Babis

I could not help but notice that, 6 months prior to AAG, I had first developed hypothalamic hypogonadism (testosterone deficiency). I always thought this was no coincidence. I also noticed that when I received testosterone therapy, my symptoms of AAG got better too. I have been taking a university course in immunology, and I think I finally know why! http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2200579/pdf/cei0106-0410.pdf "It is thus suggested that reduced levels of testosterone, together with an abnormal level of oestrogen, may trigger the onset or exacerbate the course of human autoimune diseases" Some neurologists had suggested cortisone for AAG, but endocrinologists warned against it as it would worsen hypogonadism, so I avoided it. That was a good thing, as the above article suggests that (male) patients with hypogonadism and autoimmune disease need testosterone, not cortisone, to moderately supress their immune system. If you have endocrine problems, get tested and treated by a competent endocrinologist (I had to see 10 to find a good one), as it may help your AAG.

I got horrible headaches from Privigen (it has the highest headache incidence among all brands), but none from Octagram and Gamunex. And Gamunex seemed more effective, despite my neurologist thinking the contrary. Have you considered switching IVIg brands?

Hi, looneymom. I have not taken Rituximab yet. I have to apply for funding and it will take some time. And the side effects can be serious (cancer, JC Virus, etc). My neurologist suggested it is more dangerous than IVIg and PLEX, but less than Methotrexate, Cellcept and Cytoxan. I want to try safer alternatives first. One thing I intend to try is LDN (Low Dose Naltrexone), which supposedly helps with autoimmune disease. I am suspicious about the claims but I do not have much to lose by trying LDN before toxic alternatives. I just got a prescription and will start in a few weeks, but do not expect results in less than a few months.

Note that there is a huge difference in effects and side effects between different brands of IVIg. I had very different responses to Privigen, Octagram and Gamunex. diabeticgonewild My neurologist recommended against Plasma Exchange, as it depletes blood volume and causes hemodynamic instability and arrythmias, something that people with dysautonomia cannot tolerate. So I'm surprised you were under Plasma Exchange, I'm not surprised you didn't torelate it. I'm also surprised you are receiving such a cytotoxic drug like Cytoxan. Do you have cancer? Cytoxan kills many kinds of cells and can cause infertility, among others. Ironically, although it is used for chemotherapy, it is itself carcinogenic. Why not opt for a more specific drug that targets only B cells, such as Rituximab? This is what my neurologist recommended as the 'least evil' immunosupressant (it is also mildly toxic and carcinogenic, no immunosupressant isn't, but much less than cytoxan). There are a few articles out there documenting its efficacy for AAG, and you only need one cycle every six months. Ideally, one would want an even more specific drug, that targets only the B cells that produce ganglionic antibody, but science is not there yet.

Well, it's been two years since I started IVIg (three years that I have had AAG), and I am afraid that, for me too, IVIg is not as effective anymore as it was initially. I receive IVIg every 8 weeks. It is still somewhat effective but, compared to the beginning, it takes longer (2 weeks) to feel the positive effects, and stops working earlier, 3-4 weeks before the next cycle. That leaves me with only 2-3 weeks that I feel "good" between cycles, and it is only half as "good" as I felt a year ago. So, overall, I have started to get worse, or the IVIg is not as effective anymore. Moreover, I have now moved to the UK, and it is quite difficult to get IVIg here. I may have to seek alternative treatments, but immunosuppressants are highly carcinogenic, so I do not want to go there. The neurologist I saw in Greece warned me that Plasma exchange can have nasty autonomic side effects. I am afraid and not sure what to do... I have done some research, reading many pubmed articles, regading substances that help with autoimmunity in general, without causing cancer. I also looked at myasthenia gravis in particular (its AChR antibodies are in some sense similar to those of AAG, though they target the neuro-muscular junction, rather than the neuron-neuron junction). It seems that vitamin D3, vitamin K2, and an antioxidant/mitochondrial cocktail (ubiquionol, alpha lipoic acid, vitamin B complex, vitamin E, glycocarnitine, selenium), can help reduce inflammation and autoimmunity in various ways (e.g. inhibiting interleukin-5, etc), if dosed properly. This cocktail has indeed helped me, and I get worse a week or two after I stop it. It is not a cure, but at least it does not cause cancer. The literature said that AAG usually lasts 2-5 years, with slow but incomplete recovery, but it seems many people have it longer than that. I just feel exchausted and want to find a way to put AAG into remission... Has anyone else taken immunosuppresants for AAG, and for how long? Did you have toxicity side effects, or develop cancer?

