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StillSteve

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PALS
Diagnosis
02/2011
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US
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MN
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Twin Cities
Once again, I find myself unable to briefly summarize my situation. Feel free to skip to my questions at the end of this long post. You've all probably read hundreds of desperate histories like mine.

I do not have a formal diagnosis, but two neurologists have now told me that they suspect that I have ALS. The second of these two doctors, a very well-qualified specialist in neuro-muscular diseases, told me that "the constellation of symptoms you are presenting are highly suggestive of ALS." He held out some hope for monomelic amyotrophy (but said my widespread fasics are a counterindication), and discounted multifocal motor neuropathy (the diffuse involvement in my left arm does not look to him like MMN). He did order another round of blood tests (including for Lyme antibodies), a 24-hour urinalysis to look for heavy metals, and another EMG (my third, which I expect will be my most comprehensive).

He told me that my progression seems to be slow. My first symptom, cramping of the left hand, occurred about a year ago (it's fuzzy). I definitely had weakness in my left hand (noticed I couldn't use it to trim fingernails) about eight months ago. My GP noticed visible atrophy of the right arm (upper and lower) and hand during a routine physical four months ago.

It wasn't until my GP referred me for an EMG after that physical that I noticed the fasiculations. Not sure how long prior to that they may have gone undetected, though I can say that they were pronounced and fairly widespread at that time, and certainly couldn't have been occurring at that level for long at all without my taking notice of them. These fascics continue to occur everywhere from my shoulders to my thighs. Rarely do I notice them below my knees (but not never). Much more consistent and pronounced in my left arm than anywhere else.

The weakness in my left hand is significant, but I am still using it (for example) to touch-type fairly effectively. I play softball and am able to catch the ball with my left hand, but I can't really squeeze the glove shut. I'm still playing volleyball, but I never was very good with my left arm, and I'm certainly not getting any better with it.

I'm tired a lot. Hard to know how much of this is due to my neuro-muscular condition (whatever it is), and how much is due to stress and (most likely) mild depression.

Until last Thursday's appointment, I believed that I was not showing any upper motor neuron signs. However, the doctor finds (somewhat) brisk reflexes in my left foot, knee, and (especially) wrist. This is complicated by the fact that I was diagnosed with Adie's syndrome in 2007. A feature of this is the absence of these reflexes. My doctor read in the report of the neuro-ophthamologist in 2007 that "reflexes absent from all seven locations tested." During Thursday's testing, I showed no reflexes on my right side, but some (fairly brisk, I guess) on my left side. The difference was striking. The bottom line is that my neuro does believe I'm showing upper motor neuron signs. (In my only other post to this site "Is this PMA?" I was confident that I was not showing upper motor neuron signs.)

Other info includes: no Babinski's sign, no clonus of which I am aware, no swallowing or speech difficulties, no fasics or atrophy of tongue (according to doc's visual inspection), no problems with balance, and (measured) normal lung capacity. Two EMGs have not detected anything "significant" (doc's word) in other limbs, though these were far from comprehensive anywhere other than in my left arm. One fascic was detected in my back during second exam (both neuros have seen fascics visually in my right arm during visits). No objective atrophy or weakness found outside of my left arm, but I have felt for some time that my left leg is not right. I feel a "deadness" in it almost all of the time.

I have some "pins and needles" sensations in both feet, especially my left. These are worst after a workout (I occasionally use an elliptical machine in an attempt to get some aerobic exercise; I've gained about eight pounds in the past few months). In addition to my lack of exercise, I've been eating more than I should, and I drink a protein drink every day for breakfast.

The neuro told me that (if this is ALS) my progression has been "slow." The ALS clinic coordinator/nurse told me and my wife that the good news is that my progression has been slow and that in most cases ALS tends to stay on the same trajectory. Slow onset=slow course of disease. She then told us of three or more patients they are seeing who have lived more than 20 years with the disease. One, diagnosed for 34 years, is "still moving," she said.

I am 49 years old, and other than this (apparent) ALS, am in generally good health.

Three questions:

How true is it that a "slow onset/progression" tends to stay that way? Is this consistent, or just a hopeful generalization?

Does my progression (as I've outlined above) really seem slow, or just somewhat on the slow side of things, but not very unusual?

Is there any consensus on life expectancy? This site (in the "Facts About ALS and MND" article) claims that "90% of patients die within 3-5 years of symptoms." This is the least hopeful timeline I've seen anywhere. The "Facts You Should Know About ALS" article on the ALS Association website claims that "about twenty percent of people with ALS live five years or more, up to ten percent will survive more than ten years, and five percent will live 20 years." These two sites don't seem to agree. I read statements in (many) other places such as "usually ranges from about 3 to 5 years after diagnosis." "After symptoms" and "after diagnosis" are two very different things. Where's the truth?

