2 (excitatory amino acid transporter 2) glutamate transporter may contribute to a reduction in glutamate uptake with subsequent overstimulation of glutamate receptors, resulting in neurotoxic effects". I hope this helps.

Fasciculations detection and ALS Top

Question

I've read the material on this site regarding EMG, and have even had an EMG, but I still have some questions.

1. In the diagnosis of ALS an EMG is administered in order to detect fasciculations. Since fasciculations come and go and are not continuous, as I understand them, what would happen if the EMG were performed on a day or a time of day when the fascics were not present? Would you get a false negative?

2. When receiving my EMG the neuro had me exercise against resistance each muscle being tested (i.e. the muscle w/ the needle in it). Is this in an attempt to generate and then detect resulting fasciculations? If not, what is the purpose of exercising the muscles?

3. I postponed my 2nd EMG because of a lack of fascics that day. Now that I'm exercising more the fascics are recurring. I'm scheduled for a 03 May EMG. Does this strategy make sense or are there certain EMG-detectable symptoms that may not manifest themselves to the extent that I can physically sense them?

Answer

In response to your question:

==> The EMG diagnosis of ALS rests on much more than fascics alone. It looks for denervation, active and chronic and the widespread distribution of the findings including in bulbar muscles such as the tongue. So fascics alone are not a diagnostic criterion to diagnose ALS by EMG.

==> Yes, fascics are sometimes seen easier after exercise, so in all likelihood that's what your doctor was looking for.

==> Again, while it is good to see and study fasics by EMG, the diagnosis is not usually based on fascics alone.

Widespread muscle twitching & scared to death of having ALS Top

Question

Hello. I am a 27-year-old female who has been having muscle twitching in numerous locations on my body, for 8 months now. I’ve been to 1 neurologist who did the in office neuro exam (testing of reflexes, heel to toe walking, walking on heels & toes, he tested my feeling on arms, legs etc. and also my pupil dilation, along with my strength). When I asked him of the EMG ,he refused to perform one on me stating that "from what I observed it is unnecessary, and I am not going to put you through the pain. "Well, since then, I have tried my best to believe him, but I had finally built my courage up to going to have the EMG test done that day, I was so scared to walk out of that office without one because I knew that an EMG is what really determines if you have ALS, not a neurologist’s observance. I’m going for a 2nd opinion at the end of the month (another neurologist), and once again I am sick with fear. My twitches happen more on the left side, but do also happen on the right. It seems every muscle (butt, back, thighs, top of foot, wrists, eyes, jaw, and I could of sworn I've even heard them in my ear drum) has twitched at one time or another. The ones that occur on my left foot really scares me, because my big toe on that foot doesn't point up as much as my right big toe does, leading me to think I have foot drop/big toe drop. A few of my toes went completely numb when I tried running a short distance one day. My left foot cramped up when I turned it suddenly while rollerbladig one day also. This all leads me to think there is something horrible going on with my left foot. Both my feet get so sore on the bottoms just from walking on them. I’m only 27 and I love to dance, walk, roller blade, and try to live good, but I feel like my body is old before it's time. My twitches do sometimes get "calmer”, but they never stop completely, there’s usually one happening somewhere on me. That’s the way they've always been for 8 months now. Although my left foot, on the bottom, becomes awfully twitch-happy sometimes. I'm so frightened of ALS and have diagnosed myself as having this disease because with all my symptoms how could I not? My husband refuses to go to another neurologist with me, although he told me I needed to go because he is tired of seeing me cry. I know I should feel ashamed of diagnosing myself via the internet, and I do, but can you offer me some hope anyway or maybe a little encouragement to not be soo scared. I am a military wife with no family near to help me through, my husband is disgusted with me refusing to accept the benign diagnosis without the EMG, and I feel like my life will never return to normal again if I don't get an EMG. Sorry so long. Please ,what advice would you give to your loved-one in a similar situation?

