Idiopathic Neuropathy: What do I do now?

A good start is to read Dr. Norman Latov’s book “Peripheral Neuropathy: When the Numbness, Weakness and Pain Won’t Stop” published by the American Academy of Neurology Press, available at Amazon for under $15.

You should also read, and provide your doctor with a copy of, the article “How to Diagnose Peripheral Neuropathy – No Simple Answers” published in Neurology Today, March 15, 2012, volume 12(6); pp 30, 32-33 by Mark Moran. Read

Unfortunately, this great article does fail to mention the diagnostic value of the spinal tap, a procedure that would have resulted in my earlier treatment years before my disability was serious.

It is important that you work with a Neuromuscular Neurologist who is Board Certified as they may have more knowledge regarding the clinical diagnosis and treatment of neuropathy.  Want to know much more?

If the blood work is normal and the EMG or Nerve Conduct Study shows no damage to large fibers, then request a Skin Biopsy to rule out a small fiber neuropathy. Learn more about a Skin Biopsy:

If you have symptoms of Autonomic Neuropathy affecting the bladder, digestive system, sweating, problems breathing, non-cardiac tachycardia, request a test called Evoked Potentials. Learn more about living with Autonomic Neuropathy

If you are progressively getting worse and none of the tests make sense or point to the problem, then request a spinal tap to check for an immune mediated neuropathy.  These are treatable with IVIg and other options the doctor may consider. Learn more about IVIg treatments

Many veterans’ exposed to Agent Orange, it is likely that you will be given a diagnosis of idiopathic. You must understand that the VA now will recognize Chronic Neuropathy as presumptive to exposure to Agent Orange under a new VA law as of 2012. If all other causes of your symptoms have been ruled out, then it is more likely than not that your chronic neuropathy is due to your exposure to Agent Orange. For guidance on applying to the VA for disability, Read Guidelines for Veterans VA Proposed Law on Agent Orange and Peripheral Neuropathy:as it will provide guidance and references to support the above and provide insight on how to appeal the VA ‘early onset’ requirement.   If you want a summary of the approach and references to use to support your claim, send an E Mail to gene@neuropathysupportnetwork.org.

Doctors View:

Idiopathic Neuropathy according to medical experts writing in the Journal of the Peripheral Nervous System should be considered as a disease entity in and of itself.  It is recommended that this condition be diagnosed as Chronic Idiopathic Axonal Polyneuropathy or (CIAP) as a major health problem.  (See reference 1.)

Patients View:

I would ask, what does the word ‘idiopathic’ adds to this diagnosis?  Why not use Chronic Axonal Polyneuropathy or (CAP) as this alone provides the doctor with enough information to help the patient.

I. The Problems:

> A diagnosis of idiopathic neuropathy can be a sign of a poor attitude about neuropathy on the part of the doctor.

> A diagnosis of idiopathic neuropathy can be a sign of an unwillingness or inability to spend the time thinking about a more helpful diagnosis on the part of the physician.

> A diagnosis of idiopathic neuropathy can be a sign that the doctor lacks the clinical training, knowledge and skills to interpret test results to diagnose and treat Peripheral Neuropathy.

> A diagnosis of idiopathic neuropathy can be a sign that the doctor is unaware of other diagnoses that are more helpful to the patient based on the testing that is available.

> A diagnosis of idiopathic neuropathy can be used by lawyers to claim that there is no proof that you have a disease called peripheral neuropathy.

> A focus on the CAUSE of neuropathy sets up the patient and doctor for incorrect assumptions and failed treatments or affirmation.

SOLUTION:  99% of these problems can be resolved by visiting a Neuromuscular Neurologist who is trained to understand neuromuscular diseases!

II. Problems Begin with Poorly Made Assumptions Without Knowing the Facts:

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ASSUMPTION: The doctor assumes that since tests done by the doctor appear normal, that I do not have peripheral neuropathy.

FACT: Peripheral Neuropathy is diagnosed primarily based on the patient’s symptoms and medical history and must be accepted from the patient at face value if the doctor is to help the neuropathy patient.

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ASSUMPTION: The doctor assumes that testing can diagnose Peripheral Neuropathy and did an EMG and Nerve Conduct Test and stated that I do not have Peripheral Neuropathy.

FACT: When considered along with the patients’ health history and symptoms, except for the Evoked Potentials tests for Autonomic Neuropathy and the Skin Biopsy for Small Fiber Neuropathy, there are no tests to diagnose Peripheral Neuropathy.

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ASSUMPTION: The doctor after blood testing concluded that I do not have peripheral neuropathy.

FACT: All the doctor can conclude from these tests is that whatever was tested is not the cause of your neuropathy.  It proves or disproves nothing else.

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ASSUMPTION: The doctor found a known cause for neuropathy and assumes that it is the cause of my neuropathy.

FACT: If a cause is found that is identified with neuropathy, it is then assumed that this is the cause of your symptoms, but this is still an assumption unless the cause is treatable and the neuropathy resolves upon treatment of the cause.

