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CPS: a disease made difficult because of diversity

January 17, 2012

While I was in the hospital, Jake brought me a book I’d ordered before I got ill. It’s the only medical textbook in existence on Central Pain Syndrome: Central Pain Syndrome: Pathophysiology, Diagnosis and Management, by Sergio Canavero and Vincenzio Bonicalzi. This is the first edition, published in 2007, and has since been superseded by a revised edition published last month. But that change in editions meant that I could actually afford to buy the book, which had formerly been $100, for a mere $25.

One of the great things that the authors did was to survey every single piece of documentation ever published in any medical textbook or journal about Central Pain Syndrome. They start from its earliest citations in the mid-19th Century, and trace the documentation and description of Central Pain up through the articles published in the 2000s.They discuss all the various terms by which CPS has been known – as many terms as there have been names given to Satan in his history,  Just as you can theoretically find Satan entering your life in myriad ways of temptation, so does CPS have its many ways to insinuate itself into your body. Unlike sin, however, there are extremely few recorded events of “cures” for the stain of CPS, and all of them have been caused by … another stroke.

So how is CPS defined, in this textbooks? “CP [Central Pain] is pain due to a CNS lesion along the spinothalamoparietal path.” So a lesion at any point along the path of sensation-transmission, from your spine, to the thalamus relay station in your brain, to your parietal lobe of the cortex, can give you Central Pain. At the same time, you can have Central Pain before the lesion is apparent. “Sometimes pain is the presenting symptom [my emphasis] and remains an isolated finding, as occurs in syringomyelia, and exceptional other disease (e.g., spinal cord tumors.)”

How many different ways can you acquire these lesions? Well, how many different ways can you acquire an auto-immune disease, for instance? At this point there are at least 85 different auto-immune diseases; Wikipedia lists only a portion. But you can develop an autoimmune condition to any kind of cell or tissue that you have in your body. I’ve got at least two: Hashimoto’s hypothyroidism, and Mixed Connective Tissue Disease. But who knows how many may await? Once you’ve got one, the odds are good that you will get another. The inflammation that is the cause of one autoimmune disease can spread, and begin to inflame some other tissue or organ.

I raise the autoimmune disease metaphor to demonstrate that just as there are many causes for such diseases, there are many ways to acquire CPS. Moreover, each of those autoimmune diseases will have different symptoms, depending on the tissue or cell being attacked. It’s the same with CPS: depending on the type of nerve tissue that has been injured, your CPS symptoms will be different from someone else’s. Different medications and treatments will be used with different autoimmune diseases, but the old standbys, such as prednisone, will always be the major forms of treatment. It’s the same with CPS: we have our old standbys, the anti-convulsants, anti-depressants, and opiates. One thing that you can say with certainty is that what ever the cause of your CPS lesion, you will always have a burning-freezing-electrical, unrelenting, torturing extreme pain as its primary effect and symptom.

So what are some of the ways that you can develop CPS? Well, the first thing that you have to realize is that there are actually two types of CPS, as Canavero is quick to point out. There is CPS of brain origin, and CPS of cord origin.

Brain CPS is primarily caused by hemorrhagic or ischemic stroke, but also by closed-head injuries, tumors, epilepsy, and even spontaneous (“iatrogenic”) lesions. Usually the stroke will be in the thalamus of the brain (like mine was),  but it can be cortical, usually in the parietal lobe, or in the midbrain. Brain-CPS can be caused by closed-head injuries; we have several Iraq and Afghan War vets, who were injured in IED explosions,in the CPSA. But there will also be boxers, football and hockey players, concussion victims, and anyone else who takes blows to the head, who will develop CPS. There is Brain CPS caused by penetrating injuries, or by inflammation. You can develop CPS as the result of any disease that attacks or repeatedly injures the brain, such Multiple Sclerosis, or epilepsy. You can develop brain-CPS as the result of infection: an abscess from something like toxoplasmosis, or from tuberculosis or encephalitis. Brain tumors also can cause CPS; approximately 2% of patients with brain cancer develop it (p.28).

Cord CPS has even more causes – as many causes as there can be injuries to the spine. CPS “has been reported with virtually every type of disease or lesion affecting the spinal cord substance, be it a complete or incomplete lesion.” This means that a person may develop Cord CPS with any injury to the spine, from a complete break, to a mere grazing. Trauma and concussion are the leading causes of CPS worldwide. Civilian gunshot wounds and car accidents are the main events that cause CPS in the Western world, but there are also horseback and bicycle accidents, falls – one study found that almost 20% of Spinal Cord Injury patients had developed CPS by 6 months after the injury. There are a vast number of causes: ischemic or hemorrhagic injuries due to dissections, embolisms, cavernomas, etc; rheumatological and degenerative diseases such as ankylosing spondylitis; tumors; congenital and developmental problems; infectious and inflammatory causes, including MS, herpes zoster, HIV and polio; degenerative CNS disorders; and radiation. Any surgery on the spine, such as a spinal fusion or surgery for a cervical disk injury, can result in CPS. Even lumbar punctures can go wrong, and result in CPS; we’ve got one member whose CPS dates to a spinal tap she had done for a completely different issue..

