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BernardBlack

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I posted a while back about my mother who had trouble swallowing and partial loss of speech / dysphagia back in december 2013 and we have now been through two EMGs, as her symptoms are more or less the same (although worsening a little bit).

The results look really bad yet the doctors do not want to give a diagnosis.

She's scheduled for another EMG in a couple of months but I would appreciate it immensely if someone could take a look at the results.

I apologize for the wall of text in advance. Although I understand that each persons time is precious I hope anyone has the time and opportunity to read this and give a comment.

First EMG (2013-12-19):

M VOCALIS BILATERAL, M CRICOTYROIDEUS BILATERAL
No certain spontaneous activity. All muscles show increased duration, amplitude potentials, sometimes of complex or polyphasic characters. At maximal activation a sparse activity was displayed with probable light to moderate loss of units. Changes were equal on both sides but the right vocalis muscle showed more rebuilt motor unit potentials.

ASSESSMENT
Moderate changes of neuro character in all examined muscies with loss of units and rebuilt motor unit potentials. Especially on the right side, it was clearly displayed and the EMG speaks of a change which is not recent. (Older)

Second EMG (2014-02-20)

M TRAPEZIUS diagnosed, TONGUE, M ORBICULARIS ORIS SIN, M ORBICULARIS C(cuts off)
No spontaneous activity. Substantially normally configured MUP. Normal interference pattern at maximum activation.

M ORBICULARIS ORIS diagnosed:
No spontaneous activity. Many MUPs were normal, but there was a sparse to moderate occurence of split duration MUPs with sometimes increased amplitude. In some needle positions there was a smal reduction at maximum activation, in other needle positions there was a normal interference pattern.

JITTER ANALYSIS WITH CONCENTRATION NEEDLE
M ORBICULARIS ORIS diagnosed: 20 potential pairs were registered with mean MCD 43 microseconds 18 of the potentials showed normal jitter. 2 showed increased jitter at 122 and 91 microsecond. One of the potentials was heavily split. (split may mean polyphasic)

REPETITIVE NERVE STIMULATION
Stimulation was done at 3hz during rest, directly after activation and 1 and 3 minutes after activation:

N FACIALIS WITH REGISTRATION FROM M NASALIS SIN:
Stable muscle responses without significant decrements or increments.

N ACCESSORIUS WITH REGISTRATION FROM M TRAPEZIUS diagnosed:
Stable muscle responses without significant decrements or increments.

ASSESSMENT:
- Repetitive nerve stimulation showed normal readings with registration from m nasalis and m trapezius.

- EMG showed normal readings in tongue and other bulbar musculature, aside from m orbicularis oris diagnosed. In this muscle there was a sparse presence of neurogene changes, mostly of chronic character.

- Jitter analysis using needle shows increased jitter on 2 of 20 potential pairs. This is considered related to the neurogene changes.

CONCLUSION:
No electrophysiological basis for neuro changes considered to be Myasthenia Gravis.

In denervation in bulbar musculature. Small neurogene changes in M orbicularis oris sin.

In conclusion with the previous EMG there is no evidence or basis for motor neurone disorder.


These are translated from another language, but it should be factually correct, aside from some clumsy wording.

I have read the results and compared them to this booklet:
http://www.zielinskifam.com/lit/neuro/als/emg-early-als.pdf

I'd love for this to be anything but ALS; but there are a lot of checkboxes ticked in the booklet. We haven't even been scheduled yet for the next EMG (they keep saying it's a couple of months away), and we are thinking about going abroad for help. We are located in Europe, thinking about traveling to Israel where there seems to be some exciting progress being done there. If anyone has any tips for European ALS clinics that can provide a better diagnosis and/or are doing experimental research, please write as well.

Thank you for your time.

PS2.
Previous thread here:
https://www.alsforums.com/forum/do-i-have-als-als/24455-possible-pbp-als-need-help.html
 

BernardBlack

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There is a small typo in the second EMG, CONCULSION section, second line.

The sentence should read:

No denervation activity in bulbar musculature. Small neurogene changes in M orbicularis oris sin.
 

Nikki J

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Which parts worry you particularly? It does not seem to have the characteristics of an ALS EMG to me but your physician should be able to explain the findings.
 

BernardBlack

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Which parts worry you particularly? It does not seem to have the characteristics of an ALS EMG to me but your physician should be able to explain the findings.

Thanks for answering. In the first EMG it says "All muscles show increased duration, amplitude potentials, sometimes of complex or polyphasic characters."

Is that a clear sign of motor neuron disease, or could it be attributed to something else? They've already pointed out its not MS.

Also the increased jitter on 2 of 20 pairs in the second EMG.

When I read the booklet I linked, it may qualify as early ALS.

If you have a different interpretation, feel free to share it..
 

Nikki J

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It is way beyond the scope of this board to interpret EMGs but I believe there are certainly other causes of the findings in EMG 1 and it lacks other characteristics needed for the diagnosis of ALS such as fibs and positive sharp waves. I don't see that those muscles were retested in EMG 2 but I think part of the report is missing? Increased jitter is not usually talked about in ALS. I think they were looking for Myasthenia and did a single fibre EMG? I do know elderly have somewhat increased jitter ( how old is she?) and it looks like what they saw was attributed to the chronic neurogenic changes. ( and apparently that cause is undetermined). You need to ask your physicians to explain this to you and tell them why you are worried. That paper was a highly technical academic paper and not intended as a guide to interpretation for the non professional. I am guessing you are not a neurologist?

