message for Arron - BCFS & CPK levels

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message for Arron - BCFS & CPK levels

Postby dwl on November 19th, 2002, 7:34 am

Arron,

You mentioned in your excellent "in a nutshell" post that CPK levels can be raised in BCFS. I was quite please to read this as mine are 20% above normal, BUT any neurology textbooks I have looked up BFS or BCFS in say that CPK levels are normal. Could you let me know what your source was?

Also - is anyone able to hazard a guess as to how common BFS is compared to ALS/MND?
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Postby Guest on November 19th, 2002, 1:01 pm

there are no real stat's on the distribution of Bfs in the general population. it seems that in most cases neurologists see as many als patients as they do see bfs sufferers. keep in mind that all als patients will and up in the doctors office while probably the majority of twitchers do not bother.I would not worrie about your cpk level.
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Postby Nole on November 19th, 2002, 2:03 pm

What is cpk??
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Postby Asterix on November 20th, 2002, 8:22 am

dwl,

I too, have slightly increased CPK level (275) and this freaked me out quite a bit because, as you said, this is usually not the case in "textbook BFS".
Furthermore I'm very concerned about the fact that at neuromuscular information pages "slightly elevated CPK" is often mentioned as a common finding in ALS patients. (emphasis is on "slightly" increased, whereas normal or very markedly increased levels (above 1000) point away from ALS)

However, as I have learned from further research and many reassuring answers at the MGH-forum CPK is a very inspecific indicator of muscle cell damage that can be increased for many other reasons.
So, although it is true that elevated CPK is found in ALS this DOES NOT MEAN that the reverse conclusion also holds.
For example, CPK will be slightly elevated after physical exercise or
any kind of muscle injury.

How high was your CPK level ? (mine was 275, with 200 as the
upper limit of normal range)
What was your docs comment about it ?
Mine seemed not impressed and just asked if I did exercise the day before the blood test (which indeed was the case)

You can read many reassuring/interesting facts about elevated CPK
in the following thread at the MGH-forum:
http://neuro-mancer.mgh.harvard.edu/ubb/Forum65/HTML/013279.html

As for the relative frequency of BFS compared to ALS:
This is *EXACTLY* the very one question that I've been trying to get the answer to for the last 7 months.

For if we knew this number then we
could easily derive the probability of one having ALS when the only
observed symptom is body wide fasciculations.
For example if BFS was, say, 100 times more commen than the
twitching-only-onset form of ALS (about 7 to 10 percent of all ALS cases) then the probability of a twitcher having ALS instead of BFS would
be only 1 Percent.
Unfortunately there is very little information avaiable about the incidence of BFS.
From my own research I did a very rough estimate of the upper bound
of BFS incidence at roughly 1 of 100 people (although I suspect that
the actual incidence for the very profouse body-wide twitching would
be lower)

Anyone any further factual informtaion about this issue ?
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Postby dwl on November 20th, 2002, 8:52 am

My CPK level was very similar, around 240. I phoned a biochemist who confirmed that cramps and exercise can commonly cause slight elevations. I haven't had it re-checked yet.

I spoke to the neurophysiologist who did my EMG 6 weeks ago & asked him what his impression was of the rate of benign fasciculation to ALS and he said that benign fasciculation was "massively" more common and he only sees the tip of the iceberg. He actually keeps a copy of the clinical paper which demonstrates that there is no progression from BFS to ALS so that he can hand it out to people.

Having said that, it's very difficult to cope with new symptoms when they keep popping up - my latest is the rubbery feeling in my hand & wrist muscles. This morning I started getting very fine fascics in one area in my hand which have buzzed away for a few hours.

I suspect I'll probably end up going back to the neuro again & possibly request a repeat (& more detailed) EMG) - the original one sampled only 5 areas. I wonder how many paranoid BFS'ers end up having multiple EMG's!

