[Sheltiemom18]

there are probably only a handful, if that, of people here who have Sjogren's Disease as the underlying cause of their neuropathy, thought I'd post this anyway.

Saw the new neuro today who did the skin punch biopsy almost a year ago and dx'd non-length dependent small fiber neuropathy although she was not the consulting neuro back then.

She strongly urged me to try Plaquenil for the Sjogren's (had refused it from the rheumys in the fall). She said Sjogren's is "huge" for NLD SFN and stressed that treating the underlying cause of SFN is extremely important. Didn't want to take Plaquenil because I'm really afraid of the side effects of any med. But I agreed, and will call the rheumatologists tomorrow and ask for it.

She explained the diffuseness of NLD SFN and that it can attack overnight, which mine did. She actually has a few patient's "just like" me and knows a lot about Sjogren's, which made me feel as if I'd might have finally found the right neuro. Have a follow-up in three months.

She said NLD SFN etiology is dorsal root ganglionopathy. Do the gurus here have any comments about that?

Take care,

Sheltiemom

[glenntaj]

--yes, the most common type of non-length dependent small-fiber neuropathy seems to result from attack on the dorsal root ganglia, with Sjogren's being a common cause:

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


This is also referred to by some as neuronopathy (as you can see from the site in the link), as the cell bodies are actually attacked preferentially to the fiber ends.

There are some paraneoplastic cases of this, and a number of idiopathic ones; autoimmune mechanisms are suspected in these cases as well, though there's some evidence that in some people abnormal glucose tolerance may attack the cell bodies as well as the fibers. And, there are some toxic cases, particularly post-chemotherapy (hardly surprising).

The problem here is that when axonal fibers deteriorate, given the right conditions, they may regenerate, or other fibers from the same neurons may grow into the areas to take over function, but if cell bodies are damaged, recovery is a lot more difficult.

[mrsD]

I think Herpes Zoster, shingles virus, is also a dorsal root
cause.

http://en.wikipedia.org/wiki/Peripheral_neuropathy

We had a thread with a link to stem cell research for this, search "stem cells" on our forum.

This article was also posted, by Kiwiboy recently:
http://www.sciencedaily.com/releases...0615171509.htm

I think deciding to do the Plaquenil is your decision. I do know many autoimmune conditions including lupus do use it.

I guess benefit vs risk would be a tally sheet you need to do.
If you cannot stand your pain...or it is worse, that would be a big factor swaying towards the drug.

[Sheltiemom18]

Thanks very much to both of you (glenntaj and MrsD). In the meantime, researched Plaquenil and don't like what I'm reading about its serious side effects. Especially mentioned is that people over 60 should be wary. Well that's me for sure, and add another 15 years to boot. Nor do I like the side effects being posted at a Sjogren's support group site that people on Plaquenil are experiencing. Some, much younger than I, have quit it because of them.

As you say, MrsD, it's my call. Knowing how I am about meds in general, will probably avoid it because I don't have "pain." It's decreased to a hard to describe discomfort of the skin to light touch especially on the face. Also legs and arms are affected by the the brushing of fabrics against skin. Even that has decreased somewhat but might never go away altogether. However, wind and breeze in the face no longer bothers me, thank goodness.

Will try to find out what other med(s) might be used that aren't so frightening. It was recommended to me because of the rash and fatigue factor (really not so bad). So back to the drawing board.

Sheltiemom

[mrsD]

All of the immune suppressing drugs are "frightening"..I think.

If you read the MG forum here, they use Cellcept, or Imuran, (not often Plaquenil), and those have severe side effects.

Methotrexate is another. Or Cytoxan...both chemo drugs.

IMO there is no easy treatment.

I've looked at the forum you mention at times... I get the feeling some of them have mixed problems. They tend to fixate on that diagnosis only and medically several other things may be going on at any one time. Once a person gets that precious "diagnosis" they tend to ignore other things that may be outside of it, and include other symptoms by mistake. Over time with a difficult problem people learn to fixate on every little thing, and that can be counter productive too.

