Status
Not open for further replies.

tmasters

Senior member
Joined
Feb 19, 2008
Messages
531
Reason
PALS
Diagnosis
08/2008
Country
US
State
California
City
Anaheim
Hi Everyone,

I'm a 51 year old male undergoing DX for ALS. Currently I'm undergoing a series of IVIg infusions for a differential DX of MMN, but so far this isn't showing any improvement.

I have only LMN signs presently: Muscle weakness started in the right calf a year ago, has progressed to both legs, thighs and calves, and now more recently in the right arm. I also have the fascics and cramping everywhere below my shoulders but most often in the legs.

So far I've had an ultrasound to check my leg circulation, an extensive blood workup, and 2 EMGs: one by my neurologist and one by Dr. Graves @ UCLA.

I've never had an MRI, a muscle biopsy, or a lumbar puncture. I've asked my doctors about whether these should be done but given a "no reason to do that" answer twice. It seems odd to me, because it seems like most of you with a confirmed DX have had most of this done. I'm seeing my neuro again on July 1 and want to prompt him again to more aggressively diagnose me if it's warranted. And I want to be informed before I talk to him.

So my question is:
What specific things can be ruled out by each of these 3 procedures?
- MRI
- Muscle Biopsy
- Lumbar Puncture


BTW: for some reason, a search on "MRI" in this forum produces no hits! Software bug?

Thanks,
Tom
 

andyvaughn

Distinguished member
Joined
Feb 12, 2008
Messages
138
Reason
CALS
Country
US
State
ca
City
thousand oaks
Tom... sorry to hear about your process. There is some talk about using IVIG in conjunction with cyclophosphamide and rituximab in MMN. My understanding of MMN is it is diagnosed by both presenting clinical symptoms and emg. However, I have read that there is often an elevation of antibody titres, however, I haven't seen if they mean blood or spinal fluid.
So, in your question, and MRI can rule out any physical object that may be pressing on the spinal canal that would lead to your symptoms, such as a tumor... I would want an MRI and a lumbar puncture FOR SURE in your case. The lumbar puncture would rule in/out various auto-immune processes, in MMN there is typically only a rise in protein, I don't think there are rises in antibody levels.
The muscle biopsy question I can't answer.. sorry.
Andrea - wife of Pals Jim
 

Al

Moderator emeritus
Joined
May 25, 2004
Messages
7,960
Reason
PALS
Diagnosis
10/2003
Country
CA
State
On
City
NW of Toronto
Hmmm no MRI. That is strange. Even if there were no threads it should show in posts because it's mentioned just about daily. Could be a glitch. David upgraded our software recently and we lost spell check.
AL.
 

crystalkk

Very helpful member
Joined
Sep 11, 2007
Messages
1,210
Reason
Learn about ALS
Country
US
State
pennsylvania
City
philadelphia
Tom,
I don't understand that you never had a MRI of your c-spine or your brain.... especially the c-spine
if you have cervical myelopathy (spinal cord compression it can cause your symtoms) it would also
show if you have a tumor in the cord. I am not a Dr. but I would want as many things ruled out as possible.
Hopefully the IVIG's will work and you won't have to worry about what you didn't have.
Good luck and I will keep you in my prayers.
 

Sammantha

Senior member
Joined
Aug 11, 2007
Messages
501
Country
Uni
State
NC
City
Newport
I have not been diagnosed with ALS and i can tell you i have had the biopsy and two brain MRI's and two Cervical MRI's...... It sounds like your EMG has really strong charecteristics. If they thought it was a muscle disease then your EMG would show muscle disease charcteristics. If they thought it was something like MS then your nerve conduction study would point to that. Mostly they do the MRI's to look for MS, also if your neck or brain had a tumor or what not it would cause other problems then muscle weakness. The NCV would have showed something or your regular exam. It is good that they are putting you on that therapy because that means they do not think it is 100% ALS, if they did they would have said there was no need for it. Best Wishes
 

Sammantha

Senior member
Joined
Aug 11, 2007
Messages
501
Country
Uni
State
NC
City
Newport
Sorry i re read your post and want to add that a muscle biopsy can show if you have a muscle disease...... Muscles can go hay wire if like in Multiple Sclerosis your nerves are being destroyed and signals cant reach the muscle. The Nerve condunction study will show that.... Also peripheral neuropathy would show on the NCV... Now if you have a muscle disease like Myasthenia Gravis or polymyositis then your muscle tissue will show charecteristic patterns of wasting. If your signals are getting to your muscles and nothing is attacking your nerves or muscle then the axons are short in supply... Axons are motor neurons..They are found in your brain and all down your spinal cord. Lumbar punctures are used to back up autoimmune disease's like MS.... The fluid would show antibodies and the antibodies could be attacking your spine. This is the site where polio stays and causes its damage.

Basically MRI's are done before EMG's so you skipped ahead, muscle biopsy's are done when EMG's have charecteristics that could warrant one. Lumbar puncture's are done to back up autoimmune conditions or viruses like polio. It certainly does not hurt to have these done anyhow. There is no way you can have ALS without any UMN involvement, thank god!
 

wright

Very helpful member
Joined
Jan 12, 2008
Messages
1,160
Country
US
State
xx
City
xxxx
Hi Tom

I got your PM but I thought I'd answer you here, that way others can see it and maybe use the information as well.

