Positive EMG/yet since almost 4 years - looking for something else

ChristineRK

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Long story short: I had an EMG study by Dr.xxxxxxxxxxxx which showed changes in 4 segments of the neuraxis, also with peroneal nerve conduction block across the fibular head. One thought was if this could be ALS type of motor neuron disease. I had dropped about 40 pounds, had the tremors the uneven loss of muscle mass. Fast forward almost 4 years later and have a feeding tube to ensure proper nutrition and I know my muscles - they aren't even close to where they should be, but my new neuro (2nd opinion) stated that my muscles seemed fine. Whatever. Fast forward to the end of the report:


EMG showed denervation in left leg, right arm and leg and right thoracic paraspinals in 2015.

Has had work-up including HSP and ALS panel testing, however agree that with the above constellation of symptoms, atypical for ALS and need to consider other diagnoses.

Plan:
-- will review her case further and decide on next step, most likely will include repeat EMG study. Looking forward to what they may find - but has anyone EVER had this happen and what did it end up being? (I still have tremors and fasiculations - don't know if that is important or not). Could it be something totally different (if so - any suggestions? I get EXHAUSTED even after showering (once a week - gross!), or a slow progression?

I am open to hearing anything - thanks my friends!

~Christine
 

Nikki J

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I am glad you went for another opinion This is an ALS specialist I hope? Has an emg been scheduled?

supposedly ten percent of initial ALS diagnoses are later overturned so it definitely happens. There are a host of things that can look like ALS depending on presentation. Were autoimmune diseases ever discussed? MMN or CIDP?

generally these kinds of questions belong in the could I have ALS section. Did the current neurologist rescind your ALS diagnosis or was it more it probably still is, but since you are atypical we are taking another look?

it would be wonderful if this turned out to be treatable!
 

lisa g

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Christine,
I know the sense of bewilderment your feeling since I'm at the same crossroads.
In February of 2018 I began losing weight. I attributed to the fact that I changed from a vegetarian diet to a vegan diet. Than in June I started with an unsteady Gait and vertigo also at this time I noticed my speech was slower. I made an appointment with my PCP who was alarmed with what he saw an immediately referred me to a neurologist. I went to the neurologist in February of 2019 she started testing me but I didn't feel comfortable with her and felt she wasn't listening to me so I switched another neurologist in the group. It was a husband and wife team that were familiar with ALS since his father had it. I had my EMG on April 10th and returned on April 11th for the results. I was diagnosed with definite ALS and given a prescription for Riluzole.

I definitely wanted a second opinion so in July of 2019 I went to a neurologist from the Cleveland Clinic in Weston Florida. They ordered their own blood work and MRI'S all coming back normal. They then scheduled another EMG for August. Nerve Conduction was normal. EMG was clean except one area. Report stated, reduced motor unit potential recruitment and polyphonic motor units were seen in the left Abductor Hallucis and Extensor Digitorum Brevity. Increased motor unit potential amplitude was noted in the left Tibial is Anterior, Abductor Hallucis and Extensor Digitorum brevity. Increased motor unit potential duration was observed in the left Extensor Digitorum brevity. All remaining muscles sampled were within normal limits. Not being a physician I asked what does that mean? She said it was normal. She also said she wasn't going to tell me it wasn't ALS since there is definitely something going on with my symptoms but she wasn't going to label it yet. She told me to continue taking the Riluzole and that she would do another EMG in January. She also found no clinical weakness

Fast forward to today, I've felt no progression and have had some symptoms slightly improve. So now I guess I'm in limbo until the next EMG and start the process of worrying all over again when I had already gotten to the point of making peace with the diagnosis.
 

KimT

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Lisa,

If I were you I would go to Dr. Pulley at Shands in Jacksonville or Mayo in Jacksonville. Your EMG does not sound like ALS to me, especially without clinical weakness. My initial EMG was classic for ALS but I didn't have clinical weakness. The clinical weakness followed but at a very slow rate. Even so, after Mayo and Shands I went to Johns Hopkins for another opinion. I was advised by all three that if ALS is caught early, the EMG will be dirty even with lack of or slow clinical weakness. I honestly think you need someone who has seen hundreds of people with ALS and hundreds of people with mimics. Both Pulley and Mayo's neuromuscular doctors fit.
 

lisa g

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I was planning on trying to go to mayo after I had the next EMG so I would have 3 EMG's to go by. It seems like a never ending rollercoaster. Thanks for the advice Kim. Will have to plan that out mayo is about 4 hours from my house.
 

KarenNWendyn

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Christine, you clearly have a serious neurologic problem, but it is not clearly ALS. Keep in mind that what is important when it comes to diagnosing ALS is the big picture: clinical weakness as determined by a physician, characteristic EMG changes in multiple muscle areas, upper motor neuron signs, evidence of disease progression, and ruling out ALS mimics. We encourage everyone who might have ALS to be evaluated by a neuromuscular disease specialist. Occasionally people are misdiagnosed with ALS, which is why second (and sometimes third) opinions are helpful.

As Nikki says, autoimmune conditions, CIDP, and MNN are conditions that can present with an ALS-like picture. Our sticky “Read before posting” sticky lists other conditions that have been diagnosed in people who were being evaluated for ALS.

To both Christine and Lisa, I know that receiving a diagnosis of ALS is a terrible shock and it takes a lot of time and self-work to come to terms with accepting the diagnosis. I hope both of you end up having something else. Whatever any of us “have”, doing what we can still do and living fully in the moment is always a good idea.

