Ganglionopathy
 

Hi all,

Does anyone know whether full body sfn with symptoms in the face, tongue, nose as well as legs, trunk and spine point more towards a ganglionopathy? I had read somewhere that non length dependant may be suggestive of this. My skin biops results stated that nerve damage was not suggestive of that but I'm still not sure.

Can anyone advise in whether certain blood tests can be taken to determine whether ivig may help you? I'm still not entirely sure on the mechanism of action for ivig and as I'm already on autoimmune disease overload I'd not want to worsen things. My neuro does not offer any advice or suggestions on that question. I'd have to push for the tests.

Any advice would be great. I'm spending alot of energy pushing for access to ivig, although futile right now. Pain hs floored me this week.

Can rapid bowel transit be a sign if autonomic neuropathy? I have heart flutters, dizziness, and lots of tummy spasms often. Gastro test showed rapid bowel transit.

I would say, YES, that full body SFN can point toward ganglionopathy...just as many forms of SFN can point there.

I don't think there is any blood tests to determine whether IVIG will be helpful. If you have documented small fiber damage or decreased density and history of autoimmune disease, then IVIG would be appropriate to try. A 3-6 month course would be enough for you to tell if if would help. Then a repeat skin biopsy would be helpful to gauge any improvement...along with how you feel clincally.

You shouldn't have to "push" for IVIG if you have SFN and autoimmune disease...it should be suggested by your doctor, not you having to push. Maybe another opinion would be best to get you the proper treatment.

Although rapid bowel transit has been known to occur with autonomic neuropathy, the most common is the opposite...lack of motility.

As for the gangionopathy, there is a new MRI/MRN at Johns Hopkins that looks directly at the dorsal root ganglia (DRG). This is how I was diagnosed with gangionopathy (I had noth increased signal and bilateral enlargement of the DRG). I'm not sure (and sort of doubt) if you have this available in the UK, but might be worth asking about, just in case.

Hi,
ganglionic Acetylcholine receptors antibodies is a blood test for Autoimmune autonomic ganglionopathy - .
It can be done at Oxford labs http://www.ouh.nhs.uk/immunology/dia...ntibodies.aspx
"Their presence suggests an immunotherapy-responsive condition - Vernino S. et al., NEJM 2000:343 847-855"

Or as a part of the paraneoplastic evaluation at Mayo http://www.mayomedicallaboratories.c...rpretive/89904

Hi there,

I also have a non-length dependant SFN, i am curious too if this is ganglionopathy. I am not sure if i have A N, have had excess sweating and dizziness. I wonder if it is worth taking this blood test to my next neuro appointment. I too am keen to get IVIG but i understand going through NHS procedure is going to be tricky.

Keep in mind that AAG (autoimmmune autonomic ganglionopathy) is NOT the only type of ganglionopathy. You can have damage to the dorsal root ganglia and not have damage to the autonomic ganglia. This blood test is just for one type of ganglionopathy...but an important one if you have dysautonomia.

The test itself won't determine if IVIG will help. It is a diagnostic lab to determine the ganglionopathy. It is still unknown whether the IVIG will help (until you try it), but it is one of the conditions that IVIG has had good results.

They are still not even sure WHY the IVIG helps many people with different types of neuropathy...they don't know the exact mechanism of action. They have ideas how and why it works, but it's not defined just yet.

I personally, have had good results with IVIG for my ganglionopathy...but only certain symptoms. Most of my damage is permanent.

I was diagnosed with SFN in July....I am getting waaaay worse. I don't understand the terms you are using. Is there a dictionary for these terms somewhere? I need help. :wink:

Of course you can Google the terms, but don't hesitate to ask here too. Just let us know which terms you want details on and we'll do our best to help explain things for you.

Thank you en bloc and still hoping :-) No one has ever willingly mentioned ivig, it is only after my own research and then presenting it to them has it been discussed. My neuro specialises in peripheral neuropathy. It must be cost.

Phoned secretary anyway and she said the neuro is collating info for the application. It could take a bit of time though. Knowing the NHS that's not a good comment to hear. I don't know exactly what else he needs to collate.

Sadly as its the NHS it could be 6 months before I see another neuro and then I have to navigate and negotiate with them. My neuro sits on the ivig panel wherein the decision is made so I feel I need to stick with him. The NHS is a wonderful thing but presents a vast number of challenges for some patients as well.

The blood tests look interesting thanks still hoping. I'm looking more into the Oxford labs one.

I don't believe tjy do the MRI here in the UK. My rheumatic said that a basic MRI if my spine would have shown it, that's not true at all is it. Other than that no one has a clue what I'm talking about. We are clearly behind the US in this one.

