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Wright can post here in this thread or on your profile page.
 
Hello, I dont mind whereever Wright gives me his opinions. Nothing to hide, only hope he notices this post here!

Thanks so much!
 
My apologies for not getting back to you sooner and my regrets for the reason you are here . . . but you couldn't find a better place to be for comfort.

I'll comment on the EMG and then give you my opinions and impressions to your story. Before I do any of that, let me explain what a motor neuron is, what a sensory neuron is and what a nerve is and I will do it in as layman a way as I can.

A motor neuron is a type of nerve cell that resides both in the upper part of the brain, the lower part of the brain (called the brainstem) and the spinal cord. Those that are in the upper part of the brain are called upper motor neurons and those that are in the brainstem and spinal cord are called lower motor neurons. The lower motor neurons directly contact our muscles and therefore directly control our muscles. The upper motor neurons contact the lower motor neurons but do not contact the muscles directly. Therefore, the upper motor neurons control the lower motor neurons and thus indirectly control our muscles.

A sensory neuron is a type of nerve cell that allows us to sense things in our environment. Therefore, it allows us to feel touch, pressure, pain, heat, cold, etc.

A neuron (whether it is an upper or lower motor neuron or a sensory neuron) is made-up of a soma (kind of like the "head" of the cell) and from that soma are "stringy" projections called axons (kind of like "arms that stem from the "head"). These axons are used for communication. The axons of upper motor neurons communicate with the lower motor neurons and the axons of lower motor neurons communicate with our muscles. Sensory axons relay sensory information to our brain. A bunch of axons bunched together is what a nerve is. Think of a nerve as a rope and the axons as the individual fibers of the rope.

With ALS, the soma's of lower motor neurons and upper motor neurons are destroyed. If the soma's are destroyed, that will start to destroy the axons. That gives rise to the signs of symptoms of the disease. When lower motor neurons are destroyed (because the lower motor neurons are directly controlling our muscels) it causes muscle weakness, followed by muscle atrophy and fasciculations. Damage to the upper motor neurons can be detected by an EMG. When upper motor neurons are damaged, it causes brisk reflexes, pathological reflexes, spasticity (very stiff muscles) and muscle cramps. The reason is because upper motor neurons inhibit lower motor neurons a bit. Therefore, when the upper motor neurons are destroyed, that inhibition is lost and that results in muscles that are over-stimulated and reflexes that are over-active, too.

With ALS, the sensory neurons and nerves are unaffected.

Now for the EMG:

Under "Sensory Studies" and "Motor Studies":

This is the NCS part of the EMG. "mV" is the size of the electrical activity of nerves and "ms" and "m/s" are the speed at which that electrical activity travels along the nerves. A decrease in the size of the electrical activity indicates damage to the nerve itself and a decrease in the speed indicates damage to the insulation around the nerves. If the values are normal, then there is no detectable damage to a nerve and the insulation is intact.

A sensory study is measuring the above electrical activity in sensory nerves. If the sensory nerves are not damaged, then those readings should be normal. With ALS, all of those readings should also be normal, because ALS does not affect sensory neurons. The readings were indeed normal with your father. Having said that, there are a number of other conditions that can spare sensory nerves but still cause the types of signs and symptoms your father has because those conditions affect motor nerves..

A motor study is measuring the above electrical activity in motor nerves. Even though ALS affects motor neurons and thus motor nerves, the size of the electrical activity should be relatively unchanged in its early stages. As the disease progresses, the size of the electrical acitivity in motor nerves will start to become evident. The insulation should remain intact with ALS (although in later stages it will be destroyed), so the speed of the electrical activity should be normal with ALS. The motor study appears to be normal with your father.

The Electromyography part of the EMG study follows, which is what pointed his neuro towards ALS.

Some terminology before I begin. Fibrilation potentials (fibs) and positive sharp waves (psw's) are a measure of spontaneous, electrical activity in an individual muscle cell (individual muscle cells make-up entire muscles) . Muscle cells should not have this activity under normal conditions. If they do, it means they are actively losing contact with the axons that control them (i.e. the axons from the lower motor neurons). This is called active denervation. This is something seen with ALS but is also seen with many, many, many, many other conditions. An MUAP (i.e. a motor unit potential) is electrical activity in a group of muscle cells and there is a normal value for this. If it is longer in duration and/or bigger in size than normal, then it is a sign that reinnervation has taken place. Reinnervation means that muscle cells have first been denervated from their healthy axons (I explained that above) but then undamaged axons take their place and recontact the muscle cells. When this happens, it causes restructuring of muscle cells and a subsequent increase in the duration and/or size of the MUAP. This is seen with ALS but again, is also seen in many, many, many, many other conditions.

