New here, plz comment – CIDP, MNGIE????

    • Anonymous
      August 13, 2011 at 11:55 am


      I have a long anc complex medical history. I have been diagnosed with chronic intestinal pseudo obstruction as age of 22 though I have had it probably since birth. This was only after having received countless misdiagnoses, mainly of psychic nature, and years of suffrance.

      CIPO is rare and is a movement disorder of the whole GI tract, hence there is no proper peristalsis to move the food/stools through.

      I have had three major abdominal surgeries.Now, I dont have any colon any more but an end-ileostomy. In addition, I have to wear a permanent bladder catheter as my condition affects the bladder as well.

      Ok, this was intro.
      Since approximately 1.5 years I have been developing progressive polyneuropathy. Now, it is very painful, and barely controllable as I have so many drugs I cant use due to my bowel issues.
      I also have local numbness, paresthesias, tingling, etc with my neuropathy.

      The last few months it seems to have progressed very fast.
      Last year, I had an IvIG therapy for a different reason: weh hoped to get the frequent infections (bladder and hickman catheter infection with sepsis) under control.
      Yet, I had multiple severe complications under IvIG therapy, hereunder aseptic meningitis and some other strange symptoms.

      I did not feel any improvement, and my neuropathy even got temporarily worse.

      First, we suspected the permanent antibiosis to cause the neuropathy. This might be true for the first months; at least the neuropathy worsened/improved with increase/reduction of antibiotics.
      Yet, the first symptom (throbbing pain in feet) started already after 4 weeks of antibiotocs which resolved by its own after a while and was then followed by the typical stabbing pain.

      Nonetheless we do not kow if the antibiotics were only the match for an already smouldering fire.
      I have had noticed muscle weakness since 1,5 years.

      Anyway. In beginning 2011 I had what seemed to be a flue-like bug. It could have been a bug, but its uncertain as well.
      During this bug I had acute onset of a weird and worrying sensation: there was a feeling of almost painful heavyness in my upper arms.
      I did not care any more when the symptoms resolved completely after a week. Then, I finally succeeded in getting of the antibiotics. One week later I noticed a marked imrovement in stabbing pain in feet.

      Then I got a vitamin coktail injection. And from then on my symptoms progressively worsened. First, I had again the throbbing pain in my feet which gradually moved to my distal legs.
      After three weeks my fingertips got painful blisters. The throbbing pain was unbearable. My veins in the feet were protruding and I got even more spider veins (I have had some before as I have autonomic dysfunction which also causes vasospasms).
      I then got cortisone which seemed to improve the throbbing pain and the blisters but made the stabbing neuropathic pain even worse.
      I finally came off the cortisone but ever since this episode I have had throbbing pain on and off in my fingertips and toes, sometimes feet and hands.
      Also, sine then my neuropathy has been progressing fast. Whereas before I did not have my hands involved I now do have neuropathy pain in my hands and even lower legs as well.
      I do not have just stabbing pain, but numbness, tingling, and burning. all pretty severe. pain is often unbearable.

      Now, since three days I have had this feeling of painful heaviness, particularly in my upper amrs.
      My neuropathy has not been exmained by a neurologist yet as in Germany, many neurologists are rather psychiatrists and with my history of psychiatric misdiagnoses…

      I may have finally got an appointment with a good neurologist some time next week. I am so terrified.
      and confused. i cant see a straight line in my symptoms. I thought about CIDP but why worsened cortisone my neuropathy pain while it helped with the strange throbbing pain nobody could explain so far? I visitied rheumy docs and they excluded with blood samples the option of an autoimmune disease, rheuamtological disorder etc.
      I dont exclude the option of an autoimmune/inflammatory issue yet.

      there are so may question marks and contradictions. I feel as if everything repeats itself: I have been through this with my CIPO. I had to go abroad to be diagnosed.
      Fighting the unknown is the worst at all.

      Now, I would be gratfeul of you could tell me your impression. Do you think CIDP may be an option?
      I still have touch with the Italian expert who diagnosed me with CIPO and there are some multisystemic diseases that can have CIPO as their first manifestation. Particularly mitochondrial diseases.
      There is a speficic type of mitochondrial disease called MNGIE which also causes CIPO, myopathy and peripheral neuropthy.I should get tested for, he thinks. But this is very difficult here in Germany.

      In addition to the nruoplogical problems I have developed endocrine issues, and other stuff, which might point towards a mitochondrial disease.

      i once read an article describing MNGIE patients who were misdiagnosed with CIDP at first.