Thanks again everyone for the detailed replies :-)

My neuro suggested IVIG right after I got a positive skin biopsy for SFN. I don't have a specific autoimmune diagnosis and I am still struggling with the HMO to get the IVIG.

If you already have a definite diagnosis of autoimmune disorder it might be easier to justify IVIG without doing more tests.

The bureaucracy took some time, my neuro asked me to fill some forms and they searched for papers supporting IVIG for SFN in autoimmune disorders.

Good luck!

Thankyou for your help and info. :-)

my neuro diagnosed me with SNF and then said he couldn't do anything so discharged me 😒 He has been pretty ineffectual to be honest.
I have since seen a rheumatologist and been diagnosed with sjogrens but no offer of treatment for the SFN other than the hydroxychloroquine for the sjogrens which will apparently help stop the SFN getting worse.

This makes me so angry I could use alot of expletives right now. I have read numerous reports / studies stating that ivig has improved outcomes for some with sjorgens related sfn. Is this a big conspiracy or are some of the neuros stupid? It just has to be the cost and lack of clinical trial data.
I do understand concerns regarding risks but they seem to be far less than some other immune suppressing treatments.
Can you change neurologists?
Feel your pain - literally. My neuro has just sent me away with 6 month check ups but I have been emailing persistently to ask for Ivig based on my own research.

Ok.....what is IVIG? I'm seeing my THIRD neuro doc on Oct. 14. I've learned more on this board than from ANY neurologist I've seen. They tried to tell me I had anxiety!! I have SFN and I believe I may have autonomic neuropathy too....2 phys therapists (1 is a Dr) have told me something is affecting my diaphragm. .......????????

I have Sjogren's related SFN/PN and receive IVIG. I'm sure it is the cost factor that keeps doctors from using this treatment more often. They are probably 'encouraged' to avoid prescribing IVIG.

You are correct about the risk, but as you say, 'some' other treatments actually carry more serious risks than IVIG.

One of the other reasons I think IVIG is held back from treatment is availability. It is a blood product and therefore subject to shortages. So criteria for prescribing is tough. If they gave it to everyone with even mild cases, then there would not be enough to go around. But the catch 22, is that if they used it for early/mild symptoms, they would probably have excellent results (catching it early).

IVIg is Intravenous Immunoglobulin. It is antibodies from blood donors. It takes about 2000 donors to make ONE dose of IVIG. That is why the cost is so high. My treatments run over $30,000 each (once a month).

The reason it is helpful for autoimmune based neuropathies is that IVIG is believed to help modulate the immune system...that is attacking its own body. The exact mechanism of action is unknown, but this is how they believe it helps the patient.

Hopes this helps.

Thanks en bloc. My neuropathy came on about 1 year ago. It was mild until December when the pain started. Does that mean too long for any hope. I worry that I haven't been caught early enough and now may have to wait few months more if I am able to access it
My symptoms are severe and debilitating so feel I am way beyond mild stage sadly.
Appreciate that it all depends and my question may not be easy to answer.
Thankyou en bloc for the really useful info :-)

Katekline, I don't know if you got my private message but I have covered some of the acronyms I have used in there.
Jo :-)

I don't think that is too long at all for possible improvement. Of course, the earlier the better, but starting agressive treatment by that first year is pretty good...being that many wait MUCH longer just to get a diagnosis.

It's just sad that you are having to fight for a treatment that can possibly help and should already be prescribed for you with your documented conditions. But it's all about cost and being a blood product drives that cost up. I have heard about others on NHS that cannot get IVIG...even though they have well documented conditions. Just keep pushing until you get it. Go ahead and get on the list for another neuro opinion (if you can) while you continue to fight with the one you have. Don't give up!!

In the meantime, you may want to consider other treatments (immune suppressors, and/or steroids)...if you haven't already tried them. Anything to help slow or stop progression and/or reduce the amount of damage/inflammation.

Thank you en bloc..I will keep on fighting to access it. I was taking entanercept ( enbrel) for my other problems but stopped it as I was worried about the nerve damage I already have. I get very frightened about making things worse. Problem is I am now in an awful state with severe inflammation in my eye stomach problems and awful neck back shoulder and joint pain from the AS. It has shown me that the enbrel was suppressing that. I fear I may not get ivig if I stay on enbrel as neither the neuro nor rheumy have a clue about taking both.
Things are just horrific right now to be honest. I can't even take naproxen or tramadol as I think one of them is causing my awful stomach problems.

