December 7, 2011 at 10:44 pm
Hello everyone! So I went to the university ALS/neuromuscular clinic today.. everyone has told me along the way I sound like CIDP, but there was also a concern for possible ALS.. We were there for almost FIVE hours! over 3 hours with the dr’s, which was great and extremely rare these days- conclusion was huh…. They all looked perplexed confused and really didn’t even give us a differential at this time, which is frustrating.. however they did repeat a now 3rd EMG that was normal- they even tested the muscles that have obvious atrophy and clinical weakness in my hands- normal.. it seems like with muscle atrophy and weakness it would reflect in the emg.. sooo I’m STILL a mystery, which is scarey!
However they are the ALS specialists and they sais it was not als.. pheeeeww!!! we are SOOOOO Thankful for that news!
Finally, to my point though- they ordered more tests including a skin/nerve biopsy.. Has anyone had one of these- sounds like a punch biopsy. Do yall know what exactly they are looking for , what disease processess can be found or ruled out with this? It looks like cidp may be one of them, though with a negative emg and protein of 31 in my csf i’m not a typical cidp if I do have it.. not sure where that leaves me!
December 7, 2011 at 10:58 pm
good news 🙂 that it is not ALS.
I had the sural nerve biopsy at the Mayo clinic. This is the nerve in the ankle. I, personally had no problems with it. It was done under local anethesia, had no problems walking on it immediately after. For the first week i had shooting pain in the area of the biopsy whenever i flexed my foot. since then, no pain. minimal scar, barely noticeable now. Mayo did a good job putting the stitches under my skin and steri strips on the outside which minimized scarring.
I have numbness on the rt side of my foot from it. I was skeptical about the nerve biopsy, but it did show a pattern of demylination/remylination and an inflammatory process. So in my case it was helpful in confirming the cidp diagnosis.
December 10, 2011 at 12:18 am
Lori- Thanks for your reply!! I wonder what the difference is between the sural nerve type biopsies and the punch skin biopsies like the one I will be having. They said It will show nerve damage in neuropathies.. I wonder if the skin biopsy can show neuropathies like CIPD? I think they are trying to get a diagnosis so that we can start an IVIG trial I sure hope it helps me!
December 10, 2011 at 2:11 pm
Very glad to hear it is not ALS! I have been dx since Jan 2008 with CIDP- first dx with GBS after ending up in hospital for one week in Sept. 2008. I had an excellent response to IVIG and when I kept relapsing, was placed on maintenance IVIG. Like you, me nerve conduction studies were normal, as was my CSF. I also had some atrophy and clear classic symptoms and severe weakness. I was treated with IVIG maintenance despite based on my clinical response and dx with “possible atypical CIDP” – My MRI showed some small amount of nerve root swelling in lumbar spine. I finally went to Mayo clinic for consult and they performed standard nerve conduction, emgs which were again normal,but in addition they were able to do studies of the nerve root ( the proximal portion) called nerve root stimulation. It was painful but finally showed the nerve conduction block (60 percent in lumbar spine roots and 30 percent in cervical spine roots). Because my area of demyelination was so proximal ( meaning high up in the peripheral nerve right after it exits the spinal cord) it was not picked up when the checked the usual distal ( towards the end) nerve conduciton of the nerve. This finally confirmed that this is indeed cidp – you are not alone in having an atypical presentation and I hope they get to the bottom of things for you so you can start treatment! By the way , I am now on IVIG every 10 days (20 grams), 50 mg Imuran daily for cidp and doing pretty well – hope this helps.
December 10, 2011 at 10:06 pm
Wow Shellbones, THank you!!!! That REALLY gives me hope!!! How were your reflexes?? Mine are “normal” I have always had brisk reflexes though, and now are just normal, though several neuros have said they are almost absent or only 1/4, but then another neuro will come and and be able to get more of a response, so who knows!
How long did they do IVIG on you initally before you had the actual CIDP diagnosis? Sounds like they are going to do a IVIG loading dose trial for a bit, but they aren’t sure how it will be covered by insurance without a diagnosis (it’s now polyneuropathy with possible autonomic involvement), did you have insurance that covered your trial of IVIG?
That’s really interesting about Mayo performing those tests, I will have to ask them about the nerve root stimulation, they just looked so confused by it all, and kept saying I just seem like GBS/CIDP..
I have been so confused to how I can have visable atrophy and weakness but a normal emg!?! Were they confused about that with you as well?
Did they ever give you a specific name to your varient of CIDP? I hear there are at least 18 types that they know of.. seems like they should name yours a new type if it hasn’t been documented before! 🙂
Thank you so very much for replying, I really really appreciate it, and your story really gives me hope!
December 11, 2011 at 10:00 am
Hi there – to answer some of your questions my reflexes have been normal or near normal also! The only time they were decreased is when I was hospitalized with diagnosis of GBS – at that time I was essentially paralyzed from the chest down also unable to lift my neck- some involvement of breathing and swallowing and facial muscles . Because I was hospitalized and unable to walk and diagnosed with GBS, insurance did not give any problem with ivig loading dose ( 5 consective days of treatment) . I subsequently relapsed 7 weeks after my first episode and was treated with another 5 day loading dose of IVIG this time at an infusion center . My diagnosis at this time was still GBS. After my third relapse I was re classified as possible CIDP and recommended to go on maintenance IVIG 2 days every 2 weeks. My health insurance fortunately has never questioned the use of IVIG ( I have aetna ).