I have a 10 month old daughter to worry about & this is starting to take over my life again :-(

I believe you mentioned a paper which showed that 6.7% of ALS starts with fascics alone. I mentioned this to the neurophysiologist & asked him if he could tell me what the negative predictive value of a normal EMG was, but he didn't know. I haven't been able to locate this paper, only the abstract. Do you know if these 6.7% had normal EMG's in the early stages?
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Postby Asterix on November 20th, 2002, 11:20 am

dwl,

good to hear about your neuros statement about the BFS incidence
and the biochemist's confirmation of exercise related CPK elevation :)

I assume the clinical paper that yor neuro mentioned was the well
known Mayo Study (a follow up of 100+ BFS-patients (by definition
all with clean EMG!) over an avaergae of 7 years where *none*
developed any form of MND)
you can read the full text here:
http://neuro-mancer.mgh.harvard.edu/ubb/Forum65/HTML/003918.html

As for the other Study about the 6.7% twitching-onset ALS cases,
unfortunately I haven't been able to get a copy of the full text yet.
It is not avaiable online nor have I found a copy at local medical libraries.

What I do know from "second hand" (older posts at the MGH forum about the study) is the following:
1. those 6.7% were the ones that presented with fascics as the only
complain (i.e. they went to the doc beacsue of fascics only)
2. with theses patients fascics were also the only *physical sign*
upon neuro examination (I do, however, not know wether this also included a negative EMG (I strongly suspect, however, that the EMG
would not have been negative in this cases)
3. The mean time before the onset of more serious symptoms like weakness was 7 months (2.4 to 13.4 months)

Anyone any more informations about this study ?

The third point I find rather discuraging, since it sets the time frame
for our "BFS waiting game" to over a year before you are finally in the clear. (not to mention those anecdotal stories on the web, where
PALS report two or three years before weakness onset :(

However, I think with clean EMGs you can put your mind at rest much earlier (I assume most of the 6.7% twitching-onset PALS would have shown EMG-abnormalties much earlier before weakness onset)
Generally, as far as the negative predictive value of EMG tests is concerned, there is contradictory information:
Among forum members the consensus is that every EMG will almost always detect any MND, no matter how soon in the desease process or
how many (or few) muscles tested.
However, there are more prudent statements from neurologists or ALS specialists (for example at the Cleveland Clinic neuro forum) stating
that only after 6 months to 1 year after sx onset could a EMG completely
rule out ALS...
Peronally, I feel that the truth lies somewhere in between and that
a clean EMG (even if done soon after sx onset) should at least very very reassuring. Note that in the Mayo study out of 100+ clen-EMG-twitchers not a single one developed ALS !

Unfortunately, I have not had an EMG yet out of fear (partly of a
possibly positive result and partly out of fear of a false positive result)
but thats another story (see my separate post from NOV 10)
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1 in 2 million?!

Postby Rar on November 20th, 2002, 2:09 pm

If the chances of getting als are 1 in 100,000, and the chances of als appearing with twitches only at onset of disease is 6 to 7% of all als cases, then that means the chances of someone getting als that presents itself with twitches only at onset is somewhere around 1 in 2 million!
Are my figures wrong? What are you guys worried about? Even I can't worry that hard.
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Postby jblack on November 20th, 2002, 2:23 pm

Oh, we'll find a way, reality be damned!

;-)

just had my first neuro apptmt yesterday. He was very nice about it, and referred me to someone he called one of "the global experts" in ALS to ease my mind. He didn't do an EMG, I imagine he'll let the specialist do that. That said, without me even mentioning ALS, he knew what was going on..."We get a lot of people in here with fasciculations, who have been reading things on the Internet..." *grin*. To be honest, it's anecdotally comforting that my situation is common enough that he recognizes it on sight, and ALS is so uncommon, I wonder if he's ever had a case, much less a case with someone under 40.

Fingers still crossed, but feeling a bit better!

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Postby Guest on November 20th, 2002, 5:37 pm

jblack

Are you going to see Dr. Miller. If so, is it hard to get an appointment?
He is supposed to be one of the best Als researches arround.

