I just tested positive for AChR Ganglionic Antibodies!

crypt0cu1t

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I just had a positive result of Ganglionic AChR Antibodies earlier today!

That now makes 3 positive antibodies that I have;
AChR Binding Antibodies, AChR Ganglionic Antibodies & Salivary Protein 1.

Does anyone know if AChR Ganglionic Antibodies can be correlated with autoimmune encephalitis? I'm hoping this may explain some things.
 

kangaSue

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Which Lab did the testing? A leading expert in this field (Autoimmune Autonomic Ganglionopathy (AAG), as caused by AChR ganglionic antibodies), Dr Steven Vernino (who has his own lab facility at UT Southwestern) has stated in a webchat in the past that some labs do not do the test well.
http://www.dysautonomiainternational.org/pdf/AAG_Webchat.pdf

An association between AChR ganglionic antibodies and autoimmune encephalitis is rarely reported but it has been known to be the case. Paraneoplastic syndromes are another to be aware of with AChR ganglionic antibodies
https://n.neurology.org/content/90/15_Supplement/P4.188
 

crypt0cu1t

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So have you looked much into Autoimmune autonomic ganglionopathy (AAG) yet?
That's actually the diagnosis that got me IVIG & Plasmapheresis. It was considered seronegative but surprisingly the antibody just showed up.

I don't have many dysautonomia symptoms, more encephalopathy symptoms, but I do have dry eyes, dry mouth, fatigue and occasional tachycardia.

I have been researching this antibody a lot and it seems this DOES cause encephalopathy in some cases
 

sometexan84

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Did you go through the whole Dr Vernino Q&A in the link @kangaSue sent?

I was 100% convinced I had the Ganglionopathy, but at the time I didn't realize sensory symptoms (numbness, cold feet, etc) were not a part of Ganglionopathy. I have some sensory, but main autonomic symptoms.

Tested positive for the TS-HDS antibodies. So, yours is attacking ganglia, where mine is attacking the fibers. But both could be causing damage in very similar areas, leading to similar symptoms.

What are the encephalopathy symptoms?
 

sometexan84

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Have you tested all the following?

Anti-IgLON5
GAD-65
- glutamic acid decarboxylase (islet cells)
AMPAR2 (Anti-Glutamate Receptor 2) – (Hashimoto's encephalitis)
NMDAR
Caspr2
Myelin Oligodendrocyte Glycoprotein (MOG)
Myelin basic protein (MBP) IgG
Myelin proteolipid protein (PLP or lipophilin) IgG
Myelin-associated oligodendrocytic basic protein (MOBP) IgG
Crystallin Alpha-B (CRYAB) IgG


That's all the Encephalitis-related antibodies I've documented. But it's for sure a partial list only, since I've not researched many Encephalitis related diseases/conditions.
 

kangaSue

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A 2009 Mayo AAG paper suggests that encephalopathy is more commonly found in some of those with higher titres of ganglioninic AcHR antibody ;
[Of 85 patients with medium antibody values (0.10–0.99 nmol/L), neurological presentations were more diverse and included peripheral neuropathies (36%), dysautonomia (20%, usually limited), and encephalopathy (13%). Of 58 patients with low antibody values (0.03–0.09 nmol/L), 54% had a nonautoimmune neurological disorder or no neurological disorder.]
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3764484/
 

Gingergrrl

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I just had a positive result of Ganglionic AChR Antibodies earlier today!
That's pretty rare! What were your Ganglionic levels?
So have you looked much into Autoimmune autonomic ganglionopathy (AAG) yet?
I have a close friend who I originally met here on PR in 2014 (who has not posted here in about 4 yrs) who tested positive for Ganglionic AChR Antibodies and was given a diagnosis of Autoimmune Autonomic Ganglionopathy (AAG) among several other diagnoses. I can ask her more about this if it would be helpful for either of you. She and I both got tested w/the same Mayo Panels by Stanford in 2016 but each got different results (I tested positive for N-type CA+ Channel Autoantibody and Anti-GAD65 and she tested positive for Ganglionic AChR Antibodies).