In community work, I rarely saw Plaquenil used. It is not a common drug anymore. (in the past it was, but treatment protocols do change). It was most commonly used for Lupus.

[invisable]

Originally Posted by glenntaj

--yes, the most common type of non-length dependent small-fiber neuropathy seems to result from attack on the dorsal root ganglia, with Sjogren's being a common cause:

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


This is also referred to by some as neuronopathy (as you can see from the site in the link), as the cell bodies are actually attacked preferentially to the fiber ends.

There are some paraneoplastic cases of this, and a number of idiopathic ones; autoimmune mechanisms are suspected in these cases as well, though there's some evidence that in some people abnormal glucose tolerance may attack the cell bodies as well as the fibers. And, there are some toxic cases, particularly post-chemotherapy (hardly surprising).

The problem here is that when axonal fiber deteriorate, given the right conditions, they may regenerate, or other fibers from the same neurons may grow into the areas to take over function, but if cell bodies are damaged, recovery is a lot more difficult.

In non-length depend. the cell bodies are damaged, and slim chance for recovery?

[glenntaj]

--that is correct; when cell bodies die, they die.

Not all non-length dependent neuropathies are necessarily dorsal root ganglia attacks, though. This is somewhat controversial theoretically--there was a lively debate about it going on at Johns Hopkins, particularly, among the research faculty for some years (see papers by Drs. Moghekar, Cornbluth, McArthur, etc., especially as regards efficacy of skin biopsy to determine these conditions)--but it is thought that is some autoimmune, nutritional, and toxic conditions the biochemical reaction may occur at any point on the axon, not necessarily at the tips--but this is hard to distinguish from the idea that, all other things being equal, the nerve tips farthest from the seat of circulation would be more vulnerable to metabolic disruptions, simply because oxygen/nutrients have to travel more to get there, and more effort is needed to get rid of waste buildup.

Among the conditions in which there may be non-length dependent, non dorsal root ganglia involvement, besides the ones mentioned, are the ANA-associated autoimmunities (such as Lupus or Bechet's), ciguatera toxin, leprosy, (not common, fortunately), and celiac.

Also, of course, localized nerve compression or injuries can result in non-length depedent damage, such as in a brachial plexopathy or thoracic outlet syndrome. These are usually fairly evident if the right diagnostics are applied, though.

[invisable]

Originally Posted by glenntaj

--that is correct; when cell bodies die, they die.

Not all non-length dependent neuropathies are necessarily dorsal root ganglia attacks, though. This is somewhat controversial theoretically--there was a lively debate about it going on at Johns Hopkins, particularly, among the research faculty for some years (see papers by Drs. Moghekar, Cornbluth, McArthur, etc., especially as regards efficacy of skin biopsy to determine these conditions)--but it is thought that is some autoimmune, nutritional, and toxic conditions the biochemical reaction may occur at any point on the axon, not necessarily at the tips--but this is hard to distinguish from the idea that, all other things being equal, the nerve tips farthest from the seat of circulation would be more vulnerable to metabolic disruptions, simply because oxygen/nutrients have to travel more to get there, and more effort is needed to get rid of waste buildup.

Among the conditions in which there may be non-length dependent, non dorsal root ganglia involvement, besides the ones mentioned, are the ANA-associated autoimmunities (such as Lupus or Bechet's), ciguatera toxin, leprosy, (not common, fortunately), and celiac.

Also, of course, localized nerve compression or injuries can result in non-length depedent damage, such as in a brachial plexopathy or thoracic outlet syndrome. These are usually fairly evident if the right diagnostics are applied, though.

My favorite of all! Is idiopathic common in non-length dependent?

[mrsD]

You know I think the whole point of using idiopathic is to get the insurance code for reimbursement.

Without it, how would you get a claim thru? I sometimes think too much emphasis is placed on this term. It is really just a handle for billing in many cases.

[Raglet]

I have been on plaquenil for over 15 years, and have had no problems with it. Can't say it's done anything for my neuropathy though. I have regular eye checks, which have all come back normal.

hth
raglet