Why Is An Idiopathic Neuropathy Diagnosis A Problem?

Today's longer post from neuropathysupportnetwork.org (see link below) is written by Lt Col Eugene B Richardson, who is a well-known and respected activist when it comes to dealing with neuropathy, especially when related to agent orange. Here he discusses the diagnosis 'Idiopathic neuropathy' which basically means that they cannot identify or trace the origin of your neuropathy. The problem is, that this can then encourage assessors to see your neuropathy as being more psychosomatic than genuine. Lt Col Richardson quite rightly takes them to task for this and again quite rightly insists that just because doctors haven't got a good enough testing system to accurately test for the cause of the nerve damage, doesn't mean that the nerve damage is in any way lessened for that. There are descriptions of a person's neuropathy that are far more accurate than just 'idiopathic'. The type of neuropathy can be identified and named, it's just the cause that can't be definitively established. This article is useful reading for everybody who is living with neuropathic symptoms - definitely worth a read.
 

The Problem with a Diagnosis of Idiopathic Neuropathy!
Posted November 15th, 2013 by LtCol Eugene B Richardson, USA (Retired) BA, MDiv, EdM, MS 

 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)?

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.

Read on to understand your patient mission: Help influence better awareness, clinical thinking and knowledge about Peripheral Neuropathy and the available tests in the health care system by learning, sharing documents, information, sources, and asking good “I” questions of your doctor. Become a partner in your own health care and a facilitator of change. In the process you may even get the help you need for your neuropathy.

We patients understand that there are NO tests to diagnose Peripheral Neuropathy per se, as this must be done based on the patients symptoms and medical history. The available tests can only either rule out a cause for the symptoms or confirm that damage has been done to the large or small fiber nerves.

However, failure to find either a cause or damage does not rule out a neuropathy. The symptoms of Peripheral Neuropathy are often present without the damage to the axon (nerve) or myelin (nerve covering) as that may occur later in the course of the disease. It is this focus in searching for a cause that sets the patient and doctor up for a diagnosis of idiopathic. As noted below, medical science does not know the cause for many diseases, yet they do not use the term idiopathic!

If you read the transcript of a recent Facebook chat on Idiopathic Neuropathy lead by Dr. Shanna K. Paterson it highlights a serious problem in using such a diagnosis as every patient question and the doctor’s excellent responses are focused on getting a “real” diagnosis and “real help” for the symptoms of peripheral neuropathy. (Dr. Paterson is the Assistant Clinical Professor of Neurology at Columbia University Medical College and Neurologist at St. Luke’s Roosevelt Hospital Center).

Medical experts must be trained to adequately interpretation the available tests whether it be the EMG, nerve conduct test, skin biopsy, blood work, spinal tap, evoked potentials, doctors examination, and the patients’ medical history of symptoms.

Understanding the results and meaning of these tests and information, combined with patient medical history often point to a more precise diagnosis, possible treatment options or at a minimum an idea that might help the patient.

I always loved Mims Cushing’s’ definition of idiopathic. In her book You Can Cope with Peripheral Neuropathy she defines it as being “idiot” for ‘idio’ and “pathetic” for ‘pathic’. In fact this bit of harsh humor hits the nail right on the head.

From the real world of the neuropathy patient it is difficult, with all due respect to health care professionals, to not agree with Mims definition of the term.
 
Idiopathic to doctors of course means of “unknown cause” and is a legitimate word when seeking the cause of some disease, but it is never used in MS or cancer and other ‘recognized or accepted’ diseases when the cause is not known! Why? Does research not focus on the cause of all disease to find answers that we still do not have?

The other issue is that for lawyers’ idiopathic means “no objective proof of a problem” providing the foundation for legal denial of help for the patient. Talk about frustrated patients! In both situations, this leaves the neuropathy patient without help that may be possible with a more precise diagnosis.

Medicine must cease at the clinical level the focus on neuropathy as simply a component of another disease. Peripheral Neuropathy must be acknowledged as a disease entity in and of itself, a disease of a major system of the human body.

Based on the available tests the doctor should be able to diagnose any of the following rather than fall back on the term Idiopathic Peripheral Neuropathy.

