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Are aquaporin 4 antibodies appear in any other context than Devic's syndrom?

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    Are aquaporin 4 antibodies appear in any other context than Devic's syndrom?

    Hi,

    Bit more than a month ago my mom developed burning sensation on her back and stomach, she went to a doc but he said its nothing lifetreatening and she waited for for her turn for the tests, it was like this for a week or two.

    Few weeks ago the burning sensation became too much to bear and my mom went to a hospital.
    They took her in and the next day she had issues walking and day later she couldn't walk on her own at all.
    After a few days they put her on some steroid IV thing for 5 days and now, couple of weeks later she slowly started to walk again but lost lot of sensitivity in her legs and her skin still react to touch with pain.

    Couple of days ago doc come, gave her envelope with test results and told her to wait one more day for another doc to interpret what it all means.
    She called me scared and asked to google all this and what it all means and when I started to google I almost lost it.
    There were some oligoclonal bands in her cerebro spinal fluid, Antinuclear antibodies ANA, oligoclonal protein IgG Type2*
    and something that freaked me out, in her blood test there were aquaporin 4 antibodies.
    I searched and searched but from what I could learn those are synonymous with Devic's syndrom.

    However, instead of Devic's Syndrom the doctor labeled diagnose under code of G36.8 as Other specified acute disseminated demyelination.
    My sister was talking with a doctor and also was worried about NMO, from what we googled mom would need treatment but doc said that until second attack comes there is no treatment apart from rehabilitation and second attack might not come at all, she also didn't called it Devic's syndrom but NMO spectrum diseases (its a rough translation english is not my native but she was ambiguous).

    I googled and googled and yet I cant seem to find those aquaporin 4 antibodies in any other disease or context but this horrible NMO thing.
    Are those aquaporin 4 antibodies synonymous with Neuromyelitis Optica or there are other cases when they can appear in tests?
    If its only NMO thing why no Devic's disorder diagnosis then?

    What treatment my mom should receive in case its Devic?
    Technically she is in hospital that considered top notch here and specialises in MS and Devic syndrom but getting doctors to talk is very difficult so there is no clear understanding of the situation.
    Last edited by KoKo; 05-06-2017, 02:52 PM.

    #2
    Hi Sasha:

    Unfortunately, MSWorld calls out NMO for discussion. NMO is so specialized and different from MS that it should never be discussed in an MS forum. People with no real knowledge of NMO get into discussions based on information that is usually completely wrong, and it ends up misleading everyone and being a disservice to everyone.

    There is a charitable foundation for NMO that has the best and latest information. So after this thread, please go only to the Guthy-Jackson Charitable Foundation at https://guthyjacksonfoundation.org/. The foundation also has a Facebook page at https://www.facebook.com/guthyjacksonfoundation.

    I do have knowledge about NMO, so I'll address some of your questions until you can get to the Guthy-Jackson website.

    Originally posted by Sasha84 View Post
    However, instead of Devic's Syndrom the doctor labeled diagnose under code of G36.8 as Other specified acute disseminated demyelination.
    First, Devic's Disease, or Devic's Syndrome, is an old, outdated name that shouldn't be used anymore. The doctor would not and should not make a diagnosis of Devic's Disease. The disease has been called neuromyelitis optica for about the last 15 years. As more information became available about NMO, researchers realized that NMO is actually a spectrum of presentations, and the more recent name is Neuromyelitis Optica Spectrum Disorder, or NMOSD.

    Second, NMO/NMOSD doesn't have its own diagnosis code. G36.8 is the closest code that exists, so the doctor used the correct diagnosis code. (Sometimes G36.0 is used.)

    Originally posted by Sasha84 View Post
    she also didn't called it Devic's syndrom but NMO spectrum diseases.
    As I said earlier, NMO Spectrum Disorder is the correct name. Devic's Disease is obsolete.

    Originally posted by Sasha84 View Post
    from what we googled mom would need treatment but doc said that until second attack comes there is no treatment apart from rehabilitation and second attack might not come at all,
    Sometimes a second attack never happens, but not all specialists agree that people with a first episode don't need to be treated. NMO frequently has recurrent attacks, and many (most?) NMO specialists feel that treatment should begin as soon as possible. It's a good idea for your mother to find a different neuroimmunology specialist and get a second opinion.

