ALS/EMG Advice

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Fasciculation1

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Hi All:

I have read the sticky (several times actually to make sure I absorbed it all). I’d much appreciate any feedback/recommendations. I am a 42 year old male and my symptoms started about eight months ago. I know everyone’s time here is very valuable, so i really appreciate you taking the time to read this.

My symptoms started with wide spread muscle twitches and debilitating fatigue, severe insomnia (I can’t sleep w/out sleep meds, tightness in muscles of upper back/neck and muscle pain in thighs, arms and shoulders and some minor tingling in fingers (which subsided in first month).

I had a brain MRI which was clean to rule out MS. I had two EMGs, both negative (or clean as others say) within the first four months of symptom onset (the 2nd by a general neurologist who by no means specializes in ALS and didn’t even do a written report and the first by a physiatrist at a sports medicine clinic where all he does is EMGs and wrote a very thorough report). By the fifth month (January/19), I was able to be seen at the Stanford ALS clinic and had very noticeable bilateral muscle shrinkage in my shoulders, arms and upper back, although the neurologist there didn’t even want to look at it, as he said he couldn’t tell anything b/c he didn’t know my “baseline.”

Muscles in arms have shrunk and are softer. No amount of weight lifting rebuilds bulk. About this time, began experiencing shortness of breath as well, but no “weakness” as measured during neurological exam.

Stanford did an EMG (this would be my third) and did right arm and leg as well as tongue and low back muscles. This time the needle EMG showed evidence of “denervation with reinnervation changes in the right tibial anterior muscle with associated reduced recruitment.” It also showed the tongue had “poor relaxation”. EMG was otherwise “clean” as was nerve conduction and they chalked the right tibial issue up to an old L5 radiculopathy, however the first EMG only five months early didn’t note anything dirty in right tibia at all and that physician was a back/sports medicine specialist, so he should have detected it if it were really “old”. He was very thorough in his exam.

Stanford Neuro recommended a rheumatology eval and labs, which I also had done at Stanford, given past positive ANA, but titers were low at 1:40. Rheumatologist said my issues aren’t rheumatological in nature (per exam and a ton of labs, including R/O polymosyits), and if he didn’t already know I had been seen at Stanford Neurology, thats exactly where he would have referred me next, as he felt my issues are really “neuromuscular” in nature. He recommended a second opinion at another teaching institution with an ALS center.

Fast-forward two months, symptoms continue to progress. Muscle between my right thumb and forefinger have atrophied noticeably (about 50%) and skin on right hand in general is as wrinkly as my 93 year old great aunts, from the reduction in hand muscle mass/volume. It also wants to curl when relaxed. Right thigh is noticeably smaller than left, RT tibial muscle nearly gone when I try to flex foot back compared to left leg, girlfriend continues to note that my shoulders bilaterally are getting “boney” when she rubs back and my muscle tone in arms has turned to that of jello.

Shirts are fitting loosely in upper body, especially around arms and chest and Ive actually gained 40lbs. When symptoms first started, I lost 20lbs in two month. Due to severe insomnia, was put on Remeron for sleep and have since gained 40 lbs. Muscles directly under my arms around rib cage area are almost completely gone and replaced by fat. I have loose skin in back now as if I had gastric bypass surgery, but did not.

I am now experiencing excessive salivation, neck fatigue in holding neck up, difficulties swallowing liquids and even saliva gurgles back up upon swallowing, globus sensation (prior barium swallow in Nov/18 showed ‘early presbyesophogus’ which I find concerning as that would mean swallowing muscle is weak—endoscopy scheduled next week), arm and legs feel very heavy and tire easily, have to use hands to push myself up getting out of car, very taxing on thighs to walk up stairs and they feel weak, arms fatigue easily and I cannot hold book up to read in bed for more than a minute without having to put it down.

Resting pulse ox. at home 94-97% on average and had pulmonary function test last week (results still pending). I know my body very well and am a licensed psychotherapist, and know darn well my symptoms aren’t stress and anxiety induced, as alluded to by Stanford Neuro, although will own the anxiety I am experiencing.

Fast forward two months, I was able to get into UCSF ALS clinic for second opinion (last month) as recommmded by Stanford Rheumatologist and saw a resident for most of my appt., who left several times to consult with head physician, and then the actual als clinic doctor came in at tail end of appt who did a very, very, mini exam. Their thoughts (UCSF) are they “don’t really think” I have ALS, although they couldn’t explain my evident RT foot drop (verified by both) nor serious atrophy in my right hand, which they both acknowledged. They ackowledged muscle twitches in back upon observation, and said there does seem to be some possible “lower motor neuron” involvement. They want me to wait six months and return for another EMG. Ugh. Six months?

