Being a patient with CIDP/Autonomic PN for over 45 years due to exposure to Agent Orange in Vietnam, this is a subject with which I have a lot of hard earned experience.
So, let me be clear. The over two hundred doctors I saw for my symptoms from 1969 to 2003, including those noted below are highly trained medical experts. So what was the problem with getting an accurate diagnosis and treatment during those decades?
For over three decades, upon complaining of symptoms since 1969, I was told by the doctors, “All tests are normal”. They implied, and I assumed this meant, I did not have anything wrong or did not have neuropathy.
One neurologist in 1979 did hedge his opinion stating “probably not neurological.” The word ‘probably’ without providing a medical opinion as to what may be wrong was not very helpful. So what was the problem?
In 1983, fifteen years after the horrific symptoms began; I became upset when the doctor said to me, “Why do you want something to be wrong?” Now I knew something was wrong and I wanted it fixed? Of course the doctor meant it was a mental problem and I concluded at the time I surely must be crazy. So what was the problem?
In 1999 after a doctor finally told me I had idiopathic peripheral neuropathy, I was sent to an expert neurologist who tested my reflexes and with compression on the leg he managed to get reflexes. Based on his special reflex test, he dismissed the diagnosis of idiopathic peripheral neuropathy, stating “patient has reflexes upon compression – patient is claiming to have something he does not have”, almost destroying what remained of my mental stability!
Not until years later did I understand that even the diagnosis that included the word ‘idiopathic’ was part of the problem involving the lack of clinical medical training in understanding the tests and what they do mean or do not mean or measure!
A neurologist in 2005 seven days after receiving gamma globulin for my diagnosed condition and at the peak of the infusions positive documented effect on my neurological illness, dismissed the diagnosis stating, “I cannot find any reasons for patients symptoms.”
Today, we have come a long way in the increase in information on Neuropathy, the increased understanding of neuropathic pain, and the skill of physicians to interpret the tests that are available. But it still takes a highly trained neuromuscular neurologist with special training in this area to diagnose and treat neuropathy. To understand the complexity of getting a diagnosis, perhaps read what Waden Emery III, MD Clinical Neurologist states about What is Neuropathy?
So the question I asked was – if you have neuropathy – so why are tests normal? Thank heavens that because of the hard work of many, we can answer that question clearly.
Here are TEN CURRENT FACTS every neuropathy patient must know!
FIRST, there are no objective tests for diagnosis of neuropathy and early in the illness; the doctor may have only your medical history and a subjective description of your symptoms. The current tests measure any damage to the large or small fibers, axon or myelin, and whether it is a single neuropathy or a polyneuropathy (affecting many nerves).
SECOND, neurology does not have the tools to diagnose peripheral neuropathy objectively, UNTIL damage can be measured or its affects are seen on the body (i.e. deformed feet in CMT, GBS patient can’t breathe, MMN patient can’t move an arm, or multiple issues with digestion, urination, sweating, etc.- See Dr. Norman Latov’s book for definition of acronyms.)
THIRD, peripheral neuropathy is diagnosed by ruling out possible causes of your symptoms. The tests done only look for that cause, not whether you have neuropathy or not, thus many tests are normal! Yet the type of neuropathy, which can often point toward a cause can be determined by these tests and the neurologist must indicate the type, not idiopathic which is not helpful. The failure to find a cause from the tests conducted rules out that cause, not your neuropathy.
FOURTH, testing is done to determine any measurable damage to the nerve or covering of the nerve and perhaps point to the possible type of neuropathy, not whether you have peripheral neuropathy or not.
In 2015 more doctors are understanding the need for the skin biopsy if the EMG or Nerve Conduct Tests do not show damage to the large fibers. More doctors are understanding the value of the spinal tap in diagnosis of an immune mediated neuropathy or the value of the genetic testing that will provide the patient with important affirmation!
FIFTH, most objective tests only determine the extent of damage to the peripheral nervous system, but if you have no or little damage this does not rule out a peripheral neuropathy, as it many chronic progressive neuropathies it can take years before damage can be measured.
