LMND - what is the difference with ALS/MND?

[KarenNWendyn]

Maybe to further clarify and perhaps confuse ...... a peripheral nerve is a conglomeration of various nerves leaving the spinal cord. Eg the sciatic nerve is formed by nerves exiting the spinal cord at several different levels.

Sensory nerves can also be peripheral nerves, as shown in the diagram.

Shiftkicker, I think we’re both right. I was trying to present it in a very simplified (maybe too simplified) way.

 

[affected]

Karen you have nailed it for me, and I can now see where I was confused by what you originally said, and on top of that, I have a better 'picture' of which are which.

all the upper motor neurons originate in the frontal cortex (motor strip) of the brain. Their cell bodies are in that strip of cortex.

the neurons exiting the brainstem are LMNs

So it's about the cell bodies and where they start, but of course (do'h moment for me) there are regions where both UMN and LMN are so they can synapse.

What a great team effort to get that clear until next time I confuse myself :lol:

 

[Clearwater AL]

I need some help following this... there has been mention of the frontal lobe, frontal cortex
and (motor strip). Is the "motor strip" mentioned the Primary Motor Cortex?

My simple understanding of UMN is that the role of the Primary Motor Cortex is to generate
neural impulses that control the signals to muscle movement. Thus... Primary Lateral Sclerosis.

Flawed or weak signals to the muscles... or both.

?

 

[Foxmark]

Hi jethro,
Your situation seems very similar to what happen to my mum.she started in 2013 with a problem in walking,then has a foot drop that claim for an AFO in 2014.the situation more or less is the same until last year that she spoke about a little down in syntomps, only to leg (especially left). In the end of the year after neurologist talked to possible patrikios variant she came to Turin Als centre and in february 2018 she ha diagnosis of 2nd motor neuron disease (2nd means lower).from feb to june she notices that things going down slowly but down,always related to legs.she is taking riluzolo and in my mind it is a possible side effect of the pills this weakness in legs.the other district are fine,now she use a cane,a walker and a weelhchair.in april to the als centre they wrote mnd without any reference to upper or lower.but she has problem only in legs.our worries are about the future...everything started in 2013 so don't match with pure als but doctors cannot say no it isn't....difficult to understand and also difficult to live without any clear view about future...

 

[jethro]

@foxmark thanks for sharing your experience. i scream, but nobody hears me. i know that "this" is a variant of ALS, little less terminal, but obviously nobody knows details, even wikipedia. my neuro allways extracts negative elements to fill up the form/diagnose when he asks me "what's new?" . to change dr. in such a small country is imposible. we have Hospital Information System (HIS), so wherever you go, dr. has access to it and you are labeled. nobody wants to waste energy to hear you, not to mention - to correct dg. or put something in HIS what is contrary to expert work. just look into HIS and you don't have to say even word.
obviously we have same variant of disease which is very rare IF SHE IS NOT MISDIAGNOSED (what emg says?). i'm more likely to have a pure ALS, cause i have it for a 3 years, she has "it" for a 5 and didnt spread! ALS is a rare disease, app 2-8:100 000, but this is 1-4:100 000 0 as far as i find (5% of pure ALS). this depriciate all achieved on the (battle)field trying to define what ALS actually is and it's mechanism. i got recommandation to go to italy, Casa Sollievo della Sofferenza Hospital-operapadrepio while i wanted to confirm diagnose, or contact xy (i'll send you private message), unsuccessfuly. stay in touch! i'm not expert, but i'm sure it is not side effect of riluzole. if she has not any problems with liver enzimes (AST, ALT), she shouldn't stop use it even benefit is not remarkable.

 

[Nikki J]

Jethro and foxmark. Many of us are confused about both of your situations. I know they are complicated and I know language can be difficult to translate when we are discussing extremely technical situations. It would help if you could answer directly a few questions ( jethro for yourself, foxmark for your mother)

1 does the emg show widespread acute and chronic denervation in any area? If so which one(s)? Widespread means arising from more than one nerve root

2 do you have upper motor neuron signs on examination other than any hyperreflexia? ( clonus more than 6 beats, Babinski, Hoffman, spasticity, jaw jerk)

Thank you both in advance

 

[Foxmark]

hi Nikki!

1 as i remeber denervation is present in legs, severe in left, moderate in right. no other signs, only some sporhadic fasciculation in left shoulder present in EMG
2 no Babinski, no Hoffman, no spasticity, no jaw jerk, some hyperreflexia in arms

thanks in advance!!