I of course pray that I don’t have ALS, and I’m so sorry for those struck my this god awful disease, but living 40 plus years of life and knowing my body really well, tells me that something is seriously wrong, and most signs point towards motor neuron disease, IMO. I’ve always worked two jobs, and had tons of energy, but and as of late, not only is it hard to make it through a day of work, just taking a shower totally exhausts me.

I’ve been dropping things with right hand if I’m not purposeful and focused (silverware when putting in drawer, car keys, etc). My tongue is now twitching, as is my lower lip and neck. The twitches initially started as quick moving hits all over the body, but now, they seem to be more focal and persistent (long lasting) in one specific area, versus wide-spread, quickies as they were when they first began. The ones in the right calf are non-stop and more quivering than twitches. It hurts to chew and jaw/face muscles are always tight and sore.

The waiting an additional six months, as recommended by UCSF, is emotionally causing extreme distress. I have major life decisions are major on hold (marriage and kids), while waiting to see if this ugly monster fully shows its head.

I know that there is no cure for ALS currently, but I also know that early intervention may be key in slowing progression (clinical trials, supplements, THC/CBD etc). I have virtually all of the tale-tell signs of early ALS, IMO and symptoms only started 8-9 mos ago, so things are still fairly new, especially given the fact that I’ve read it usually takes 12-18 months on average to receive a Dx.

All autoimmune and rheumatological issues have been ruled out at Stanford and A TON of bloodwork is all normal. Only abnormal labs are borderline low testosterone (300) and borderline low Vit D ((which I’m supplementing). Cervical and Lumbar MRI showed some minor issues with discs, mild spondolysis, but nothing that UCSF doc thought would contribute to my L5 radiculopathy/foot drop.

I’m very type A personality wise, so I’m not really a wait and see type guy and this a sooo hard, being in limbo like this. It’s defintely forcing me to grow.

My major questions:

1) can an EMG really tell if a raduculopaty is old or new? Stanford said EMG CAN definitely tell and they chalked it up up to an old L5 radiculopathy? UCSF doc said you CANNOT tell if the denervation/reinnervation is old or new, which is why she wants to re-do EMG in 6mos. If it were really “old”, the first EMG I had done on right side should have picked it up.

Any other thoughts about the Stanford EMG findings or language used (I’m not EMG literate)? “Denervation/reinnervation with associated reduced recruitment”—is this usually what is seen more widespread on EMGs when ALS is present?

2) I don’t feel like any of the docs I’ve seen really care to help me find answers and “wait and see” with something like ALS, can mean significant functional decomp., without any early intervention. Given the budding hopeful treatments in trail now (brainstorm and others), early intervention is so important. Therefore, should I seek a third opinion? If so, does anyone have any recommendations? I’m in California, but am willing to travel anywhere for real answers.

3) Would you recommend I be supplementing with anything specific now, even though I am in diagnostic limbo?

4) At what point does one ask about a lumbar puncture? No doc has mentioned it yet.

5)Any other thoughts, opinions or suggestions?

I know everyone’s time here is so valuable and I thank you in advance.
 
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Cali, can you post your latest EMG w/ identifiers removed? And the PFTs when available?

As to your questions, my responses are
1) Yes

2) Hard to say without better understanding differentials at this point, but to manage your expectation, you're not missing out on some sure thing disease-modifying therapy, and self-care is always the best palliation

3) Not knowing your history/what you take besides Remeron (which I would consider tapering/replacing if possible on the theory that you are experiencing side effects given the timing of everything as CNS issues are always possible with ADs), a RDA-level B complex can do no harm. When I hear possible dysphagia/dyspnea of uncertain origin, I don't want to talk too much about some of the supplements that can be sedating, for example.

4) An LP is only needed to help dx ALS if there are mimics it can rule out. I can't see any such in your case. But again, what are the differentials?

5) There is more going on here than meets the eye.

Why would Remeron be the first move for insomnia w/o concomitant depression, and if mood was a pre-existing issue, is there a contribution to the insomnia (chicken and egg) and potentially some of the other physical issues, that must be considered in light of rapid weight gain, etc.? For example, as you know, dysphagia can be exacerbated by anxiety, the latter of which you acknowledge.