The specialists were all confused as to why my testing did not show usual findings. I have not been given a name to my particular case but they have ruled out a number of other variants. I have been back to the mayo for a second visit with essentially the same findings. I am now 45- was 41 when it first started and have a history of other autoimmune disease ( lupus). When I asked them if they have seen cases like mine, they responded ” sometimes”. I saw Dr. Dyck at the mayo. I think it helps to see a referral center where they see alot of this, as most small centers or practices see only a few cases. If my nerve root studies did not show findings the next step for me was biopsy. Aslo in my case my weakness was more proximal muscles ie hip flexors . I was told that not everyone is trained in nerve root nerve conduction studies especially in small centers. My insurance covered my consult at the mayo clinic, but I had to pay for travel and hotel expenses. It was worth it for me in terms of knowing I was on the right treatment, the validation ( knowing I was not losing my mind!), and to help make sure insurance could not decline ivig treatment. I have also been fortunate to have had doctors who listened, and were very supportive. At this point, I am stable on treatment and doing much better – I can lead a fairly normal life working part time, raising 2 kids, but can no longer do sports ( cycling, running, skiing etc ) which I used to do and of course IVIG treatments. I wish you luck with your upcoming testing and keep going! Have you had a sensitive MRI to look at your nerve roots? One of the criteria they can use for diagnosis is to find nerve root swelling. they need to use the new more sensitive MRI – good luck and please feel free to ask me any more questions you may have!
December 11, 2011 at 10:12 pm
Shellbones- thank you so much!! Wow, sounds like it has been quite the journey for you! I will ask them about the nerve root stimulation for sure, if we can get some answers, I’d be SOO excited!! I also like that you say you have had normal reflexes too! I defintely have pretty substaintial proxmial weakness- my hips and shoulders are very weak, but its both of my hands that have actual motor loss that is permanant- the tips of all of my fingers are floppy, and the base of my thumbs are weak.. I don’t drop things and can still grip ok, but typing, I type with floppy fingers, which is odd, and my find motor skills have gone out the window!
I also have slurred speech at the end of the day, along tip of tongue numbness and pretty extreme all over muscle weakness – including my face , I can only smile or laugh a few times before I can’t do it for a while.. (my myasthenia panel came back normal, but I think I’m going to request a Musk MG panel too).
Did you have any muscle atrophy?
I’m glad that IVIG is working so well for you! I have always been a runner, skier and surfer, and with all of this it’s looking likely that won’t happen again, but after being told that ALS was a possibility, I can live with that for sure!!!
I think a trip to MAYO may be in our future.. Do you know if the initial IVIG loading dose is usually done in the hospital, or do they start it outpatient? Thanks again, I REALLY REALLY appreciate it and you really do give me some much needed hope!
December 12, 2011 at 9:10 am
Hello again! It has been quite a trip indeed- I think the MUSK panel is a good idea- they ruled me out for Myasthenia too- I did have atrophy especially in my legs in my quads and calves were most prominent – that has improved since I have been on IVIG- I believe they will start ivig as an outpatient but may have you go to an infusion center attatched to the hospital for first try- make sure they test your IgA levels as people that have a deficiency of this can not recieve ivig- ( this is a type of immunoglobulin your body normally makes but rarely there are people that have a deficiency of this) – I ended up with a medi- port when ivig became a regular thing for me. My advice if you get it is to start low and go slow! I found that my side effects were greatly reduced when I lowered the rate of infusion. I dont go past 60 cc/hour – it takes me a little less than 4 hours from start to finish . Do you have a date for your biopsy?
December 12, 2011 at 11:36 am
thank you, they haven’t checked my IgA levels yet, thats interesting, what happens if someone with an IgA defiicency receives IvIG? You know, I have always had negative antibody testing, even to things that I know I have been exposed to (immunizations, chicken pox etc etc- my titers are always negative even after getting multiple injections of the same immunization to try and acheive a titer) I wonder if that could be a cause? I worry that due to me never mounting measurable antibodies that all this bloodwork I have gotten might not mean a thing!
We dont’ have a date set yet for the biposy, the Dr who performs it (I guess there is only one around here), is booked out until march!! They didnt’ want to wait that long, they are trying to get it all done in the next week or two, so they were going to talk with him and get back to me…. I sure hope IVIG helps!! I’m SOOO nervous it wont help and i’ll continue to decline.
Before you had the port, did they use a picc line or just place an IV everytime you went in? Sorry – so many questions!!! I really appreciate your help!!!!
