Hi, this is my first post, am totally new to this sort of thing. I started reading your posts over the last few weeks and decided I should join. You all have provided so much helpful information, very appreciated for the newly diagnosed.

I began having back pain and nerve symptoms last October, and after months of tests, was diagnosed in january through skin biopsy with idiopathic small fiber neuropathy. I have gripping pain in my feet and freezing burning pain in my feet, legs, torso, back and neck. My hands mostly just go numb periodically, mostly at night in my sleep. In the midst of this, in December, I had an extremely painful attack in my face which took me to the ER and was diagnosed with trigeminal neuralgia. In hindsight, my doctors do not believe its that but somehow related to the rest. Have any of you had this connected to your trigeminal nerve?

I am taking 2700 mg of gabapentin, Tramadol, Nortriptyline at night, and just finishing 2 month trial of steroid infusions. None of it seems to help.

I have seen mention from a few of you about a smaller subset of non length dependent type of SFN, given my body wide symptoms, am wondering if I fit in that category. And if so, is this something to pursue that might lead to an identification of an underlying cause or different treatment plan?

Thanks for listening,
SFNgirl

Hello SFNgirl.

I'm sorry to hear about all the pain and crap you are experiencing. The face incident sounds really sore. As I understand it, anything close to a diagnosis of "trigeminal neuralgia" is about as bad as it gets.

The typical portrayal of symptoms in cases of SFN begins with a "glove or stocking" pattern, meaning the symptoms typically begin in the hands or the feet (or both). The progression over time includes further symptoms going from these peripheral areas up the arms or legs towards the "trunk" of the body. In other words there is a natural progression which follows a systematic "length dependent" pattern.

The "non-length dependent" SFN diagnosis does not follow the typical progression noted above. One way of figuring out if you have non-length dependent version is to systematically review the presentation of your symptoms--did they start in other places on your body before they started in your hands and/or feet? Did they jump to your torso/face before they worked up your arms and/or legs?

There is a good possibility that the nerve biopsy you had done during earlier testing will confirm your diagnoses of NLD SFN/G. You should ask your doctors. The word "idiopathic" in your diagnosis likely refers to either: a) an unknown etiology of your SFN, b) aytpical progression of your SFN without confirmatory evidence of NLD SFN/G; or c) NLD SFN/G as confirmed by the biopsy.

Most physicians don't have an understanding of NLD SFN/G because this is a rare diagnosis. I can't remember the specifics but I seem to recall that it affects only about 5% of the population of those referred to specialized neurology clinics. Most regular MDs just never run into it, even those who specialize in diabetes where SFN is unfortunately fairly common. Where did you have your testing done? I know that UC has the Center for Peripheral Neuropathy which might be good for a second opinion, etc. I'm sure there are other great clinics in your area but I was just reading about this one.

Anyway, hang in there. Which reminds me that the primary treatment for NLD SFN/G is pain management and trying to live a healthy lifestyle. For pain management people often end up having to go with time-release tramadol or time-release morphine (Avinza) or time-release Oxycodone (OxyContin). Hopefully you don't have to go there, but if you do, I hope it helps.

I'd be happy to talk more if you need a friendly sounding board,

Mike

Thanks for the very helpful reply. It sounds as though you have nld SFN, and is the /g for ganglionopathy? Since I posted my question, I had a follow up w my neurologist and it seems in his notes he now defined mine as nld SFN. So I guess thats sort of official. I am considering going for second opinion, but thinking mayo or Hopkins which my primary doc recommends.

In my case, it's hard to tell based on your explanation of nld pattern. Everything all started in my low back, and as I am (was) a runner, I assumed and my primary doc assumed it was a disc issue. After a while, I started having weird pains and freezing sensations down my left leg and then more significantly in my left foot. The strange freezing feeling then was going across my low back, felt like I had used Ben gay or icy hot cream, like a cold burning feeling. I then can't say the exact order as to upper back, right leg and foot and hands beginning to go numb. But it certainly did not follow a systematic pattern. In the midst of testing and meeting a neurologist, I had the face thing, still a mystery as my neuro does not know how it could be connected. It seems to me it must either be related to SFN or was actually a fluke jaw thing, because during the attack my bite was actually off and then settled back to normal after the attack subsided. Was absolutely the most pain I have felt in my life.

In your case, is it idiopathic or have they determined a cause? My neuro has ordered a few last tests, CT scan of chest and abdomen (looking for malignincies which he said he does not believe will be the case thankfully), but he ordered paraneoplastic panel and another autoantibody panel. He said he is now looking for very rare things, but most likely going to be a focus on pain management as you suggest. I am taking time release Tramadol and it helps a bit but not nearly enough. I have just weened off gabapentin (from 2700). And about to try lyrica to see if better results w less side effects. Have you had luck w any particular pain mgt strategy?