      Does anyobdy know here anything about MNGIE or mito?
      thanks for reading. sorry for the length. would be so grateful for answers.


    • Anonymous
      August 13, 2011 at 11:57 am

      just another quick though: can antibiotcs or even IvIG therapy cause CIDP?

    • Anonymous
      August 13, 2011 at 3:30 pm

      thanks for answering. which tests do you mean? as far as I know demyelinating neuropathy is a feature of both CIDP and MNGIE. I do have intestinal dysmotility which could support the idea of MNGIE, but once again there dont have to be a connection between my neurological issues and my CIPO.

    • Anonymous
      August 13, 2011 at 5:51 pm

      thanks.unfortunately, MNGIE patients can have elevated CSF protein as well.
      I dont know if there is a difference in EMG and nerve conduction studies between the two conditions as the neuropathy is a demyelinating neuropathy both times.

    • Anonymous
      August 14, 2011 at 12:48 pm

      The article linked to above seems quite definitive.

      mngie is Autosomal recessive. That means “…it is one of several ways that a trait, disorder, or disease can be passed down through families.

      An autosomal recessive disorder means two copies of an abnormal gene must be present in order for the disease or trait to develop.

      Further, the article states: “The diagnosis can be confirmed by measuring TP activity in buffy coat or plasma levels of thymidine and deoxyuridine.”

      Anybody, anywhere in your family tree have this trouble?

      Has your TP been measured?

      For all acronym definitions please refer to the article.

    • Anonymous
      August 14, 2011 at 2:07 pm

      I have no idea what is happening to you, my poor dear. I will lift you in prayer to God and hope for answers for you. That is THE BEST thing I can do for you.
      Your situation sounds awful and I hope the doctors can find something to help you. God bless!

    • Anonymous
      August 14, 2011 at 8:30 pm


      There are things that your gastroenterologist and neurologist should look at to arrive at a diagnosis on MNGIE. Quoting from [url][/url] :
      [INDENT][B]Diagnosis/testing.[/B] The clinical diagnosis of MNGIE disease is based on the presence of severe gastrointestinal dysmotility, cachexia, ptosis, external ophthalmoplegia, sensorimotor neuropathy, asymptomatic leukoencephalopathy as observed on brain MRI, and [URL=””%5Dfamily history[/URL] consistent with [URL=””%5Dautosomal recessive[/URL] inheritance. Direct evidence of MNGIE disease is provided by increase in plasma thymidine concentration greater than 3 µmol/L and increase in plasma deoxyuridine concentration greater than 5 µmol/L. Thymidine phosphorylase enzyme activity in leukocytes is less than 10% of the control mean. [URL=””%5DMolecular genetic testing[/URL] of [I]TYMP,[/I] the [URL=””%5Dgene%5B/URL%5D encoding thymidine phosphorylase, detects [URL=””%5Dmutations%5B/URL%5D in approximately 100% of [URL=””%5Daffected%5B/URL%5D individuals.
      [/INDENT]The website I found above has a lot of good information; it would be worthwhile to read it. One thing, though, is that it is exceptionally rare. There are only about 70 reports of the syndrome since its description. I could not find out when that was, but it was early than 1997. Still, the odds of both CIPO and CIDP should be very low too, so if the shoe fits…

      Godspeed in finding a diagnosis and relief,

    • Anonymous
      August 14, 2011 at 11:15 pm


      thanks so much for replying.

      so is CIDP rather rare? as you say it was unlikely to have both CIPO and CIDP? Are you aware of drug-induced CIDP?
      Of course, I have taken numerous drugs, mainly antibiotics.

      I think I might have mild ptosis, too.
      Enzyme TP has been never measured so far.

    • Anonymous
      August 20, 2011 at 3:48 am

      Hello pelztier86,

      Yes, CIDP is rare. The prevalence estimates range from 1 to 8 cases per 100,000 people, skewed toward the low end. If you use 3 per 100,000, that means there are 9000 cases in the USA at the moment.

      I could not find prevalence numbers for CIPO, but it cannot be that common. if it is 1000 times more common than CIDP, the likelihood that somebody would have both is in the neighborhood of 1 in a million. If it is 10 times as common as CIDP, the likelihood is 1 in a 100 million. The statistics get a little more difficult when applied to such small numbers, so the likelihood might be higher than what I say here.

      I don’t know of any confirmed drug-induced case of CIDP. There was a thought that statins might cause it, by analogy to rhabdomyolysis, but I don’t think it has proven out.