Unusual source of CIDP or PN symptoms??
 

Haven't posted for quite awhile since I'm waiting for test results from my November trip to Johns Hopkins and I'm now back in the job market after 28yrs.

Up front I defer to forum members w/ much more expertise than I have in CIDP but this article caught my eye this AM. I don't really know what to say so nothing at all seems to be a prudent course of action.

http://news.yahoo.com/s/ap/20071207/...rhouse_illness

Alkymst

Thanks C!
 

I've been reading about this on a bunch of different boards...all a re-hash of what you've provided. EPIDEMIC? Maybe, the brains? Maybe. Ties to other aspects? NOT. Only that a bunch of folks who happened to work in one place at a particular period of time are now diagnosed w/CIDP. I'm finding little info from the CDC, nor about how the processing plant is well...'processing stuffs'. Disinfectants, water sources for cleaning that kind of stuff. All are probably playing it all very close to their vests...Think of the liabilities?
Then there are those epedemial issues about HOW the pigs were raised, what fed, innoculations ad nauseum that well, we aren't gonna know a THING for the next 10-15 years...when more damage has been done.
Yes I am becoming cynical about this aspect. But I truly appreciate your posting this!
My cyncicism arises from a spate of bacterial and auto-immune issues popping up in a small area of my own neighborhood in the last 10+ years...All resulting in severe to less severe damage[s]. I have the least of the 'damages' comparatively-truly scary that. I have tried to get the CDC, State Health Board and locals involved all to 'no-go', no response, nor even interest...Sad but true. Either public panic or property values dictate the agendas...not public well being in the long term.

NOW to get off MY soapbox? How did your testing go, from what all that transpired? As you have read, Fanfaire went thru the Mayo 'processing'...can you compare? Any analogies? I for one, sure would like to hear and know what all that went on! - j

I would wonder about prions.

I wonder if a lot of folks with CIDP now, would have been diagnosed with MS 20 years ago. Plus, if you don't get a sural nerve biopsy....I don't think they can diagnose CIDP. Don't most folks with CIDP get that diagnosis off the sural nerve biopsy?

They don't want to do sural nerve biopsies anymore, unless really pushed for some compelling reason to do so. If you only have a skin nerve fiber density biopsy, such as I have, they don't diagnose CIDP...at least not to my knowlege. Please correct me if I am wrong, because CIDP responds to IVIG, and I no longer get IVIG, as my SNF was deemed not to be inflammatory.

Cidp--
 

--is turning more and more into a clinical diagnosis. True enough, a nerve biopsy (rather than a skin biopsy) can sometimes clearly show the trophic changes associated with a relapsing-remitting demyelinating inflammatory neuropathy, but plenty of people have been given the diagnosis without one, based on blood/spinal fluid work-ups catching protein/antibodies of one sort of another, response to IVIg, or just plain EMG/NCV results.

Truth be told, CIDP is a rather broad and catch-all term. Many neuros use it to refer to any long-term demyelinating neuropathy in which there is evidence of inflammatory process. But there are many other conditions that can directly cause a chronic demyelination, especially toxic or hereditary ones:

http://www.neuro.wustl.edu/NEUROMUSC...dem.html#table

http://www.neuro.wustl.edu/NEUROMUSC...er/myelin.html

All too often, if the doctors are not being thorough in their investigations, the evidence of demyelination leads them to designate CIDP without looking for the patterns that may indicate how the process is occurring, and where along the myelin, which might point to a more specific disease. The classic presentation of CIDP involves macrophage-mediated stripping of myelin (to greater or lesser degree) all along the axon, but irregularly spaced damage or involvement of the Schwann cells might suggest something that is more specific.

As cycleops notes, the definitions of these conditions are very fluid as medical and genetic research goes forward, and we are likely to see redefinitions/reclassificiations in the future.

Thanks, you answered my question. My spinal fluid was normal, my blood markers have never showed inflammation (except one really high ANA elisa which ended up negative upon repeat), more sophisticated testing, my EMGs and evoked potentials were normal. IVIG did not change my autonomic testing. On that basis, they must have ruled out CIDP.

I believe the sural nerve is thinly myelinated, thus, demyelination would be noted if CIDP.

I know they are doing fewer of the sural nerve biopsies as they cause further nerve damage, and if there are other ways to come up with CIDP diagnosis, well then it makes sense to skip the sural nerve biopsy.