 

[Foxmark]

jethro no liver problems from february...

 

[KarenNWendyn]

Al, to answer your question, yes, the motor strip and motor cortex are the same. PLS is a pure upper motor neuron disease. And then the lateral corticospinal tracts get into the picture as well.

The corticospinal tracts contain the upper motor neurons that originate in one area of the primary motor cortex. These axons synapse either directly or indirectly on the anterior horn cells in the spinal cord. A single corticomotoneuronal axon synapses with many anterior horn cells of its own motor neuron pool and also with those of agonists and antagonists, allowing for coordination of highly skilled movements.

Now ain’t that confusing.

 

[Nikki J]

Thanks Foxmark! If the denervation is acute and chronic then it sounds like what your doctors call second motor neuron disease is what we call PMA. They used to think it was slower progressing than ALS but I believe the current thinking is that that only holds true if only one leg is affected for several years

 

[KimT]

I'm lower motor neuron dominant BUT Mayo and U of FL thinks I have something else going on as well. Comorbidity can confound trying to figure everything out. I'm going back to see a rheumatologist in August. I was diagnosed with fibromyalgia in 1999 at Mayo but they want to do some other bloodwork to figure out why my joints ache so much and I have so much pain. They don't believe ALS can cause this type of pain when one is still able to move about freely.

Laurie and I discussed this a year or so ago. I just decided to treat symptoms but Mayo seems to think we need to be sure there isn't something else that might respond to a different type of treatment. They have a two-day program for fibromyalgia that they think might help with pain, range of motion, etc. They thought it would be well worth it considering my slow progression. They said nothing I would do in the program would be contraindicated for ALS.

 

[Nikki J]

Kim if there is a chance of improving your quality of life do it! I am LMND and I think we are not dissimilar in our mobility and I have never had any pain ( knock on wood). Unfortunately there is no rule that you can’t have multiple issues as you know already

 

[jethro]

@nikki appreciate a lot for assistance! i'm affected, but foxmark is a cals.
i'll try to decribe exactly what i have in diagnosis following your question's direction. i found last emng.
1.
* "severe loss of motoneuron" found on right foot muscles, posterior tibialis,
less severe(?) loss of motoneuron found on left foot and left post&anterior tibialis, right anterior tibialis, both quadriceps AND MUSCLES IN UPPER RIGHT ARM AND FOREARM AND RIGHT TENAR
*. moderate in muscles of left tenar, both hipotenar and left forearm.
----dg: examination shows mainly LMND, mild decline according to emng done 8 months before.
THIS IS REUMATOLOGY-PHISICAL MEDICINE AND REHABILITATION DPT, not neurology, but emng is well done.
2. NEURO: hyperreflexia, 3+ . clonus is rare, but present. fasciculations are non stop all over body except head (it started on a right eye blinking in 2015)., babinski negative, hoffman have not examined, spasticity positive, jaw jerk negative. saliva seem to be started, same as slowly speech (not nasal by now, but slowly). fasciculations not clear, no visible sign of atrophy.

as everybody can read, it shows tipical ALS,

but i have no problems of anykind, just legs. (cramps i have in right hand for 21 years, can not write with pen for 21 years.)
**

NEURO: last noticed 3 months ago, "left arm atrophy". (now it looks like it looked as usual.)
go to gym 2x week, doing everything like before, exept legs. at once max. 500met can walk. right hand is a little bit weaker than right, but it is 21 years like that.
subjective: legs atrophy (i have knee pads, so i can compare how they fit now and 6 months before), hands - no atrophy

thank you.

 

[Foxmark]

My mum feel the sensation of when she is on standing position,after few minutes she needs to sit to avoid any falls.she lives in the 3rd floor of a building without elevator and until now she has to climb this stairs. We know that in the future we have to move from here. also aspect on mental side affect sensation and feeling from my point of view

 

[Nikki J]

Hi Jethro. Loss of motoneuron is not terminology used here so guessing that means acute and chronic denervation? Did you have fibs and psws? Comments about nups and recruitment?

ReUMN you have spasticity, 3 plus reflexes and “ rare” clonus. If the clonus is not sustained it is really the spasticity that counts but yes that is UMN and if combined with a diagnostic emg and mimics excluded would be ALS. It is not unusual to have EMG findings beyond your symptomatic area(s)

Thanks for sharing