Answer

First things first: If you perform a search in this TeleEMG forum using the word ALS, you will find many postings dealing with this subject which would be of help to you (search button is on the top of this page)

Second, fasciculations and cramps are no the only signs of ALS, there are many others as you will see when you perform the search so your neurologist's reluctance to call them ALS may be based on what else he/she found in the exam and history.

If you are concerned however, it is not a bad idea to seek a second opinion, and if you still hear this is not ALS, ask your doctor to explain to you what these symptoms are.

Does Clean EMG in one limb exclude ALS? Top

Question

I was just wondering does a clean EMG/NCV on the L limbs after 6 months of fasciculations r/o motor neuron disease, or will it ONLY r/o LIMB ONSET MND? Would bulbar ALS be r/o with this EMG or do the areas corresponding to bulbar onset have to be tested? I have read conflicting information on this. Some say, bulbar is rapidly progressive, and would show some spread into the upper limbs. Others have responded to the contrary. Seeking clarification, thanks.

Answer

If the EMG is done in the comprehensive way, then a negative result is against MND. so, a clean EMG of one leg does not rule it out. The rule is, if an EMG is normal in one side go for the other side if MND is suspected. Also, to answer the second point, if the patient has presented with bulbar symptoms, then we examine the tongue muscle in addition to the extremities, even laryngeal muscles if needed (if no other EMG abnormalities are seen). I do the later muscles with help of an ENT surgeon.

Comment

Just a slight correction, I had my ENTIRE L side studied. I have a hx of Sjogren's syndrome and was hospitalized 6 months ago with L sided weakness, and have residual hemianopsia in my L eye. During my hospital stay, I developed some ugly symptoms such as hyper-reflexia bilaterally, fasciculations, clonus and was on IV Solumedrol 1000 mgs x 5 days. The Neurologists and docs suspected neurogenic vasculitis. To this day, I have fascics, but have regained my strength and show no s/sx of atrophy. I still have residual hyper-reflexia, and b/c of the persistence of these sx an EMG was indicated. It showed, normal NCS and no fibs, PSWs or fascics and good motor unit potentials.
With this history, and the reason for my initial post, I feared MND. Am I correct to assume that you feel further EMG testing to be done? The entire L side being normal, would not r/o ALS? And if it were bulbar onset, I have some dysarthria, wouldn't it be readily apparent through some limb denervation? (Especially, upper limb?) After 6 months time, with my strength returned to normal with no wasting or atrophy, and fascics declining, and a clean EMG on my L side (upper and lower). ALS is STILL a possibility? I'm concerned. Thank you. Karen, RN

Answer

The EMG is an extension of the clinical history and examination. At this stage with this history background, I am skeptical regarding the diagnosis of MND.

I have fasciculations without weakness, do I have ALS Top

Question

I went to my neurologist for an EMG and 2nd opinion of my fasciculation. My fasciculations, which have been ongoing for 10 months now, the results were normal but a fasciculation was picked up by the needle in my calf that I did not feel. I assume this was normal, Am I correct in doing so? I know people that have ALS sometimes progress slowly, but my neurologist said that after nine months of twitching I would be showing some kind of symptoms somewhere if I had ALS. So far no weakness, no atrophy, normal LAB/blood work, and clean EMG except for fasciculations recorded and one on thigh seen by neurologist. The neuro also proceeded to show me this calf twitches, which said, had occurred since his late 20’s, he is now in his late 70’s. I asked him why mine have happened in every muscle possible, if they’re benign, and not just in the calves and feet like his. He had no idea why and also couldn’t tell me why my feet get sore; he just said ALS does not cause pain. When can I feel reassured that I don’t have ALS? It is so hard to do when the fasciculations never leave. I’m 27 and I have gained 15 LBS. Do people with ALS have trouble putting on weight when they can still eat? I want to diet and get back into shape, but have been too afraid to lose weight with this constant fear of ALS symptoms kicking in. What does slow progression mean. When it comes to ALS. It several months of years of fasciculations and then weakness? Please answer my questions if you can, I want so badly to get on with my life. Thank you again.

Answer

I cannot do better than your neurologist. I agree with him that you do not have ALS. He has given you the proper opinion.