Small Fibre Sensory Neuronopathy
 

Hi all,
New to this, first post, I am a 57 year old woman who lives in Scotland. I was diagnosed last year with small fibre sensory neuronopathy after being diagnosed 8 years ago with peripheral neuropathy, I cannot find much information on this condition as it is very rare, it has worsened over the past couple of years which promted my neurologist to send me to see a professor in London who did some tests and came up with the diagnosis, I would be very grateful if anyone has any information they can give me as I do not know if my condition will get any worse. Thank you for taking the time to read this.

Welcome to Neurotalk--
 

--And you should definitely come on over to the Peripheral Neuropathy section here, and read and read and read:

http://neurotalk.psychcentral.com/forum20.html

By the way, small fiber sensory neuropathy is far from rare. It is the most common presentation of neuropathy among those who are diabetic, and it is also the most common idiopathic presentation (as many as a third of those with small fiber neuropathy have no discernable cause for it).

When you were diagnosed with peripheral neuropathy, you were just being diagnosed with damage to nerves outside the brain and spinal cord--the term doesn't indicate type or cause. Small-fiber neuropathy, which is by definition sensory and/or autonomic (unmyelinated or "small-fiber" nerves are not involved in motor functions) is a bit more descriptive, but not much more.

See:

http://neuromuscular.wustl.edu/sensory-small.html

http://neuromuscular.wustl.edu/senso...tml#idiopathic

Small Fibre Sensory Neuronopathy
 

Hi Glenntaj thank you very much for the welcome and the information, it was my neurologist that informed me this condition was very rare and that is why there has not been much research done. :confused: I am glad I have found this site there seems to a lot of very helpful people on here and a lot of useful information. My condition affects my hands ,feet ,face bowel and bladder, I have been tried on every medication there is but some had no effect and the rest I had very bad side effects, six months ago they decided to try me on Ivig(I get every three weeks) although not fantastic but helps keep the pain bearable, if anyone has the same symptoms as me please get in touch thanks again xx

Neuronopathy is a form of neuropathy.

I hope there is no confusion about that?

I put up a post with a link for you Villier, on PN forum here:

http://neurotalk.psychcentral.com/post893155-36.html

So it might be a good idea to check your spelling, to see if there has been a confusion?

small fibre sensory neuronopathy
 

Hi Mrs D
Thank you so much for your info my spelling is not wrong my condition is neuronpathy I have looked at dorsal root ganlia which was stated in a letter from the Profesor I saw in London I cannot find too much information on the subject that is why I have contacted this site to see if anyone can give me some answers :grouphug: xxx

Links have been helpful thank you for your reply just getting to know how this works

Neuronopathy--
 

--as a subset of neuropathy is rarer than other kinds of neuropathy, but likely not as rare as many people think; many have evidence of attack on the dorsal root ganglia.

Sjogren's syndrome is the most common association with this, but there are plenty of idiopathic cases.

See:

http://neuromuscular.wustl.edu/antibody/sneuron.html

Small Fibre Sensory Neuronopathy
 

Hi Glentaj thanks again for the information, I have been tested for sjogrens as for everything under the sun but nothing has shown up as to what is causing this, I have had very specialised blood tests done mri scans, emg, skin biopsy and the paper test in my eyes(can't remember what it is called) so do not know if there is much left to test, I will probably see my consultant on Wednesday when I am on the ward to get my Ivig I must ask him if it is idiopathic.

Small Fiber Ganglionopathy (Neuronopathy)
 

I am a 64 year old male and I am new here. In January of 2011 I started having burning around my trunk at the level of my navel. Over the next two months the burning involved my entire trunk and began moving down my legs and arms. A skin biopsy confirmed the dying back of small nerve fibers. After another month the burning covered my entire body including my scalp, face and tongue. I also experience disturbances to my sense of taste and smell. The biopsy and the involvement of my trunk and face confirmed the diagnosis of Small Fiber Ganglionopathy (Neuronopathy). The tricyclic Desipramine is providing significant relief from the worst symptoms but this Neuronopathy has dramatically affected my life.