SIXTH, the medical literature, including statements by Dr. Norman Latov of Columbia University, note that in Peripheral Neuropathy, reflexes may be absent or diminished, but not always, so alone this is not a standard for diagnosis of or dismissal of neuropathy.
SEVENTH, symptoms including painful skin on touching or sensations of pin prick, or sensitivity to hot or cold, as well as other symptoms, remit and relapse in patients with peripheral neuropathy and thus are only reliable for diagnosis when correlated with ALL available information including the patient’s medical history.
EIGHTH, treatment by gamma globulin does reduce the symptoms of immune-mediated neuropathies and this positive response confirms the diagnosis, thus a physician should not dismiss an existing diagnosis based on the absence of the symptoms following the infusion of gamma globulin or other effective treatments.
NINTH, as noted by Russell Chin, MD, Neurologist in New York City, we need better clinical training for doctors including neurologists both in the medical schools and updated clinical training for practicing physicians. The clinical training of all neurologists and physicals working with neuropathy patients must be a top priority for medical and neuropathy organizations.
TENTH, we desperately need MORE neuropathy research seeking better diagnostic tools for doctors, more treatment options, and improved approaches to treatment of chronic neuropathic pain.
Pam Shlemon, President of the Foundation for Peripheral Neuropathy is working with researchers to deal with the problem that very few research projects even make it to phase 3 trials and the question is why? Hopefully, her team will discover some answers and provide better direction for research.
An early diagnosis of the cause of a neuropathy and approaches to treatment are critical for many patients to prevent serious disability or worse from damage to the peripheral nervous system.
One could call these the TEN COMMANDMENTS for diagnosis of peripheral neuropathy!
Gene,
First, thank you, outstanding information. And second, any recommendations on who are the best doctors for PN in the Boston and New York areas? Thanks again!
John: Let me go look and get back to you.
Dear Sir:
Thank you so much for the single most comprehensive and compassionate site on PN. I am a complicated case and just received another devastating eval from a top neuro. I would really appreciate the opportunity to speak with you if that could be arranged.
Thank you
Sir:
My neuro just dismissed me as “dramatic”, even though my ENFD dropped from “within normal” to “low normal”, with axonal swellings in a few months.
I have two questions I cannot figure out, even from Donofrio’s book.
Based on your history and knowledge ( I know you are not a doctor)..
1. Would a spinal tap be useful in a suspected Toxic Neuropathy
2. Wouls a spinal tap be useful in a suspected disseminated HSV if I have been on Valcyclovir for 6 months.
thanks
Kim
I removed your last name from the previous post. Hope this is okay. A spinal tap is helpful in diagnosis when inflammation or infection is suspected according to Dr. Latov. Most immune mediated neuropathies will show an elevated protein in the spinal fluid, however not all… it is like the other tests…. but if the abnormal proteins do not show up it DOES NOT rule out an immune mediated neuropathy. Absolutely it would help in any toxic neuropathy, but I stay away from the use of the word toxic. It is better to just use immune medicated because toxic neuropathies are immune mediated and toxins attack the immune system. Lots of political influence out there that down play toxic neuropathies to protect chemical company gifts to institutions. My opinion and experience. In Dr. Donofrio book look in the index under Cerebrospinal fluid. As for the use of Valacyclovir for herpes here is what I found. See http://www.drugs.com/sfx/valacyclovir-side-effects.html. Hope something here helps as the side effects for this drug would certainly confuse any doctor as to where they are related to your neuropathy or to this drug. In the opinion of many doctors, if you stop the use of the drug and the side effects decrease or result then it was the drug.
Lt Col: Thank you, sir for your reply. I JUST now found it, as I was looking for something else…
I have learned a lot, and have a lot of questions as well. Donofrio’s text is stellar.