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ASSUMPTION: The doctor states all tests are negative and normal so you are told you do not have peripheral neuropathy.

FACT: When considered along with the patients’ health history and symptoms, the available tests either search for a cause or attempt to determine if there is damage to the axon or myelin and/or to the large or small fiber nerves.  If all these tests are negative, then all they prove is that whatever was tested is not the cause for the neuropathy and that until now there is no sign of damage to the peripheral nerves.  These tests do not rule out neuropathy only the cause tested or if there is damage, nothing more. They do not disprove your neuropathy if negative. Furthermore there are other causes for many of the symptoms that look like peripheral neuropathy and so the symptoms must be consider for what else may be happening to the patient.

FACT: The EMG and Nerve Conduct Tests only measure damage to the large fibers and the only standard for measurement of the small fiber damage is the Skin Biopsy.   Many patients are told they do not have neuropathy based on these two tests without the skin biopsy for small fiber neuropathy which is immune mediated and treatable.

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ASSUMPTION: The doctor assumes that since reflexes are normal on testing, that I do not have peripheral neuropathy.

FACT: Reflexes in neuropathy are sometimes diminished, sometimes absent, but not always and so reflexes are not the criteria in and of themselves for the diagnosis or dismissal of Peripheral Neuropathy.

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ASSUMPTION:  The doctor orders a spinal tap upon suspecting an immune mediated neuropathy and when it is negative assumes that this rules out such a neuropathy.

FACT: While the spinal tap confirms an immune mediated neuropathy, it is known that sometimes the elevation of the protein and other facts that are looked at does not occur, so a negative test, does not rule it out.

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ASSUMPTION: Agent Orange exposure does not cause neuropathy because of the blood/brain barrier, low levels of exposure, initial (so-called) research done on the issues of Agent Orange, and many other claims by the medical establishment and the VA.

FACT: This toxin and toxic carcinogen is now known to cause chronic peripheral neuropathy usually of the immune mediated variety.  Indirectly chronic neuropathy is caused by diabetes associated with Agent Orange exposure and by many cancers or other diseased now acknowledged are due to AO exposure.  Treatments for the cancers also cause chronic neuropathy and so this is indirectly related to Agent Orange exposure.

As Dr. Norman Latov MD PhD notes in his book, many patients have a genetic predisposition for these neuropathies which supports the fact that the blood / brain barrier is often crossed in many diseases due to toxin exposure.

Testimony before Congress in 2000 provided by a research expert from the University of Kansas noted that the initial research on the effects of Agent Orange on the human body is so statistically flawed to render the findings of no value.  This testimony was ignored by the medical establishment and the VA.   (Dr. R.W. Trewyn, PhD, (Holds a doctorate in cellular and molecular biology and was Vice Provost of Research at Kansas State University  Research Foundation) on March 25, 2000 provided testimony to the House of Representatives, Subcommittees on Veterans Affairs, in a hearing on Air Force Ranch Hand Study on the Health Effects of Agent Orange.  In his report in March of 2000, Dr. Trewyn notes the scientific shortcomings of the studies and noted the flawed mandated study of Army Chemical Corp personnel in Vietnam. He concluded the studies are statistically meaningless yet they have been the ‘gold standard’ for VA decisions for decades.)

These facts were supported by the findings of the Institute of Medicine in 2010 and confirmed by Veterans Affairs Administration law in 2012 as Chronic Neuropathy is now presumptive to Agent Orange exposure with the VA requirement of an early onset (symptoms within one year of exposure) during a period when medical science could not diagnose PN and did not fully understand the symptoms!

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ASSUMPTION: The doctors’ examination during my visit showed little or no signs of peripheral neuropathy, as I was able to walk better during the visit and he concluded that I do not have Peripheral Neuropathy.

FACT: Symptoms of Peripheral Neuropathy remit (come) and relapse (go) and it is always possible that when you were examined, your symptoms were in remittance and thus were not present during the visit. This is similar to relapsing and remitting of MS for whereas the cause in MS is different it affects the same Peripheral Nervous system as PN with similar symptoms! To understand the remitting and relapsing patterns of neuropathy read Will My Neuropathy Get Worse?

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ASSUMPTION: Only diabetics have neuropathy and you are not a diabetic, so you could not have neuropathy.

FACT: While it is assumed that diabetes is the leading cause of neuropathy, all diabetics do not get neuropathy, and there are over 100 causes for neuropathy.  With the failure to diagnose neuropathy, the truth may be that diabetes stands out as a cause, only because it is diagnosed and the other causes are not.

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ASSUMPTION: Idiopathic means that there is no proof of any disease.

FACT: Idiopathic means of unknown cause.  It does not prove that you do not have a disease called Peripheral Neuropathy.   Any attempt by lawyers to prove that idiopathic means there is no objective basis for disease, is a misuse of the term.

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ASSUMPTION: Idiopathic neuropathy is a helpful diagnosis.