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.

peripheral neuropathy or polyneuropathy
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)
Guillian 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 go to this link: http://neuropathysupportnetwork.org/blog/2013/01/guidelines-for-veterans-va-proposed-law-agent-orange-and-peripheral-neuropathy/#more-1086 and send an E Mail to gene@neuropathysupportnetwork.org for more guidance.)

When left with a diagnosis of Idiopathic neuropathy, the patient is left without affirmation that is important in the strange world of neuropathy symptoms, robbing patients of self-esteem.

To name something that is real to the patient, is to provide an emotional/cognitive handle on the strange world of the neuropathy patient and their families.

From the experience of some neuropathy patients, a diagnosis of idiopathic often results in a not too subtle a suggestion that the patient has a mental illness (since all tests are normal and there is no cause in must be mental) and the statement is used as a club against the patient to send them on their way. Many neuropathy patients have been here including me.

I even remember the doctor in the military who asked me, “Why do you want something wrong with you?” when he said, “All tests are normal.” It was easier to attack the patient than acknowledge a lack of clinical knowledge and skill or that all they had determined was that these tests were normal, nothing more.

Even more shocking, an esteemed teaching neurologist at a major University Neuropathy Center in 2001 noted that the patient had reflexes upon compression. He then dismissed the obvious diagnosis, attacked the patient by writing in the medical record “patient is claiming to have something they do not have”, without knowing that sometimes reflexes are absent, sometimes they are diminished, and sometimes they are normal. He ignored all the objective tests to reach this conclusion and he is a highly respected teacher of neurology.

The impact on reflexes is hardly a standard on its own for the clinical diagnosis for the neuropathy patient and to ignore the actual results of the objective testing that was done and the long medical history was incompetence at the highest level.

To further frustrate the patient, if an employer finds about such a diagnosis, this can cost the employee their career. Been there and done that in my military and civilian career with supervisors who wanted to play doctor? Since the cause is unknown, it must be failure to handle stress both supervisors concluded.

Understand I did not receive even a diagnosis of idiopathic in the 1970’s all the way to 1999, so maybe with this use of idiopathic for a diagnosis, we patients can celebrate progress?

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. This diagnosis fails the patient clinically in both diagnosis and any possible treatments or responses to the neuropathy.

Medicare in 2013 still leads the way in supporting the dismissal of neuropathy by not paying for the blood tests that a doctor orders under the neuropathy profile!

POINT: The real value of this chat on Idiopathic Neuropathy is that it raises the question as to why do we need such a diagnosis, when testing in 2013 combined with the patients’ medical history, a more precise and helpful diagnoses is possible, even if “chronic axonal neuropathy” is used?

The Peripheral Nerve Society has developed a great source of reliable information and challenges via their journal and other experts have done the same. (See the Journal of the Peripheral Nervous System.)

Have you ever heard of idiopathic cancer? How about idiopathic MS or idiopathic Alzheimer’s? Okay, with MS there are liaisons on the brain so there is a test to confirm the diagnosis, but there is no focus on the cause. With cancer the same is true, but with Alzheimer’s the symptoms are there as in PN, but tests are not available to actually confirm the diagnosis until autopsy. So the real problem is that we do not have an actual test to diagnose Peripheral Neuropathy so we focus on CAUSES.

Over the year’s comments such as peripheral neuropathy is best thought of as something caused by other disease processes and therefore is not really a disease encourages the wrong focus. Every disease is caused by or involves other disease processes and these ‘accepted’ diseases do not use the term idiopathic even when our knowledge is limited or absent.

There are many diseases for which we do NOT understand or fully understand the cause or causes and that are caused by or involve other disease processes.

As Dr. Thomas Brannagan of Columbia University states, the simple reason is that too many health care professionals do not have the clinical training for the proper diagnosis and treatment of the neuropathies.

The challenging question is, “Why not train them?”

I recently went on several health care websites and with very few exceptions, every major disease is listed, but there are too many who do not even list Peripheral Neuropathy and I struggled to have these sites to consider listing it when there are more patients with Neuropathy than MS. They always list MS. Why? (See the Journal of the Peripheral Nervous System Vol.17, Supplement 2, at Page 44 top left hand column.)

So why is idiopathic neuropathy even necessary when a more helpful diagnosis is possible when a trained physician knows how to use and interpret the EMG, the Nerve Conduct Study, the spinal tap, the nerve/muscle biopsy, the blood work, a skin biopsy, a genetic test, and actually think about the subjective information provided in the patients’ medical history?