Were sleep-disordered breathing and RLS ruled out? How much objective validation of clinical weakness and loss of musculature is there -- like what has the muscle strength testing said? Does this coincide with changes in diet/exercise regimen? Not knowing your baseline BMI, you're aware that rapid weight gain can contribute to some of the issues you describe.

Where is your PCP in this? You should have someone who does actually have a baseline.

I could go on but I don't really have much to go on. All I'm getting is that you're scared of ALS, there's deconditioning involved, and EMG(s) show tibia-area nerve issues. You wrote a lot, but there's just not that much to grab on to (actually a good thing in this case). Chart notes would be helpful as well in parsing potential differentials and how plausible ALS is at this point given the body of objective evidence.

Best,
Laurie
 
Wow, that’s a lot of scary symptoms.

The history raises the possibility of ALS, but the EMGs apparently tell a different story and two top-notch academic centers felt ALS was unlikely. I take it UCSF didn’t do an EMG because you had the three already?

If you post the summary and conclusions of the EMG you had at Stanford (black out identifying information), we could give you an opinion. Stanford has a great reputation and I would tend to believe them if they say they can distinguish between an old and new radiculopathy.

The “wait and see” approach is pretty typical, particularly in ambiguous situations.

My only suggestion would be to take the assumption that you do not have ALS as there are a number of disease mimics, and why assume you have the worst possible thing? It’s better for your mental health to assume you have a treatable condition. And go out and spend as much time doing fun things with family and friends. Meanwhile your PCP or general neurologist might advise on symptom management.

If your symptoms appear to be progressing at an uncomfortable pace, then your choices would be to seek a third opinion or to see if you could return to Stanford or UCSF in 3-4 months rather than 6. You could call them up in a couple months and explain what you’re noticing.

Unfortunately it can take months and sometimes over a year to confirm an ALS diagnosis — longer in slowly progressing cases. A clean EMG rules out ALS. I wouldn’t put as much stock in the EMG done by the physiatrist. they do a lot of EMGs but they tend to have a different focus and perspective. Even a general neurologist might miss things if s/he doesn’t see a lot of neuromuscular disease.

Best of luck to you.
 
Thanks so much for your reply Karen and Igelb.

Igelb-yes, I had a sleep study and apnea was ruled out but I do have some mild RLS stuff going on. The Remeron was only started in December, about four months after symptom onset due to the severe insomnia. I take half a tab of the lowest dose PRN and it works great. The only drawback is the weight gain. The muscle stength testing has been fairly unremarkable (although I had a very strong baseline) absent the right foot drop (can’t stand in RT heel). The concerning part is that I could stand on RT heel at Stanford, two months earlier.

Karen, yes, UCSF, said they wanted to do an EMG in-house, but wanted to wait six months from the last. The doctor said that if I had an L5 nerve root impingement upon MRI, she could said with certainty I didn’t have als. Unfortunately, the MRI, didn’t show an L5 impingement. I was praying it did as I’d take back surgery over MND, obviously, if I had a choice.

The actual summarized report was VERY scant as said the following:

Interpretation: motor and sensory nerve conduction studies are normal. Needle EMG shows evidence of chronic denervation with reinnervation changes in the right tibial anterior muscle with associated reduced recruitment.

Conclusion: These electrodiagnostic studies are suggestive of a chronic L5 radiculopathy. There is no evidence of widespread neurogenic abnormalities. Clinical correlation is advised.

I attached pictures of the needle EMG results.

Thoughts?
 

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My thought is that my initial impression that you don't have ALS is supported by the January EMG, and it's hard to believe anyone told you differently.

So my 2nd thought is that scans don't show all the spine issues that manifest as some of the kinds of issues that you describe, and therefore, I'd ask about a PT referral, esp. with new extra weight. And maybe breath training for the breathlessness. There are good devices for that, too.

Have you had a recent A1c done? Your risk for diabetes is certainly greater than before and of course diabetes can affect the nerves.

And as I said, with so many antidepressants and sleep aids out there, if Remeron helped you pack on the pounds, I would still reconsider it.

Summary: looks like you're in the wrong place, a very good thing. It's very common at an academic medical center to say, "Come back in six months," but if you want to feel better and have a plan, I'd go back to your internist at the moment.
 
Looking over your EMG and rereading your history, I have to agree with Laurie.