Have you tested all the following?
Anti-IgLON5
GAD-65
- glutamic acid decarboxylase (islet cells)
AMPAR2 (Anti-Glutamate Receptor 2) – (Hashimoto's encephalitis)
NMDAR
Caspr2
Myelin Oligodendrocyte Glycoprotein (MOG)
Myelin basic protein (MBP) IgG
Myelin proteolipid protein (PLP or lipophilin) IgG
Myelin-associated oligodendrocytic basic protein (MOBP) IgG
Crystallin Alpha-B (CRYAB) IgG

That's all the Encephalitis-related antibodies I've documented. But it's for sure a partial list only, since I've not researched many Encephalitis related diseases/conditions.
I know this was addressed to @crypt0cu1t but I was surprised to see anti-GAD65 linked to AE and I hadn't seen that before! Anti-GAD65 seems to link to so many different things. I tested positive for GAD65 at a high level on Mayo Panels in 2016 and then a few years later my level was significantly lower (but still positive) after IVIG & Rituximab. We measured it again out of curiosity to see if it was lower but it was never the focus of my treatment and it was the autoantibody that I was never certain what it linked to (in my case).

Anti-GAD65 most commonly links to diabetes and SPS (Stiff Person Syndrome) but I never had either of those. I later found info that Anti-GAD65 also links with Dysautonomia & Hashimoto's (and I had both of those) and we assumed that was the link for me. I also had an extremely exaggerated startle reflex (which links to higher glutamate and lower GABA which is the exact mechanism of Anti-GAD65 in that it attacks the enzyme that converts glutamate to GABA). The IVIG completely eliminated my exaggerated startle reflex although this again was an extra bonus and not the focus of my treatment.

On a side note, my doctor decided that it was not worth it for me to continue to test for additional autoantibodies after we found that I had 11 different ones (in 2016). He said that it was interesting on an academic level because I had the most severe/clear-cut autoimmunity of all of his patients, but that it wouldn't change my treatment plan (which was high dose IVIG & then Rituximab) no matter how many additional autoantibodies we found.

I always wondered if I had (or still have?) additional autoantibodies but was not sure if having that info would make any difference, especially since my treatment led to remission of all of my core symptoms. And (so far), I have maintained the remission for over one year after stopping Rituximab. One thing we were curious about (in light of other problems not related to my core symptoms) is if I have autoantibodies against the pituitary which affect my ability to create ACTH. But there is not a commercial test for this plus it is too challenging to pursue during COVID.

I am similar to @crypt0cu1t (although we each have different diagnoses & symptoms) in the sense that I had the symptoms of LEMS (but never tested positive for the most common LEMS autoantibody which is the P/Q Calcium Channel Ab and instead tested positive repeatedly for the N-type). And @crypt0cu1t (if I understand correctly), you are negative for anti-NMDR but positive for other Auto-Abs that link to AE? I think there are just people who are atypical cases of everything even if many neurologists cannot understand this.

Edited to Add: One area where testing for the specific auto-antibodies is crucial is with the paraneoplastic autoantibodies that link with cancer b/c then you can have the corresponding tests to rule out cancer as the cause (versus autoimmunity as the cause).
 
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sometexan84

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I was surprised to see anti-GAD65 linked to AE
Actually, I think I only put that because I had a note that GAD-65 Ab was linked to "limbic encephalitis". In addition to cerebellar ataxia and diabetes.

Anti-GAD65 most commonly links to diabetes
I'm negative for GAD65. But since testing positive for TS-HDS Ab, I've noticed that diabetes is very much linked to autoimmune and autonomic neuropathies. In fact, most of my research today was regarding the link between diabetes, prediabetes, and impaired glucose metabolism with Small Fiber Neuropathy and Autoimmune Neuropathy.