I am curious as to the conclusions of this, my last (for now) adventure in diagnostics...more mystery, probably.

In ways MS has had a 'leadership role'
 

for us all? In that we both suffer from degrees of 'demeyelination'. Just from different starting points in the body?
Cycleopes: Two key points in this all are that 1]demeyelination in MS occurs in the brain...and will usually show up in an MRI..tho not always[PN'ers are of course only 'findable outside that brain'?] and that 2] PNer's and MS folks both react poorly to anathesias of most types. ON the plus side tho is MS research has brought lots and heaps of info about the demeyelinating process [tho no cures...yet] into light..treatments are at the 'mouse model' stages...but PN'ers also have now their OWN MICE! That is not a 'resource' we had even five years ago. I am grateful for every mouse who is giving his life for all of us.
All sorts of PN conditions are being added to the St Louis site each year...I am surprised at how many are being added or 'clumped' or shoved into the square pegs that sort of 'fit'. When I was first diagnosed, I read extensively, and intuitively figured that dozens would be added in the next decade-that is happening. IN that respect, Glenn you are right...stuff is being 'dumped' into the PN categories, when they should be more rightfully be pegged as auto-immune or toxic. Further, those of us lucky enough to find good docs and get good medical diagnostics and then treatments are not the NORM! Currently it seems to me that the medical community has no desire to listen to general overall field concerns by patients..at all. That is sad, as they could learn LOTS from US...There are no vehicles for us to ask why 'docs always say..."It's in your head"...duh? based on what?'
Cycelops, I believe fewer sural biopsies are being done for the following reasons, as I have discerned from Insurance Companie's Policies and Procedures...found if you 'web' deeply enough? 1] it is an invasive procedure, leading to potential total paralysis if not done expertly and also severe infections 2] the procedure is only valid IF the biopsy sample is expertly handled from extraction thru analysis [rates last I read = 60% adequate handling rate...lo in my humble...] 3]Procedure should only beconsidered an option of last resort if multiple criteria [each company's #'s are different] are inconclusive. Lastly the Punch biopsies are only viable for small fiber neuropathies...to see what, essentially isn't there ...that should be. A sural biopsy only reinforces as a confirmation of what 13-18 other tests should already show. I declined and argued successfully as to why I was declining such a procedure to a top region's neuro department head! But then He also said I couldn't have CIDP because I wasn't in a wheelchair...Boy I set him straight on that notion! Like, DUH? I got diagnosed and TREATED IN TIME?
BTW only 50-60% of folks who try IVIG respond favorably...that doesn't mean they do or don't have CIDP or MMN or any others of the alphabet soups- IVIG is used for a multitude of immune issues as therapy...It just means, like all else we are all different, have a whole different set of other factors coming into play, and we all respond differently. Also, one can have nothing or a negative reaction to ONE BRAND of IVIG, but respond favorably to another...
Oh well, enough on my part here. On your part, I just wish there were more of the key 'positives and or negatives' that would lead to a clear cut diagnosis? I have learned tho, that sometimes, testing a few months later...say after some medication courses....and at different labs...things CAN come out different. I know folks with Lymes' and that is very often the case. Of course, my take on all this is one of the 'mere mortal- a patient', one who like many here THINKS about it all awake and asleep. Can't help it?
Silly :hug:'s to one an all - j

If one has good diagosis to establish a reason for small fiber neuropathy, I would treat the disease diagnosed and see if it the small fiber gets better.

CIDP seems to be a disease entity all on its own...more so than the ethereal small fiber neuropathy that accompanies many diseases, and is also 'idiopathic'. I think years ago CIDPer will probably plopped in MS with disregard to it being a separate disease.

I do not want a sural nerve biopsy if other tests confirm an absence of inflammatory markers that would indicate CIDP. You are correct, sural nerve biopsies are quite damaging. Nerves do not take lightly to manipulation.

20 years ago, diagnostics were more simplistic, if not more inaccurate....now so many factors play in...it is almost overload at times.

25% of all small fiber neuropathy is 'idiopathic', for at least a while.

I believe, that most people do not get the incredibly sophisticated care I have had recently since being seen at the tertiary center, where I have not encountered even a rude clerk.

Muscle biopsy is really last ditch effort to find a cause.....very few people get it, and if any one thought lip biopsy was bad...I don't recommend this. Not to mention compiling a 20 pound genealogy was not effortless either. My symptoms lately cross over into a myopathy category.

