• Welcome to Phoenix Rising!

    Created in 2008, Phoenix Rising is the largest and oldest forum dedicated to furthering the understanding of and finding treatments for complex chronic illnesses such as chronic fatigue syndrome (ME/CFS), fibromyalgia (FM), long COVID, postural orthostatic tachycardia syndrome (POTS), mast cell activation syndrome (MCAS), and allied diseases.

    To become a member, simply click the Register button at the top right.

Postural tachycardia syndrome (POTS) with anti-NMDA receptor antibodies after human papillomavirus v

Kati

Patient in training
Messages
5,497
The scary part is if you even suggested that, most often you would be believed to be hysterical, crazy or both. My current health care system guidelines calls for less testing for people like me, not more.
 

Gingergrrl

Senior Member
Messages
16,171
The scary part is if you even suggested that, most often you would be believed to be hysterical, crazy or both. My current health care system guidelines calls for less testing for people like me, not more.

I know that is absolutely true re: your system but I think (hope?) that the paraneoplastic syndromes are an established fact now vs. something like ME/CFS or MCAS where a doctor can just decide that they do not exist and you are crazy. At least the doctors that I saw here (even the one who decided my entire case was "psychosomatic" b/c he did not believe MCAS was real) was adamant that I now need ongoing cancer checks b/c of the antibody that was found.

So, the cancer piece interests the doctors (so they will not get sued) but the Paraneoplastic Syndrome and how it can affect the patient neurologically, autonomic, autoimmune, etc, is getting lost. You can have the PNS with a benign tumor, or with no tumor ever being found, so attacking or lowering the autoantibodies is still the right intervention. In the true story of the girl in the book I read, she would have died without attacking the NMDA autoantibodies.

That's why I feel if mine are weakening my lungs/breathing, who knows how far it could go or when it would plateau? So I am attempting to do the same interventions (IVIG and RTX) and hoping it will help me like the girl in the book b/c they are the only interventions that make sense for me.

Sorry, this was not directed at Kati, I just ended up typing more than I'd planned!
 

Sidereal

Senior Member
Messages
4,856
@Gingergrrl,

I know nothing about this subject other than having read that book, but the book mentions Dr Josep Dalmau at the university of Pennsylvania, which has expanded its research and clinics in autoimmune neurology. Here's a tidbit from his bio on their website:



Here is a page from their website that describes some anti-bodies and associated diseases:

http://www.med.upenn.edu/autoimmuneneurology/classification.html

According to this piece published earlier this month

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4974842/

Dalmau also has an institutional affiliation in Barcelona, Spain. Email address is listed. May or may not be helpful for patients based in Europe who suspect they may have this condition.
 

Gingergrrl

Senior Member
Messages
16,171
I noticed that the University of Pennsylvania's Center for autoimmune neurology Website said that they were finding two new autoantibodies a year. It's nice to see that somebody is out there researching this area.

Agreed and I retract what I said that no one in the US is investigating this. I may contact them at some point and if I do, will report back what I learn, but can't do it yet.
 

Gingergrrl

Senior Member
Messages
16,171
I just looked at the website for that center: http://www.med.upenn.edu/autoimmuneneurology/research.html and it mentions my two autoantibodies (GAD65 and VGCC) in a detailed chart along with many other autoantibodies: http://www.med.upenn.edu/autoimmuneneurology/classification.html.

It describes the types of treatments that they do:

Treatment
Antibody mediated neurologic diseases can improve with treatments that influence the immune system. Each disease is different in terms of how it responds to treatment, and each patient requires a treatment personalized to his individual situation. Some common treatments for these diseases include, IV steroids, IVIg, plasmapheresis, rituximab, cyclophosphimide, and Cellcept. Additionally, some diseases are known to be associated with tumors. Therefore, if a cancerous tumor is found treatment may include surgery, radiation, and/or chemotherapy. For more specific information on treatment, please see the section on associated syndromes.

It also explains how to contact them:

New patients should call 215-662-3606 to register and request an appointment. We require patients to send medical records prior to scheduling, these records should include a cover sheet with contact information, recent neurology clinic notes, MRI/EEG/EMG reports, and antibody test results. They can be sent to Dr. Lancaster via Fax (215-349-5579) or Mail (3400 Spruce Street; 3 W. Gates Building, Neurology; Philadelphia, PA 19104)

I wish they were on the west coast but if I was closer to them or able to fly, I actually would go see them now that I have read more. I wanted to post this in case helpful to anyone else.
 

Sidereal

Senior Member
Messages
4,856
So this POTS patient who had a low titre of NMDA receptor antibodies (and no encephalitis) was also positive for the antibodies recently reported by Scheibenbogen in a subset of ME/CFS patients.