I’ll also add that we are always happy to take a look at someone’s EMG. Just black out identifying information and post it.
 

lisa g

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Thanks to you both Karen and Kim for your insight. I definitely plan on following up with the neurologists. Like I said I had already come to terms and peace with the initial diagnosis so to hear that the last EMG is considered clean really puts a spin on things and once again leaves me second guessing any thing I feel. I guess the only thing to do is wait and see how everything plays out.
 

lgelb

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To Karen's point, Christine, your EMG reports both mentioned the possibility of multifocal motor neuropathy (MMN) and suggested depending on clinical features that blood testing for anti-GM antibodies (part of the ALS dx process anyway) and possibly an IVIG trial could be warranted.
 

KimT

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Lisa,
Have you had a lumbar puncture? Don't worry about having three EMGs. I've had a dozen, some for training purposes for new doctors. Also, Mayo has a hotel on site so if you go, you might want to consider staying there. Four different doctors at Mayo did EMGs on me and it didn't even hurt. Not so at other places.
 

lisa g

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Kim,
Never had a lumbar thought about asking the neuro about having one. Not worried about the EMG pain is temporary. Last EMG was tolerable until the inner foot area. You get through it. I will definitely be looking into mayo. I think Dr. Pulley diagnosed Kevin M also.
 

ChristineRK

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08/2015
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I am glad you went for another opinion This is an ALS specialist I hope? Has an emg been scheduled?

supposedly ten percent of initial ALS diagnoses are later overturned so it definitely happens. There are a host of things that can look like ALS depending on presentation. Were autoimmune diseases ever discussed? MMN or CDIP?

generally these kinds of questions belong in the could I have ALS section. Did the current neurologist rescind your ALS diagnosis or was it more it probably still is, but since you are atypical we are taking another look?

it would be wonderful if this turned out to be treatable!
Hi Nikki - sorry for the late response - I've been AWOL for awhile. No, my current neurologist did not rescind my ALS diagnosis - that is what she said almost exactly - 'you probably still have ALS, but since you are atypical - we are going to take another look'.
 

ChristineRK

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Joined
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Messages
55
Reason
PALS
Diagnosis
08/2015
Country
US
State
OH
Christine,
I know the sense of bewilderment your feeling since I'm at the same crossroads.
In February of 2018 I began losing weight. I attributed to the fact that I changed from a vegetarian diet to a vegan diet. Than in June I started with an unsteady Gait and vertigo also at this time I noticed my speech was slower. I made an appointment with my PCP who was alarmed with what he saw an immediately referred me to a neurologist. I went to the neurologist in February of 2019 she started testing me but I didn't feel comfortable with her and felt she wasn't listening to me so I switched another neurologist in the group. It was a husband and wife team that were familiar with ALS since his father had it. I had my EMG on April 10th and returned on April 11th for the results. I was diagnosed with definite ALS and given a prescription for Riluzole.

I definitely wanted a second opinion so in July of 2019 I went to a neurologist from the Cleveland Clinic in Weston Florida. They ordered their own blood work and MRI'S all coming back normal. They then scheduled another EMG for August. Nerve Conduction was normal. EMG was clean except one area. Report stated, reduced motor unit potential recruitment and polyphonic motor units were seen in the left Abductor Hallucis and Extensor Digitorum Brevity. Increased motor unit potential amplitude was noted in the left Tibial is Anterior, Abductor Hallucis and Extensor Digitorum brevity. Increased motor unit potential duration was observed in the left Extensor Digitorum brevity. All remaining muscles sampled were within normal limits. Not being a physician I asked what does that mean? She said it was normal. She also said she wasn't going to tell me it wasn't ALS since there is definitely something going on with my symptoms but she wasn't going to label it yet. She told me to continue taking the Riluzole and that she would do another EMG in January. She also found no clinical weakness

Fast forward to today, I've felt no progression and have had some symptoms slightly improve. So now I guess I'm in limbo until the next EMG and start the process of worrying all over again when I had already gotten to the point of making peace with the diagnosis.
Hi Lisa!
That 'limbo' is the absolute WORST!! I find it is really messing with my mind. I'll even tell myself on certain days 'I don't have it!' - then I will go up and down some stairs and really feel the fasiculations and weakness afterward. What I am also not a fan of - was that my new doc said 'there doesn't seem to be much muscle mass loss' - but she didn't even KNOW me before! I used to have 'huge' calves (which was the first thing I noticed that was really much 'smaller' than before - along with losing 40 lbs. I was also VERY shocked when I saw an actual picture of myself - my friend got me a new t-shirt for my birthday and I posed for a pic - and when I looked at it, my shoulders are SOOOOO much more 'boney' than they were before along with my chest muscles. I mean I was SHOCKED. It's very frustrating when you hear back 'well, you're probably smaller because you stopped lifting weights'.....................noooooooooooooo..........I stopped lifting weights because my muscles were getting weaker. That was another 'tip off' that I knew something was wrong. I couldn't increase my weight lifting poundage and actually had to decrease it. Is this a point where I find another doc - but no one knew me before. I don't know.....frustrating and disappointing. My next appointment, I'm taking a friend of mine who knows the 'old me' and is going to give her opinion so the doc doesn't think I'm 'over exaggerating'. So, because of comments like these, the weight loss, the inability to weight lift any more, I get tired very easily (I sleep A LOT), and 2 dirty EMG'S. Yet I'm still able to 'do things' that people who weren't as in good of shape (NOT bragging) and ability than I was and I'm progressing slower - this last 4 years has REALLY been a 'mind-bender' almost all of the time. :-(

~Christine
 
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