Is neurology justified for ignoring central pain for so long? You decide.
Significantly, fifty percent of those responding to the survey, indicate that their present doctor believes what they are told by the CP patient and does not discount the pain. That leaves fifty percent, who are still not communicating with their doctor. We know CP is tough. There is such a spectrum and few clues as to whether the patient is in agony, or just likes to gripe, or neither.
In the end, it is a matter of just relying on the patient’s word, until the day when fRMI or something like it can grade pain severity. There is no doubt some high scores will go to CP patients, and everyone will be on much firmer ground. We have known those who spoke in moderate language, only to take their own lives, and those who speak bitterly of deep pain, which is nevertheless relieved by Ibuprofen. No wonder the clinician feels he/she is on quicksand.
The danger of ingratitude makes it hard to write this article, since we have been the beneficiaries of some of the most compassionate, intelligent, and wonderful care by neurosurgeons, neurologists, and anesthesiologists, whom we would not consent to be criticized in any way. Nevertheless, at a CP site, it would be wrong to ignore those who do not have access to the better pain clinics.
We praise the fifty percent of doctors who have taken the trouble to know about Central Pain and address these remarks to the fifty percent who are still having problems. Since these doctors are generally neurologists, we conclude that this is the specialty that most often becomes the interface between patient and medical care. Neurosurgeons and anesthesiologists also see pain patients, and there have been some very fine compliments to them as well.
We posit that neurology is not very good at detecting partial sensory loss and hyperpathia. “Not very good” as in leeches are not very good at relieving peptic ulcers. If the loss is total, you’ve got it. But the real measure is what you do with thirty percent sensory loss, or thirty percent ultimate pain. If all you can say with the tuning fork is “Do you feel that”, then you aren’t going to get very far with partial sensory loss. If your only interest when sticking us with a safety pin is, “Does that feel sharp”, then you don’t know how to test for dysesthesia/allodynia. Sorry. You just don’t.
Let us say at the outset, that neurology is a very difficult specialty. Those in this profession have words to fit diseases, but the diseases often refuse to fit the words. In no other profession, do doctors have such a hard time agreeing on the definition of terms. In few other injuries is the medical history more essential than in CP. Dejerine concluded the exam was not very helpful, but the unique medical history was the key. The medical history is NOT difficult, however, as Dejerine said. Bizarre burning pain, centripetally located, after injury to the CNS, makes the diagnosis.
Unfortunately, Dejerine’s criteria (AND Bowsher’s criteria) are just too easy. No, no no, this is just too odd Pain from light touch but not deep pressure. Preposterous! Taking off his white coat and putting on his vestments, the doctor transforms into an agent of the Spanish Inquistion, an honorary member of the Reverse Torquemada Society. “Under no circumstances wiil you claim to be in pain. You are an orthodox patient!”
Because pain, even intense pain, is usually minor and transient, we do not believe cases where it is extreme and permanent. The problem is especially acute when the patient is the type to sit stoically, rather than burst into tears.
Given the difficulty of getting to the office, if the CNS injured patient is there for pain, there is probably something going on. The SCI patient has other things to worry about beside pain, things that have credibility because they can be seen. Is it not foolishness to talk about malingering in a quadriplegic? Is it not inevitable that terrible wrong will be done to the pain patient if medical McCarthyism is the order of things. If the patient will not admit to a pain benefit from morphine, do we “have a fit and fall in it” and begin to cry “fake”, or tear our clothes and hold our ears as if blasphemy has occurred?
The doctor must be very careful before he starts the admonishment. Some “fundamental” assumption may simply have been disturbed by the CP history. It is the assumption that nerve injury pain must be like noxious pain. It is not unheard of to find a casual clinician, seemingly indifferent about seeing patients in wrinkled scrubs, who turns into a prissy Miss Manners when it comes to what he will accept from a CP patient about pain. We won’t mention the blood that IS on your gown if you won’t mention the tears that are NOT on our face.
The problem is that the doctor has to believe the patient. Since the clinician does not know Dejerine’s sufficient tests, to ask if the pain is “centripetal” (worse distally) and whether the zone of pain and non-pain is indistinct, and to test with von Frey hairs for sensory loss, the physician begins to act like a medieval cleric in an insane asylum, attributing everything to “evil” (in this case, lying).