With all these myriad causes, it is easy to see why CPS was though of as a myriad of diseases for decades.The name of “Central Pain Syndrome” as a recognized group of similarly-caused pain disorders really didn’t come into agreement until the end of the ’00s. Canavero’s textbook had a great deal to do with delineating the field. Even the CPS caused by just one type of injury has been known by a variety of names. For instance, CPS following a thalamic stroke was the first “central pain syndrome” recognized by physicians, back in 1906. Its first name was “Dejerine-Roussy Syndrome,” after the two doctors who first identified it. Dejerine–Roussy syndrome has also been referred to as: “Posterior Thalamic Syndrome”, “Retrolenticular Syndrome”, “Thalamic Hyperesthetic Anesthesia”, “Thalamic Pain Syndrome”, “Thalamic Syndrome”,  and “Central Post-Stroke Syndrome”. “Post-Thalamic Stroke Disorder” was the name of the disorder that I was first given, back when I had my stroke in October ’07. By the time I had my first visit with a neurologist, four months later, in January ’08, it was finally being called “Central Pain Syndrome.” “Yes,” my neurologist told me, “That is the name that we are using now.” And that is with just the one type of CPS which is most famously associated with the syndrome.

There are millions of CPS patients all over the world, suffering from this pain, due to dozens, if not hundreds, of causes. Each cause will bring its own attendant issues, problems, and sensations. In addition to the burning-freezing-electrical pain, there are also sensations of stabbing, crushing, grinding, aching – as many sorts of pain as the human body can sense. Then add to this the fact that the time that it takes for CPS to arise from the precipitating event or cause may vary – anywhere from immediately to ten years. The sheer number of causes, the variance of onset, and the variety of sensations, has made the recognition of Central Pain, as an entity unto itself, practically impossible until now. One cannot help but feel that the medical profession can be forgiven for being so slow on the uptake as to finally comprehend that all of the patients, all the across the world, who are suffering from pain related to some injury to the Central Nervous System as suffering from a common illness.

I say they may “almost” be forgiven, because I know the exhausting, suicidal horror of living with this scourge. The only terrestrial metaphor that I can think of the even comes close are those descriptions I’ve read of life in the Nazi or Khmer Rouge concentration camps, where lack of food and sleep, and sheer overwork, caused a perpetual waking agony that would only be ended by death.I keep thinking of the images of Treblinka, which I first read in Steiner’s book, which was serialized in Life Magazine back in 1966. That was the first detailed examination of a Holocaust camp I’d ever read. Although it may have its accuracies, it stuck with me in its evocative portrayal of human misery, abject suffering, and cruelty. While it probably opens me to criticism – and I am by no means equating CPS with the Holocaust, the intentional torture and murder of millions, I do find that there are useful metaphors to be found in those annals of human agony. It is not just the physical anguish. There is also the complete sense of entrapment, as if we ourselves are behind barbed wire, cut off from all other human beings. The fact that no one but us can see the very real effects of the whips, lashes and barbed wire isolates us even more

Another salient difference is that CPS patients do not believe that other human are torturing them intentionally. We all have a deep belief that the “normal” humans would help us, and ease our pain, if they could – if they only understood what it is like. I cannot conceive that physicians would not do everything in their power to break the back of this monster if they knew of its nature and frequency. The only way to guarantee that they know about it is through education, not only of them, but of their patients. To think of all the hundreds of thousands of Americans, and millions of people world-wide, who dwell in the grey nether-world of CPS pain without any relief, – with, perhaps, the worst-case scenario of being told that they are imagining their pain, because there is no evidence of injury to the limbs that are burning, makes me determined to bring the facts of this cruelest of all non-fatal diseases to the recognition of doctors and laypeople everywhere.

There may not be a cure for us today, but there will never be a cure until there is recognition that one must be found.

8 Comments leave one →
  1. yolan..... permalink
    January 18, 2012 6:57 AM

    Good post Louise.

    I’m one with an autoimmune disease – paraneoplastic encephalitis. 😦

  2. January 18, 2012 2:29 PM

    Holy Mackerel! I’ve never heard of that particular form of encephalitis. You *truly* had a rare disease. How did you acquire that, if I may ask? Is your CPS Brain or Cord as a result? What a monster you’ve got on your back.

