Diagnostics & Testing~~ for dorsal root ganglia and damage:
 

I haven't seen much on diagnostics & testing, so hope this opens the door for discussion about what people have found helpful and what's new.

When it comes to PN, many different tests can be done to look for the culprit....sometimes (actually, many times) still without finding an answer. They can include EMG and nerve conduction studies, skin biopsy for small fiber neuropathy, sural nerve biopsy, and of course basic xrays and MRI's to check for mechanical/structural problems where nerves may be damaged as a result.

I have had all of the above over the years, but minimal findings here & there didn't account for the severe nature of my autonomic neuropathy and now more pronounced PN affecting proprioception, balance, mild sensory, and pain in the neck, back, legs, and bottoms of my feet.

However, my most recent test provided the answer. It was the results from a new type of MRI to specifically look at the dorsal root ganglia, and referred to as the DRG protocol. This MRI w/contrast apparently uses a standard process (not the experimental water excitation) with a 1.5 tesla magnet. They could clearly see the ganglia...enough to determine there was bilateral enlargement and had an increased signal, both consistent with ganglionitis.

I also have the autoimmune disease, Sjogren's, which has known neurological manifestations to include a direct relation to ganglionitis. So this finding actually made sense and put many unanswered pieces together.

Anyway, the test may prove helpful for others where a definitive diagnosis has not been determine. It will likely not be available at your community hospital, but certainly worth checking out if you have access to large university hospital like Johns Hopkins, Mayo, Cleveland Clinic, UCSF, Duke, etc. Like I said the MRI is still "standard" in most regards, just utilizes a new protocol, so my insurance (Medicare) picked up the cost without question.

Of course, since there are MANY causes of PN, other simple tests should be checked first...starting with basic blood work. Maybe mrsD will pipe in with a good list of the basics in regards to diabetes and vitamin/mineral checks. I'd also like to hear if others have found specific tests (new or otherwise) helpful. Word of mouth is sometimes the best source of information.

I completely understand the frustration of not-knowing what's causing all the symptoms & pain. I struggled with finding a proper diagnosis for over 15 years. Therefore, maybe asking about what new testing is available at academic facilities can open the door for not just a definitive answer, but better treatment options as a result.

Thanks for making this post, and sharing information about the new MRI protocol available here.

Glenn is our "test" expert, having had many of them himself and informative discussions with his doctors.

This will be a great thread for new posters to consult!

Edit to add: This video explains the dorsal horn of the spinal cord:
http://www.medscape.org/viewarticle/754961
If you don't have a free membership, this link will prompt you to make
one. It is easy and free and Medscape has wonderful information worth signing up for on many health topics.

Let me add by saying that this new MRI (as well as any other MRI) is now available to patients with pacemakers.

I have a pacemaker and was a part of their pilot program. However, this is likely ONLY still available at Johns Hopkins, but they told me the study papers were being finalized very soon so that all facilities can utilize the same protocol.

http://www.hopkinsmedicine.org/heart...rillators.html

Again, it is NO fancy or different type of MRI, just using a different protocol (with very strict monitoring) so that pacemaker patients can benefit from these diagnostic tools also.

En bloc--
 

--have you seen the Liza Jane spreadsheets?

This was put together by Liza Jane--with considerable input from a number of us--to be as comprehensive a series of possible tests for neurological symptoms as current science allows, and it gets updated periodically:

www.lizajane.org

The Useful Websites "stickies" also has a number of entries at which dignostic protocols for neuropathy can be found--those given by Washington University's Neuromuscular conditions website and by Dr. Latov of Cornell Weill and Dr. Poncelet of University of California/San Francisco most prominently.

Thanks Glenn.

Yes, the "stickies" have lots of good info and links. Many of these things would have been sooooo helpful to me back in 1996 when i started my diagnostic journey. The Lizajane charts are a good idea for those who are just getting started and those with many questions about what needs to be or what has already be done...with places for comments on results.

Being that my problems include autonomic neuropathy, others systems (cardiac, GI, etc) are greatly effected. Therefore I use the same concept of file keeping as Lizajane suggests, just breaking them down by specialty.