This is not the extreme case. In this relatively easy to diagnose condition (bizarre burning pain after CNS injury), after nearly 700 completed surveys, we are still waiting for the first respondent who says they received a correct diagnosis, and credibility, at their first doctor visit. We know of patients who were diagnosed correctly, right off the bat, by a General Practitioner, with Idiopathic Thrombocytopenic Purpura, Turner Syndrome, West Nile Virus, Paroxysmal Nocturnal Hemoglobinuria, and even premature puberty from an AV malformation in the brain, all of them extremely difficult diagnoses, but we have yet to find ONE outside a major medical center who was initially diagnosed with Central Pain, which is like falling off a log to detect.
The idea that there are varieties of Central Pain and that job one is to sort out the pains from spinothalamic tract and posterior columns does not occur to many. The entire time is spent performing a neurological exam that has little to do with central pain, and satisfying any skepticism about veracity. This last step, the veracity part, is usually only conditionally satisfied, as the bizarre story maintains the skepticism almost during the entire length of the professional relationship. First impressions form lingering attitudes.
How does this help the patient? She already knows she has pain and would like to learn more. In this case, the introduction, the part where the doctor decides whether the patient is actually sick, which may take five or six visits, eats up the time that should have been spent learning more about Central Pain. Granted the doctor must decide whether the pain is profound agony, in which case radical means must be considered, or if the patient is merely in pain, which is always bad, even if it is not bad.
Far better to take two minutes to get past Bowsher’s criteria (see at this site) and then move on to the very difficult job of finding a common vocabulary by which the patient can talk about bizarre pain. Satisfying the doctor’s skepticism is NOT the delivery of health care. Putting the doctor at ease does nothing for the patient. The great historical figures in CP research do not regard the diagnosis as anything special, and can generally get through it in one or two minutes. It is the understanding which is challenging and should take up the time.
The CP patient typically does not bother to accurately describe pain quality, which is another clue. CP is so unique the patient assumes it is some well defined malady which the doctor will know about, avoiding the necessity of explaining what the patient has no words for. The doctor assumes that if it isn’t worth the patient’s time to describe quality, the pain cannot be severe. You see, even terrible historians do a pretty good job describing pain and can tell where it is, when it started, what makes it worse and the quality of the pain. (The CP patient may foul up on one or all of these questions.) Sometimes, even unconscious patients can give helpful responses to painful stimuli.
Mozart and Bach are recognized still for their genius. Music, poetry, and art are not better today. They are just different. We have been conditioned to see progress and improvement, particularly in medicine. However, there is a part of medicine which is an art. It has mainly to do with listening carefully to patients. Admittedly, this is less productive in Central Pain than in other conditions. There is nothing in colloquial English to describe the central pains. They are a confusion and mix of neurotypical pains. However, despite the uncertain vocabulary, very clear constants in the medical history emerge. It was this fact that led Dejerine and Roussy to conclude that the examination was nothing compared to the history in diagnosing pain of central origin.
Both Dejerine and Roussy listed only three questions that needed to be asked of a CNS injured patient:
1) are disorders of superficial sensibility present;
2) is there burning pain
3) has the perception of the skin surface been altered.
Riddoch simply determined if the quality of the pain was difficult to describe.
Ironically, some of today’s doctors make the ability to describe the indescribable pain the first requirement for any credibility. Catch 22. “You have been preapproved to be believed, provided you make your descriptions in accordance with my narrow idea of what CP might be like, and how severe I think it might be.” Yes, words are very important, and the patient is not the one who sets the verbal context.
Time honored “reflex sympathetic dystrophy” had to be changed to “complex regional pain syndrome”. The thalamic syndrome turned into central pain.
Terence Anthoney’s famous book on the terminology of neurology and the obvious conflicts and inconsistencies in the way words are used in neurology stands alone among the medical specialties. It is the only such book in any field. What makes neurology so difficult to talk about? If we say spinothalamic tract pain, do we mean the anterior ST tract, or the lateral ST tract. No one really knows.
First, the causation of neurological disease is obscure, the manifestations are obscure and the language is obscure. Did we mention obscure? What is a stroke? Is it occlusion of a vessel, constriction of a vessel, or is it a vessel blown to kingdom come. How about the location. Which of the thousands of brain parts can become injured from loss of blood supply. We just as well call it a “sudden” for all the good the term is for information. All CP sufferers ask is that you accept equal ambiguity in what you hear from central pain patients. If you want, you could also learn a little pain chemistry too, just because it is so dang interesting, and it may raise your curiosity about us. Are you listening?
Development of MRI, especially functional MRI, which reads blood flow/oxygen consumption, and by extrapolation, the metabolism of a brain area, has put a big dent into some of the mystery. For example, central pain is often associated with injury near the thalamus, hence the alternate name, the thalamic syndrome.