Thanks for the info

I pulled the following quote out of a 2010 article by Kenneth C. Gorson from Tufts University. His earlier work (2008) describing the NLD SFN/G (yes the G is for Ganglionopathy, and the N is for Neuronopathy rather than Neuropathy which is used in typically used to describe LD SFN) was the first article which explained adequately the symptoms that I had/am experiencing. (Sorry, I’m pretty sure that last sentence was too long and hard to read.)

Here the reference to the 2008 study (I think you might be able to get it off the internet if you are interested):

Gorson KC, Herrmann DN, Thiagarajan R, et al. Non-length dependent small fibre neuropathy/ ganglionopathy. J Neurol Neurosurg Psychiatry 2008;79: Pages 163-9.

And finally the 2010 quote:

“A distinctive clinical pattern of burning neuropathic pain and small fibre sensory loss involving proximal regions of the limbs, trunk and face has been characterized clinically and by the spatial distribution of skin biopsy abnormalities as a non-length-dependent small fibre neuropathy / ganglionopathy.

“The cause of this curious disorder remains unknown in the majority of patients, but investigators have postulated that the pathology may be localized to the dorsal root ganglia subserving small nerve fibers. The condition has been associated with diabetes mellitus, Sjögren syndrome, celiac disease and other inflammatory or autoimmune diseases in some cases.” [Note: some other association has been with rheumatoid arthritis, hepatitis C virus, Lymes disease, Crohn’s disease, and cancers/chemotherapy but many cases are “ideopathic”]

“This disorder, which is rare but probably under recognized, can be distinguished from the all too- frequent length-dependent, distal small fibre neuropathy (SFN) by the topographical distribution of abnormalities on skin biopsy; an abnormal intraepidermal nerve fibre density (IEND) of proximal and distal regions, or a disproportionately reduced IEND at proximal sites, suggests a non-length-dependent process.”

Yes I have non-length dependent small fiber neuronopathy / ganglionopathy. Mine is likely from the association with Type II diabetes but who knows?? You have to be willing to to have a high tolerance for dealing with ambiguity, chaos and conflicting opinions when having this problem, working with MDs, trying to figure out which way is best path forward, etc. I’m not sure if you will have a similar insight at some point in your struggle, but a year or two ago, I realized that I had more knowledge of this than the MDs I was seeing. Right now I would be happy finding an MD who would be willing to make an honest effort just to learn about it. Sounds like you Docs are better.

I have a burning face pain and so I pay attention to information on trigeminal neuralgia, etc. FYI, and based on nothing specific, my brain seems to recall at this time that some people have head pain a) in the forehead, around the eyes, nose, and cheek area, b) in the scalp and “hair” area; and c) inside the mouth and on the tongue. I am sure I have read about other areas (neck, shoulders) involved as well.

One time my wife stopped me from going out the door to get an extreme haircut. I came to the conclusion that a haircut would stop my head from hurting. I guess now it sounds pretty stupid but at the time it "sounded like a plan!"

The thing with face pain is that you become a walking set-up man for every wanna-be comedian in town:
Them, "How are you today Mike?"
Me, "My face hurts."
Them, "Yeah, (insert your joke here)".

Moving on, here is to the start of a great week!!

--on neuronopathy/ganglionopathy at Johns Hopkins is Dr. Abhay Moghekar (who I have personally corresponded with in the past); he's written a number of papers about this and how hard it is to diagnose definitively, at least until we get better ways of imaging the dorsal root ganglia--although there are some new technologies that have improved such imaging (though they are not yet widely available).

We've certainly discussed this in a few threads in the past:

http://neurotalk.psychcentral.com/ar.../t-110411.html

http://neurotalk.psychcentral.com/ar.../t-142219.html

I have only been dealing with this since Sept 20, 2013, but it came on fast and severe.. From my experience, something unknown attacked my body while I was abroad (toxic, virus, internal malfunction) Initially I was left with Constant Bilateral Facial pain that started in my left clogged ear, but when they irrigated it it spread to my left side of my face and quickly to both sides. It stayed like that for 3 months until my feet, legs, and hands started to get involved.


I'm 25 and was very healthy, but that part does not matter as much anymore, I would trade my healthy look a million times over to actually feel healthy,, but of course I have completed hundreds of tests and been to many neurologists whom until recently said it was all in my head..


It wasn't until I had an appointment with an Autonomic Nervous System Neurologist @ the Mayo Clinic did I get some answers. Of course again all the blood work comes back normal (but there were some oddities during my tilt table test) and per the post above the Dr. confirmed they just can't look into this as detailed as they would like due to lack of technology.

What they could tell me was that my autonomic nervous system was affected, and that was why myself and someone could have free floating adrenaline rushes or other strange sensations. The hard part with the small nerve fibres is that they literally run up and down the entire body.