Diagnostics is an ever changing field. I had been diagnosed for several years with multifaceted autonomic neuropathy, Sjogren's, and APS. However, as you know, PN pain can be difficult to pin down. Due to my pacemaker being implanted 7 years ago, I was unable to have an MRI...which excluded looking for a wide variety of possible causes for my PN.

Being that Sjogren's can attack both the brain and spine it was always felt there was inflammation in these areas. However, it is only now that I have confirmation of the ganglionitis thanks to 2 new protocols in MR imaging: allowing MRI's to be done on patients with pacemakers and also newer/closer looks at the DRG itself. My goal in posting this was to let people know that these new protocols exist and can help in the diagnostic process. I hope it's helpful.

Here is a new article explaining more issues with dorsal root ganglia and sensory neuropathies:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3287412/

more on dorsal root damage:

http://www.hindawi.com/journals/ad/2012/873587/

This explains damage very clearly:

http://www.myelitis.org/newsletters/...tter4-2-06.htm

I started reading about dorsal root ganglia since Mrs. D updated your thread. I inquired last Friday from the clinic where I normally get my MRI about the DRG protocol. The technician I spoke to is not aware about a special protocol to specifically look for the dorsal root. However, he said that if the doctor will say what particulars to look for, they might be able to do so.

As you may understand, it is so difficult not knowing what is causing the changes in your system. When you continue to feel the pain, you start the route of diagnosing yourself, which I am very much aware is not healthy.

I believe you said in other threads that your skin biopsy was done at Hopkins, so I'll assume you are referring to a clinic at Hopkins for the MRI. I don't know whether a 'technician' will know about the DRG Protocol. The radiologist that actually read my MRI was Avneesh Chhabra, MD. He specializes in MR neurography (even wrote a book with the same title). I don't know if you mention a name whether it would help get you this specialized test or not...but it can't hurt. I would ask your neurologist about it (if he is at JH), not the MRI clinic. My test was ordered through my neurologist.

I will add that my neurologist specifically asked them to look at the DRG due to my Sjogren's diagnosis. I have also asked a radiologist on this forum about this protocol. He said he had not heard of it specifically under that name, BUT that a strong enough magnet could definitely see the DRG.

I understand (I really do), the need for a 'cause', but treatment is the key. IF you can find something to alleviate your pain/suffering, then the cause is just a name. It took years and years for a diagnosis of Sjogren's for me. Even though I had known in my mind that there was ONE cause for all my secondary diagnosis, I had given up hope of ever finding out what it was. I was always more concerned with finding a means to control my symptoms. I think we have ALL considered diagnosis for ourselves at some point in our medical journey. Being your own advocate with your doctor is the best thing you can do for yourself. Stay on top of your testing and learn about your symptoms so that you can have productive discussions with your doctor.

My skin biopsy was done here in Jacksonville by my neurologist. However, i was informed the specimen was sent in John Hopkins. The result has the name "THE JOHN HOPKINS MEDICAL INSTITUTION" on its written report . I called the Cutaneous Nerve Laboratory of JOhn Hopkins to get my report which shows the "numbers", but I was asked for a reference number from my doctor.


I agree with you that treatment of the symptoms is very important. But, I am really hoping, while I am still in the "early" stage of my neurological symptoms, to at least find teh cause. I always read that treatment of the cause of the nerve damage is crucial. I am thinking that if we could find the cause now, I may still have the chance to improve. Along side the efforts of finding the cause, I am taking taking actions to alleviate the symptoms by taking the supplements that can help heal the nerves.

Thank you en bloc for all your answers. I hope you will not get tired of replying to my inquries.

Dear Mrs.D,

Please bear with me, I just wanted to make sure my understanding on DRG is correct - damage on DRG does not reflect on skin biopsy? I want to tell this to my doctor in my next visit.

You know sometimes, when I read I understand the words (as individually written) but i could not somehow connect their meaning in the whole context of the idea:D

Thank you.:hug:

In humans so far we don't know the answer to this question.

But I did find a link to a study in animals suggesting that
dorsal root damage precedes sensory neuron death in the periphery.

http://www.ncbi.nlm.nih.gov/pubmed/21924225

Thank you very much Mrs.D!

