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Information for Medical Providers

Central Pain Syndrome (CPS)

(AKA- Thalamic Pain Syndrome, