Tentative CIDP diagnosis (sorry…long!)

    • Anonymous
      May 2, 2008 at 11:48 am

      Hi everyone. My name is Margaret and I am a 60 year old with a couple of health problems, one of which may be CIDP.

      I have a dilated cardiomyopathy and severe GERD. I have had exertional shortness of breath that, after 5 years, was determined to not be related to my heart failure. Last April I was diagnosed with significant respiratory muscle weakness, with maximal inspiratory effort about 45% of predicted and maximal expiratory pressures about 40% of predicted. These were definitely accurate studies.

      I have had a zillion negative blood tests for MG, Lupus, mitochondrial myopathy, etc., etc. Mostly the only positive thing was consistently elevated sed rate in the mid 50’s. CK neg.

      I also have proximal muscle weakness with very easy fatigueability, although the neuro tests my objective strength as normal. For instance, when I dry my hair it is a real hassle as I have to keep stopping and resting or I get a lot of burning pain in the arms and shoulders. It is mostly upper body. I have significant respiratory muscle fatigue, and have been on oxygen at night for almost 5 years and now on bipap at night to give my resp. muscles a rest. It helps a lot. I have a normal gait.

      I had a muscle biopsy in the fall which was essentially negative. The neuro thought I had a mito myopathy. My EMG and NCS was negative. About 6 weeks after the EMG which was right side only, I developed a neuropathic pain in my left foot. I am not a diabetic. Several weeks ago I had an EMG of the diaphragm which showed dennervation but also re-ennervation with positive sharp waves and fibrillations and large motor unit potentials with normal duration and normal interference pattern.

      My neuro, who is at a large university medical center and specializes in neuromusc. disorders now says I have CIDP, even though phrenic nerve involvement is rare. She wants to start a course of IVIG asap, which I gather can also be diagnostic if it helps. She did not mention doing an LP although she noted that protein is elevated in the CSF with CIDP. I had another EMG/
      NCS yesterday, of the left leg this time, which was normal! I have no numbness but I do get occasional severe muscle cramping and an odd random sensation of something crawling on my legs. 🙂

      So, after all that history, do you guys think I really have a CIDP? The neuro who did the testing yesterday did not think so, and said he had no idea what is wrong. I WOULD JUST LIKE SOME ANSWERS!! We are getting closer to an answer, though. BTW, the upper body issues started about 6 or 7 years ago and has very gradually been progressing. The heart failure and the resp. muscle fatigue started almost 6 years ago.

      Any input would be greatly appreciated!

      *I get by with a little help from my friends*

    • Anonymous
      May 2, 2008 at 1:05 pm

      That doesn’t sound like CIDP to me. A lumbar puncture would show elevated proteins I believe and that should help confirm CIDP. From the sounds of it though, no tingling ,weakness of hands/feet – hmmm… I would say more tests are needed to confirm.


    • May 2, 2008 at 6:50 pm

      I was curious, why the muscle biopsy to dx cidp. Usually if any biopsy is done it is a nerve biopsy. The nerve biopsy would show each demylienation process you ever had. You have had so many tests, I guess that would be the last in order. An elevated protein with a l/p is not always present. However, usually with so much time with issues (you state about 5 years) I would expect the ncv/emg would have some indication of conduction blocks or slowed velocities. As well, my son had upper and lower body weakness WITH gait issues, bowel, bladder and respiratory issues w/in a 6-8 week time frame.

      Regarding trying ivig and using it as a diagnostic tool, I suppose you could do that. If you were having symptoms and already had a confirmed dx of gbs, and suspected cidp, I would say for sure, do it. But you are not firm with a dx. and I suspect with the mild weakness issues, it might be hard to notice a difference. People with cidp like my son for example are not able to hold a pencil, walk up stairs, button pants get up from a sitting position (and he was considered mild) so once ivig is administered, the results were obvios to decipher. We did use ivig as a final dx tool, as there was confusion as to if it was gbs or cidp. There was an immediate response after a few relapses and administration of ivig and the ivig would not have helped if it were gbs residuals.

      Is it possible you at one time or another had a mild gbs attack and are now years later feeling residual feelings? Good luck in finding answers to your questions, the process can be very frustrating!
      Dawn Kevies mom

    • Anonymous
      May 2, 2008 at 8:06 pm

      Thanks for the responses.

      The muscle biopsy was to try to diagnose a mitochondrial myopathy which is what the neuro initially thought I had. It wasn’t until the diaphragmatic EMG showed what I understand are the typical CIDP changes that she suspected CIDP. Because of that, I think I would be able to tell relatively quickly if I improved on IVIG, because I get so short of breath with any exertion. I realize that my upper body weakness is nothing compared to what it could be, but I think I would notice right away if there was any improvement. My adl’s are impaired. Who knows? She does not know the results of yesterdays EMG, so maybe she will head in yet another direction. I really do not want a nerve biopsy, I know that! The positive EMG did not show any sign of myopathy, just neuropathy with dennervation plus re-ennervation. And nope, never any hint of GBS, ever. There are just so few demyelinating diseases that I don’t know where else she will look. I’ll let you know if some miracle happens!


      *I get by with a little help from my friends*

    • Anonymous
      May 2, 2008 at 9:07 pm

      In reading your story, I wondered about MMN, multifocal motor neuropathy which is a chronic immune mediated polyneuropathy, but one that classically affects the upper extremities more than the lower and usually is predominantly motor. You have very little sensory involvement, it seems, although you could have more than just the left foot neuropathic pain that you mention (or this could be really bad and you not saying that). This is also treated with IV IgG. Sometimes it helps to look for GM1 antibodies in MMN. I do not know details of MMN, but it might be worth asking your doctor about since the upper body is affected more than the lower.

      A couple of questions/suggestions. Have you ever had an EMG of the arms? Often it is best to look at the places most affected by symptoms and I would wonder about your arms since there is so much fatigue there.

      Is your GE reflux time course associated with the respiratory muscle weakness and have you had other “autonomic” changes such as slowing of internal organ function (constipation, trouble to empty the bladder) or alterations in blood pressure or heart rate.

      There are a few case reports of cardiomyopathy and Guillain-Barre/inflammatory polyneuropathy. I read about this long ago and do not remember if there was an explanation as to why they might be associated, but you/your doctor could search for the combination on Google or PubMed to see if that gave any information that might help you.

      My final comment, is that many of us on this site have had a long time from symptoms to diagnosis and some remain a mystery. If the suspicion is an immune mediated process, sometimes it is worth a trial of treatments for such (Iv IgG or steroids, for example). As well, almost everyone that comments here will attest to the fact that they are not like the textbooks. There is a lot of variation in what symptoms people have, severity, and course.

      WithHope for cure of these diseases

    • Anonymous
      May 12, 2008 at 11:11 pm

      With Hope:
      I really appreciate your comments. I don’t think it could be MMN as all my symptoms are bilateral except the left foot neuropathic pain. I do have a lot of pain, esp. in my arms and shoulders with activity, and the outside of my hips when I walk more than 2 minutes, and that is not a joint problem. My hands CAN hold a pencil but start cramping if held too long. I get frequent jaw pain when chewing that requires me to stop eating for a while before the pain stops. I do not have any numbness, but I do get some tingling in the upper back. I have read that not everyone has both motor and sensory issues, and that actually about 50% of CIDP patients present atypically.

      I had an EMG and NCS on my right side all the way up to the forehead last July that was negative. The neuro now thinks that the cardiomyopathy is not related at all and just bad luck to have also.

      All I know is that there is some process demyelinating my phrenic nerves for certain, and I do not want to end up on a ventilator! Any medical people on this site that can help some more? Or anybody have any more input? Please.

    • Anonymous
      May 12, 2008 at 11:49 pm

      Hi Maggiemag, Welcome to The Family. In my opinion, yes it could very well be cidp. You sound like you have been in my body for the last 15 years or soo! I still don’t have a def dx from a neuro, getting closer also! But my pcp has dxed me with cidp, and he has known me since before all this stuff started vs my former neuro who screwed up big time because of other drs’ stupidity. I see my neuro next month for a followup and hopefully a dx. Have you had any treatments or have had any improvements over the years, or is it all downhill symptoms flaring all the time? Not All patients with cidp have high protein numbers from the lp, 5% have normal levels. Do you take any meds like neurontin for your nerve pain? Take care.

    • Anonymous
      July 2, 2009 at 11:00 am


      I know this post is old.. but have you heard of something called Myasthenia Gravis?

      It causes proximal muscle weakness, and muscle fatigue in doing things like walking or brushing hair or teeth.
      And breathing difficulty is one of the first signs.

      When I first had my sx’s they were testing me for MG.

      There is a blood test for it, and then they take a medication used to treat it, and if you respond to it, then you have it. (it has something to do with the receptors not catching up, as they are being bombarded with too many signals)

      I hope you figured it out.
      Best of luck to you.