Hi Wayner my thoughts are with you, I know exactly how you feel, I to was diagnosed with the skin biopsy, my problems started eight years ago with a tingling in my fingers which progressed to my toes a while later, a couple of years later it had turned into a burning throbbing pain in hands and feet and by this I had a tingling in my face and perineum also a dryness in my mouth which I was tested for sjogrens came back negative, a year and a half ago I started having problems with my bowels and bladder, over time I have been tried on every medication from pregabalin, gabapentin, amytriptiline, lidocaine infusions, mexilitine you name it I have tried it, I either had bad side effects or they didn't work, ten months ago after seeing a professor in London who diagnosed me, his suggestion was to try me on Ivig which I get every three weeks it helps a bit it brings my pain to a bearable level although it dips just after two weeks and then it is back to a lot of pain and sleepless night until the next infusion, as you say it dramatically affects your life, I am so glad I found this site as there are so many helpfull people with a lot of knowledge,I have found out more about my condition from here than I have from my neurologist it has given me an insight into questions I will be asking him in a couple of weeks time. I am glad you are getting significant relief from your meds. Take Care Marie

I'm curious villier what testing you had for Sjogren's. I have both Sjogren's and ganglionitis/neuronopathy from it. As you may be aware up to 40% of people with Sjogren's are sero-negative, so you can test (lab) negative yet still have Sjogren's. So I was curious if you had a lip biopsy that was negative also?

Hi en bloc, Sorry just seen your post, I have only had the eye test for Sjogrens which was negative and have not had a lip biopsy done, I think the only reason they did the eye test was I have a dry mouth, living in the UK we do not get to see reports of tests that have been done as you do in the States, so regards to blood tests I dont know any results or what they entailed all I know was that some were very specialised one in particular a porter arrived with a vacuum flask with hot sand and had to wait to take the blood straight back to the lab(my GP had never even heard of it), I personally do not think I have Sjogrens my condition is Idiopathic what I can make out. Since I have had the skin biopsy done I feel they are pretty sure they have now got the diagnosis right, I am so glad I found this site I now know the right questions to ask my neuro when I see him in a couple of weeks as I am getting more symptoms. What are your symptoms and what meds do you take? Marie

Since you have dry mouth and neuronopathy, you should not discount Sjogren's with just a Schirmer's test. You could try to inquire about labs drawn for Sjogren's (SSA & SSB). I believe even with the NHS you can get lab results...just not as easily as in the US. The lab where they put the specimen in the sand was likely for cryoglobulin.

My symptoms include dry mouth/eyes, neuropathy pain, and severe autonomic neuropathy (which involves heart rate, BP, temp control, urination, gastroparesis, balance, vascular spasms...and the list goes on). I also have APS (antiphospholipid syndrome) which is a clotting disorder that has caused 3 strokes.

I have a whopper list of meds...6 just for the heart & BP. I also take Plaquenil for the Sjogren's, several GI meds, blood thinners, and minimal pain meds. I did IVIG for 9 years but stopped after a severe case of aseptic meningitis from it. I may try again one day. I also had fairly good results from Cellcept (cancer drug/immune suppressant) for the short time I took it. I did not have luck with antidepressants, as you have. I also tried anti-seizure meds like lyrica and neurontin, but again they didn't work. The damage caused to the dorsal root ganglia from Sjogren's is irreversible and there is no known effective treatment as of now. The objective is to reduce pain, which you apparently have been able to do to some extent.

Your treatment may not be different if you pursued the cause of your diagnosis...other then plaquenil to treat and slow progression of the Sjogren's itself, or use Rituxan.

I am curious about one other thing. What test confirmed the neuronopathy? I am not aware that a skin biopsy can diagnose this by itself as it is used to determine small fiber neuropathy. Is this the test they said confirmed it, or was it a conclusion?

Hi
 

En gosh you have such a lot to contend with I don't know how you cope with it all, I thought I was bad with the neuropathy, palpatations idiopathic, beta blockers doing the job fine leave alone, multi nodular goitre, monitored for a year doing nothing fine leave alone, for a while though I have been having dizzy spells lately getting more frequent and low and behold palpatations, also, had a few UTI's as well, two weeks ago was shopping passed out and hurt hand taken to hospital, was kept in till next day repeated bloods done heart monitor on nothing, was at hospital today for Ivig spoke to ward doctor.....oh dizzy spells will be due to you fighting off infection thank goodness I see my neuro in two weeks time sorry for rambling on needed to get things off my chest.

You asked what test cofirmed the neuronopathy I surmised it was the skin biopsy as the neuro spoke about the dorsal rooot ganglia and i hadn't a clue at thetime what he was talking abot and because I still don't understand it all yet, I am a bit nervous as I destroyed the letter I got from the professor which mentioned anti-Hu antibodies and cannot remember whether it stated that I showed with or without so now thinking that is why the dx is neuronopathy what are your thoughts?(be totally honest), I would appreciate if you have time if you could give me a few pointers what I should be asking, my problem is I am not a pushy person and a bit shy.