Hanna, Could you update us on what happened? Did Sulfalazine work? Did you get pregnant? Thanks, Babis

Hi Hanna, Is there any chance you could get IVIg? To me it seems the ideal treatment for AAG, since you do not become immunosupressed and, in your case, I think there is no risk to the fetus. It was difficult for me to get IVIg covered in the US, but it has been much easier to get it covered in Germany (and other coutries with a social healthcare system).

Hi Arizona Girl, My doctors give me a round of IVIg every 6-8 weeks. In each round, I receive 30 gr / day for 5 days. I was told that is reasonable, since the half life of IVIg is about 3-4 weeks. This regimen was advised by European University doctors (Greece and Germany). It is very similar to that used for Myasthenia Gravis. I am now told that the dose will be reduced to 3 days (30 gr / day) every 8 weeks (probably to save money). I am a little scared this might be insufficient, but we will see. The protocol suggested by Mayo was a little different (5 days of IVIg initially, then once a week for 5 weeks, then stop IVIg and start immunosuppressants). The sides of immunosupressants are steep, so I have managed to avoid that so far, sticking to the (much more expensive) IVIg. The Mayo neuroimmunologist (Dr Andrew McKeon) said the European protocol is also reasonable. The symptoms that I had and improved dramatically with immunotherapy were disabling & numerous. They included sympomts of dysautonomia (dizziness, faintness, POTS, crashing fatigue, heat intolerance, GI dysmotility, irritable bowel, aerophagia etc etc). They also included symptoms of encephalitis (sensory integration dysfunction; severe vision problems such as "frame-skipping" or jittery vision, inability to process moving or bright or complicated patterns, palinopsia; sensitivity to sound; brain 'numbness' or extremely uncomfortable "cotton brain" sensations, etc etc). My doctors did not take these symptoms seriously and for a year thought they were migrainous or psychological. I kept insisting it was not migraine nor psychological but nobody listened. Even Dr McKeon at Mayo thought these symptoms were migrainous initially. But then the Mayo radiologist did an excellent software analysis of my brain PET scan, which showed hypometabolism in the visual cortex and elsewhere. Then, given the AChR antobody positivity, Dr McKeon immediately suspected autoimmunity could cause the visual problems (in addition to dysautonomia), and suggested trying IVIg. This worked, which proves that his second hypothesis was right, finally...

Just wanted to note that I had a further 25% imrpovement one week after my third round of IVIg. I am now 75% recovered :-) I hope this continues after the next round of immunotherapy. It looks like I will be receiving 5 days of IVIg every 6 weeks, for at least a year.... From my experience, and my delayed diagnosis (which happened only after I insisted for the autoimmuned dysautonomia evaluation), I think AAG is massively underdiagnosed, and many people suffer unnecessarily.... Many autonomic labolatories, such as Froedert Hospital, are sometimes able to tell that someone has POTS, but do little to discover the underlying cause. So they give people symptomatic treatments, instead of the therapy that could actually cure them. I am lucky that I ended up in Mayo clinic neuroimmunology, and evel luckier to be in Europe now for treatment, where doctors are much more willing to offer IVIg, giving me my life back...

Steven Vernino, MD, University of Texas at Dallas, neuroimmunologist, worked at Mayo clinic, specializes in autoimmune dysautonomia.

Sue, the autoimmune dysautonomia evaluation detects roughly 50% of cases of autoimmune dysautonomia. Than means there is another 50% of cases in which the antibodies are not known, and yet may still respond to immunotherapy. So, if you had subacute onset and there is suspicion of autoimmunity, a couple of months of IVIg may be worthwhile to try, even if you are antibody negative... Since this conclusion comes from articles written by Steven Vernino himself, you could ask him if it might be worth trying IVIg even if you are antibodies come back negative negative. You can also have a look at this . Autoimmune dementia shares many similarities with autoimmune dysautonomia and autoimmune encephalitis (they all have subacute onset, they are commonly missed treatable conditions, they are immunotherapy responsive, etc).