I guess I'll take your tests in order.

MRI: when MND's (or similar diseases and syndromes) are suspected, these are typically done on the brain and cervical spine (usually those two are done together), thoracic spine and or lumbar spine. What they look for is impingement of the nerve roots (nerve roots innervate the muscles, skin and organs of our body), which could explain denervation and weakness in the limbs and thorax.
An MRI done with contrast is used to show signs of MS, which is a demyelinating disease of the central nervous system (i.e. brain and spinal cord). Myelin is the fatty insulation (otherwise known as white matter) around nerves and tracts (nerves are called tracts if they are located in the brain or spinal cord) of the nervous system. If demyelinization has taken place, then you will see dark spots on the MRI.

Lumbar puncture / Spinal tap: this is done to look at the contents of the spinal fluid or the pressure within the central nervous system. Elevated levels of protein in the fluid could indicate hyperthyroidsim, Guillian-Barre syndrome, CIDP, as well as other autoimmune disorders. If they find IgG antibodies in the fluid, that could also indicate Guillian-Barre syndrome and CIDP as well as multiple sclerosis. Having said that, sometimes if someone does have these diseases and disorders, their fluid could actually be normal.

Muscle biopsy: when testing for MND, this is done to look for Fiber Type Grouping. Let me first explain how muscle fibers are innervated by motor nerves. A motor nerve is made-up of a bunch of axons that are all wrapped together (think of a nerve like a rope, and each individual thread of that rope is an axon). Each individual axon comes from a single motor neuron and each of those axons is responsible for innervating either one or up to a thousand muscle fibers (that depends on the muscle of the body). When that individual axon innervates the muscle fibers, it does so in a scattered pattern. In other words, the muscle fibers that each axon innervates are not next to each, but rather relatively distant from each other. Because of this, muscle fibers that are next to each other are innervated by different axons and typically have different characteristics from each other and therefore are structurally different. That causes a "checkerboard-like" look to the muscle if you biopsy it (that would be normal). With a motor neuron disease, when the motor neuron dies, the axon dies and therefore an adjacent axon has to innervate the muscle fibers. Now you have a group of muscle fibers that are next to each other that are being innervated by the same axon. Because of that, they will take on the same properties and therefore will look the same structurally. Now the "checkerboard-like" appearance will be lost and you will have FIBER TYPE GROUPING. In other words, a group of muscle fibers that look the same (that is not normal). Fiber type grouping would be caused by chronic denervation, which is a characteristic of MND. However, chronic denervation can also occur due to nerve impingement, slipped discs, etc. so it doesn't necessarily mean someone has an MND. One other note: the muscle biopsy isn't full proof and should be used as a last resort. Many docs won't even do them.

As far as nerve conduction studies and MS: those are not used to diagnose MS. MS is a demyelinating disease of the central nervous system and those damaged axons do not innervate our skeletal muscles, so it would be normal with MS.

Bottom line Tom: it sounds as if you need to have more tests done. The only thing I can think of, that would point them away from an MRI, is the involvement of your lower limbs and now upper limb. That would mean a disc problem in your neck as well as your lower back. However, stranger things have happened and so I would insist on getting it done. I would also insist on the spinal tap.

I wish you all the luck, Tom. If you have any more questions, please ask. Take care.
 

wright

Very helpful member
Joined
Jan 12, 2008
Messages
1,160
Country
US
State
xx
City
xxxx
Hey Tom, I forgot to include something concerning the muscle biopsy

Myopathies or dystrophies or certain types of metabolic disorders will preferentially "attack" certain types of muscle fibers (we have 3 main types of muscle fibers). A muscle biopsy would be able to see which of these types of fibers has atrophied or been affected. There wouldn't be any type of fiber type grouping with these disorders but the biopsy would certainly not be normal (it would just be abnormal in a different way).
Having said that: the EMG abnormalities with chronic denervation would show large motor unit potentials (MUP's) and a myopathy for example would show diminished MUP's. A motor unit is made-up of a single axon and the muscle fibers that it innervates. Motor unit potentials (potential is a fancy word for electrical activity) are generated by all of the muscle fibers that are innervated by individual axons. The reason the MUP is large with chronic denervation is that the surviving axons (recall that axons die and adjacent axons have to take up the slack with chronic denervating diseases) have to innervate more muscle fibers. Therefore, the greater number of muscle fibers in the motor unit will generate more electrical activity. The reason the MUP is diminished with myopathy, is because muscle fibers are lost in the motor unit and thus there will not be as much electrical activity.

There isn't any one test that is going to diagnose ALS or PMA or PLS or CIDP or MS, etc. Many tests have to be done along with clinical exams to properly diagnose. So again, I encourage you to press your physicians for further tests.
 

hopingforthebest

Distinguished member
Joined
Feb 20, 2007
Messages
355
Diagnosis
02/2007
Country
US
State
NJ
City
Mays Landing
EMG very important

Tom

Go easy on insisting on all the tests. Hopefully you have started with or will see an ALS University department to begin with extensive history and physical. Honestly, spinal tap can be done but the EMG and neurologist doing your physical work up will tell them alot! Family history, etc. very important.