    Originally posted by Sasha84 View Post
    Technically she is in hospital that considered top notch here and specialises in MS and Devic syndrom but getting doctors to talk is very difficult so there is no clear understanding of the situation.
    Where is "here"? I'm having a little trouble understanding how a hospital that specializes in MS and NMOSD would let a person with NMOSD go untreated when the chance of recurrent attacks is so high. A second attack could be devastating, and many (most?) NMO specialists feel that treatment is mandatory. Even people who are on treatment can have very serious attacks, so letting someone go untreated is considered to be too risky. So even if your mother is already in a hospital that specializes in MS and NMO, a second opinion from an equally or more qualified hospital is highly recommended.

    Originally posted by Sasha84 View Post
    Are those aquaporin 4 antibodies synonymous with Neuromyelitis Optica or there are other cases when they can appear in tests? If its only NMO thing why no Devic's disorder diagnosis then?
    The aquaporing 4 antibody is highly specific for NMO. There currently isn't any other condition known to involve it. So it sounds like your mother has correctly been diagnosed with NMOSD. Again, your mother isn't being diagnosed with Devic's Disease because that term isn't used anymore.

    Originally posted by Sasha84 View Post
    What treatment my mom should receive in case its Devic?
    From what you've said, your mother has been diagnosed with NMOSD. There are a few different medications being used for NMO. You can get the latest information from the Guthy-Jackson foundation website.

    I hope you and your mother can get the information you need from the Guthy-Jackson Foundation website and that she can get into the care of an excellent NMO specialist. I wish you and your mother all the best.

    Comment


      #3
      Originally posted by Sasha84
      Is it still possible the second attack wont come despite all that?
      Yes, that is still possible. Some people have one attack, recover pretty well, and never have another problem.

      Originally posted by Sasha84
      I read that this happens only when spine and optic nerve get attacked simultaneously.
      That was found to not be completely true. That's why the name of the condition was updated to NMO Spectrum Disorder -- because there can be a spectrum of different presentations. Some people never have eye problems from NMO.

      Even though the hospital your mother was in is supposed to specialize in MS and NMO, their beliefs about NMO are not up to date, and neither is the Polish NMO website you were reading.

      Rituximab may be the most commonly used medication for NMO, but there are other medicines your mother can take if you can't afford rituximab. Another medicine used for NMO is azathioprine, a powerful immunosuppressant. It's been used for decades and is very well understood, at least in the United States. That may be different in Poland. But it at least gives your mother a treatment option that's more affordable.

      And she can always be treated with high-dose steroids again if she has another attack. But the next time, the treatment should start immediately, not after several days.

      Hopefully your mother will get more information when she sees an MS specialist in a few weeks.

      Comment


        #4
        Hello Sasha84,

        I have had NMOSD for twelve years and am seropositive for the Anti-Aquaporin-4 Antibody (aka NMO IgG). I just wanted to add to the excellent information that jreagan gave you in his/her replies to your initial post. In regards to the antibody---a seropositive test result is highly predictive of relapsing disease, and a full 90% of NMOSD patients (both seropositive and seronegative) have relapsing disease. In recent years they have found that only about 10% of cases are monophasic (one attack only---previously that percentage was believed to be about 25%).

        Please be sure to visit the Guthy-Jackson Charitable Foundation for NMO Spectrum Disorders at the link that jreagan provided. They are dedicated to finding a cure for NMOSD and have funded many research projects---all a collaborative effort.

        Wishing your Mother well and will be hoping that she receives adequate care and treatment.

        Grace

        Comment


          #5
          Originally posted by Sasha84
          Are there some exercises that person with NMO can do at home that can be a good substitute for hospital rehabilitation?
          Hi Sasha:
          There are exercises available on the Internet that your mother can do at home. Probably the easiest to find are exercises to do after a stroke. The cause of the problem is different than NMO or MS, but the principles of using muscles in rehabilitation are the same. Try to find a website that's as "medical" looking as possible, and avoid anything that doesn't make sense. It would be great if your mother could at least get some medical advice and guidance while she's waiting to get into rehabilitation, but it doesn't sound like her doctors are being very helpful.

          If your mother can walk, the most helpful thing she can do is to walk, as long as she can do it safely. If she can't, then the next best thing is just to move her legs regularly through as big a range of motion as possible. Then the same with arms and rest of the body. Work on whatever is weak, but don't forget to keep whatever is working well stay strong too.

          This is also very important: Someone needs to be available to help her with her exercises to prevent her from being hurt by falling or using her muscles in the wrong way. With some help, your mother should be able to have some improvement in functioning.