Gabapentin has given me minimal relief as well, for it seems whatever is going on inside of me is more powerful than the medicine and just runs it over unless i'm drugged to the point of questioning the point of existance.

The neuronapathy is destructible in my instance so far only my clonazepam. But be ready for some changes.. It might not happen to you, but I had sever self derealization to begin with and led me to try to get off of it 3 times before i realized it's pain relief along with its side effects were still worth it.

I understand it is an addictive drug, but it is long lasting and if it works for you I think it is the best option until medical community gets a better grasp on how to treat this disorder.

Some other things that work for me is that I am in bed by 9pm and usually sleep restlessly till about 6:00 I than stay in bed another hour or two and try to enjoy the fact that my nerves are quieted.

This gives me a few hours of nice relief until I start to have to rely on my medication to get through the rest of the day.

6:00 am - Gapapentin 300mg
9:00 am - Clonazepam .5 mg
12:00 pm - Gapapentin 300mg
3:00 pm - Gapapentin 300mg
6:00 pm - Gapapentin 300mg
9:00 pm - Clonazepam .5 mg


p.s. does anyone know the best doctors or technology available to actually look into the dorsal root ganglion or whois leading the way in how to best to treat central pain syndrome.

Thanks
Casey

Hi everyone,

Well, after a year-long medical saga, idiopathic SFSN is finally my diagnosis. I'm not sure how "standard" my symptoms are though. I'm just going to list them as briefly as I can:

- My lower left leg has tingled and twitched for about 30 years. No known cause. I described the tingle as being more like I was standing on a vibrating floor. Sometimes it was painful at night.

- Last year, it felt like it was getting worse, and then all of the sudden it moved in to my right leg as well.

- An additional symptom is now that I have referred sensation into my feet. If I rub any body part from my mid-torso on down, the electrical sensation in my foot on that side of my body gets stronger.

- I'm also lucky to be "blessed" with this prickly feeling from head to toe on occasion, and when it's at its worst, I literally feel like I'm plugged into an electrical socket and am buzzing. And also when at its worst, I have bizarre sensations in my temples.

- Another feature is cold sensitivity. I'll get chilled to the bone for no reason.

Do these symptoms sound familiar to anyone? I've seen anyone report a decade-long problem that suddenly got worse.

I've had all the usual tests to rule things out, but have not had a punch test. My neuro said it would confirm the diagnosis, but wouldn't tell us why this was happening. The only stone left unturned in my neuro's mind in the possibility of an activated virus like Epstein Barr or Herpes Zoster, both of which are known viruses I have. (The symptoms in my left leg started about two years after a multi-month bout with mono.)

I'm also not ruling out some poorly understood metabolic issue, a hormonal issue, or even something going on in my spine that's impacting my spinal cord/CNS function even though MRIs show nothing. (Why mid-torso down for the referred sensation?)

Has anybody had any luck going from idiopathic to a known underlying cause? As I've read something 50% of all cases of SFSN are idiopathic, there must something big they're missing.

Any thoughts appreciated. Even just having people to commiserate with would be comforting. I've felt like I was losing my mind for awhile.

Janie
Baltimore

Welcome janieg.

Not sure how many replies you will get as it has been awhile since anyone posted. Hopefully, someone will be along.

Welcome!
I did have the punch biopsy done and was diagnosed with SFN. I understand how it feels to be told it is idiopathic. I've actually come to hate that word. That being said I have come to realize I can't drive myself crazy trying to find out what caused all this.

I've had SFN for 6 years now. In the beginning I went to many different doctors. I even went down to Hopkins. All this with no real answers. Every doctor has a different opinion (guess) and wants to try a different med. I can't take most of the meds so I take a few and try to do my best with what I have.

Don't get me wrong, I still search for answers but once in a while. Not every minute of every day.

I totally agree with you. They are missing something that causes neuropathy. My GP ( who left sadly) told me they are only beginning to locate antibodies that cause this. He said maybe mine was caused by an antibody they don't know about yet.

Could be true!

Janie
Baltimore[/QUOTE]

Has anybody had any luck going from idiopathic to a known underlying cause? As I've read something 50% of all cases of SFSN are idiopathic, there must something big they're missing.

Hello Janie

Confirming my dx was what drove me crazy. I wanted to know what type of neuropathy I had. I was pretty sure it was due to being pre-diabetic and then having 6 rounds of chemo (taxol/carbo) since the sudden and horribly painful onset was almost exactly a month after chemo ended.

I asked a foot and ankle dr I had seen 2 times to do a skin punch biopsy and he did.....severe small fiber neuropathy.

Most of the dr's I've seen chalk it up to diabetes so who knows ?

Take care and keep us posted.

Debi from Georgia