Hey Sue, If the neurologist you refer to is Steven Vernino, he seems an excellent choice. He has published many articles on AAG and is among the leaders in the field. He was in the team that discovered the ganglionic acetylcholine receptor autoantibodies, and designed the essay used to detect and measure them at Mayo. And yes, most doctors I saw shrug their shoulders or offered my salt and water or symptomatic treatments; nobody was willing to test for antibodies; I had to see tens of doctors and beg them for testing me for a whole year, before somebody agreed to order the test. Even after it came back positive, they thought little of it and were unwilling to offer immunotherapy. Extremely frustrating. They said many things that contradicted the literature and common sense, just to avoid the responsibility of immunotherapy. I explained to them I had severe visual symptoms and many things that my brain could no longer do, and they told me I was stressed and depressed, even though they knew I had dysautonomia and ganglionic antibodies. Luckily, I trusted myself and the literature more than these doctors. With the positive result in my hands, I thought I had wasted enough time trying to educate them, and decided to go to someone who is educated already. So I e-mailed one of the neuroimmunologists at Mayo involved in publications on these antibodies. He was kind enough to offer me an appointment in 2 days (as opposed to 3 months I had to wait to see the incompetent doctors at Froedert Hospital). Once I got to Mayo, I asked if immunotherapy could help, and he said yes, and offered to do a month of IVIg right there and then. Since I was scheduled to move to Europe, it could not be done, so I asked him to write his regimen in detail in his summary letter, which he happily did. He also sent me a revised interpretation of my brain PET scan (which had been misinterpreted as normal at Froedert Hospital) and was strongly suggestive of autoimmune encephalitis, mainly in the visual cortex. This is seen in a small percentage of people with AAG. After moving to Europe, I showed my Mayo records to the neuros at the hospital, and luckily they agreed to follow the Mayo suggestions for immunotherapy and even improve them further. My dramatic response after 2 months of IVIg shows that both the encephalitis and the dysautonomia were indeed autoimmune. I now have my life back, thanks to Mayo neuroimmunology, and thanks to trusting myself and the literature, instead of some illiterate "doctors". My advice: do not settle for salt & water or symptomatic therapies. These are useful but not enough. Seek an accurate diagnosis of the underlying causes, and therapies that treat those causes, not the symptoms. From my experience (saw over 50 doctors) 90% of doctors do not really know what dysautonomia is, 95% of them cannot properly diagnose it, and 99% of them cannot properly treat it. So your best bet is to go to pubmed, seek doctors with extensive publications on the subject, and pay them a visit. This has worked remarkably well for me, both in terms of diagnosis and treatment.

I received 5 days of IVIg (Privigen), and had no improvement for the first 3 weeks, but I saw a mild (25%) improvement the 4th week. Then I received 5 more days of IVIg (Gamunex), and felt nothing for a week, but I had further 25% improvement after the 2nd week. My symptoms of dysautonomia and encephalitis (severe very strange vision problems, confirmed in a PET scan as hypometabolism in the visual cortex) both improved, which proves that both problems were autoimmune. I am very happy about this response, I hope I continue to improve. I am scheduled for another round of IVIg in 6 weeks.

Hey, Erika: How many rounds of IVIg did you receive? How many days was each round? And how long after the symptom onset was your IVIg treatment? I ask because many rounds of IVIg may be needed to see a result, and because shorter delay for immunotherapy leads to better results. I also have the neuronal ganglionic alpha-3 AChR autoantibody. I received 5 days of IVIg (Privigen), and had no improvement for the first 3 weeks, but I saw a mild (25%-50%) improvement the 4th week. Then I received 5 more days of IVIg (Gamunex), and felt nothing for a week, but I had significant improvement (50-75%) the 2nd week. If I add physical therapy for POTS and further rounds of IVIg that are scheduled, I think I may fully recover... The literature suggests that if a single round of IVIg has no result, then many rounds of IVIg, or combined immunotherapy (IVIg, Plasma Exchange, Immunosupressants) plus physical therapy can lead to a response.

Have you had an electromyogram (EMG)? It is important in order to exclude ALS, muscular dystrophy, etc. Experts (hard to find in the US) can also do a special form of EMG to rule out mitochondrial disorder. I have muscle loss and fasciculations so I went to a Professor of Neurology with significant expertise in neuromuscular disorders. She performed an EMG and found some abnormalities but was able to determine that their cause is benign and to rule out any of the above serious diseases. It seems that the atrophy is due to hypopituitarism (low testosterone and growth hormone). The fasciculations may be part of AAG. Muscle fasciculations are common in Guillain Barre syndrome, and AAG is a rare variant of Guillain Barre.