          Comment


            #6
            Sasha84, I thought of another good resource for you in addition to those mentioned by jreagan. Dr. Michael Levy, Director of the Johns Hopkins NMO Clinic, has a Facebook group. The name is Johns Hopkins Neuromyelitis Optica (NMO) Clinic. Dr. Levy, and his associate Maureen Mealy, answer questions from NMO patients. He is an excellent NMO clinician and researcher, and is part of the international panel that revisited and revised the NMOSD diagnostic criteria. You might consider visiting his group.

            Grace

            Comment


              #7
              Hi Sasha:

              I'm glad you're getting some good information about NMO. CellCept is a good medication, and it's very encouraging that it's available in Poland. But azathioprine is also a good medicine, which is why it's still used so much all over the world. So your mom has two good prevention medicines available even if you can't afford rituximab. And prednisone will always be available if it's needed. Proper treatment can cut the chances of another attack in half. And that's all very good news.

              Also, methylprednisolone (the IV steroid) is available. And, if the hospital is as specialized as its reputation says it is, plasmapheresis might be available for trying to speed up recovery from another episode. So things aren't as bleak as they first may have seemed.

              The rest of my advice is for you. You know how on an airplane the flight attendant tells everyone to put on their own oxygen mask first before assisting anyone else? It's time for you to do the equivalent of that.

              Your family has had quite a shock. But to help your mom the most, you have to take care of yourself and have a clear head. You can't help anyone if you're panicky and sad and your thinking is clouded by worry. Worrying won't give you any control over the future, plus it steals your happiness now. That's a double loss for you and your mom. But you can prevent it by steering your attention toward the right things.

              So, seriously, it's time to take a deep breath and calm down. There's medical care available for your mom. Today was already better than yesterday, and tomorrow will be better than today.

              Comment


                #8
                Originally posted by Sasha84
                Mom finally met a doc and we learned that that rituxan is not confirmed by a study to work on NMO (Maybe the study must be done here to count)
                That is incredibly hard to believe. Numerous studies have been done in the medically advanced countries showing that Rituxan works best and is the drug of choice for NMO. (Studies can be found on Dr. Levy's and the Guthy-Jackson websites.) But we already know that Polish medicine is a bit behind the times.

                Originally posted by Sasha84
                As for now its encorton+Imuran for 2.5 months then next visit to the doctor.
                And that's OK. Imuran is a good drug. I took it without any trouble for years until my liver couldn't tolerate it anymore. As long as your mom is taking a prevention medicine, that's a step in the right direction. And if other drugs become available, she can change drugs.

                Originally posted by Sasha84
                mom worked as a teacher, kids constantly coughing and everything. Is it save for person on Imuran to work as a teacher?
                Maybe, maybe not. It might be too risky to be around little children, who spread germs around pretty easily. It might be safer around teenagers, who tend not to spread illnesses from person to person so much.

                Originally posted by Sasha84
                I read that treatment with rituxan is only two IV's every 6-8 months and nothing else, what is the size of each IV?
                If it works as expected, Rituxan is given in two doses every six months. Some people with NMO need it more often because their immune cells regenerate so quickly.

                The dosing is based on a person's weight, so it isn't possible to know how much of the medicine will be needed until the dose is calculated for the person. That may or may not affect how the drug is priced for the patient. It sounds like you may have to wait to figure out the cost until your mom finds a neurologist who will actually prescribe it.

                For now, it sounds like things are starting to go in the right direction for your mom.

                Comment


                  #9
                  Originally posted by Sasha84
                  I suspect studies must be conducted here in order for them to be recognised and until then it is impossible to get doctor to treat you officially with a medicine that wasnt officially approved for the task. (just a guess)
                  I think you're probably right about that. The pharmaceutical system works similarly here in the US, although it may be a little different.

                  In the US, a drug first has to be approved by our Food and Drug Administration (FDA), based on the results of studies and a risk management program, before it can be sold commercially. And when a drug is approved, it's approved for a specific purpose. (More purposes can be approved later if the drug makes wants to conduct the necessary studies.) So it sounds like that part's the same in Poland and in most other countries.