Apparently, there's even a thing called Diabetic autonomic neuropathy

Dysautonomia & Hashimoto's (and I had both of those) and we assumed that was the link for me. I also had an extremely exaggerated startle reflex
Me too on those 3

Did you ever find out the cause(s) of all the antibodies?
 

crypt0cu1t

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Have you tested all the following?

Anti-IgLON5
GAD-65
- glutamic acid decarboxylase (islet cells)
AMPAR2 (Anti-Glutamate Receptor 2) – (Hashimoto's encephalitis)
NMDAR
Caspr2
Myelin Oligodendrocyte Glycoprotein (MOG)
Myelin basic protein (MBP) IgG
Myelin proteolipid protein (PLP or lipophilin) IgG
Myelin-associated oligodendrocytic basic protein (MOBP) IgG
Crystallin Alpha-B (CRYAB) IgG


That's all the Encephalitis-related antibodies I've documented. But it's for sure a partial list only, since I've not researched many Encephalitis related diseases/conditions.
Yes, I've tested for all of them. The only antibodies sk far that I've come across are AChR Binding, AChR Ganglionic & Salivary Protein 1.
 

crypt0cu1t

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I have a close friend who I originally met here on PR in 2014 (who has not posted here in about 4 yrs) who tested positive for Ganglionic AChR Antibodies and was given a diagnosis of Autoimmune Autonomic Ganglionopathy (AAG) among several other diagnoses. I can ask her more about this if it would be helpful for either of you. She and I both got tested w/the same Mayo Panels by Stanford in 2016 but each got different results (I tested positive for N-type CA+ Channel Autoantibody and Anti-GAD65 and she tested positive for Ganglionic AChR Antibodies).



I know this was addressed to @crypt0cu1t but I was surprised to see anti-GAD65 linked to AE and I hadn't seen that before! Anti-GAD65 seems to link to so many different things. I tested positive for GAD65 at a high level on Mayo Panels in 2016 and then a few years later my level was significantly lower (but still positive) after IVIG & Rituximab. We measured it again out of curiosity to see if it was lower but it was never the focus of my treatment and it was the autoantibody that I was never certain what it linked to (in my case).

Anti-GAD65 most commonly links to diabetes and SPS (Stiff Person Syndrome) but I never had either of those. I later found info that Anti-GAD65 also links with Dysautonomia & Hashimoto's (and I had both of those) and we assumed that was the link for me. I also had an extremely exaggerated startle reflex (which links to higher glutamate and lower GABA which is the exact mechanism of Anti-GAD65 in that it attacks the enzyme that converts glutamate to GABA). The IVIG completely eliminated my exaggerated startle reflex although this again was an extra bonus and not the focus of my treatment.

On a side note, my doctor decided that it was not worth it for me to continue to test for additional autoantibodies after we found that I had 11 different ones (in 2016). He said that it was interesting on an academic level because I had the most severe/clear-cut autoimmunity of all of his patients, but that it wouldn't change my treatment plan (which was high dose IVIG & then Rituximab) no matter how many additional autoantibodies we found.

I always wondered if I had (or still have?) additional autoantibodies but was not sure if having that info would make any difference, especially since my treatment led to remission of all of my core symptoms. And (so far), I have maintained the remission for over one year after stopping Rituximab. One thing we were curious about (in light of other problems not related to my core symptoms) is if I have autoantibodies against the pituitary which affect my ability to create ACTH. But there is not a commercial test for this plus it is too challenging to pursue during COVID.

I am similar to @crypt0cu1t (although we each have different diagnoses & symptoms) in the sense that I had the symptoms of LEMS (but never tested positive for the most common LEMS autoantibody which is the P/Q Calcium Channel Ab and instead tested positive repeatedly for the N-type). And @crypt0cu1t (if I understand correctly), you are negative for anti-NMDR but positive for other Auto-Abs that link to AE? I think there are just people who are atypical cases of everything even if many neurologists cannot understand this.