When denervation is confined to one muscle group, this suggests a localized process. You had so many other muscle areas which had completely normal EMG findings, i.e. no positive sharp waves or increased insertional activity, etc. and this is with you being symptomatic in so many areas.

Someone with ALS would have abnormalities in most muscle tested.

Also, two top notch university programs have told you no ALS. I’d have to agree with them.

You’ve got a life to live. Why spend it looking for trouble?
 
Greetings Karen and Laurie: it’s been three months since I’ve posted and six months since my last EMG.

Laurie, on your suggestion, I talked with my neurologist and she discontinued my Remeron and started me on Gabapentin (300-600 mg at bedtime) instead to help with my sleep and RLS Sxs. It has helped with my sleep, but weight gain has remained. My A1C was also fine. Had a pulmonary function test, which was mostly normal. Expiratory muscles were a couple points lower than expected, but they called it a normal PFT. I’ve also done some biofeedback breath training, as you suggested.

I saw a non-surgical spine physician who reviewed my MRIs and didn’t recommend any treatment, and said he’d have recommended only Gabapentin, if I wasn’t already taking it. He said the lumbar MRI didn’t show any L5 nerve root impingement after reviewing the cd copy in his office with me, and therefore doesn’t feel current lumbar issues are contributing to my tibialis anterior atrophy and inability to stand on right heel. He strongly recommended I continue pursuing the ALS rule-out. Ugh. My general neurologist and primary care physician have run out of ideas, or directions to go, to figure out what’s wrong, but something is definitely wrong.

I’ve continued to see my general, private practice neurologist, monthly. No clinical weakness detected, except inability to stand on right heel and noticeable atrophy of the tibialis anterior muscle on rt leg and right Calf is about 1/4 inch small than left. The interesting thing is, that the tib. ant. muscle is also somewhat numb to the touch (about 50%). I know numbness points away from ALS, but the atrophy and weakness also don’t appear to be lumbar spine related, so, that poses a problem of what’s causing it. CK is high normal (178) and last Aldolase was very slightly elevated at 8.2. Reflexes are intact, and a few are hyporeflexive, if anything, according to general neuro. Hoffman and Babinsky negative. My RT ankle is now feeling weak and sole of RT foot is very tender and appears to be atrophying as well. I’ve also noticed some slight atrophy in RT tricep. I continue to twitch in a wide-spread fashion, but right calf and now left calf twitch, virtually non-stop. RT thigh also appears smaller in mass than left thigh, and I continue to feel slight weakness in both thighs symmetrically. RT thenar muscle is VERY soft when compared to left.

I can still button my shirt, stand on toes (just not RT heel) and raise hands over head. My confusion surrounds the sticky saying “if you can continue to do normal things, you don’t have ALS”, but I can’t help but wonder if some people who have als had weakness first and muscle didn’t just stop working all of a sudden without any weakness or indication whatsoever, especially in slow progressors.

I continue to notice excessive saliva and have globus. Barium swallow study showed “early presbyesophogus” while in laying position, which is concerning as that would indicate weakness in swallowing muscle. I’ve also had an endoscopy which was unremarkable for acid reflux or any other issues.

It’s now been about a year since symptoms first started and six months since my last EMG. On August 20th, I’ll have my fourth and hopefully final EMG at UCSF ALS Clinic. My questions are:

1) First two EMGs were totally clean. Third was done at Stanford and showed denervation/reinnervation in RT tibialis anterior, which first two EMGs didn’t detect (although done be very competent and experienced professionals), yet they (Stanford) said it was from a chronic injury, but if it were chronic, the first two EMGs should have detected it. I know the sticky says, it can never be too early for an EMG to detect ALS, but in my case, something was detected on the third EMG, that the first two didn’t show. Does it ever happen where only one dirty area is first detected on EMG, in which a subsequent EMG then show more wide spread issues indicative of ALS?

2) Does ALS tend to present widespread (head to toe), just on one side of the body first (atrophy/weakness)?

3) Do you have any other recommendations on tests, rule-outs, differentials, or specialists I should potentially see? The primary care, two rheumatologists, and spine doctor have all said I should be pursuing the neuromuscular route, but then neurologists have said they don’t think it’s ALS, but want to take the wait and see approach to be positive.

Thanks so much for your time and assistance. It’s very much appreciated.
 
Your emg did not have an ALS pattern so no to question one

Question 2 ALS generally starts in one area a hand or a foot often and spreads

Question 3. Was your ncs utterly normal if not have you had a ganglioside antibody panel?
 