Actually--
 

--the original paper by Dr. Abhey Mogehkar of John's Hopkins on small-fiber neuropathy secondary to damage at the level of the dorsal root ganglia (which I haven't been able to find an active link to this morning, though I think I actually have a hard copy somewhere in my files) does mention that one of the distinguishing characteristics leading to suspicion of DRG involvement is when the skin biopsy results are NOT length-dependent.

Also, see this description from the Washington University neiromuscular website:

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

When you say "NOT length dependent", does it actually mean that the result of the skin biopsy is normal? Sorry, just want to make sure that I understand it.

No, not necessarily--
 

--the more likely possibility is that samples taken from all areas will show intraepidermal nerve fiber density reduction, and that there will not be a pattern of greater reduction in samples taken farther from the center of the body (such as at the ankle) vs. more proximal areas (thigh, upper arm).

In length-dependent neuropathies, it's the fibers that are farthest from the body that show damage first, and that show greater damage as time goes on. this is thought to be due to the fact that much of the damage, whether diabetic, toxic, autoimmune, etc., is thought to be primarily circulatory/ischemic in nature, in that damage to small blood vessels results in nerves not getting enough nutrients/oxygen and being unable to efficiently remove waste products, and the farther this is from the center of circulation, the more likely this is to occur--it takes efficient circulation to do this in tissue in the feet, for instance, where blood also has to fight gravity.

non–length-dependent small-fiber
 

I encountered an article today about non–length-dependent small-fiber sensory neuropathy. I could not copy it in the Forum.

http://onlinelibrary.wiley.com/doi/1...mus.22255/full

The article says that patients with NLD-SFSN, often develop SFSN symptoms and signs in a patchy distribution. This can include the face, upper limbs, or trunk before the lower limbs, or the latter may be involved simultaneously. This pattern of distribution suggests that NLD-SFSN most likely represents a ganglionopathy.
Also, it says that non-length dependent neuropathy shows reduction of IENFD on the skin biopsy.

Now I wonder, if my normal skin biopsy would fall under the non-length?

Thank you Glenntaj.
My skin biopsy says normal epidermal nerve fiber density and appearance from both distal leg and proximal thigh. Though, there might be a possibility that my coondition is DRG, but I am still hoping its not so I am still trying to find rhyme and reason on it.

I had no idea PN could affect proprioception , this was the first neurological symptom I actually had some 8yrs before my pn became apparent, is that possible ?

m

Why not? If there's a tissue in the body, there's a few diseases to match! I sometimes have phantom vibrations and my neurologist suggested it could be due to proprioceptive nerves getting hit. (But I mostly have the tingling/numbness/pain/burning thing going on.)

Thank you so much for the links! These are the clearest explanations I have seen. I was diagnosed last year with SF Neuronopathy (Ganglionopathy). It was confirmed by skin biopsy and the fact that my entire body is affected including my face and tongue. One thing that continues to baffle me is if the C fibers have indeed died back why do I not experience any deficit in my sense of touch? I only have these awful perceptions of burning and pins and needles. It would be awesome if you might point me to that type of information. It is a remarkable disease... Thank you in advance.

It depends--
 

--on which fibers are preferentially affected; there are subcategories even within the smaller fibers.

Most actual mechanical touch receptors, though, are thinly myelinated fibers, not unmeylinated "small" fibers, which generally subsume the sensations of pain and temperature.

Thank you so much. I assumed that it had to be something like that. It is strange, however, how stretching the skin slightly like closing my hand or the breeze on my face or the movement of my clothing can be painful.

Any idea what contrast is used? I had a contrast MRI (not nerve related) that used gadolinium. Nothing bad happened, but I was definitely worried about injecting a heavy metal into my body and would like to avoid it if possible.

Hi. I have the same over-sensitivity in my skin - legs, arms sometimes back and face. I also have painful feet. So far, there has been no cause found in my condition. Mine started in all the areas I mentioned. Do you feel pain with the touch of your sheet? I do.

I don't know if this common, when I wake up in the morning I don't feel the stinging (like sunburned skin), burning sensations. But once I get up, there it goes again.