                  But once a drug has been approved by the FDA, doctors can prescribe the drug for any reasonable purpose. The uses aren't restricted only to the approved uses, and those are called "off-label" uses. In the US, Rituxan is approved to treat lymphoma, leukemia, and rheumatoid arthritis (the drug manufacturer applied for all of those approvals.) It's also prescribed off-label for NMO and MS, among other things. It sounds like Poland doesn't allow off-label prescribing, and that's why Rituxan and CellCept can't be used for NMO.

                  However, even though doctors in the US can prescribe drugs off-label, medical insurance companies aren't required to pay for drugs used off-label (although some do). So that sounds like it might also work that way in Poland.

                  As far as leg cramps, it's hard to say what's causing your mom's. Once a spinal cord has been damaged (from any cause), spasms can just be a leftover. I still have really painful leg cramps 15 years after having a large transverse spinal cord lesion. The cramps don't necessarily mean that there's more damage occurring. They can just be the result of nerves misfiring due to damage that's already occurred.

                  I'm not aware of any way to tell if they're part of the healing process. So your mom should probably consult with her neurologist, who will have a better idea of what might be happening in her particular case. Maybe the cramps will slow down when she gets into rehab. That would sure be nice.

                  Comment


                    #10
                    Hi Sasha:

                    I'm so sorry to read that your mom has had another attack.

                    Originally posted by Sasha84
                    How NMO relapse/attacks are diagnosed? Is it some reliable urine and blood tests, interview with a patient?
                    Attacks are diagnosed mostly by spinal cord MRI, and then by signs -- what the doctor observes in the physical exam of the person's motor and sensory function -- and symptoms -- what the person is feeling. Blood and urine tests don't tell anything useful about the attack itself, but they can give clues about other things that may be going wrong as a result of the attack.

                    Originally posted by Sasha84
                    or just pump patient full of steroids whenever there is suspicion of another attack?
                    That's it in a nutshell. Steroids are the only treatment for an acute relapse, even when people are on a maintenance treatment medication like Imuran or Rituxan. Steroids can be started even before the MRI is done if the suspicion and evidence are strong enough. Otherwise, steroids should be started as soon as possible after the MRI and physical exam.

                    After that, the cycle repeats itself, hopefully not very often. I hope your mom's neurologist will make her a priority now, and she can get treated quickly and into rehab.

                    Comment


                      #11
                      Hi Sasha:

                      I'm glad to hear that your mom's neurologist has been to see her. I appreciate why the slowness of her MRI is frustrating you. In the US, a person who has already had an episode of transverse myelitis and is positive for aquaporin antibodies would be tended to much more quickly at an "expert" hospital. But we know that the medical system in Poland appears to not be quite up to speed on NMO.

                      It isn't so much the cramps that would make me suspect another attack, because cramps can occur in the absence of inflammation. It's the burning that would make me suspicious, because that would be the recurrence of a sensory symptom. Your mother already knows what an attack feels like, so burning might be a important symptom she should pay attention to. The MRI may look better, but symptoms are also important clues. As long as things don't get worse, she'll have to rely on the doctor's judgement. If she starts to feel worse and lose some function, then she needs to speak up LOUDLY to her neurologist to have him pay attention and re-evaluate.

                      I'm also glad that you found a place to vent on an MS forum. But I can't say strongly enough that an MS forum is the WRONG place for people to discuss NMO. The truth is that you got very lucky that there happened to be a couple of people who post here who know a lot about NMO. If you look back at other posts just here at MSWorld (not counting other MS forums) about NMO, they're rampant with misinformation and people basically just making things up and speculating and misleading each other to wrong conclusions.

                      People with MS trying to advise a person about NMO based only on their knowledge -- and often misinformation -- about their own MS symptoms and treatments is just WRONG because they're so very different. That can be discouraging and harmful for someone with NMO or someone caring for one.

                      So for your mom, if she wants to get into an online forum or a Facebook group, she should make every effort to find an actual NMO group, whether it's one in Poland or internationally. The Guthy-Jackson foundation has a page of support groups at https://guthyjacksonfoundation.org/support-groups/. Maybe there's one or two groups that might be a good fit for your mom and even for you.

                      For general emotional support, of course you should post in any forum that is beneficial to you. But if the need for support also involves the need to understand the disease, then an NMO forum is more advisable. Your mom and sister may likely get better information and support on an NMO forum, but I hope that you will continue to post here as long as you would like to.

                      Comment


                        #12
                        Hi Sasha:

                        You and your family are in shock, and youíll have to go through all of the stages of grief. There really arenít any shortcuts through it.