How are things with you at the moment, are you in a lot of pain, how do you cope from day to day, do you have family living with you, do they understand? Hope this finds you in not to much pain..........tc .......Marie

Forgot to add also have had a few bouts of reflux which resultedi in a hoarsenes, doc gave me meds for stomach never had indigestion in my life.

First, it sounds like you could use a good cardiologist to isolate the cause of the palpitations. Keep in mind, it's easier for a doctor to attach the label of idiopathic vs take time to find the cause. If you have any autonomic dysfunction (which can be common with certain types of neuropathy--ie, autoimmune), then palpitations (AND accompanied dizziness) are likely a result of that...and in most cases easily treatable with medicine. It's a matter of proper testing to identify the arrhythmia. This goes way beyond a simple heart monitor.

Don't buy the "dizziness is from infection" unless you have a raging infection and are severely dehydrated or something. You need a doctor to start investigating the cause of these things.

BTW, the reflux can also be related to some delayed emptying...another autonomic dysfunction. Is the medicine he gave you helping?

It really sounds like your doctor drew a conclusion about the neuronopathy based upon your history of PN and then the addition of SFN (dx from the skin biopsy). The anti-Hu antibodies woudl also play a role. And it sounds like his conclusion may be/probably entirely correct. Just from reading your posts, I think (guess) you may also have some autonomic dysfunction going on as well. If I were you, I'd ask about some testing for the palpitations, dizziness, GI, etc. This would include a tilt table, 24-48 hr holter, 30 day event recorder (all of which needs to be done by a cardiologist). If you have any early fullness when eating or nausea/discomfort after eating, I'd add a GI empty test to check for delayed emptying. And knowing how common this type of neuronopathy is with Sjogren's, I'd get them to follow-up with more testing...maybe even a lip biopsy to confirm or deny the condition.

As for me, my pain, coping, etc. It's been a struggle recently, to be honest. I have continued progression and what is like a game of medical mgmt to keep up with it. And of course, pain can be very draining. But that said, I drive on because attitude and outlook is half the battle. I do have major surgery next week to replace my pacemaker, so that will likely set me back in other ways as well...as things like surgery can cause a flare of symptoms with autoimmune conditions. I am lucky though that my husband and son (a Marine who happens to be home right now on leave) are both very supportive.

Other then my comments above about requesting testing, you should just tell your doctor that you want to look at the "whole" picture and how your neuropathy can/will effect other systems (some that it already appears to be doing) and that you want to be pro-active about mgmt to stay on top of your condition as best possible. Your interest expressed in this way might encourage him to agree to be more thorough.

Anti-Hu antibodies--
 

--as a mediating cause of neuronopathy are usually a sign of an occult cancer; this is considered a paraneoplastic syndrome:

http://neuromuscular.wustl.edu/antibody/sneuron.html#hu

If you did show up with Anti-Hu antibodies there should be an immediate search for a primary tumor. But this would likely have caused the doctor to get in touch immediately. I would suspect that means you're negative for these, but it wouldn't hurt to check. (You do have to be your own advocate. And get copies of all test results.)

Hi en thanks for all the advice I appreciate it so much, I am at my brothers for a few days at the moment and wont be back till Tuesday, I wish you well for next week and hope everything goes well for you, hopefully there will be no flare ups and you will recover quickly, I am really pleased you have a very supportive husband and son at home, I will be thinking of you and keeping my fingers crossed that everything goes well for you keep me posted when you feel up to it.......tc...........Marie

Hi Glen thanks for the info I have actually came across paraneoplastic syndrome which I admit when I read about anti-Hu antibodies I did panic a bit as I remembered there was something about that in the letter but cannot remember what, I am sure now that if I had been positive they would have had me checked out by now, I really need to grow a backbone and stop being so soft thanks again...............tc...........Marie

First Post
 

Hi

I am new to this forum and i have exactly the same diagnosis. It is a rare condition - Can I email you direct to discuss further.

Thanks

please don't post your email on the forums, as it will appear on Google right away for the world to see.

New members cannot use the private message function here for a short while until they have posted a few times. Then the private message will open for you. Then you can exchange emails safely that way.

So you can discuss things here on this thread or make a new one on the PN (Peripheral Neuropathy) forum:
http://neurotalk.psychcentral.com/forum20.html