Already we see the problem. The vocbulary is deficient. No one can locate their bearings until more about mechanisms is elucidated. Making things worse is the inarticulateness of patients. Strangely progessing diseases with unexplainable manifestations leave the patient as speechless as anyone else.
The history of neurology is peppered with stories of one or two doctors who listened to patients and came up with breakthrough understanding. Conversation about the unspeakable somehow produced results. Struggling patients and struggling doctors combined with post mortem pathologists to derive remarkable insights into the nervous system.
On the other hand, great clinicians have always been doctors who learned to LISTEN. As the Johns Hopkins great, Dr. Sir William Osler told endless numbers of students, “If you listen to your patient long enough, he will tell you what’s wrong with him.” That is pretty much it. Easy to say. Apparently impossible to do; or else there would be tons of good clinicians, and every doctor knows these people are rare. Doctors are often too busy reading reports, going to meetings, and filling out forms laid on them by H.M.O’s and insurance companies to have much bedside time.
Now, we want our gripe. After all, as a group, we of the most serious pain state known to man have been brutalized, ignored, and tormented by the ignorance and arrogance of large numbers of those claiming to understand the nervous system.
When one of the editors acquired central pain, the leading neurology textbook, Adams and Victor, did not discuss central pain. An entire generation of neurologists was trained with a textbook that did not have information on this serious condition, affecting many of those with SCI, MS, and stroke. Entire laboratories study mad cow disease, when there are more people acquiring CP every day than have had mad cow disease in the entire world.
Somewhere in all the carnage, we also have a few heroes and angels. It isn’t that hard to become a legend to C.P. patients. All you have to do is acknowledge that CP exists and that we have it. This already puts you in the top ten percent. If you attempt to help us, you move into the top five. If you cannot help us but refuse to put the blame on us, you merit a top one percent award. And if you go out and do some serious research on nerve injury pain, then we will nominate you for a front row seat in heaven. For reasons we don’t expect anyone to understand, pain relief happens to be the most blessed act on earth.
As described by Lawrence McHenry, President of the American Association of Neurology, S. Weir Mitchell, (the great Civil War neurologist, and originator of much of the Hopkins concept of medical education), was years ahead of his time in understanding pain of central origin. So little was he listened to, that when Dejerine, Egger, and Roussy, in France, described the “thalamic syndrome” 32 years later, it was taken as something that had never before been glimpsed in the history of medicine, when really, it was just the first showing that the thalamus was involved. Dejerine and Roussy were themselves forgotten until the great British neurologist, George Riddoch, described a “pain beyond pain”. Yep, you guessed it. He was talking about central pain.
Pain is just so darn boring. Riddoch’s series of articles in the Lancet and Riddoch himself, were in their turn forgotten and buried in the sand until the amazing Ron Tasker, a neurosurgeon at Western Toronto, redescribed the whole business and determined that central pain included signals carried in the spinothalamic tract AND presumably, the postetior columns. Tasker was part of a great tradition of British brain scientists, which gave rise to men like David Bowsher, and even Patrick Wall, who, along with Melzack in Montreal, wrote the magnificent “Textbook of Pain”.
Wall, our originator and the patron saint of painonline, showed in 1985 that the spinothalamic tract actually was composed of at least SEVEN smaller tracts, each of which presumably subserved some special type of pain. Joining the pain Hall of fame was John Bonica, the inventor of the epidural block, Dr. Bonica discovered the epidural block in the 1930′s, an dgave it to his own wife, but widespread use of it was delayed for fifty years or more by the medical profession. Was this delay fostered by the pseudo-religious notion that women were “supposed to suffer” in labor? The source of this myth was supposedly the Bible, but inspection there reveals there is no mention of this anywhere. It does say Eve would have sorrow in conception, but that phraseology has nothing to do with labor. It probably refers to dirty diapers, beans up the nose, and wayward children.
Dr. Bonica inspired many of the current pain researchers, including some at painonline. He endured more than 14 surgeries himself for back pain. He also wrote a mongraph on central pain, and was a champion of those with CP for most of his later life. We are confident he has joined the saints as a great benefactor of mankind. His wonderful wife died only a month after Dr. Bonica. In life they were not separated and in death they are not apart. We hope his work is not buried, nor Patrick Wall’s, who began the database which serves as the basis for painonline.