                        But after a while youíll be able to center yourselves and regain some perspective. Hereís some perspective for you to consider. This is something I learned from years of living with health challenges of my own as well as helping loved ones through theirs.

                        The laws of mathematics and physics apply equally to everyone. But humans, as wonderful as we are, like to gum up the system with existentialism, emotions and value judgements that nature doesnít intend or imply anywhere in the universe. Why? Why me? This isnít my fault, so someone with evil intentions must be doing this to me! And all we do is make ourselves miserable.

                        Because there are no judgements in the universe, there are no evil forces doing anything to us. Those beliefs are instilled in us very early by societies and religions, which want people to be afraid of things. Because when people are afraid, theyíre easy to control.

                        There are no evil forces any more than you can walk into a room and turn on a dark switch instead of a light switch. If anything, the order of the universe is well-being. The earth rotates and revolves around the sun. The sun rises and sets every day. Water moves around the planet. Plants continue to grow and babies of all kinds keep being born. As the saying goes, your cork will float as soon as you stop holding it under the water.

                        If thereís a higher power thatís perfect and loving and the model weíre trying to aspire to, then itís completely impossible for that loving being to also be punitive or vengeful. Thereís nothing to fear from that higher power. If that higher power were to be arbitrary or vengeful, then itís no better than humans and, therefore, not really a higher power. Oh, the stories humans make up. As long as weíre making up stories, wouldnít it be better for us to make up stories that are happy and empowering?

                        Since nature doesnít make judgements, all of the interpretations come from us. We have the choice to judge events harshly, look for the worst and fight against things we donít like. Blaming someone/something -- or everyone -- is an acceptable coping mechanism while working through shock and grief, but after that it's a poisonous way to live. Or we can choose to reserve much of our judgement, look for the best, and stop pushing against things (which tend to push back).

                        There are people who have had very difficult things happen to them who become angry and bitter, and other people in exactly the same circumstances who remember that all of the beautiful things that existed before are still here to appreciate, and they focus on the good things, find their happiness again and carry on.

                        My sincere wish for your mom, your sister and you is that, after a while when things stabilize and the shock wears off, youíre all able to get centered again and find some perspective. Because itís out of that perspective that youíll be able to find comfort and strength that you may not even realize you already have.

                        Comment


                          #13
                          Hello again Sasha84,

                          I wanted to let you know that the Guthy-Jackson Charitable Foundation for NMO Spectrum Disorders Research has a Clinical Consortium (Johns Hopkins, UTSW, Mayo Clinic), the members of which will confer with the neurologist of any NMOSD patient free of charge. It might be worth your while for your Mother's neurologist to confer with an expert. If you contact the GJCF they will be more than happy to get you pointed in the right direction. Right now they're likely to be unavailable as they're currently holding their NMOSD roundtable and hosting their annual Patient Day in Los Angeles.

                          Also, the term NMO has been replaced with NMOSD. The diagnostic criteria was recently revisited and revised by an international panel of experts and this term was deemed preferable because it's inclusive of all presentations.

                          Please try to think positive. While it's a difficult disease, it can be managed. I was diagnosed twelve years ago and experienced numerous bad attacks during my first two years, but since starting Rituxan in 2007, I have been neurologically stable for the most part (only two minor blips in the ten years that I have been using Rituxan). Recently, I have been able to extend my infusions to every four months as opposed to every three (I relapsed at 0 values) with the ultimate goal of being infused every six months.

                          Wishing you and yours well.

                          Grace

                          Comment


                            #14
                            Re: Rituxan dosage. It's not based on weight. The recommendation as per Johns Hopkins
                            for NMOSD patients is one full gram followed by another full gram two weeks later. Thereafter, it's one infusion every six months as long as the CD19 stays below .1%. In the event that the cells have started to recover, two infusions (one full gram each) are given, separated by two weeks.

                            If you're unable to get it in Poland, you might consider contacting the manufacturer of Rituxan. Genentech fully covered mine for the first year I used it, after I had been denied by my insurance company. They sent me paperwork for the second year in the event that my insurance denied me again. Fortunately, my insurance decided to cover it and has done so since then.

                            Grace

                            Comment


                              #15
                              Re: NMO Diagnostic Code

                              It has had it's own ICD code for some years now. It's currently G36.0 Previously, it was 341.0 for some years. Both codes were/are specific for neuromyelitis optica.

                              Grace

                              Comment

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