I think I was not clear – I believe this is (“immune mediated” – TOXIC – THANK you for the distinction, i found it confusing that you called your “immune mediated” when you KNOW in your heart it was that Evil Chemical) and can trace onset 14 months ago back to courses of Nitrofurantoin, which I have used for DECADES for chronic UTI’s. I have a very complicated history prior to that – have been disabled for 30 years ( use a wheelchair) due to neuropathic pain from bilateral sciatic nerve trauma (described as “tortuous” when they tried to fix it and found extensive AVM’s within and around the nerve). But I managed, I swam thousands of miles, and I coped. 14 months ago all I wanted was to fix my shoulder tendinosis so I could keep undependent with my wheelchair and keep swimming. Then, this sudden, abrupt onset of Bodywide ( started hands and feet – now full arms, legs, mouth and head) neuropathy. As if someone had set my body on fire. – severe pain along with profuse sweating, heart rate and other autonomic issues For months I sought diagnosis, all the while still taking more courses of the Nitrofurantoin. FINALLY was referred out of town for Punch Biopsy. It was looking back that I realized CRAP! it started coincident with a UTI, and progressed with each course ( I had 7 full courses in the 6 months of onset). 2 punch biopsies – I am watching my IENF drop, and axonal swellings, etc., but my “top neuro” at a teaching hospital tells me that the severity of my complaints are inconsistent with his objective findings. Finding NO relief from now the 3rd drug I am on and am heading down the dreaded Narcotic trail. I have many comorbidities, HSV2, HLAB27, etc. I have some questions, but would really like to take them “offline”.. With much MUCH gratitude, Sir.. for the work you do. Please email me if you reply
P.S. having a Spinal Tap this week.. a bit apprehensive .. your thoughts on that were very helpful. thanks
A spinal tap is rather simple…. the trick is to lay very still for a period that they will tell you to prevent headaches afterwards. Sounds like you are getting some help? Are you seeing a Neuromuscular Neurologist? It would be better if we had this conversation via E mail at prcgene@aol.com
An immune mediated neuropathy is caused by many things… and one of them is toxins as in CIDP or MMN and in GBS it is an infection they think! Immune Mediated only means that something is causing the immune system to attack your peripheral nerves.
I c cannot look in Donofrio’s book. I borrowed it… it is pricey, and wort.h every penny for those who can afford it!
Even though I have had two EMG tests that were normal, I have definite neuropathy symptoms. I feel like I am walking on pepples, my legs tingle constantly. My hands and feet get hot, bright red and painful in the evening, along with painful long lasting foot cramps. I have had nerve pain in my cheek and am constantly constipated. Since my EMG test came out normal, my doctor has dismissed my complaints and is unwilling to listen. I am desperately in need of a neurologist that can test me for other forms of neuropathy, such as those involving the small fibers. It might also be important to note that my father suffered from the same problems. When he died, it could only sleep in a reclining chair with a sheet tented over his feet .
I have been unable to find a physician in Connecticut that performs skin biopsies for other forms of neuropathy. It is my understanding that you can have normal EMG tests and still have neuropathy. Can you provide any assistance to me?
Susan: I am going to send you an E Mail with information that may be helpful. It is quite possible that you have a hereditary neuropathy and it is affecting the Autonomic Nervous System. You need to have a test called Evoked Potentials to look at this issue. You also need to find another doctor!!! A Neuromuscular Neurologist is often able to diagnose when others cannot but if you have this in your family the diagnosis is staring them in the face! But I will send you some information in an e mail that will be helpful. Gene
Col Gene,
I was a CWO Cobra pilot and flew “cover missions” behind the Ranch Hands spraying Agent Orange in RVN in 1970. We inhaled that stuff and knew no better in those days as you know. I have had burning legs starting from bottom of feet to back of buttocks for over 30 years. I have been diagnosed with neuropathy by 2 neurologists and the VA has denied me any benefits except the 10% for Coronary Bypass connection to Agent Orange. Two weeks ago I had a spinal nerve stimulator implanted to hopefully help so I can at least get better sleep. So far, very little help but am still working with technician to find sweet spot on stimulator setting. I also take Gabapentin which helps a “little” for my shaky hands and arms but not the burning legs. Any advice dealing with VA and any on the spinal implant.