EPS is being done, because I have a history of nonsustaining ventricular tachycardia, on top of usual bradycardia. When they first found the nonsustatining VT (2000) they did not know I had SNF. (That clinic had me pegged as fibro) Nonsustaining Vtach, with neuropathy and myopathy, can add up to any number of neuromuscular diseases, basically genetic, or hereditary myopathies, which will in the long run all be declassified, reclassified and renamed, as all include some degree of neurological dysfunction. But if the muscle biopsy come up abnormal it gives even more importance to doing the EPS. I am losing some distal and a bit of proximal muscle strength.

We shall see what emerges from this. I approach it fairly neutrally. What will be, will be.

The neuromusular disease specialist who did my biopsy was very throrough on history, changed the original site of biopsy based on my history, and this is cute...said if 'this is of muscular origin' the biopsy will indicate it''

I told her my neuro will argue with her that the myopathy is really caused by the neuro, not the myo...she laughed and said I was right...and she agreed that it is a point of much discussion and research. This analysis will take 2 to 3 weeks and should yield some info.

Worst case scenario is I needed a pacemaker and they decided not to put one in...right? But then I won't be around to worry about that!

The uncertainty factor which has plagued my case all along. Global anhidrosis is unusal and generally not good....usually an MSA associated phenomenon. I don't have the other MSA problems, yet.

There is no denying the difficulty of autonomic neuropathy. Now I am having functional problems in some muscles, places not typical of SNF...

It will take time to figure this out. Because I get all my care now at the same tertiary clinic, I end up with many great minds chatting about the case now and then....all with acess to the same tests and records, I'm cool with my care. It's fine....they are smarter than I, but don't treat me as if I am too dumb to understand things. I can't ask for more...I am at the end of the internet...

My center is Mayo influenced and the protocols very similar.

hmmm
 

I don't think it's likely to be prions; those diseases take a long time to develop, and there isn't an immune response at all. Kind of like Alzheimers, amyloid, and maybe HIV--the proteins just start misfolding other proteins. This sounds like theyr'e talking about something more acute, and more motor than sensory. Since it's the Mayo that's seeing them, I'd guess they're getting mega-works. Maybe Fanfaire has run into them in the waiting rooms?

Awful. Blowing the meat off the bones the way they do increases the chance of brain and spinal cord meat contaminating the carcasses, and it just shouldn't be done, or way more carefully than what I've read about it.

Please forgive me for sounding stupid but why on earth would anyone shoot air into the head of a pig and let the brains fall out.

I must be completely clueless here. I just walked over to Alan and asked "Forgive this question, but have you ever been around pigs brains in a slaughterhouse. The look on his face was priceless.

But honestly, why would anyone work there, and what is the end result??

I'm very serious here.

Is this a food item or something??

Thanks.

mel

could be...
 

You know a study was done on kids in Denmark who had genetic tendencies to Juvenile diabetes. 1/2 were allowed to drink cows' milk, and 1/2 not.

The ones that did not use cows' milk, had significantly lowered rates of diabetes. This was traced to the bovine insulin fragments in milk, stimulating
antibodies which then attacked the pancreas.

This grim slaughterhouse article mentions inhaling aerosol mist of brain tissue which would contain myelin. Since inhalation is a known sensitizer for drugs, it could very well be that this process engaged the immune systems of the workers. Add to that the vibration from the air guns, if held in the hands could be a trigger. If minute amounts of myelin were inhaled, and the body reacted to it, then it might react to other myelin like in the diabetes study.

You know, inhalation is an interesting topic. How many of us use spray bottles and cans of products. I have limited my use quite a bit, as I overtly react now to most cleaning products sprayed out of anything....you gotta think...of course we inhale droplets!!! I just can not imagine aerosolizing pig neural tissue!!!

I bet that
 

we will not find out the whole 'processing' procedures at that plant until we are far more older and 'grayer'! That plant will probably close down and be demolished before an appropriate investigation is done. That's the way it is in this world. It really could be something else entirely, it depends on what CDC preliminary investigations reveal about plant safety, hygiene and where/how all the affected folks lived. Could be other 'common' factors and the plant is just a 'co-incidence'. I would be tho, whatever the results? We won't know about them until some nasty law suit about 5-10 years from now in some kind of Erin Brockovich way.
More important is clear analytic diagnosis and environmental assesments, for those affected. IF they truly have CIDP, We know their employer is NOT gonna cover IVIG treatments, no way no how, not for that number of folks! Then they are left disabled and to fend for themselves. Right now, IG is only covered adequately under medicare/medicaid disabilities with one heck of a hefty copay each infusion[that only pays for inherited conditions, not acquired]...often at a fee more than my own insurance pays for each treatment. There are lots of strange things going on in this world and the ripple effects for those families are going to be horrid. Many of us know the repercussions of the CIDP neuropathies on our lives...Essentially turned inside out and upside down? I, for one, wish I knew how to help and support them, while not financially at least information-wise.
Good thoughts to all otherwise? - j

I have a shred of optimism...
 