A previously healthy 18-year-old woman developed fatigue, pre-syncope, dizziness and nausea 48 h after vaccination with HPV vaccine, Cervarix. The symptoms were initially mild and were not attributed to vaccination. She subsequently received a second HPV vaccine 6 weeks after the first injection. Her symptoms immediately intensified, and she became bed-bound with pre-syncope, dizziness, nausea, sore throat, difficulty falling asleep, frequent awakenings during the night, visual disturbance, transient episodes of confusion and difficulty concentrating. She was unable to attend school and after seeing numerous specialists was diagnosed with chronic fatigue syndrome.

Autoimmune markers were negative for antinuclear anti- bodies (ANA), anti-SSA and anti-SSB antibodies, anti- TPO antibodies, anti-thyroglobulin antibodies, anti-phos- holipid antibodies and anti-DS DNA antibodies, but posi- tive for low level of anti-NMDAR Ab (performed by Oxford University Hospital Laboratory, UK; normal range: no antibodies detected). Further testing demonstrated pos- itive beta 2 adrenergic and M2 muscarinic receptor anti- bodies (Berlin Cures, Germany). A paraneoplastic panel, including ganglionic AchR antibody, was negative. Serum anti-NMDA Ab were repeated 2 months later and were once again mildly elevated. To rule out a paraneoplastic syndrome with positive anti-NMDA Ab, a pelvic ultra- sound was obtained and did not reveal any ovarian or pelvic masses. Subsequently, a full body FDG-PET scan was performed and was also unremarkable. CSF analysis was negative for anti-NMDA Ab and other neuronal anti- bodies and showed normal cell count, glucose and protein. The patient received 5 treatments with plasmapheresis followed by a daily dose of prednisone, and her symptoms improved significantly for about 7 weeks after the treat- ment. When plasmapheresis was stopped and prednisone was tapered, the symptoms recurred. Following plasma- pheresis and prednisone treatment, a repeat serum anti- NMDAR Ab were negative.

Strange paper entirely focusing on the NMDA antibody which seems unrelated to her symptoms instead of the adrenergic and muscarinic antibodies which could plausibly account for her POTS symptoms.
 

Gingergrrl

Senior Member
Messages
16,171
So this POTS patient who had a low titre of NMDA receptor antibodies (and no encephalitis) was also positive for the antibodies recently reported by Scheibenbogen in a subset of ME/CFS patients. Strange paper entirely focusing on the NMDA antibody which seems unrelated to her symptoms instead of the adrenergic and muscarinic antibodies which could plausibly account for her POTS symptoms.

I agree it is strange that they focused on the NMDA antibodies but maybe it is b/c they view them as more serious than the two that (as of now) can only be tested in Germany and are less well-known world-wide? We were discussing this in another thread but I am attempting to obtain the info from Dr. Scheibenbogen's lab to send a blood sample to Germany for these tests (am still waiting for some additional info to give to my doctor before I can proceed).

In any case, if the above paper had realized that the adrenergic & muscarinic Abs were important, do you think they would have changed their treatment to something like IVIG and RTX (vs. plasmapheresis and Prednisone)? My very limited understanding is that plasmapheresis can get rid of the antibodies very quickly but they always return and is never a cure. In a life-threatening case, this is often necessary but then some additional treatment like IVIG or RTX is still done later.

Do you think in the case of this girl if she had received IVIG and RTX (or a similar immuno-therapy) that her symptoms might not have returned in seven weeks? I was initially very disappointed that no US doctor would allow me to try plasmapheresis but after more research it seems like the benefits are very short-lived vs. something like IVIG the benefits could be permanent or RTX they could last six months (vs. seven weeks) or in theory could also be permanent? My IVIG is moving at a snail's pace but maybe this is better in the long-run than plasmapheresis? I really do not know but it seems that IVIG changes the immune system and antibodies vs. PP just eliminates them very fast and then they come back?

I'd love to hear any thoughts on this (@Sidereal or anyone) and these autoantibodies just seem to be really crucial for a huge group of people. I wish there were more than a handful of researchers worldwide studying them.
 

Sidereal

Senior Member
Messages
4,856
In any case, if the above paper had realized that the adrenergic & muscarinic Abs were important, do you think they would have changed their treatment to something like IVIG and RTX (vs. plasmapheresis and Prednisone)? My very limited understanding is that plasmapheresis can get rid of the antibodies very quickly but they always return and is never a cure. In a life-threatening case, this is often necessary but then some additional treatment like IVIG or RTX is still done later.

I wonder if the decision to use only steroids after the plasmapheresis was more financial/insurance-related vs. clinical? After all, prednisone costs peanuts whereas IVIG and RTX cost an enormous amount.
 

Gingergrrl

Senior Member
Messages
16,171
I wonder if the decision to use only steroids after the plasmapheresis was more financial/insurance-related vs. clinical? After all, prednisone costs peanuts whereas IVIG and RTX cost an enormous amount.

Yes, and if the girl was in the US (I think she was in the UK b/c they mentioned Oxford) but if in the US, then absolutely her insurance would go for the cheapest route possible if she or her doctor were not advocating for other treatments. There is a program to get RTX for free through Genentech but you still pay for the infusion costs which can be thousands of dollars.