AnonymousDecember 12, 2011 at 4:25 pm
I do not have CIDP. Had mild GBS and now chronic fatigue syndrome/fibro. I had a nerve biopsy in the beginning, from 2 places on my leg. I am not sure if it is the same one that you are talking about, but they were looking for autonomic nerve disorders I believe. They just took 2 small patches of skin. It did not hurt much, and healed up right away. People have talked on here about some other kind of nerve biopsy where they have had residual effects at the site of the biopsy. If it is that kind, then you may want to think twice about it. I would ask more questions about the biopsy, whether there would be any residual effects, and what they are looking for.
December 12, 2011 at 4:50 pm
please no apologies for questions! You are asking very good ones and the more info you have the better! In terms of IVIG and IgA deficiency, you can have a severe, anaphylactic type reaction as IVIG contains small amounts of IgA and apparently if your body will not recognize and accept it and it can be very serious. they would only check your levels if you are going ahead with IVIG as it does not have any bearing on cidp itself. Intersting that you don’t develop measurable antibodies to vaccines – I have not seen anywhere that this might be related to CIDP but it is a disorder of a dysfunctional immune system so who knows? I had a normal IV the first times I received it in the arm for 5 day loading doses, but my veins did not tolerate well and I am petite 5″ and 105 lbs so my veins are not that big- Hang in there!
December 12, 2011 at 9:45 pm
Thank yall so much for your time!!
Jessicah- Sounds like I’m scheduled for the same as you had – punch skin biopsies, I think is what they are unofficially called, but my dr called them an epidermal nerve biopsy, not an actual- cut out the deeper nerve biopsy like others I have read about that can have nerve pain and may not even yield great diagnositic results either.. From the sounds of it it seemed this punch skin nerve biopsy was a newer thing, the one dr that does it is booked up until march, but looks like as of now we are going in Feburary- she said it would look for nerve damage/neuropathies etc…. Do you know , does it just look for small fiber neuropathies, or can it look for CIDP type neuropathies too? (not really sure if cidp is small or large- apparently on my exam, I have both abnormal). They think this whole thing has affected my autonomic nervous system and are going to be doing additional testing for that, other than the biopsy. I hope it gives us answers about it all – especially my motor dysfunction!! Did you get any answers from it?
Shellbones- thanks, that is good to know!!! I’ll make sure that they check it before proceeding with the IVIG! I’m a tough stick for an IV apparently, I have these huge veins, but they all tell me how “valvey and knotty” they are ha.. but I tell ya, someone can poke me with a needle a million times if it means that I can start getting better from all of this! I’m READY to get back to feeling decent and on with life again!
I really appreciate yall, thank you!
AnonymousDecember 13, 2011 at 12:02 pm
My biopsy was negative. Actually, one of them the doctor kind of screwed up and they could not get a good answer from it, but essentially, I did not have small fiber neuropathy. I had a lot of autonomic nerve symptoms so that is why they were looking. I went to 6 neuros and one of them told me that I had P.O.T.S. an autonomic nerve dysfunction. I went to 2 rheumys. I ran the gamut with tests, except for spinal tap. All “normal.” My ANA was slighter higher and some other inflammatory markers, but nothing that would indicate any diagnosis of an autoimmune disease.
Like you, I thought I had GBS/CIDP, then some other autoimmune, like RA, lupus, etc. I had a lot of neurological symptoms in the beginning, lost reflexes, weakness in arms, severe nausea (25 lb wt loss), tingling /numbness etc. That all got better over time. What I am still dealing with is fatigue, and joint pain and rashes. I finally went to an immunologist in Miami (I am in Chicago). Another member on this forum had an H1N1 vaccine reaction at the same time I did and he works for U Miami. They have an immunologist there who specializes in Chronic Fatigue syndrome and other immune disorders. I finally went down there and the doctor found that I have a low immune system. The vaccine triggered latent viruses (I have 3 out of the 5) and that continue to cause symptoms when your immune system is not functioning properly. I am treating now with supplements and immune boosters.
I wish you the best of luck. It took me a year and a half to finally get to the right doctor who can help me.
December 14, 2011 at 11:32 pm
Wow Jessicah, sounds like you have been through a lot!!!! It really amazes me, especially with this neurological stuff, how so many people have to go to so many different doctors!! I could never be a neurologist since it seems like it’s all so incredibly subjective! I feel like most things you dont have to go and get multiple opinions, mabye a 2nd opinion, but it seems like a lot of people on here and that i’ve talked to see 4, 5 or more neurologists before nailing down a diagnosis.. I have seen.. humm, gotta count them all- 6 totat neurologists!!! 3 that were neuromuscular specialists, and I sit here worsening with no one really knowing what is going on! I have told my husband a few times – I wish I could just Pee on a stick like a pregnancy test and we could just get our answers! haha.. Wish it was that simple!
With all of this going on, I have opted to NOT get my flu vaccine this year! I don’t give my kids the flu vaccine (they do get everything else though), and I never got it until I worked where I am very exposed to it daily, I started getting it so I wouldn’t bring it home to my kids (if the vaccine was effective that year), but not this year- no way! I did make my husband go get one though, if the flu hits us, we need one man left standing to take care of us haha.. that is IF it even works!
It’s crazy what can set off our immune systems! Hope your doing well! Thanks again so much for your help!
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