Thanks for reading and for any info you can provide,
B
 

halfin

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Joined
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Messages
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PALS
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08/2009
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CA
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Hi Bachster - sorry you are on this road, hope you find a way off it soon -

The disease is highly variable but yes, broadly speaking there are slow progressors and fast progressors. Some people have a pattern of plateaus interspersed with periods of relatively fast change.

Your progression definitely seems slow. Hand cramping a year ago, hand weakness 8 months ago, a little more hand weakness now plus some UMN signs. I think most people would have had at least progression into the other hand, and possibly to other body parts.

As far as survival, apparently different researchers have gotten different results, depending on the study population. I guess it's not that clear whether 10% or 20% survive beyond 5 years. Given the relative rareness of the disease, there wouldn't be that many examples of long survival to make precise statistics possible. I would imagine that most analyses would use time post diagnosis, because that is a well defined date, while symptom onset may be vague and subject to patient recollection.
 

JeffP

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08/2009
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MI
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Iron Mountain
cant say any better than hafin .you really cant say how this disease will go but try to live for today and try your hardest of the time value .no matter if your healthy or not we dont know when are time is to come so do your best to enjoy the moment you have today godbless
 

StillSteve

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Joined
Apr 28, 2010
Messages
41
Reason
PALS
Diagnosis
02/2011
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US
State
MN
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Twin Cities
Thanks, halfin and JeffP, for your responses and well wishes. I am trying to remain as optimistic as I can. Don't know if I'll ever get off of this road; just hope that I can handle whatever is to come, at least for awhile.

I can understand some of the variation in the life expectancy statistics (the difficulty of pinpointing onsets, especially), and I know that I shouldn't try to apply "average" and "median" numbers to my individual case anyway. But it's bewildering to see such disagreement, even in what seem to be the best sources available to us. I just wonder where these numbers come from, since there is rarely ever any reference to sources or studies. (This includes this site's "Facts About" claim, which may be the most pessimistic of any I've seen.)

B
 

charlottecorday

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I understand your frustration with all the time spent......At least, you wil probably get an answer with an extensive EMG.

I had two that were both dirty in one limb, and they did not check anyplace else. I am real angry about it.

Getting my third EMG in six months,this month. Maybe we will both get some answers.

Life is about luck sometimes
 

StillSteve

Active member
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PALS
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02/2011
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US
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MN
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Twin Cities
Thanks for the reply, charlottecorday.

I'm not feeling particularly lucky these days, but yes I expect that this third EMG is likely to lead to a definitive diagnosis. The doctor I'm now seeing (second neuro) is seeing me at the U of MN's ALS clinic. He is an expert and I have no reason to doubt his ability to interpret my results. He (in a surprise to me) found UMN signs. If "fasciculations potentials" found during an EMG count, I'm pretty sure he'll find involvement in three or more regions.

Best of luck to you.
 

halfin

Senior member
Joined
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Messages
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PALS
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US
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Generally fasciculation potentials are not as significant as some of the other EMG findings, like fibrillations or large motor units. Fasciculations are often benign so if that's all they find that would be a relatively good sign I think.
 

dp1969

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Apr 23, 2010
Messages
46
Reason
PALS
Diagnosis
06/2010
Country
US
State
CO
City
Denver
Bachster -
Your symptoms and progression are fairly consistent with mine...I started having weakness in my right hand in July 2008. At this point I have atrophy and fairly limited use of my right hand/arm. Starting to have weakness in my left arm and stiffness in my legs. I also demonstrate brisk reflexes and fascics throughout. I was recently diagnosed with ALS (sort of...long story); the neuro stated that generally slow progression in the beginning indicates slow progression throughout the course of the disease. Not certain if that is helpful...it didn't do much to put my mind at ease.

Hal -
I was concerned about my fascis (increasing in duration and location) and asked the neuro if another EMG was warranted. He stated infatically that the fascics were a sign of denervation...is he reaching that conclusion only because of signs of denervation in my right arm on the EMG I had in January?

Thanks to both of you.

Dan
 

StillSteve

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Joined
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Messages
41
Reason
PALS
Diagnosis
02/2011
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US
State
MN
City
Twin Cities
I do hope that that fascics (or FPs) found during EMG aren't as significant as other findings. But I just can't get my head around the fact that atrophy, camping, and trembling all occurred in my left arm *before* I noticed any fasciculations. So why fascics *before* atrophy, etc in other limbs if deenervation/reenervation is the cause? I know that I may have been unaware of some, but no way I wouldn't have noticed the types of fascics I'm getting now in all four limbs, back, and stomach. And yet I don't have detectable atrophy anywhere other than left arm so far, and no "significant" abnormal findings outside of left arm during two EMGs.

My third EMG (next week at ALS clinic at U of MN) will seal my diagnosis, I'm afraid.