I am hoping this horrible incident paves the way to explain autoimmune
PN for others. This is unfortunately how medicine works. Some pay a price
to point the way to knowledge that ends up helping others.

I get a bit confused on some of this CIDP. I assume that if you have Idiopathic Small Fiber Neuropathy, they have ruled out CIDP. I wonder how the distinction comes in as small fibers are c fibers and alpha delta (thinly myelinated fibers)....so myelinated fibers are affected in small fiber neuropathy. It must be based on lymphocytic infiltration, and if your epidermal nerve fiber biopsy does not show lymphocytic infiltration and/or you have no other inflammatory markers, it must be dubbed 'idiopathic SNF'. Is this a correct deduction?

The below quote is from

http://www.emedicine.com/neuro/topic467.htm

"The term chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been used to identify patients with a chronically progressive or relapsing symmetric sensorimotor disorder with cytoalbuminologic dissociation and interstitial and perivascular endoneurial infiltration by lymphocytes and macrophages. In many ways, CIDP can be considered the chronic equivalent of acute inflammatory demyelinating polyradiculoneuropathy (AIDP), the most common form of Guillain-Barré syndrome (GBS).

A number of variants of CIDP have been described that have immune or inflammatory aspects and electrophysiologic and/or pathologic evidence of demyelination in common. No consensus exists on the best approach to the nomenclature of these disorders. CIDP is a major subset of chronic acquired demyelinating polyneuropathies (CADP). In this context, CIDP is considered when patients have a symmetric proximal and distal motor predominant disorder.

CIDP variants include patients with predominantly sensory symptoms, those with a distal symmetric disorder (DADS), those with multifocal sensorimotor neuropathy or sensorimotor mononeuropathy multiplex with prominent conduction block (also known as Lewis-Sumner neuropathy), and those with CIDP with associated CNS demyelination or with other systemic disorders.

The following disorders are considered distinct from CIDP because they have specific pathophysiologic features and respond to treatments differently than do patients with CIDP: Demyelinating neuropathies associated with immunoglobulin M (IgM) paraproteins, including those with anti–myelin-associated glycoprotein (MAG) antibodies; polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome; and multifocal motor neuropathy."

I've read a lot fo the literature in this area--
 

--and my understanding as to the distinctions and nomenclature is pretty much along the lines of that emedicine.com summary.

But--and this is an important caveat, gleaned not only from looking at a lot of the small-fiber skin biopsy material (especially the early work from the Johns Hopkins researchers) but from talking this over with Drs. Latov, Chin, et. al.--inflammation is likely a component of almost any of these immune-mediated neuropathies. Without getting into heavy biochemistry, the distinction is basically between those neuropathies that, like CIDP, have more classic "cellular" (macrophage/lymphocyte) infiltration, and those, such as the ones associated with M-proteins, in which the attack is protein/antibody mediated. The processes are biochemically dissimilar--you get "key in the lcok" receptor site cross-reactivity in the latter--but the result is still inflammatory, although it may be harder for measures such as C-reactive protein to pick up the latter. One of the most common findings on skin biopsy, for those who have small-fiber neuropathy, is swelling of the axons, and that's an inflammatory antecedent.

The example of small-fiber gluten neuropathy is instructive--in those with gangliosides along their C-fibers that are structurally similar to the gliadin molecule, antibodies formed to gliadin cross-react with those gangliosides, with the resultant small-fiber axonal dysfunction. There is generally a period of swelling and excessive branching before the fibers discorporate.

mel
 

Did you get the answer to your question?? Look how many years before you walked into a Dr. office before you heard have you ever been asked have
have you ever had Black Lung Diease?? People did and still do
worked in those mines,why now good money.. And in the past it was a way
to buy food...It was years befor men or women wore masks on
asseembly lines,for years they couldn't be force..They claimed they were hot.
But they supported there family's...I would imageine the same for these Mn.
workers..Oh I know this is a simple way of answering the question,you
would think things would have changed but unless some lawsuits are invoved,
it's sad because even in the health field patients and heath care workers
lives are shortened why years of not wearning gloves are washing there hands.