If cost/insurance were not the issue, do you think given her adrenergic and muscarinic Abs, that IVIG and RTX would have been the better treatment clinically? I know each case is unique but this is what I am leaning toward. Prednisone and all these steroids have a temporary benefit but am not sure if they can shift the immune system or attack the Abs like IVIG and RTX can. I know it is all a gamble but some treatments just seem more solid to me than others.
 

Sidereal

Senior Member
Messages
4,856
It's been a couple of years since I read Brain on Fire, the memoir of the woman who had anti-NMDAR encephalitis, but as far as I recall huge IV doses of prednisone didn't help and she had to get IVIG. Unfortunately I've forgotten the specifics but recently my other half came down with a sudden-onset post-viral mysterious neurological syndrome and gigantic doses of IV prednisone did nothing but make him severely agitated, diabetic and full of candida.
 

Gingergrrl

Senior Member
Messages
16,171
It's been a couple of years since I read Brain on Fire, the memoir of the woman who had anti-NMDAR encephalitis, but as far as I recall huge IV doses of prednisone didn't help and she had to get IVIG. Unfortunately I've forgotten the specifics but recently my other half came down with a sudden-onset post-viral mysterious neurological syndrome and gigantic doses of IV prednisone did nothing but make him severely agitated, diabetic and full of candida.

I have not read "Brain on Fire" but recently read "The girl on the 6th Floor" and in her case they tried everything and it was not until Mayo diagnosed her with anti NMDA encephalitis and put her on IVIG and then RTX that she made a solid recovery. Am sorry about your other half, that is awful. I have never had Prednisone and am getting Solu-Cortef as pre-med for my IVIG and so far have not had any problems from it. Hoping he is better now?
 

Sidereal

Senior Member
Messages
4,856
If cost/insurance were not the issue, do you think given her adrenergic and muscarinic Abs, that IVIG and RTX would have been the better treatment clinically?

I think at this point in time no one could responsibly answer that question because these antibodies are only beginning to be researched. All we have to go by at the moment is that one paper I gave you months ago published by Germans and Norwegians. In that study a subset of patients were treated with RTX and the antibody levels did decline in some of them. I'm aware of anecdotal reports on dysautonomia forums/groups of IVIG being useful for some people but I don't know if those people had these antibodies. There's no established protocol for testing or treatment of POTS out there. It seems to be extremely hit-and-miss, ranging from being left to rot in bed for the rest of your life with no testing or treatment to million dollar workups for these autoimmune neurological channelopathies. Personally, I had very severe POTS when I was diagnosed and I was offered zero testing or treatment, just told to exercise.
 

Sidereal

Senior Member
Messages
4,856
I have not read "Brain on Fire" but recently read "The girl on the 6th Floor" and in her case they tried everything and it was not until Mayo diagnosed her with anti NMDA encephalitis and put her on IVIG and then RTX that she made a solid recovery. Am sorry about your other half, that is awful. I have never had Prednisone and am getting Solu-Cortef as pre-med for my IVIG and so far have not had any problems from it. Hoping he is better now?

Been meaning to read that book. It's encouraging to see that even a severe, often fatal, disease like anti-NMDA encephalitis can resolve fully with time and the right treatment. It just goes to show you what is possible when doctors actually try to help instead of dismissing the patient as crazy.

It's been eight weeks since onset. He was no better with any of their treatments (they had tried IV acyclovir, abx and prednisone) until I started him on a couple of Japanese meds I bought online. He's objectively much better now but not yet able to return to work. Of course, I am not encouraging anyone to self-treat, I'm just reporting what I did when the alleged "top neurologist" turned out to be disinterested and incompetent, as they usually do. I also caught him lying about some autoimmune testing he claimed had been done and turned out not to have been.
 

Gingergrrl

Senior Member
Messages
16,171
Been meaning to read that book. It's encouraging to see that even a severe, often fatal, disease like anti-NMDA encephalitis can resolve fully with time and the right treatment. It just goes to show you what is possible when doctors actually try to help instead of dismissing the patient as crazy.

It's a great book, written by the girl's father and documents the entire experience from start to finish and she had amazing doctors who did not stop looking until they figured it out. It was at a small hospital who consulted with Mayo who figured it out. Her initial symptom was seizures vs. psychosis which also worked in her favor b/c she was put on Neuro unit vs. psych from the outset.

What was intriguing to me is that is seems no matter which autoantibody someone has, the core treatments (at this time in science) are the same. I have a friend (not on PR) who has a known antibody that affects the brain (not NMDA) and now dx'd with a PNS and his tx's are steroid, then IVIG, then RTX (and we are not in the same country and do not have the same antibody). So it makes me feel that I am on the right track.

He's objectively much better now but not yet able to return to work.

That is good news and hoping he will make a full recovery. Too bad he could not get the autoimmune or antibody testing.