Astrix
Here is a response from the wellknown emg expert P. Fabre to the "famous" 6.7% study you are so concerned about
hope it helps

Yes, I agree that patients with ALS may present with fasciculations. In one report (letter), 6.7% of ALS patients had fasciculations as an isolated, initial manifestation of the disease (Eisen and Stewart. Ann Neurol 1994;35:375-376). """Practically, all the patients I have seen got the fasciculation (if present) with other signs of denervation and neurogenic MUPs of variable degree""". [/b]
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Dr. Miller

Postby jblack on November 20th, 2002, 5:51 pm

I am, yeah. How did you know? did I mention that in an email? Anyway, I think it's very hard to get an appointment. The guy I did the initial consultation with was very nice, however, and got me something pretty quick (3 weeks) because he didn't want me to worry. I didn't even ask for it -- he just thought it would be a good way to relieve my anxiety.
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Postby Guest on November 20th, 2002, 6:55 pm

My neuro was going to refere me to dr. miller with almost the same wording as the neuro you saw . the medical center i am with sent me to their own mnd specialist instat.

here is a statements from Dr. Miller:
SUBJ (04/96): ALS DIAGNOSIS/DRUG TRIALS
I have the following questions:
1. My neurologist has done several EMGs and says they are normal. Do normal EMGs rule out or lessen the likelihood that one has ALS?
2. How does a neurologist determine whether or not fasciculations are benign or characteristic of ALS?

REPLY[1] from MDA: Robert G. Miller, M.D., California Pacific Medical Center, San Francisco

1. Yes, a normal EMG casts doubt upon the diagnosis of ALS. Several normal EMGs virtually exclude the diagnosis. When symptoms are present for more than six months, a normal EMG adds tremendous confidence that fasciculations are benign. When symptoms have been present for more than a year and the EMG is still normal except for the presence of fasciculations, then a diagnosis of ALS is untenable.
2. It isn't possible to state with certainty whether fasciculations are benign or characteristic of ALS in and of themselves. The real issue is whether the rest of the EMG and the clinical examination are normal; and with the caveat above, the diagnosis of benign fasciculations can be made.

And here a doctors visit from someone reported in another forum:

Oct and Nov where a living hell to me. I was so convinced I had the disease I was planning my will. I am in my thirties. When I visited the Doctor Miller in San Fransico I thought it would be so apparent to him that I had ALS. He asked me "Who told you about ALS?" "Have you been on the internet researching this disease?" "Do you know how many people I see that come in here who are convinced and terrified they have the disease and do not?"
He put me through such a through muscle evalutaion. Made me do 100 pushups, 50 deep knee bends and hop on one heal. He said there is no way you have the disease.

Well I hope your fit for the visit...

What is the distrubution off your twitches?
Mine had a very fast onset.,got bodywide within days?
Is this the case for most of you?
I heard it is most likly benign if the fasc. come out of the blue with a wide distribution? I find it hard to believe.
TH
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100 push ups

Postby jblack on November 20th, 2002, 7:06 pm

Now I know why my neuro sent me there! For punishment and humiliation! ;-) I'm pretty fit ... but 100 push ups!!??

Mine are by far at their heaviest in the right lower leg and foot, but I do get them other places (left calf, fingers of right hand) less frequently and in a few places (biceps, quads, tongue) very occasionally (a handful a day). What has me worrying (like I need any help worrying!) is that my right lower leg muscles, front and back, do feel tired and stiff and weak, even though I can quite easily do exercise. Probably just the BFS, fatigue-related weakness.

I'd say that if yours came on all of a sudden and all over your body, that almost has to be anxiety or stress. I wouldn't worry if I were you. Certainly ALS isn't gonna kick in everywhere at once at full strength!!

Thanks for the post. Wish me luck!

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Postby paranoid on November 20th, 2002, 7:55 pm

Jesse,
I just cant believe how similar our symptoms are. Almost all my twitching is in my right calf and right foot with occasional thumpers elsewhere. There is not any of the moment of the day I cant look down and see the twitching. I also suffer from the weak feeling or to me a " heaviness". Though not weaker, it seems to take more effort to do things with this leg? Are your feelings similar? I also went through a period of major cramping in this leg, though thankfully that has subsided. I have had my symptoms since mid June, had a normal clinical neuro exam but am still awaiting my E.M.G as it has been rescheduled till Dec 16th. Please continue to post your experiences and any info from your neurologists and your tests. I and I am sure others really appreciate your input.
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Postby jblack on November 20th, 2002, 8:24 pm

I'll definitely keep posting, this place has been a lifeline for me at times. Plus, I'm less than 2 months from my first symptoms, so my worry feels fresher. My appointment is the 11th -- not sure he'll give me an EMG, maybe I'll have to ask for one. Did you ask for one, or did they recommend it?

yes, more effort might be a decent description. My leg hasn't been "heavier", but, for example, when I do the heel and toe walking, I can really feel it in those muscles, even if I'm perfectly capable of doing them. I have no idea if this is typical of weakening muscles, but I certainly hope not!

have faith...