You obviously care very much for your mother and she is lucky to have you. It is frustrating to have no answer but it sounds like they are following her closely. Sometimes it takes time for neuro conditions to declare themselves fully. The longer it does not declare itself ALS I think the less like ALS is. A wise person said no need to chase ALS, ALS will find you.
 

BernardBlack

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It is way beyond the scope of this board to interpret EMGs but I believe there are certainly other causes of the findings in EMG 1 and it lacks other characteristics needed for the diagnosis of ALS such as fibs and positive sharp waves. I don't see that those muscles were retested in EMG 2 but I think part of the report is missing? Increased jitter is not usually talked about in ALS. I think they were looking for Myasthenia and did a single fibre EMG? I do know elderly have somewhat increased jitter ( how old is she?) and it looks like what they saw was attributed to the chronic neurogenic changes. ( and apparently that cause is undetermined). You need to ask your physicians to explain this to you and tell them why you are worried. That paper was a highly technical academic paper and not intended as a guide to interpretation for the non professional. I am guessing you are not a neurologist?

You obviously care very much for your mother and she is lucky to have you. It is frustrating to have no answer but it sounds like they are following her closely. Sometimes it takes time for neuro conditions to declare themselves fully. The longer it does not declare itself ALS I think the less like ALS is. A wise person said no need to chase ALS, ALS will find you.

Thank you so much for answering Nikki.

They did do a single fibre EMG, but it showed no signs of Myasthenia. My mother is 47 years old, so not that old..

You are right, interpreting is about more than just reading the words. I'm trying to put it aside, but my mothers condition isn't improving (maybe even worsening a little) and we're all very afraid.

They're doing another EMG but not until sometime october-november. I'll be sure to catch up here regardless of what happens. Thanks to you and everyone else volunteering your time to this, it means the world to me.
 

nebrhahe53

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We are not doctors here, just people either caring for dying people or dying people. I think the one sentence I caught was > In conclusion with the previous EMG there is no evidence or basis for motor neurone disorder.

It would seem to me the doctors do not believe ALS is present. However I will give you a piece of advice I followed when I was being diagnosed:

My body did not feel right to me-there was clearly something wrong. The first neuro I went to did an EMG and gave me a clean bill of health-she was not experienced with ALS.
I paid my own way to a nationally known ALS clinic and was seen by the clinic head. Within 5 minutes, even without an EMG, he diagnosed me with ALS. He did EMG the next day to confirm and it was dirty everywhere he looked. This was 6 days after the other neuro cleared me, so if in doubt, see a neuro at an ALS clinic.
I wish you well.
 

MaxEidswick

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>I paid my own way to a nationally known ALS clinic and was seen by the clinic head. Within 5 minutes, even without an EMG, he diagnosed me with ALS. He did EMG the next day to confirm and it was dirty ... see a neuro at an ALS clinic.

and *that* is the truth! Damn Stanley ...
 

BernardBlack

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Hi everyone,

My mothers condition has worsened with more bodily symptoms such as fatigue, weakness and fasciculations.
We've got an additional EMG (with more detailed single fibre testing). They have tested additional muscles like arms and legs and have found issues in those as well. (Polyphasic MUP)

However, they only found increased jitter in 6.4% of single fibres tested (threshold is 10% according to a diagnostics manual linked earlier).
They are also claiming there is no spontaneous activity and no "active" denervation. Due to this they don't want to commit to an ALS diagnosis.

It's really depressing that her symptoms are spreading, and awful not to have a diagnosis. They don't even want to do any further testing and are offering immunosuppressants and cortisone. We read about IVIG treatments so we're gonna try to suggest that but it's really just random stabs at the problem.

One question that I have is how there can be polyphasic MUP:s and increased jitter -without- deenervation? I thought that the presence of polyphasic MUP and increased jitter was the hallmark of deenervation...

Thanks everyone for reading.
 

BernardBlack

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I rephrase my question a bit:

Is it possible to have atrophy without active denervation? My mother has severe atrophy in the bulbar region and has a hard time eating anythin but liquids. Now body issues as well. Dirty EMG:s. So how can it be suggested there is no active deenervation with these symptoms...
 

Atsugi

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If your mother doesn't trust her doctors, she could get another doctor, who might commit to a diagnosis.
We're not qualified to second guess her doctors.
 

affected

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Is your mother being seen by an ALS specialist neurologist?

If not, that is what I would do next, but sorry I'm not at all good on answering EMG questions. Maybe Dusty will have some comments on that side of things.
 

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Hi affected,

He's a respected neurologist but I don't think he is specifically geared towards MND, although he has published papers on myopathy and similar disorders. We live in the small country of Sweden, which doesn't have a dedicated ALS clinic at all, only some specialized departments at hospitals. I think our neuro doc is probably one of the leading neuros in Sweden and every EMG gets a second opinion by another doctor. There's really nowhere for us to go.
 

affected

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Thanks for explaining that Bernard.

In Australia there are not many neurologists at all experienced with MND either. We had to travel 3 hours to see on, many rural people have to travel much further. The next closest one would have been 8 hours travel away.

We did know however that seeing any other neuro would have been a waste of time as they would not have the expertise.

Have you asked him then about your questions you are asking here concerning denervation? Maybe asking him to really sit down and explain exactly where they are at. I know that if they don't know the answer they don't want to do deep discussions. I know how it was for us when doctors we were seeing didn't know what was wrong. They didn't want to talk too much about it, just try and send us on to someone else.

Did you find out if there are any other European ALS clinics you could try and get her to?
 
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