    Thank you so much for sharing this. I’ve been “collecting” the reasons people acquire CPS, since I was originally told how “rare” it was – only happening after a few strokes. The more I investigate, the more CPS sufferers appear. I believe that by pointing out the volumes of people suffering with CPS, the greater the incentive for research, especially on the part of drug companies. After all, they see “patients with life-long, non-fatal diseases” as “cash cows.” Plus, the incentive for us to pay for their newly-developed meds will be astronomical – so they can charge astronomical sums.

  3. February 3, 2012 8:45 AM

    Kudos for posting such a useful blog. Your weblog isn’t only informative but also particularly artistic too. There usually are incredibly few people who can write not so easy articles that creatively. Preserve up the terrific work

  4. Alison Martin permalink
    March 22, 2012 9:56 AM

    What a great blog – we have both the books by Canavero and Bonicalzi and can thoroughly recommend them … they take a bit of concentration though, so it was enormously useful to read your summary. I am going to print it off to show to my husband’s GP. My husband has suffered from right-sided central (thalamic) pain for 10 years and more recently has also developed Parkinson’s Plus disease, probably Multiple System Atrophy (Parkinsons is yet another cause of central pain). We are determined not to give up and it is great to know that there are others who share our desire to raise awareness and to seek a solution for this horrendous suffering. My husband is currently taking part in a research programme involving transcranial magnetic stimulation.

    • March 22, 2012 1:35 PM

      Alison -We here in the States would love to hear more about the research project!What exactly does it involve, and how is it working for him? Who is sponsoring this research?

      • Alison Martin permalink
        March 22, 2012 3:56 PM

        Hello Louise – Good to hear from you. The research is being carried out by the Pain Relief Foundation, which is allied to the Walton Neurological Centre – part of University Hospital Liverpool. I was interested to see links to them on the CPA website. If you Google Prof T Nurmikko and the Pain Relief Foundation, you will come to it. It involves using transcranial magnetic stimulation – he is only partway through the study, so would rather wait until all the outcomes are known before drawing conclusions. He can certainly feel some difference and also in relation to his Parkinsonism. We have to spend several days in Liverpool each month (160 miles north of where we live) and, if the treatment works, he will have to make monthly visits as the treatment “wears off” – for the first 4 months, he has to have 5 treatemnt sessions (spread over 3 days) each month, focusing on different areas of the brain, identified through functional MRI scanning and brain mapping. It has provided quite a breakthrough for some people – they are also working on techniques for amputees with phantom limb pain (including the military) involving visualisation techniques and also seeking ways for people to have home treatment. The big drawback is that patients have to travel long distances quite frequently to “top up” the treatment. My husband was very lucky to be accepted – he was one of only 50 from 500 referrals! The criteria were central pain that has not responded to other treatments. We are keeping our fingers crossed but realise that, if the treatment works, it will probably be something that goes alongside other things. We and our pain specialist heard about it from a t.v. programme.

      • Alison Martin permalink
        March 22, 2012 4:04 PM

        Hi Louise – me again! I should also have mentioned what a wonderful team the people at the Pain Relief Foundation are – they are absolutely dedicated and extremely supportive. It makes such a difference to be with clinical people who not only have the medical knowledge but also truly understand what central pain is about. (Not to mention of course our local pain specialst, who saw the same T.V. programme and immediately thought of my husband and our GP who supported the referral!). As you know the enormity of central pain is such that 3 days in a hotel each month is a small price to pay if some help is to be gained (even though we can entirely sympathise with the lady who has a blog about the nightmares of travelling by car – even 160 miles is an enormous ordeal for my husband). Our thoughts are with you. Alison & Peter

  5. Bill O'Brien permalink
    November 3, 2013 7:36 PM

    I appreciated reading the posts. I suffered a rt thalamic stroke in conjunction with a heart procedure to correct an A-Fib situation. My pain grew in intensity over a period of years. I was a EMS helicopter mechanic and came to understand that my short term memory had been affected. This forced me to raise the safety flag on myself. After being evaluated I had to retire. It has been 6 years since my stroke, three years since retirement. I pain has grown in intensity as each day goes by. For many years my pain was managed with Cymbalt, fentanyl patch and pain killers. This worked but I felt I needed to correct the skeletal issues acquired over the years. I had concerns corrected and those areas don’t cause me difficulty any longer but now my lower half of my body feels like it is being destroyed without break. I live in Kentucky where pain bills were abused. So now I face each day without any pain medication except for Ibrofen, takes edge off for a brief period but the pain is so great that I can’t be on my feet for long but yet lying on bed will only last for a short time also. Currently I have been to numerous neurologists, each with a different approach, I hate suffering from all the side affects of the drugs which they want to try. I personally believe that they do not believe me because I look healthy and fit. Thanks for letting me vent. Bill

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