Edited to Add: One area where testing for the specific auto-antibodies is crucial is with the paraneoplastic autoantibodies that link with cancer b/c then you can have the corresponding tests to rule out cancer as the cause (versus autoimmunity as the cause).
Hey! Yeah so I never did have NMDA antibodies, I just misinterpreted stanford's test. I have AChR Binding (myasthenia gravis) Salivary Protein 1 (sjogrens) AChR a3 neuronal (AAG/Rarely AE).
 

Gingergrrl

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Actually, I think I only put that because I had a note that GAD-65 Ab was linked to "limbic encephalitis". In addition to cerebellar ataxia and diabetes.
Thx for clarifying and I think I must have just assumed AE when you said "encephalitis". Also, I just sent you a PM re: some of the questions you asked me in other threads re: high dose IVIG & autoimmunity.

I'm negative for GAD65. But since testing positive for TS-HDS Ab, I've noticed that diabetes is very much linked to autoimmune and autonomic neuropathies. In fact, most of my research today was regarding the link between diabetes, prediabetes, and impaired glucose metabolism with Small Fiber Neuropathy and Autoimmune Neuropathy.
What is "TS-HDS Ab"? There is definitely a link between GAD65 Ab and diabetes and when I first tested positive at a pretty high level for GAD65 Ab in 2016, my Endo ran every test he could think of for diabetes (the common ones and some more obscure autoimmune ones) and I was negative on everything. So in my case, we suspect that the GAD65 Ab was due to Dysautonomia and/or Hashimoto's (which have both also been linked with it).

Did you ever find out the cause(s) of all the antibodies?
We will never know for sure and can only speculate but my main doctor, my MCAS specialist (who is now retired), and my Endo all felt the most likely culprit was prior severe Mono from EBV in 2012. Unfortunately (not too long after the Mono), I ended up living in a rental with toxic black mold for 3 years which also sent my immune system into chaos with MCAS/allergic reactions. So both EBV and mold pushed my immune system into autoimmunity.

Hey! Yeah so I never did have NMDA antibodies, I just misinterpreted stanford's test. I have AChR Binding (myasthenia gravis) Salivary Protein 1 (sjogrens) AChR a3 neuronal (AAG/Rarely AE).
That is what I thought but wanted to make sure I remembered correctly. Didn't you also have a diagnosis of LEMS or MG at one point, too? Can you remind me, did you ever have muscle weakness?
 

sometexan84

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What is "TS-HDS Ab"? There is definitely a link between GAD65 Ab and diabetes and when I first tested positive at a pretty high level for GAD65 Ab in 2016, my Endo ran every test he could think of for diabetes (the common ones and some more obscure autoimmune ones) and I was negative on everything. So in my case, we suspect that the GAD65 Ab was due to Dysautonomia and/or Hashimoto's (which have both also been linked with it).
I'm noticing all these weird connections between glucose stuff (diabetes, impaired glucose metabolism, etc), gluten stuff (celiac, thyroid, etc), thyroid stuff, arthritis (RA, ankylosing spondylitis, etc), and autoimmune peripheral neuropathies. It's weird.

TS-HDS seems kinds of new. An autoantibody discovered in 2003. It's now part of the CellTrend testing, in "Small Fiber Neuropathy". Seems to be able to cause sensory and autonomic dysfunction.

TS-HDS (Trisulfated heparin disaccharide IdoA-2S GlcN-S-6S)
TS-HDS is a disaccharide component of glycosylation of heparin and heparin sulfate.
  • Loss of peripheral autonomic nerve fibers, usually unmyelinated (without insulation, called myelin, around the nerve fibers)
  • Small fiber loss with IgM deposits around the outside of medium- & larger-sized capillaries with C5b-9 complement deposits
Interesting, because I'm in a group of TS-HDS positive folks, and they all keep talking about MCAS, which I have somewhat ignored.