No one listened better than Dr. Wall. He never bought it all, particularly hasty assumptions we had made about the pain, but what he did buy kept us alive and in hope. His jaunty sharpshooting at our undisciplined ideas were never offensive. That he realized CP patients need structure in how they view their own pain shows what a great clinician he was. When his lips moved, we could hear heaven’s voice. We thank him for the good he did us and all pain patients everywhere.
Now we have a new generation which is incredibly bright and smart. It was a bright new world when nerve injury pain caught the attention of the PhD chemists. An animal model for CP has been developed, largely begun at Karolinska under the amazing Jorgen Boivie and expanded in the U.S. by Claire Hulsebosch in Galveston, one of the angels of painonline.
PhD’s accept CP to the extent of trying to solve it. We can ask nothing more. The animal model makes skepticism obsolete as does functional MRI and PET scanning, begun in CP by the great Ken Casey at Ann Arbor and developed further by Sergio Canaverods and Carlo Pagni in Italy. These were and are some of the pioneers in nerve injury pain. Many of them deserve Nobel prizes, but pain is not a populist malady, and the Nobel committee has yet to promote the angels of pain mercy to their proper place.
We would be remiss if we did not mention Carl Saab, at Brown, who pushed the early studies to locate pain inhibition in the brain and offended the majority of researchers for a few years with his findings, until they discovered he was right. Carl has been kind enough to write here, and we thank him for that also.
Marshall Devor in Israel, one of the brightest scientists on the face of the earth, and a compassionate soul, has continued the work of Patrick Wall, and much of our destiny rests in his hands, and in the hands of others at IASP and in the hands of NIH associated researchers who push the envelope of knowledge every day. Praise the Maker, the list is long and growing of people determined to defeat mankind’s greatest enemy.
Now down to earth. What about YOUR doctor. Does he listen? Does he demand you help him understand. Does he/she compel you to explain what your pain is really like. Doctors have to listen to every flavor of CP, from the person with nothing more than a neuropain “sunburn”, to those who are sprayed continually in “acid”, and would be like as not to bite him so great is the agony. None of it fits into ordinary words, so the doctor is not going to bat 1000. We have a responsibility to speak well and inform the doctor, even if it is over his dead body. If the skeptic would read Mitchell or Dejerine, it wouldn’t be necessary to convince anyone, but the pain prophets of the past are ignored by their own.
Increasingly sophisticated testing for pain is becoming available, but the nostalgia surrounding the tuning fork, the reflex hammer, and the cotton ball is so great that they continues in use, even in conditions such as CP, where there are better ways. “To a boy with a hammer, everything looks like a nail.” To a neurologist with a reflex hammer, everything looks like motor loss. Von Frey hairs and pieces of plastic wrap are better for CP testing, and temperature and sharpness standards, essential for detecting PARTIAL deficit would also be nice, but the history is sufficient for a knowing listener.
Tests in neurology tend to be black and white. If things are not entirely absent, dont’ waste my time. Never mind that CP tends to live in the middle, with intermediate injury and incomplete loss. The profession still has a hard time measuring, and a hardheaded difficulty in accepting, partial loss of things.
In other words, “How can you be in pain?”
You #1 “look so good”; and,
#2 “can’t have pain if you are paralyzed”.
Not to forget, “Why are you smiling if you are in pain?” (Hint. We are trying to act like we still have some social skills).
“Significant” loss to many doctors is loss which THEY can detect. What the patient alone can detect is not worth the paper which it isn’t written down on. The patient’s perception is too often ignored. The person who cannot wear clothing knows full well things are really bad, but if the old MRI happens to turn up blank, the doctor’s eyes may glaze over as he performs a watch check, and communication essential for care become water under the bridge.
STOP. The significance of diseases is not determined by tests. It is determined by what it is doing in the patient. If your tests cannot measure something, that something is not going to get measured. What implement in the black bag measures pain? Your ears can measure it if you will give the patient a vocabulary with meaning to both of you.
It is rather more easy to measure partial motor loss. It can usually be seen. Sensory dysfunction is another matter. If you burn, big deal. Show me some flames. Put the conflagration where your mouth is.
Osler would roll over in his grave. Listen to us! Please. We hurt. We burn. We need your help, even if it is to inform us that no satisfactory treatment exists, especially then. For once, stop worrying about the H.M.O. monitor and remember why you went into medicine. We’re right in front of you. Blow us off and you become a businessman. Is that what you want?
CP is not black and white and that is just as hard for us as for you. If we can deal with pain that doesn’t fit our prior experience with pain, you CAN deal with gray areas. You CAN handle incomplete cord injury. PLEASE.