For all you "rare" Non-Length Dependent experts out there........

Please help me sleep better tonight.........

I read a previous post from Glenna saying her Doc at John Hopkins, feels this type of neuopathy may not have the possibility of healing as axon cells are dead.

Is this if the NLD SFN is caused from a disease property ex: Sjogens, etc..... or is idiopathic also included in this grim verdict?

2nd Question: Is it likely that NLD SFN can progress to Large Fiber Neuropathy/Sensory and or Motor?

As of now, I do not have large fiber (1 1/2 years after onset).

Any info would be greatly appreciated. I have been doing a lot of internet searching only to get more confused and upset.

I would ask how this doctor knows the cells are dead.

I don't think anyone knows anything for SURE about many types of PN. It is a very variable problem, and there are always surprises.

Doctors NEVER know anything for sure.

Here is an article on diagnostic error rates:
http://www.patientsafetyauthority.or.../Pages/76.aspx

It something to think about.

from the above cited study: Diagnostic Error in Acute Care

"Commonly misdiagnosed conditions include cancer, infection, fractures, myocardial infarction, embolism, neurological conditions, and aneurysms."

Hi, invisable -

As one of the "rares" but definitely no expert, it had to be tough to hear what that doc said.

Have to also admit confusion, but after almost a year from the onset, have to now believe that mine is caused by primary Sjogren's Syndrome that some of my docs have Dx'd and which isn't curable - so can't imagine that the neuropathy will ever leave. I just continue with the supplements and no Rx meds.

The burning has gotten much better (except face but not continuously) and no signs of length-dependent. Did have mild balance issues early on but not anymore, and only get a little dizzy once in awhile if I move or turn too fast. Muscle strength and gait remain very good.

Sorry I can't be of any help but will definitely ask the neuro I'm seeing on the
21st whether or not NLD SFN causes cell death.

--known as NEURONOPATHY, which is associated with Sjogren's an someother autoimmune situations, in which the primary atttack is on the dorsal root ganglia and the cell bodies there, rather than on the axonal fibers:

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

In these cases, if the cell bodies are destroyed, they are gone, and that entire nerve is gone, so healingis muchmore difficult than with condtions in which only myelin of the axon is damaged; recovery of function depends on how much other nerve cells nearby that res till functioning can sprout additional connecting axons to take over some of the functions. (Possible, but difficult.)

Bingo, glenntaj! Had actually forgotten all about dorsal root ganglionitis - duh.
Another duh - one of the neuros actually called what I have neuronopathy associated with the Sjogren's (her opinion), so you're right on the money with this.

I know this is an odd question.......hope you understand what I mean........

How is distinction made between Idiopathic Non-Length Dependent Small Fiber Neuronopathy and Idiopathic Small Fiber Neuropathy?

Does Non-Length Dependent automatically put you in Neuronopathy dx. rather than Neuropathy?

--as there are certainly causes of small-fiber disruption that are non-length dependent but don't necessarily show evidence of preferential dorsal root ganglia involvement.

A lot of these are toxic in nature--chemotherapy, ciguetera, mercury. And there is thought that some might be autoimmune--some sensory variations of Guillain-Barre like syndromes (certainly one theory that was advanced in my case). It is also thought that in neurological gluten sensitivity there may be involvement all up and down the body.

But, certainly, such abnormal presentations should be investigated for conditions that involve the dorsal root ganglia.

Glenna, thx for sharing your wonderful knowledge on this subject.

A couple more ?'s, if you don't mind....

1. Can non-length dependent be idiopathic in nature?

2. What are conditions that involve dorsal root ganglia and how are they investigated?

3. With Gluten sensitivity, wouldn't stomach involvement be present?

4. Can Isaacs Syndrome be mostly sensory in nature?

Thanks so much again.........I need to be "armed" for my next appointment.

To wit, In order:

1. Absolutely--a significant fraction of non-length dependent neuropathies don't reveal a smoking gun cause when investigated (mine, for example), but one has to make sure that very thorough testing is done; a lot of doctors, even neurologists, are baffled by this entity. Particularly, the immune/toxic possibilities should be explored--paraneoplastic syndromes, nerve antibodies, heavy metals, organic industrial exposure.

2. This is sort of answered by no. 1--at least for things that are known to cause non-length dependent syndromes or neuronopathies. There are serum tests for the various antibodies, gluten, toxins, and skin biopsy patterns, if enough samples from various sites are taken, can often be helpful. I've known doctors who are confused when skin biopsy results show overall damage rather than length dependency, though--in my opinion, finding that non-length dependent pattern should prompt a further search for the types of causes listed above (if not already done). EMG/NCV studies generally are not very helpful.

3. A good question-and the answer is not necessarily. In fact, there's more and more evidence that many with gluten sensitivity may not present first with gastric symptoms. There's a whole body of work by Dr. Hadjivassiliou on neurological prsentation of gluten problems--JCC has collected a lot of this in The Gluten File (everybody should have this bookmarked--most comprehensive database on the subject yet compiled--http://sites.google.com/site/jccglutenfree/)
and even today, I found this (you need to sign up for a free subsciptionto read it, but no big deal):

http://www.gastroendonews.com/index....e%5Fid%3D16015

4. My understanding of Issacs syndrome is more limited, but my understanding is that while there may be sensory symptoms involved, the syndrome is defined by irregular activity in motor neurons that causes continuous activity in muscle fibers. It is interesting that some of the same autoimmune and toxic causes are implicated in this syndrome as in neuronopathies, though.