Here we go. I'm in NYC. Neurologist suspected SFN and sent me for a skin biopsy via another doctor in his office. Came back positive, but showed reduced nerve fiber density in the thigh, not the ankle. My Neurologist put me on Neurontin and sent me back to my Internist for blood work. The Neurologist was tough to reach on the phone and didn't specialize in this illness. I found a 2nd Neurologist who did. He put me on Gralise which is Neurontin 1x a day vs 3x a day as I kept messing up on the exact timing dosage of the 3x a day Neurontin.

2nd MD also said he doubted whether I had SFN as the test results were so unusual. Note, my symptoms were burning pain, pins and needles of my back, lower legs, feet and hands. I never heard of this illness until I was told I had it. Looked it up and seems the 1st MD was spot on. I also responded fairly well to the medicine. I had a return appointment and again 2nd MD said he doubted whether I had it due to test results. I want to kill this Neurologist. Then I re-read the lab results which said the test was taken rt thigh and rt calf. Test was taken right thigh and right ankle. I didn't tell the MD this. Obviously my test results are messed up, but that has nothing to do with the fact that I have the symptoms I have. So has anyone had 1) test results showing reduced nerve fiber in the thigh and/or 2) a bad experience with a so called expert in this field? Please help

You need to find another neurologist...one that knows about NON-length dependent small fiber neuropathy. This means that it does NOT follow the normal pattern of starting at the feet and working it's way up.

Any neurologist that deals with neuropathy should know about non-length SFN.

...who has heard of this condition.

I’d recommend you show copies of this article to your MD’s....

CHARACTERIZATION OF NON–LENGTH-DEPENDENT SMALL-FIBER SENSORY NEUROPATHY

AUTHORS: SARA KHAN, MD and LAN ZHOU, MD, PhD

JOURNAL: Muscle & Nerve, Vol. 45; Pages 86–91; Year: 2012

ABSTRACT: Introduction: Non–length-dependent small-fiber sensory neuropathy (NLD-SFSN) is not as well characterized as length-dependent small-fiber sensory neuropathy (LDSFSN). Methods: Sixty-three patients with NLD-SFSN were compared with 175 patients with LD-SFSN for their demographics and disease associations. Results: Age was younger in those with NLD-SFSN (45.5 6 13.1 years) than in those with LD-SFSN (55.1 6 11.4 years, P < 0.001). Forty-six of 63 (73.0%) patients were women in the NLD-SFSN group, whereas 84 of 175 (48.0%) were women in the LD-SFSN group (P < 0.001). Disease associations were identified in 26 of 63 (41.3%) patients with NLD-SFSN, including diabetes or prediabetes in 10 (15.9%), connective tissue diseases in 6 (9.5%), thyroid dysfunction in 4 (6.3%), sarcoidosis in 3 (4.8%), vitamin B12 deficiency in 2 (3.2%), and paraproteinemia in 1 (1.6%). Immune-mediated conditions were present in 9 of 63 (14.3%) patients with NLD-SFSN and 6 of 175 (3.4%) patients with LDSFSN (P 1/4 0.012). Conclusions: NLD-SFSN is more common in women, presents at a younger age, and is more likely to be associated with immune-mediated conditions than LD-SFSN.

You can purchase a .pdf file of the article for $35 dollars off of the website if your local university does not carry the journal. I just did

Or both!


ARTICLE: Non-length dependent small fibre neuropathy/ganglionopathy

AUTHORS: K C Gorson, D N Herrmann, R Thiagarajan, T H Brannagan, R L Chin, L J Kinsella, A H Ropper

JOURNAL: Journal of Neurology and Neurosurgery in Psychiatry, 2008; Vol 79: Pages163–169.


ABSTRACT

Objective: To describe the clinical and laboratory features of a painful non-length dependent, small fibre ganglionopathy (SFG).

Background: The syndrome of generalised SFG with early involvement of the face, trunk or proximal limbs is not well recognised and contrasts with the burning feet syndrome of small fibre neuropathy (SFN) and classical large fibre features of sensory ganglionopathy.

Methods: Retrospective case review including skin biopsies from four neuromuscular centres. Patients with pre-existing diseases associated with ganglionopathies were excluded.