Your father has fibs and psw's in both his right and left deltoid muscles (i.e. shoulder muscles). His MUAP's are also longer in duration. Therefore he has active denervation and reinnervation in both of those muscles. His left and right FDIO muscles (muscles between his finger and thumb) show the same pattern. His right and left tibialis anterior and EDB muscles (muscles on the front of his shin) also show the same pattern.. His left and right gastrocnemius (calf muscle), left and right biceps and triceps (muscles of his upper arms), left and right EDC and brachioradialis (muscles of his forearm) only show reinnervation. It also says that he has signs of denervation in his genioglassus area (i.e. bulbar region), which is related to the tongue.

With ALS, active denervation and reinnervation will be seen in multiple muscles in multiple areas of the body. In fact, there are criteria that must be met before a definite ALS diagnosis is given. There must be three different areas of the body that are affected and it must be in multiple regions of those areas. It appears that your father has met those criteria. There must also be clinical signs of upper motor neuron damage (i.e. brisk reflexes, spasticity, etc.) seen. You have not made mention of any other those. The history must also be consistent with ALS. That is where the snag is. You say that he has had bouts of clinical weakness with subsequent improvement. That is not at all how ALS works. Once an area is weakened, there is no way to make improvements because the death of the motor neurons is permanent. Having said that, it is my guess that his neuro is thinking that something else was happening simultaneously with ALS and that's how the improvement was made.

Bottom line: it is not 100% definite that your father has ALS. Even if there is a tiny percent chance he doesn't have it, you shoud get a second opinion, which is what you are now doing. Please let us know how that goes and please let me know if there is anything I need to clarify.
 
That was a great post Wight. Very informative and thank you.
 
Great explanation, Wright! Thanks! Maybe David could put it in a sticky note.
 
That was excellent! I agree it should be a sticky. I have been looking for an "ALS For Dummmies" type explanation and that gave it to me. Thank you!
 
There is a typo in paragraph number 6. I have pasted it below in italics and bolded where the typo is:

With ALS, the soma's of lower motor neurons and upper motor neurons are destroyed. If the soma's are destroyed, that will start to destroy the axons. That gives rise to the signs of symptoms of the disease. When lower motor neurons are destroyed (because the lower motor neurons are directly controlling our muscels) it causes muscle weakness, followed by muscle atrophy and fasciculations. Damage to the upper motor neurons can be detected by an EMG. When upper motor neurons are damaged, it causes brisk reflexes, pathological reflexes, spasticity (very stiff muscles) and muscle cramps. The reason is because upper motor neurons inhibit lower motor neurons a bit. Therefore, when the upper motor neurons are destroyed, that inhibition is lost and that results in muscles that are over-stimulated and reflexes that are over-active, too.

EMG's DO NOT detect upper motor neuron damage . . . but rather . . . EMG'S detect lower motor neuron damage. I also failed to say that upper motor neuron damage is detected with a clinical exam.
 
Dear Wright, thank you for all the effort that you have gone to here, as my mother said when I told her about you : You have your place ready in heaven with all the blessings that come from my heart and my family's and everyone else that you touch.
I will read this through again and again and of course update you lot on what happens in India.

On a side note, the supplements that I started my dad on seem to be kicking in.
Coq10 enzyme 60mg, I specifically gave this as loads of ALS sufferers mentioned how they felt weak without it and also as its known for some reason to inhibit dengeneration.
Ginkgo bilboa, as I was concerned about brain fog and knew from when i was 22 it worked for me. My dad figured out he was driving the wrong way in the dark and he seemed more aware today.

Ill keep you all posted, bless you all.
x
 
Ok a question please. I have read and read to understand.
and so
:When upper motor neurons are damaged, it causes brisk reflexes, pathological reflexes, spasticity (very stiff muscles) and muscle cramps. The

If this is done by clinical assessment, Im sure not of this has been detected. A clinical assesment was done.
Everythign seemed fine in that regard apart from:
upper arm and shoulder muslce atrophy.
weakness in arms as a result.