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Postby Arron on November 20th, 2002, 9:20 pm

OK, here's my input for what it's worth; There have been many posts by neuro's that said that elevated CPK levels (an enzyme in the blood) may or may not mean anything at all. There are also several articles that say the same thing. It is all relevant to other circumstances in conjunction with what is going on in your system. Not all people have perfect blood levels or blood pressure and so on. Elevated CPK levels "can" mean a NMD is happening and it can also mean nothing, deopending on the underlying cause and other symptoms involved. Just like a slightly abnormal EMG doen't positively mean you have a NMD. Some people just have abnormalities and that's that. Nothing is cut in stone. Muscles use and release enzymes to function as well as acide such as amino, Latic and so on. Depending on use, strain, stress and other factors, levels of enzymes and acids will vary. Twitching and cramping muscles are muscles that are moving and being used, although involuntarily... they are still being flexed and contracted. This in itself will release certain enzymes and acids into your blood stream, which in turn will be picked-up on a blood test. Simple enough.

Now, onto a sentence that I just have to comment on, made by "guest";
"I heard it is most likly benign if the fasc. come out of the blue with a wide distribution? I find it hard to believe."

Why would this be hard to believe? This is actually a GREAT sign. If ALS starts in one place, such as a hand and it progresses from there and migrates up the arm and to other places of the body, then why is it hard to believe that benign twitches just randomly come and go? Twitches in ALS certainly do not EVER come and go or randomly pop in one place and then other places. I mean... do you think ALS spreads like that? No way! It migrates at a pretty constant speed. It certainly doesn't pop-up here, then there, then over there and back here again and then everywhere in between. No disease does that. It starts in one place, kills the connections in the nerves between the brain and muscle. With that, the muscle starts to quiver and "twitch" as it dies and once the ALS progression has taken place, it migrates from that point forward. You don;t get ALS in your eyebrow for 3 weeks then have it in your calves, then have it in your back and arms and in the mean time, still be able to blink your eyes or raise your eye brows where it originally started. Once a nerve ending dies, it is dead, period, and in ALS, twitching ONLY comes with dying muscles, NOT before!

Onto the 6.7% of patients that had twitching only as their first symptom of ALS. Maybe that's what THEY noticed at first but facts are facts. Twitching in ALS ONLY happens because of dying muscle tissue and denervation. Twitches DO NOT, CAN NOT and WILL NOT "cause" ALS or muscles to die.

If those 6.7% of the people only "noticed" the twitches, then that certainly doesn't mean that ALS starts that way. What it does mean is that, that's all they "noticed" before any weakness was detectable by them. This certainly does not mean that a doctor performing a clinical exam couldn't find weakness or other symptoms. It simply means that these 6.7% of people were just "unaware" of what was going on with their body is all.

Just like some people cut themselves and notice blood running down their arm BEFORE they feel the pain from the cut or notice the abrasion. Does that mean that they bled before they got cut? If the blood running down their arm was all they noticed and they didn't "feel" the cut first, does that mean they only had bleeding as a first sign? Well, that is what is implied by saying that some people only had twitches as a first symptom of ALS. It may be all they noticed at first but it was NOT a first symptom! It was only what THEY noticed at first. Other symptoms and/or tell tale signs are most certainly present once twitching starts and will certainly be detectable by some tests and a good exam by an experienced neuro.

This 6.7% theory just shows that some people are just not in tune with their bodies or they just don't pay attention to what is going on around them. I mean, haven't you heard of over weight ladies that went to the doctor complaining of stomach pains and found out they were like 7 or 8 months pregnant? I certainly have heard several stories like that, so does that mean she had no first signs of pregnancy? No way. It just means she is an idiot and is totally unaware of other symptoms and signs going-on with her body or she just ignored them and blew them off as nothing.

You have to look at the whole picture and quit over analizing statistics when in reality, most statistics are way off and never take certain key factors into consideration and that 6.7% theory is one of them, so relax.
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