Dr. Norman Latov of Weil Medical College at Cornell University, shared in 2006 that for “one quarter to one-third of patients, no cause can be found and the neuropathy is called “idiopathic.” He notes that these are usually “axonal and may be sensory or sensorimotor” and “classified according to the clinical presentation”, with “therapy primarily symptomatic.” But again, I ask, why refer to them as idiopathic with the strong focus on the cause for peripheral neuropathy when this is not true of other accepted diseases processes for which we often do not have a cause?

Why not think out of the box with trial treatments such as was suggested in the article years ago in the use of IVIg? I suffered for decades and then years while a doctor with limited clinical skill thought all neuropathy patients MUST have diabetes and others used the term idiopathic. My disease was a form of CIDP and the treatment was IVIg. What other trials of treatment are available given a general idea of what is happening in the neuropathy patient?

Patient Challenge:

I strongly recommend that patients never rest on a diagnosis of “idiopathic” neuropathy, as in the experience of many neuropathy patients, it means someone may have failed to do the diagnostic work and/or does not understand the meaning of the tests we do have available. For me such thinking resulted in my severe disability and unbelievable suffering without support over decades with failed diagnosis and lack of thinking outside the box.

I remember the neurologist who was not clinically trained who wanted in a desperate way to make me a diabetic while resting on idiopathic. He delayed treatment another four years until I gave him the article on a trial of IVIg that worked for me. He was not aware of the usefulness of the spinal tap for such a neuropathy 2004. Then in 2005 for the first time my doctor, Dr. Waden Emery III, Neurologist in Lighthouse Point, Florida asked, “Why did they not do a spinal tap?” Limited clinical training!

In 2013 military veterans who are seeking help were told by VA doctors and claim reviewers that unless they are diabetic they cannot have Peripheral Neuropathy! This is frightening clinical misinformation for these patients.

You may want to provide the doctor with a copy of the document recently published in Neurology Today, March 15, 2012, volume 12(6); pp 30, 32-33 by Mark Moran. Read the entire article at: How to Diagnose Peripheral Neuropathy? No Simple Answers 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. If you want a copy of this document, send an e-mail to gene@neuropathysupportnetwork.org

If you want a good description of the symptoms and causes of Peripheral Neuropathy, read Dr. Norman Latov’s book (Listed on the Resource Tab) or go to these articles including linking to the website of the National Institute of Health. Give this information to the doctor if needed. Click here for the Link to the NIH:

Click here for the article on the NSN website:

Well, the Doctor may be insulted or get angry.

Yes, it may take a bit of skill using “I” messages such as “I wonder if it would be helpful to _____?” rather than ‘you’ messages to the doctor. ‘You’ messages are usually rejected and making the person defensive while ‘I’ messages sets the person free while stimulating thinking! This is true even if the thinking occurs after your appointment is over.

I remember when the military Men of the Chapel went fishing to catch sea bass off of Baltimore one year and we brought the large catch of fish to shore and gave it to the Priest who was feeding the poor of the city. In his office was a sign that read, “I give you fish and you sell fish. I am angry. So I refused to give you fish because you sell them. You are angry. Better you angry”.

Your mission: Help influence better awareness, clinical thinking and knowledge about Peripheral Neuropathy and the available tests in the health care system by learning, sharing documents, information, sources, and asking good “I” questions of your doctor. Become a partner in your own health care and a facilitator of change. In the process you may even get the help you need for your neuropathy.

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 by clicking here:).

Reference 3: “How to Diagnose Peripheral Neuropathy? No Simple Answers: Experts Offer Some Guiding Principles”, in Neurology Today, March 15, 2012, volume 12(6); pp 30, 32-33 by Mark Moran. (Send E Mail to gene@neuropathysupportnetwork.org)

Reference 4: Peripheral Neuropathy: A Practical Approach to Diagnosis and Management by Dr. Didier Cros, M.D. Editor, Lippincott Williams & Wilkins published 2001

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 by clicking here:).

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

PATIENT TO PATIENT – Disclaimer: Patient to Patient articles are educational, not diagnostic or prescriptive and the patient is encouraged to seek help from their own private physician.

Copyright 2013: Network for Neuropathy Support, Inc., dba Neuropathy Support Network.

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