I agree, dp1969: that the "slow progression" idea doesn't do much to put my mind at ease either. The widespread fascics make me think that the slow progression (one year to this point for left arm) has changed or is changing, and that things will go more quickly now. I feel like the story I got at the ALS clinic was just a an attempt to give me some hope (where there is actually very little).

Thanks to both of you for your perspectives, and for the information.

Best wishes,
B
 

halfin

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Joined
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Messages
540
Reason
PALS
Diagnosis
08/2009
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US
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CA
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Santa Barbara
I don't think the cause of fasciculations is fully understood. Some are thought to come from the nerve-muscle junction, others from the nerve fibers, others perhaps from farther "upstream" in the spinal cord or even the brain. The NMJ ones might be related to degeneration, but the other sources can have a wider set of causes and are often completely benign.

Many PALS do experience the pattern where they have fasciculations in an area, then it becomes weak. That would point to some process related to denervation as the cause.

My fasciculations have been more like you describe, widespread in most of the body, and not related to specific areas of weakness. I assume they are connected in some way to ALS in my case, but I don't think they are directly tied to denervation since all parts of my body twitch constantly but I am much weaker in some areas than others. I suspect that somehow it points to ill health in some part of my nervous system but not the NMJ particularly.

That is my story, but everyone is different and you may yet have a different outcome. Prepare for the worst but hope for the best, as they say.
 

AndyDJX

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They found fasiculations on my first EMG but were pretty adamant that they didn't mean anything unless they found other troubling signs. So fasics + more significant findings=bad. Fasics by themselves= of little or no concern.

I do not have ALS by the way. I think my fasics, atrophy and muscle trouble has been caused by long term effects of radiation to the spine from cancer treatment a while back. But that's purely speculation; the docs really aren't sure.
 

charlottecorday

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I would be curious to know of those that do not experience Fasciculations or rarely do. I picked up a pathology textbook at an estate sale of a neurosurgeon that was written many moons ago. Medical textbooks usually don't have major typo's. I read that ALS showed many Fibrillations(fasciculations were not even mentioned,(although I know lou gehrig had multitudes ,like I said the book was about 60 years old) and fibrillations were widespread and the most common characteristic of the ALS process.At that time I found myself wondering if fasciculations were more common in athletes. JUST CURIOUS.

Of course, fibrillations are not found in healthy individuals, whereas, fasciculations can
be(benign).

Fibrillations and positive sharp waves ARE denervation.(atrophy presents depending how long it lasts).Of course,Wright you can jump in and spar if you see fit.

Bachster,your posts are very engaging.
 

charlottecorday

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I am not implying fibs are only ALS related. You can have fibs and have many other things besides ALS. Wright, right?
 

trfogey

Very helpful member
Joined
Jan 31, 2008
Messages
2,017
Reason
PALS
Diagnosis
07/2007
Country
US
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NC
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Raleigh
I do hope that that fascics (or FPs) found during EMG aren't as significant as other findings. But I just can't get my head around the fact that atrophy, camping, and trembling all occurred in my left arm *before* I noticed any fasciculations.

Just because you didn't notice fasciculations in that first limb doesn't mean you didn't have them. Based on my own experience, it's pretty easy not to notice fasciculations, especially early, mild ones in a new region.

So why fascics *before* atrophy, etc in other limbs if deenervation/reenervation is the cause? I know that I may have been unaware of some, but no way I wouldn't have noticed the types of fascics I'm getting now in all four limbs, back, and stomach. And yet I don't have detectable atrophy anywhere other than left arm so far, and no "significant" abnormal findings outside of left arm during two EMGs.

Atrophy doesn't set in until the muscle is paralyzed, as far as I know. The muscle atrophies from lack of use, just as if the body part had been immobilized in a cast. As long as the muscle is being used, there is no reason for it to atrophy. And atrophy can take a long time to be visible to an untrained observer, especially in a large muscle. However, the weakness will be noticeable immediately, which is why we say "weakness before atrophy".

I agree, dp1969: that the "slow progression" idea doesn't do much to put my mind at ease either. The widespread fascics make me think that the slow progression (one year to this point for left arm) has changed or is changing, and that things will go more quickly now. I feel like the story I got at the ALS clinic was just a an attempt to give me some hope (where there is actually very little).

I had fasciculations visible in my right hand and arm two full years before I noticed (and the neuro measured) any kind of weakness in either. You are entitled to "feel" any way you like, but all the research concerning ALS progression indicates that your "feeling" is likely to be wrong. Why would your doctors be engaged in some kind of conspiracy to deceive you? At this pace and with your attitude, you'll "think" yourself sicker than the doctors could ever diagnose you.

If you are this pessimistic about your future and your doctors without a diagnosis, I can't imagine what you'll be like with a confirmed diagnosis.
 

Al

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Joined
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Charlotte, you're getting a bit carried away here. trfogey's post is in no way as confrontational as you took it. Disagree yes but don't trash the man and his family. We're having a zero tolerance to trashing other members.

AL.
 
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