MRI's are done to see impingement, compressed disc, black discs, and are done when a patient comes with specific symptoms, mostly come with back pain, numbness down one leg into toes, problems sleeping, due to discomfort in back, neck.

In my husbands case, MRI did not line up with his complaints (weakness, atrophy, his neck was hard to keep up, arms weak, NEVER COMPLAINED OF PAIN) We had so hoped for my husbands IVIG infusions for one year to give us a glimmer of improvement and it did not. We thought we were so fortunate to get the ALS classification of PMA (usually less agressive) but his is all upper body, legs still good; but diaphram is the muscle that controls his breathing and that is where he is in trouble. His FVC are only 24%, normal is 100%. The ALS team will measure yours with the history and physical and the follow up visit. (the little machine that you blow into). The local neuro we saw for almost 1 year NEVER measured the breathing.

I pray you do not have one of the ALS classifications, but insisting on all these medical tests is something you need to discuss with an ALS specialist. They see hundreds of ALS patients a month. A local neuro might see 2 or 3. That is something to consider.
Find the right doctor and he/she will decide exactly what you need to determine your diagnosis. Let them work for their money. They are the MD's that have 8 years or more of medical school/training. We only have internet reading. They see the real deals.

This site is the best for comparisons to other patients with ALS once you are diagnosed. The ALS patients have been a blessing to compare notes regarding care issues, equipment that is available through loaner closet at ALS clinic and they become your friends via computer.

Hope you get good results and remember not to stress as it never helps, only hurts

Patty:)
 

wright

Very helpful member
Joined
Jan 12, 2008
Messages
1,160
Country
US
State
xx
City
xxxx
If I were in anyone's shoes that had a possible diagnosis of ALS or PMA, etc, I know I would insist on all tests that would point to the correct diagnosis.
You don't have to have pain with a disc problem. It depends on how the protrusion presents itself. If it protrudes centrally, it could just press against the spinal cord and cause painless muscle weakness. The disc can also press solely on the ventral root, which is made of solely motor nerves, and therefore will again only present with painless muscle weakness and could also cause atrophy.
The EMG with ALS or PMA is very similar to the EMG with MMN. That is why those with a diagnosis of possible ALS might be put on IVIg to see if it helps. If it does, then it is not ALS or PMA.
I certainly agree with Patty in that these tests must be discussed with a specialist, but again, I think I would be anxious to explore any and all avenues before I was given any type of ALS, PMA, etc. diagnosis.
Just as another note: not one single word of what I wrote in the previous posts came from the internet; I don't ever rely on the internet for my information (too much bad and incorrect info). My information comes from the lectures I give or journal articles that I read. I'm one of the people those neuros have to get through first before they can be called doctor. I will certainly not sit here and say I know more than a well-trained neurologist does; that is their specialty and I would always defer to them. I'm just here to offer the knowledge I have accumulated in my years of schooling and training.
 

ladyk

Member
Joined
Jun 11, 2008
Messages
19
Reason
Learn about ALS
Country
US
State
OH
City
Zanesville
thanks

Thanks to all of you for the info. I'm in the process or being diagnosed. I've been twitching, numb and weak for about 11 months. I have twitching all over but the worst is in all of my chest muscles and left rib cage. It's so severe that I can't lay down but for a few minutes. I have weakness in the left side, can't feel my left hand and arm most of the time, I drop stuff frequently and have quite a lack of balance. I have a lot of pain so I was diagnosed with costochondritis, then fibromyalgia but then neuropsych said that those weren't correct diagnoses. I just had cervical MRI but after the 1st part when they put the dye in for contrast the twitching was so severve that they said they would have to stop because I couldn't be still (NOT MY FAULT!) I'm waiting to see what's next whist also dealing with waiting to be biopsied for thyroid cancer. All the info was so helpful. Knowing how the tests work, what they are for and in what order tests might happen is comforting.
 

tmasters

Senior member
Joined
Feb 19, 2008
Messages
531
Reason
PALS
Diagnosis
08/2008
Country
US
State
California
City
Anaheim
Nothing yet

Thanks to everyone for your replies. This forum is great! Where else can you go for both support and detailed information?

I went to see the neuro yesterday. He did a quick exam. Reflexes still diminished, perhaps more than 3 months ago. No brisk reflexes or any other UMN signs yet.

I asked him what my diagnosed possibilities are and he said "either MMN or ALS, there could be nothing else." He wrote me a prescription for, "...there IS one treatment, can't remember the name of it...", Rilutek, (I had to help him with the name.) He refused to order any more tests but referred me back to UCLA and Dr. Graves to recommend more testing if he sees fit. I felt very comfortable with him and I'll bring these questions up again.

My neuro said my EMG was very conclusive for ruling out other things. So MRI, biopsy, or lumbar puncture are not necessary.

Again, thanks. And I'll continue to play the waiting game and the musical doctors game...

-Tom
 
Status
Not open for further replies.
Top