I take gabapentin and a whole bunch of supplements. I just recently increased my gabapentin dosage from 1200 to 1500mg. It makes the pain tolerable. For two days now I'm using Lidoderm patch for the feet. It is not 100% pain free feet but it makes me move around.

Gadolinium:

Here is more information on this contrast agent:

http://www.fda.gov/Drugs/DrugSafety/.../ucm142889.htm

Video illustrating dorsal root pathways:
 

http://www.medscape.org/viewarticle/754961

This Medscape video is very good at describing the dorsal roots along the spine, and explaining neuropathic pain.

You may have to pause it when the slides come up listing treatments, as they are very brief.

This video requires a membership to Medscape which is free and easy to do. Medscape from then on will be accessible to you and there are many good features there on health topics in general.

Hits home with me
 

There is a strong circadian pattern to my NLD SFN/G. When I get up in the morning all is well. However across the day, burning in my thighs, arms and face gets worse. I'm typically most miserable in the evening when I am trying to relax after work. It may be that I have fewer opportunities to get fully engaged in a task that limits my awareness of pain, but watching TV at night is a high probability time to notice pain. Probably a statement on the quality of TV programming. :)

I would wonder if you have an inflammatory thing going on?

Late afternoon is when your cortisol levels dip, and they come up a bit after dinner. Then fall again around 4am. Cortisol is your natural anti-inflammatory, from the adrenal glands.

You could try our AlkaSeltzer original formula trick, to see if
it helps. If it does then you have some inflammatory thing going on with your PN. AlkaSeltzer works fast, and has aspirin in it in solution, so is less irritating to the stomach. Do not use if you take blood thinners or have a bleeding disorder.

There are several versions of AlkaSeltzer, don't choose the Cold types. Original formula is the one that works.

Inflammatory types of PN can be arthritic, lupus, compressions in the spine, etc. Eating an anti-inflammatory diet may help.
Also some natural anti-inflammatories like bromelain (enteric coated), curcumin, quercetin, etc, may work too.

Did I ask you about using any ACE inhibitor drug for hypertension? If you use one, this could be causing your problem too. They raise bradykinin in the body, which dilates blood vessels and this puts pressure on nerves.

I like the sound of...
 

... the "AlkaSeltzer Trick." It sounds like something out of James Bond. :) Is there a string you could point me to that discusses the specifics of the AT? (like when to take, what to look for?, etc,)

I am on Ramipril 10mg for hypertension. I'm not sure if that is an ACE inhibitor.

I'll try and post something in the new members section over the next day or two, as I am new to the board and just started posting in out-dated threads.

Thank you!

Mike

YIKES....you are using an ACE inhibitor and have PN symptoms?

That is a sign to get that drug changed. I am recovering from Lisinopril, which became so toxic I was having a nightmare suddenly of severe severe pain and swelling. You can get swelling in your throat suddenly, as the bradykinin builds up and have a major emergency! I'd discuss changing the drug immediately and see if your symptoms improve. I am suspecting they will.

On top the bradykinin elevation, I also had a drug induced lupus from the ACE inhibitor which made my joints inflamed and also
created a mysterious rash on both my forearms.

Your specific ACE inhibitor also can cause liver damage. Another reason to get it changed.

Please consider this, as it might fix your problems.

bradykinin:
http://en.wikipedia.org/wiki/Bradykinin

People vary in how they metabolize this bradykinin away.
Bradykinin reactions in normal people are called acquired angioedema. But if you have a genetic error in this metabolism, you could have another form of angioedema which makes you most sensitive and likely to have reactions.
This is called HAE..
http://www.haea.org/

I think anyone with PN taking an ACE inhibitor is at risk of this buildup of bradykinin and hence symptoms of that.
It took 10 yrs + for my reaction to go ballistic. So time means nothing if you have been on these drugs for a while. I am now on a very small dose of beta blocker which is working so much better on my BP and has no side effects!

As far as AlkaSeltzer goes just take one packet of two and see if things get better. If it does, that points to inflammation as a PN component. This form of aspirin is pretty safe because it is in
solution and therefore absorbed very quickly and doesn't hang around the stomach to irritate anything. If you use any blood thinning drugs, or have stomach ulcers you need medical
permission to use any aspirin product.