Results: 12 men and 11 women, with an average age of 50 years, were studied. Neuropathic pain developed over days in eight and over months in the other patients. The face (n=12), scalp (n=10), tongue (n=6), trunk (n=15) and acral extremities (n=21) were involved. Symptoms began in the hands or face before the legs in 10. The pain was characterized as burning (n=22), prickling (n=13), shooting (n=13) or allodynic (n=11). There was loss of pinprick sensation in affected regions in 19, with minimal or no loss of large fibre sensibility. Laboratory findings included abnormal glucose metabolism in six patients, Sjogren syndrome in three and monoclonal gammopathy, sprue and hepatitis C infection in one each, with the remainder idiopathic. Sensory nerve action potentials were normal in 12 and were reduced in the hands but normal in the legs in six. Skin biopsy in 14 of 17 showed reduced nerve fibre density in the thigh equal to or more prominent than in the calf. Two of seven patients improved with immune therapies, 13 symptomatically with analgesic medications and the remainder had little improvement. Ten considered the pain disabling at the last follow-up (mean 2 years).

Conclusion: The pattern of symmetric, non-length dependent neuropathic pain with face and trunk involvement suggests a selective disorder of the dorsal ganglia cells subserving small nerve fibres. It can be distinguished from distal SFN. A potential metabolic or immune process was detected in half of the cases and the disorder was often refractory to treatment.

All my doctors have some kind of hookup to get/read articles like this, so before spending the bucks (unless you want to) I'd just give the reference to the doctor. S/he might (depending on how nice a guy/gal s/he is) even copy it for you gratis...

Doc

--consistently showed reduced intraepidermal nerve fiber density at both thigh and ankle, and my symptoms were body wide in the most acute phase of my neuropathy. Technically, the symptoms still are body wide in that i am prone to compressive effects anywhere in the body although I am much less symptomatic than during the acute phase ten years ago, and my subsequent skin biopsies have reflected this, showing some degree of re-enervation in both places.

It is true that many doctors are unaware of non-length dependent small-fiber neuropathy as an entity, but anyone with skin biopsy results as you described really need to be followed by a specialty center that is aware of the research in the field (i.e., Jack Miller in Chicago, Mass General, Cornell-Weill in NY, or Washington University, from whose website and neurological database the following links come from

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

http://neuromuscular.wustl.edu/antib...uron.html#sfsn

Mine showed no discernable nerve fibers at ankle, severely reduced at above knee and segmenting and breaking down at upper thigh. That was three years ago, my symptoms have progressed to include pins and needles up to hips.

A lot of neurologists are not very helpful in one way or another. In my case I have CMT, hereditary neuropathy, so there is no treatment except pain meds, but the number of stunning misdiagnoses or dismissals of symptoms chronicled here is a real eye opener. You must advocate for yourself.

Hi, I was Dxed by dr out of WashU... Very thankful. However, just this past appt., was told that some symptoms that I understood to be part of NonLengthDependent SFN/Ganglionopathy maybe aren''t: bradycardia attacks, perceived difficulty inhaling, bladder issues, all of which I thought was part of various of my ganglia flaring up at various times... Do I need to remind doc of nonlengthdependent "status"?; is it the ganglia? Or might I have something else as well???
Advice, please

There is an autonomic ganglia...and your symptoms might be related to autonomic dysfunction/dysautonomia. Have you had any autonomic testing...like heart monitor for the bardycardia, tilt table, urodynamic studies for the bladder issues, etc? Do you have problems with GI (early fullness, nausea after eating, bloating, etc)?

If you have ganglionopathy from autoimmune disease, then I would suspect the autonomic ganglia as well. Any autoimmune work up?

I too was diagnosed w non length dependent sf ganglionapathy and also have related autonomic issues. My first neurologist seemed to have no idea about the non length dependent distinction, but johns hopkins came through. Symptoms started in my back and are now basically full body from my scalp to my toes, though each day is different as to which body parts have symptoms, generally don't have all at same time. Originally was diagnosed through a QSART test and skin biopsy, but it was the sweat glands that were most telling in my case. Tho have recently been told I have large fiber neuropathy too, so not really sure what that means to the earlier diagnosis.