motor neurones in feet & ankles

[Rhuraidh]

My consultant doctor has told me that I have an unusual presentation of motor neurone disease but it is not typical ALS. The motor neurones have died off in my feet and ankles. I am looking for other people who have the same symptoms please. Or perhaps someone can suggest a possible explanation of my symptoms?

 

[notme]

If you've had an EMG, I'd suggest posting the results to a man named Wright.

Most people have ALS start either in the feet or the hands.-- the rest normally have bulbar (mouth area) onset--so it doesn't sound that strange to me...but perhaps he saw something in the EMG that wasn't typical of ALS presentation?

 

[Rhuraidh]

thank you for your prompt reply and thanks for your suggestion which I will take up.

 

[Nighthawk]

Hello,

As far as I understand, Motor Neurons are located mainly in the brain stem and the spine. From there, they control the muscles in your arms, hands, legs, feet, etc.
Once they get affected and start dying, then your start to notice weakness and fasciculations in the muscles nourished by these Motor Neurons.

HTH.


NH

 

[SMP51]

before we went to Mayo clinic we were told as well that my husband was not "typical" ALS, even at Mayo we were told at first they were not sure because it was not typical and it would either stop at his feet or eventually reveal itself.
I don't know what testing you have done but my Husband had MRI, EMG, gamigoblin and steroid infusions to rule out CIDP, 24 vials of blood tested for god knows what and a nerve biopsy.
The nerve biopsy was the silver bullet
from my reading here I have come to think no ALS is typical and on the flip side, many things are quickly and wrongly suggested as ALS. therefor it is much safer to say something seems like not typical ALS, if they don't know for sure yet the last thing you want to do diagnose someone with it!

 

[Rhuraidh]

Hello,

As far as I understand, Motor Neurons are located mainly in the brain stem and the spine. From there, they control the muscles in your arms, hands, legs, feet, etc.
Once they get affected and start dying, then your start to notice weakness and fasciculations in the muscles nourished by these Motor Neurons.

HTH.


NH

You are quite correct. I should have said that the electrical tests showed up as the motor neurones affecting the nerves in my feet.

 

[Rhuraidh]

before we went to Mayo clinic we were told as well that my husband was not "typical" ALS, even at Mayo we were told at first they were not sure because it was not typical and it would either stop at his feet or eventually reveal itself.
I don't know what testing you have done but my Husband had MRI, EMG, gamigoblin and steroid infusions to rule out CIDP, 24 vials of blood tested for god knows what and a nerve biopsy.
The nerve biopsy was the silver bullet
from my reading here I have come to think no ALS is typical and on the flip side, many things are quickly and wrongly suggested as ALS. therefor it is much safer to say something seems like not typical ALS, if they don't know for sure yet the last thing you want to do diagnose someone with it!

Do you mind if I ask you why the nerve biopsy was the silver bullet? As my case is not clear cut, I also have had a nerve biopsy. What did your husband's nerve biopsy reveal?

 

[johnnyliverpool1]

...a neuron is a brain cell..the brain is made up of 100 billion of the little blighters......yes, a 100, 000,000,000.....stretch em out in a line , that would reach 1000 km or 600 miles..imagine if one cell was playing up, how would you find which one.....no wonder there is no cure.............johnny

 

[SMP51]

IF I am recalling it rightly, when the infusions didn't help with what they were hoping was CIDP ( a problem like LAS/MD which remains in the extrimities) the nerve biopsy was to look at they mylon sheath of the nerve and deterioration of it....it ruled out CIDP, or perhaps it confirmed ALS? the chain of events are a little blurry being as we have also now been part of a drug study, a wild ride of a year.
anyway we were left waiting for it to advance or not.....WE noticed a diagnosis on a bill at the local level before any one confirmed ASL positively, was it for filling a box for insurance or the doc here who really likes my husband couldn't bring himself to go there I am not sure.

 

[wright]

That sounds like your neuro might be referring to Charcot-Marie-Tooth disease, which typically affects the feet and lower legs. It's actually a peripheral neuropathy, though . . . so my assumption could be wrong.

I know you have PM'd me and asked how you could show me your EMG. You have two options: 1) transcribe the results and impression in this thread or 2) if you want a bit more privacy, attach it as a picture in your profile and add me as a friend so I can see it

 

[SMP51]

CIDP is what we were hoping he had, a peripheral neuropathy.
Chronic inflammatory demyelinating polyneuropathy - Wikipedia, the free encyclopedia

 

[Rhuraidh]

IF I am recalling it rightly, when the infusions didn't help with what they were hoping was CIDP ( a problem like LAS/MD which remains in the extrimities) the nerve biopsy was to look at they mylon sheath of the nerve and deterioration of it....it ruled out CIDP, or perhaps it confirmed ALS? the chain of events are a little blurry being as we have also now been part of a drug study, a wild ride of a year.
anyway we were left waiting for it to advance or not.....WE noticed a diagnosis on a bill at the local level before any one confirmed ASL positively, was it for filling a box for insurance or the doc here who really likes my husband couldn't bring himself to go there I am not sure.

I'm interested in the biopsy as this was the only test that showed up anything that was a conclusive result. Do you recognise any of the following information that was the same or different to your husband's report?

The nerve biopsy showed a normal population of large and small myelinated fibres and there is no evidence of axon degeneration. One of the epineural blood vessels is surrounded by a rather frequent and convoluting capilliaries (neovascularization) and the lymphocyte markers indicate the presence of inflammatory cells around several (other) epineurial blood vessels, but not qualifying for a vasculitic process.

and in the conclusion of this report

A sural nerve biopsy showed no evidence of neuronal loss, and the significance of the inflammatory cells is uncertain.

And in the conclusions of the report :-

 

[Annie's Phil]

Based on my limited understanding, I think the meaning of your report's conclusion is that there is no evidence of peripheral neuropathy. The new capillaries surrounding one of the blood vessels supplying the nerve, though present, do not qualify for a diagnosis of vasculitis (one cause of peripheral neuropathy.) There is also no evidence of demyelination, which ruled out CIPD as SMP51 mentioned, and other demyelinating diseases such as multiply sclerosis. So I believe that your sural nerve biopsy is evidence that the problem lies further upstream on the nerve pathways.

The biopsy of the sural nerve can not give positive evidence of als. It can provide evidence of peripheral neuropathy. Ruling out a nerve disease process in the sural nerve implies that the problem must be somewhere else. Als is one possibilty, but not the only one. That's why other tests, especially the emg, are required.

Regarding your symptoms, my wife's began the same way. First, foot drop in her left leg. Then the same in her right leg. Weakness spread up her legs, then into her torso. Then it moved down her arms and eventually hands. She never had bulbar symptoms.

Perhaps your Dr. has other data to lead him to the conclusion that you have an unusual presentation of als.

We're all hoping it's something else.

 

[Rhuraidh]

thanks for link

 

[Rhuraidh]

...a neuron is a brain cell..the brain is made up of 100 billion of the little blighters......yes, a 100, 000,000,000.....stretch em out in a line , that would reach 1000 km or 600 miles..imagine if one cell was playing up, how would you find which one.....no wonder there is no cure.............johnny[/QU

Couple of areas that I'm following are the Nogo A drug trial and the NurOwn stem cell clinical trial at the Hadassah Medical Organization in Israel.