Rhyme or Reason...
 

I too am looking for a rhyme or reason. I have an apparently "normal" skin biopsy result in that the number of fibers fall well within the diagnostic guidelines, but I do have small and medium sized axonal swellings. Without the biopsy showing a diminished fiber count, my neuro (probably rightly so) is baffled as to the body-wide intense burning and paresthesias I experience. From what I've read, doesn't involvement of the DRG have to demonstrate itself in at least a positive finding via biopsy, nerve conduction study or a deficit clinically of some kind? This thread is a little old, so I don't know if "Idiopathic PN" is still monitoring or if you have had any success yet in finding your "reason." I hope so.:)

Actually--
 

--I think you are showing some evidence of nerve damage--those axonal swellings. Large scale axonal swellings are not typical and imply some sort of immune attack, with lymphocyctic inflitration of the nerve tissue.

Also, while you may fall well within the guideline for "normal" nerve fiber density, that number is only a snapshot in time and doesn't tell us where you were before symptoms hit, and whether you would have had a higher/lower number then--I've written often on how the McArthur protocols rather arbitrarily define normal intraepidermal nerve fiber density as below the fifth percentile or above the ninety-fifth percentile for age-matched "normals", and how even if you get a "normal" finding, of being, say, in the twenty-fifth percentile we would not know if three years ago you would have been at the fiftieth percentile.

It is good, though, that skin biopsies are repeatable--often it is the tracking of percentiles and nerve fiber condition over time that allows for more of an interpretation as to progression or healing. Mine have gone from the second to the eighteenth percentile over time--so I am now technically "normal", but it is more important to note that I have gotten some re-enervation over the years.

Thanks so much for this...
 

I really am fascinated... a bit excited... about what you write about lymphocyctic infiltration or immune attack on nerve tissue because maybe it's closer to an answer. I have had a persistent skin manifestation of the virus I had over two years ago called levido reticularis and the biopsy of that (not the skin punch biopsy for the nerve fiber density) showed a chronic inflammatory perivascular infiltrate. All testing for vasculitis is negative though and the area of the nerve punch biopsy did NOT show any inflammatory infiltrate. So, conflicting results within a close time frame. I really am lost as to why my neurologist hasn't honed in on this. And, even if he did, I haven't been lead to believe there's anything available to me but symptomatic treatment (Gabapentin). It worries me that I have a progressive inflammatory condition that is not being addressed and it will eventually result in irreversible damage. Really, so many thanks for sharing all your knowledge with us via Neurotalk.

I have to ask this.... As I had a terrible skin problem
that was finally diagnosed as drug induced lupus.

The culprit was lisinopril an ACE inhibitor for blood pressure.
All that family is implicated in this reaction. I had been on
it for 10 yrs.

If you Google "drug induced lupus" you will find at the
lupus organization a long explanation about this and
a list of drugs so far identified (but it is incomplete) for
some. PubMed searches will be better. That is where I
found my data for myself. Use generic name + lupus
in the keyword search there.

Livido reticularis is an autoimmune sign. Drug induced
Lupus does not raise ANA levels so doesn't show up
in that testing.

Heb1212,

I haven't found that elusive rhyme and reason:)... yet. In fact, the pain has slowly gotten worse. The last time I saw my neuro, I was given a food supplement called Metanx. He sounded positive on the effectiveness. For someone who doesn't lose hope in getting better, I was optimistic. I only had 2 days of "feel better" after taking it. After 2 days, it came back to the "normal" pain.

Mary

Mary... gosh, so very sorry. Mine too has gotten worse over the two years since onset, which so discourages me because I worry constantly about the progressive nature. When will it level off? I'm trying my hardest to adjust to my new "normal" too. I wish you God's comfort:hug:

Diagnostics & Testing~~ for dorsal root ganglia and damage:
 

This looks very promising!


https://www.ucsf.edu/news/2012/05/12...tion-lab-study

N1

Here is another link on helping fix damage to the dorsal roots:

http://www.cellr4.org/article/809