Does upper neurone issues arise earlier, simultaneously and can they occur after the lower motor nuerone issues?
he doesnt have any spasms or twitchs,I ask the the time for that.

Thanks for all your help.
really appreciate it.
 
Upper motor neuron damage can happen prior to . . . simultaneously with . . . or after lower motor neuron damage. Unfortunately the course of the disease is incredibly variable.

I would get a hold of his neuro and ask him specifically about his clinical exam. Please let us know what is said.
 
On examination there is something of a staring expression. Mr X appears confident in his statements and his speech is clear, although there is a hint of possible pseudo bulbar palsy. The eye movements are normal for smooth pursuit and saccadic excursions, although vertical saccades downwards are of limited aplitude. There is a weakly positive jaw jerk. The tongue appears healthy and moves well. There is good power in neck flexion and extension. There is wasting of the shoulder girdle muscles and over the superior scapula areas without evidence of fasciculations. Shoulder abduction is weak at 2/5 with adduction relatively preserved at 5/5 on the right and 4/5 on the left. Elbow flexion is weak at 4/5 on the right and 4-/5 on the left with elbow extension 5-/5 on the right and 4/5 on the left. Wrist extension is weak at 5-/5 on the right and 4/5 on the left with wrist extension 4+/5 bilaterally. Finger extension is 4+/5 on the right and 3/5 on the left with grip strength 5-/5 on the right and 4/5 on the left. There is wasting of the intrinsic hand muscles with weakness of finger abduction. Strength in the legs is well preserved apart from weakness of extension of the left great toe at 4+/5.

Mr X walks well, but when walking on his heels does not lift up the left foot as much as the right. He is able to perform tandem gait forwards and backwards. The Romberg’s Test is negative, but he is unable to lift up his arm. He can hop on the right leg and finds it more difficult to hop on the left leg. The deep tendon reflexes are present throughout at 1-2+ with positive pectoralis jerks and a suggestion of crossed adductor responses. The plantar responses are indifferent. Sensation is normal throught to pin prick, joint position sense and vibration appreciation.
 
Hello again Pumpie

As I explained in my previous post, the bulbar region is the brainstem (by the way, the brainstem is called the bulbar region because it is shaped like a light bulb) and controls muscles of the head and neck.

A pseudobulbar palsy is an upper motor neuron problem and presents with upper motor neuron signs and symptoms in the region of the head and neck. A lot of times with a pseudobulbar palsy, inappropriate emotional outbursts accompany it (e.g. laughing or crying at inappropriate times). There is also something called a bulbar palsy, which is a lower motor neuron problem and presents with lower motor neuron signs and symptoms in the region of the head neck.

It appears the presence of a slight jaw jerk reflex (that is a pathological reflex) is what caused his neuro to declare a "hint" of pseudobulbar palsy. The only other upper motor neuron problem I see in that report is another pathological reflex called the pectoralis jerk (the pectoralis is a chest muscle). His deep tendon reflexes hovered around normal (2+ is considered normal and his were right there or slightly diminished at 1+, which would make sense with lower motor neuron damage). Everything else in that report indicates lower motor neuron problems.
 
Ok I guess there is evidence to show upper nuerone activity too.

We got some of the blood tests back and CK levels are elevated. This was going to be used as another evidence to point to ALS.

So I guess its pointing to ALS so far.

the other bloods ; syphilus and lyme disease are yet to come back.

I shall keep you all updated with the findings.
thanks again Wright, you're a star!
 
So my father is in India at the moment and has so far seen two nuerologists. the last one a MND specialist, head of the hospital department.

so what he says is that dad has ALS, but not the severest form and that there are different types of this disease and that there is no treatment for it.

He says as much as it cant be reversed, it maybe slowered or perhaps stopped through the various types of physio.

So dad start physio tomorrow, and he is there for another month. I will keep you updated with the progress if there is any.

it alll sounds a bit bizarre to me given what I have read about ALS, but i have always understood that Indis sometimes have a different take on Medicine. Fingers crossed that he is talking sense.
 
Mum and dad went to see a homeopathic doctor. My dad has become withdrawn, and doesn't speak that much anymore in India. Anyway the doctor prescribed these Nerve Tonics.

My mother is monitoring the change

ALSO! Dad couldn't get up off the bed without a push., now he does that alone. Fingers crossed , God willing we see some more positive things!
 
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