FACT:  Idiopathic Neuropathy according to medical experts writing in the Journal of the Peripheral Nervous System should be considered as a disease entity in and of itself.  It is recommended that this condition be diagnosed as Chronic Idiopathic Axonal Polyneuropathy or (CIAP) as a major health problem.  (See reference 1.)

I would ask, what does the word ‘idiopathic’ add to this diagnosis?  Why not Chronic Axonal Polyneuropathy or (CAP)?

When left with a diagnosis of Idiopathic neuropathy, the patient is left without affirmation, which is important in the strange world of neuropathy symptoms.  Without affirmation patients are robbed of their self-esteem.

To give an illness a NAME for symptoms that are real to the patient, is to provide an emotional/cognitive handle on the world of strange symptoms for the neuropathy patient and their families.   (For a humorous but serious approach to this issue, see The Golden Girls TV show, season five, “Sick and Tired” Part 1 and 2.)

A diagnosis of Idiopathic neuropathy misses the clues of what might be done for the neuropathy, as options to consider are lost.  It too often shows that the doctor does not have the clinical expertise to read the tests with current knowledge about neuropathy.

Idiopathic fails the patient clinically in emotional support, diagnosis and exploration of treatments or responses to the neuropathy.

With the greatest of respect for the writers of the article, following forty-four years of living with neuropathy , a diagnosis of idiopathic neuropathy is a ‘failed diagnosis’ given the diagnostic tools and information medicine has available in 2013. Having been at the receiving end of idiopathic, primarily due to limited clinical skills or failed testing and/or failed interpretation of these tests by the experts, in my view and that of many patients, the use of idiopathic in diagnosis fails the patient and doctor.

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What a knowledgeable Neuromuscular Neurologist will be able to diagnose.

Based on the tests noted in the opening of this article, the doctor will be able to diagnose any of the following:

Examples to name a few, without including the word “idiopathic” which adds nothing but uncertainty, would include the following. You can add acute (two months) or chronic (beyond two months) or progressive (remits and relapses with increasingly worse symptoms) to any of these focused findings. While not exhaustive this list will provide you with the point being made.

axonal neuropathy or polyneuropathy

sensory neuropathy or polyneuropathy

motor neuropathy or Multifocal Motor Neuropathy

sensory/motor neuropathy or polyneuropathy

immune mediated  neuropathy or polyneuropathy

large fiber neuropathy or polyneuropathy

small fiber neuropathy or polyneuropathy

autonomic neuropathy

autoimmune sensory neuronitis

entrapment neuropathy (carpel tunnel syndrome) (common in diabetic and immune mediated neuropathies)

Gillian Barré Syndrome

Distal Symmetric Polyneuropathy

Chronic Inflammatory Demyelinating Polyneuropathy and variants

Or when there is a possible suspected cause of the neuropathy:

Diabetic neuropathy

Celiac neuropathy

Chemotherapy/Radiation induced neuropathy

Neuropathy in a nutritional deficiency

Neuropathy in alcoholic abuse

Hereditary or genetic neuropathy

Neuropathy in Agent Orange exposure***

Toxic neuropathy

Drug induced neuropathy Entrapment neuropathy

Neuropathy with IgM Monoclonal Gammapathy

Vasculitic neuropathy

Neuropathy in AIDS

Neuropathy in Lyme Disease

Diphtheric neuropathy

Sarcoid neuropathy

Neuropathy in cancer

Paraneoplastic neuropathy

Neuropathy in myeloma or POEMS

Neuropathy in amyloidosis

(* **Supported by the findings of the Institute of Medicine in 2010 and confirmed by the Veterans Affairs Administration law in 2012 as presumptive to Agent Orange exposure. For guidance in submitting a claim to the VA Click on link and send an E Mail to for more guidance.)

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REFERENCES:

Reference 1: Journal of the Peripheral Nervous System, Vol. 17, suppl. 2, Page 43-49 “Idiopathic Neuropathy: New Paradigms, New Promise”, 2010 and other issues of this scientific journal of the Peripheral Nerve Society.

Reference 2: “Peripheral Neuropathy: When the Numbness, Weakness and Pain Won’t Stop” by Norman Latov, MD PhD, AAN Press 2007. (Order from Amazon)

Reference 3:“How to Diagnose Peripheral Neuropathy – No Simple Answers” in Neurology Today, March 15, 2012, volume 12(6); pp 30, 32-33 by Mark Moran.

Reference 4: Textbook of Peripheral Neuropathy, Peter D Donofrio, MD, Editor, Professor of Neuropathy, Chief of Neuromuscular Section, Vanderbilt University Medical Center, Nashville, TN Published by DEMOS Medical, 2012.

Reference 5: You Can Cope with Peripheral Neuropathy: 365 Tips for Living a Full Life, by Marguerite (Mims) Cushing (Neuropathy Patient) and Dr. Norman Latov, MD, PhD, published 2009. (Order from Amazon)

Reference 6:“Sick and Tired” Part I and 2, Season Five TV show, The Golden Girls.(Order from Amazon click here)