Hi Nikki,

Thanks so much for your reply. Roger-that on questions one and two. That makes me feel better. On #3, all three NCSs were completely normal. Should I still possibly inquire about a ganglioside antibody panel?
 
You could ask but if they say no doesn’t fit don’t push. It seemed like a long shot before I knew your ncs results. Even more now
 
Hi. I reread most of your thread. EMG at Stanford (your 3rd EMG) was done after you had complained of widespread fasciculations as well as muscles in the upper extremities getting smaller. Yet the only abnormalities on that EMG was in the right tibialis anterior muscle, and there was “no evidence of widespread neurogenic abnormalities”. Insertional activity and positive sharp waves were diffusely absent, even in the right tibialis anterior. As Nikki says, that is not an ALS pattern. It’s reassuring that EMG was completely normal in some symptomatic areas.

So, ALS appears highly unlikely. I would wait for your upcoming appointment and you can ask them what other conditions might be possibilities. Glad you’re getting this other opinion.
 
Greetings all. I had my fourth EMG today. I haven’t received the written summary as it isn’t completed yet, but was able to get the data from today’s EMG, which I’m hoping someone can help shed some light on. I’ve attached it.

The neuro doing the EMG today, talked very, very briefly with me post-EMG and said they’re going to recommend a neurogram w/contrast and have it reviewed by their neuroradiologist at UCSF. He said, not knowing my history, the EMG today points towards an L5 issue. He also said, when he takes my history into account though, knowing six months ago, I didn’t have any active denervation occurring (only old stuff) and now today I do have active denervation, and my recent Lumbar MRI not showing any L5 impingement, he’s not sure what to make of it. Especially since my first two EMGs were totally clean. He said he detected “issues” in three total muscles today.

I understand the fibs/psws in the Tib Ant. today indicates active denervation now, I’m not sure how to interpret the Volitional MUAPS and Max Vol Act and exactly what it all means. Is there old denervation in the other two muscles? The neuro doing the EMG today said that another EMG in six months is likely suggested, pending what my neurogram does or doesn’t show.

My RT Tib Ant. continues to atrophy as does the muscle in the rear of my right leg as well as RT thenar muscle and RT shoulder. The neuro today said that ALS, especially a slowly progressive case, can’t be completely ruled out (although not his first rule-in right now either) which is why he feels its smart to repeat the EMG again in six months. Your thoughts, impressions, suggestions are very much appreciated, so thanks in advance.
 

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Skimming your thread, we may've skipped your question of whether some muscles in slow progression would just stop working. It is not a muscle by muscle question, since movements involve more than one. It is a question of inability to do something, and if you have atrophy in areas that are not weak, that is a strong argument against MND. Also, it's not true that chronic injury shows up exactly the same way (if at all) on every subsequent EMG.

3 muscles with issues a year in and on the 4th EMG still seems pretty reassuring, esp. as we do not have evidence of either bulbar onset or diffuse limb onset as we would expect. You're not the first person to have an EMG that doesn't exactly match up to the MRI, but it sounds like another MRI is in your future anyway (the neurography). Still, even if it doesn't present an explanation, that doesn't mean that we jump straight to ALS. Even MRIs cannot see all, and they cannot explain everything they show, either.

The EMG itself is not a marker of disease progression as some people paint it. For example, we could see untreated tumors enlarging over time on CT or MRI, or track kidney failure with blood and urine tests. EMGs aren't like that. They're a snapshot in time, an art and science both to conduct and to interpret. That's why most PALS have no further EMGs once they are diagnosed.

You have several hundred skeletal muscles. The ultimate test for any significant muscle damage is how well they work.
 
Im sure this situation is really frustrating. The biggest thing that stands out to me is how functional you are despite the atrophy you report. When I was diagnosed there were several things I just couldn't do with my left hand but I saw no atrophy. It wasn't until the neuro pointed out to me very slight atrophy visible on my palm (slightly flat look). I would never have noticed it if she hadn't showed me, but what I DID notice were simple everyday things I suddenly either couldn't do at all, or could manage only half the times I attempted. I agree with the doctors telling you if you can do all your normal things you probably don't have ALS.

That said I would also want it ruled out unequivocally if I were in your shoes so I don't blame you for feeling anxious and frustrated. But I do think it seems highly unlikely with so many opinions from great centers, normal reflexes, mostly normal emgs, the lack of function loss, etc.
 
and i just realized i said the same thing as Laurie's first paragraph. sorry!
 
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