Have you considered a short course of steroids...to see if your neuropathy and other symptoms are largely inflammatory based? If not, then it would be a wise choice...not a long course, just a taper pack. If your SFN is more inflammatory based, then you might feel much better (immediately). It's worth a try, just to determine (and learn) the amount of inflammation you are dealing with. Labs may/may not help tell you about your level of inflammation, but they are not very accurate.

My blood shows minimal inflammation which is frustrating. My eye problem is iritis, inflammation of eye due to autoimmune attack. My joints are inflammatory reactions too. I tried corticosteroid shot and one week if prednisone which didn't help the neuropathy.
I do feel that my neuropathy is much worse since stopping the enbrel, not that I had improvements on that.

En bloc, do you know from your latest rheum visit whether a biologic may be used in conjunction with IVIG?

I actually just left from an appt at Hopkins and was told that YES, a biologic can be used in conjunction with IVIG...but it is risky in my case because my use of IVIG is not just for neuropathy. I am immune deficient and therefore use of a biologic that further suppresses my already deficient immune system is very risky. But otherwise (for most other people), biologics can be used with IVIG according the inflammatory arthritis rheumy I saw today.

As for the bloods showing minimal inflammation...this is what I meant by the labs not being very accurate. They should not be relying on them to base your level of inflammation. The one week of steroids you got may not have even been enough to know whether it would have been helpful. How much did you receive daily?

Your neuropathy also may not be inflammatory based and that should be further documentation for the use of IVIG.

Thankyou en bloc, really helpful as always, I appreciate it. :-)
The steroids tablets were 50mg I think. I took them for a week and the neuropathy was the same, but appreciate that it probably wouldn't miraculously disappear as nerve damage is nerve damage. I have an appointment this Friday at the gps to ask for a next stage pain med, I am going to ask for a corticosteroid shot to see what it does. If it helps all my other stuff that would be great. I have alot of pain in my face behind my eye and I now recall my neuro saying the last mri shiwed inflammation within the whole left side of ny face and sinus. I know I have inflammation everywhere from my other non neuropathy symptoms.

Sorry to go on about Ivig but do you know if its a non inflammatory mechanism behind the neuropathy whether ivig will then be of any use? My biopsy just showed outright reduction of nerves rather than inflammation.

Hi. I'm not sure if you know that a rare side effect of any tnf-a inhibitors, (Embrel), is neuropathy. It can be sfn, sensory, sensory motor, and cns. There is a contraindication for taking it with any type of neuropathy in some publications. There's lots of info if you google tnf-a induced neuropathy. Much info comes from chrons.

Have you had an emg/ncs recently? Do you have weakness or only pain?

Oh yes, even when PN/SFN is non-inflammatory, the IVIG can be helpful. Probably used more often in this scenario rather than for inflammatory based neuropathy (because they typically use long term steroids for this type, due to lower cost).

If you can get approval of an IVIG trial, then make sure to request (after the trial) to have a repeat biopsy. If the nerve density improves, it will help greatly to get continued treatment.

I think I need a do over.
I just wanted to bring awareness that sometimes TNF-A medications can cause an immune mediated response that may be acute or very slowly over a long period of time. Sometimes the response stops when the med is discontinued, sometimes it keeps going. If it is recognized it can be treated with ivig and the damage is stopped and reversed. I know this because it is not uncommon in the cidp community.

This article describes, 3rd paragraph from bottom

http://emedicine.medscape.com/articl...65-clinical#b5

I brought this up for awareness and I don't know your history. If there's a sliver of a chance this could help I had to post it.

Take care

I think your point was noted the first time. However she said she had the nerve damage 'before' taking the Embrel and stopped it due to worry about the nerve damage (that already existed).

Thanks for your informative link.

If she gets the IVIG then it would cover this connection anyway.

Thanks all.

February, appreciate the info. Sadly ii developed sfn before any treatment and the neuro and rheum both felt enbrel would be worth a try as excessive tnfa I have due to ankylosing spondylitis may be behind sfn. Only 3 reported cases of enbrel enduced sfn were found when neuro and I did research. It's an absolute nightmare. Sit here every day asking myself whether I should re inject the enbrel stacked up in my fridge.
Enbrel is much more commonly associated with demylinating neuropathies is my understanding. It's still a huge shot in the dark taking it in my case. It does however have really food results in treating my original disease ankylosing spondylitis.
Can I please have some new non defective genes if anyone knows where I can find some :-)

I understand the challenges of deciding the lesser evil of treatments vs disease. I didn't want to cause you any unnecessary worry. Honestly, having worst case cidp, I am not worried about demyelination because myelin heals. What does worry me is not figuring out the cause. I have been very fortunate to figure out many triggers. I wish for you the same. Good luck in your pursuit for ivig.