Jim: I will reply by E mail as the guidance I have for you is long and it will provide a way to fight back at the VA on appeal. Gene
We really need help! Jim has had neuropathy for about 2 years he’s been on gabaoentin lyrica and now cymbalta none of this works! He is a retired military vet him being 49 years old he was a airplane mechanic and took a civil service job doing the same thing. We have been to a few neurologists with no answers as to what is causing this. All blood work and Mir has come back with no issues. Help us please he hasn’t worked in a month and no one seems to be able to find out why his pain has become so severe.
Could this be caused by exposure to jet fuel over 29 years?
Shannon: I will send you an E mail with some questions and ideas. God bless Col Gene
First let me thank you for your service.
Second I’m so happy I Googled “Can a EMG text be wrong and your page came up.
I’m beside myself and feel like I’m crazy.
Let me give you some history. In 2005 after several years of suffering with bilateral carpal tunnel I had surgery on the right hand.
My hand was never right after surgery. I had severe pain ,burning like fire ,swelling and unable to touch. I was finally diagnosed with CRPS. (RSD).
I have been diagnosed with Fibromyalgia as well. My symptoms have become worse, my legs muscles are in constant pain. My feet are always on fire with numbness.
I have fallen several times.
My Cronic fatigue is horrble Fibro stinks
I have had abnormal brain scan every 6 months and abnormal scans of lower back.
I am on meds for neuropathy and Fibromyalgia and a pain medication.
Because my symtoms have declined my Doctor sent me for a Emg for legs and lower back. Guess what? The EMG is normal!
How can an EMG be normal when your feet are numb, burning and I had back pain? My Doctor thinks I may have MS. OH I forgot to mention memory loss and vision problems.
I really am confused. The Neurologist says nothing is wrong. She wants me to do physical therapy.
I know something is wrong. It’s not all in my head. So I just want to Thank you for all your information.
Deb: I am so sorry you are not getting the help you need. First let me say that an EMG and Nerve Conduct Test only measure damage to large fiber nerves and do not rule out a neuropathy. You can still have neuropathy and have no damage show up to the large fiber nerves on this test just yet. This is assuming the doctor was trained and knew how to read the test! Second, small fiber damage can only be measured by a simple skin biopsy. I will send an E mail regarding the information that you can give to your doctor to order this kit. But when it comes to testing, NO TEST CAN DIAGNOSE neuropathy as that is done by your history and symptoms in the hands of a skilled trained Neuromuscular Neurologist! If the neurologist said you are to do physical therapy at this point without knowing what is wrong, I would find another doctor. Physical therapy is good IF done by someone who knows about neuropathic pain and nerve damage with low impact therapy, otherwise it can be the worse at this point You need a diagnosis as to at least the type of neuropathy and you do have a neuropathy from the description of your symptoms. If the brain scan was abnormal why was MS not diagnosed… I know you are upset and confused, so I am going to stop here and I will send you an e mail with suggestions for help with the pain and diagnosis. Hang in there my friend.
Thank you for the great info. I’m currently dealing with the Va with numbness all over both legs with painful and burning feet as well as back pain. Have had two emgs both normal. MRIs indicated herniated disks in lumbar spine at l4 l5 as well as various bulging discs, arthritis and normal brain scan . Blood tests all normal. I have been tripping over my feet on occasion also. The Va have acknowledged my numbness but have offered no reasoning other than degenerative disk disease. I’m going on a year now and feel like there is no help as they say the normal emgs mean no neuropathy which I know is absolutely not the case. Ive had to reduce my work hours and change jobs it’s been crazy.
Chad: What this is called may be an ENTRAPMENT NEUROPATHY but it is neuropathy. This doctor needs a copy of the Textbook of Peripheral Neuropathy by Peter Donofrio MD Vanderbilt Medical in Nashville. As usually this VA doctor is not aware of the full scope of neuropathies. Normal EMG’S does not mean no neuropathy it only means there is no damage to the nerve that is recordable or they did not know how to read the tests. It could be both! I will look and see what I sent you and respond again by E mail.