Aerosol spray cans,we have known about for years ,but so many still use
them..Oil rigs next to Holleywood Highschool. Oh in Mo. for years shoe factories,which are now being converted into homes for seniors..Money
and when it effects your heath in some way you do it to feed your family..
Unless we demand better health care and working conditions,thease boards
will contiue..Not to mention weathy neighborhoods who charge home owners
to burn there garabage..Why you President will tell you it's gives people
jobs until they get sick,that we figure out..I doubt if this will be printed,
just wanted to make sure your question,was answered...To all the above you are brillant ,thank you. Keep up the battle.. Sincerely Sue :)

Thank you for your explanation
 

Glenntaj:

Thanks for your info. I appreciate it.

I checked my biopsy report. No mention is made of swelling axons, only a length dependent pattern, sweat gland density being low...calf area down to 2 fibers per mm and a -3 in sweat glands....one normal hair follicle was in the sample...one Langerhans cell was located in the thigh sample stained positive with PGP.

Nothing about onion bulbs, or axonal swelling.

I have some horizontal pattern of innervation in the calf.

Nothing seems conclusive........my biopsy was done by a very respected research physician who trained and worked at Mayo....he set up the autonomic testing facility where I go, likely based on his experience at Mayo, so next to Mayo itself, I think it is pretty reliable.

I have never been there, but, I don't hear much mention of Vanderbilt's Autonomic Center, but I read it is quite good and developed from testing astronauts autonomic reaction in space. Of course, not all small fiber neuropathy results in autonomic neuropathy.

Like I said before, I think I have just reached the end of the internet, for now.

I don't think that I can scientifically substantiate a case for molecular mimicry based on B. Burgdorferi, even tho I have the specific bands for Lyme and the clinical diagnosis from EM, and a rising titer, established at that time. Lyme testing is extremely controversial. I did have one lab that tested bands that most labs do not test, (ironically the very same proteins they used for that Lymerix vaccine.) I am positive on all bands for Bb. I am negative on the tests that test IgG, positive on tests of IgM. (Women have different immune systems then men or the fetus would not survive-I have read women have more IgM response) Early antibiotic therapy aborts the immune response to Bb....and all further testing depends on that immune response. I have had negative PCR of spinal fluid, so I do not think there is any active infection. They took 20cc of spinal fluid, so that is a lot to test for Bb proteins.

If axonal degeneration is an outcome of Bb, thru molecular mimicry, it would cost the 'system' quite a bit of money for IVIG. I do not see research proceeding in the direction of proving molecular mimicry causing axonal or neuronal degeneration. The cost of IVIG would bankrupt insurers, and drive up the cost of IVIG due to shortages.

All inflammatory markers, thus far, are negative....which is why I pushed genetic, just to see. Chances of a hereditary cause are likely low, but due to disease progression and my own work on pedigree, my doc agreed.

If I could make a case, I would likely be on IVIG...but my case would be based on Lyme, and Lyme gets a bad rap. Insurers won't buy it.

This biopsy is getting pretty old now, almost 4 years old and symptoms are much worse now then when it was done.

I think confusion comes in with abreviations....CIAP=Chronic Idiopathic Axonal Polyneuropathy, versus, CIDP=Chronic Inflammatory Demyelinating Polyneuropathy....two very distinct types of PN. Even with a medical background, I have to really double check things. I think it would be worth a posting showing all these abreviations and explaining these diseases and how many different types of PN there are.

With idiopathic cases, showing no inflammation, showing disease progression, with negative finds for any of the known disease entities responsible for axonal degeneration, (except for Lyme) I do not think it unreasonable to explore heredity.

OK the 'science' begins?
 

Or not, call me a sceptic about these issues, but here's the latest on what all is being done about this all: http://www.medicalnewstoday.com/articles/90789.php

I'd say, watch the business news for that plant's closure due to 'financial reasons'. - j

Sand but true, it is the reality of the times we live in.

Good find
 

It only takes an inquiring mind and good investigative work to locate these clues. Why can't they see the picture? We can. And hey,...I'm no Einstein. :p

Billye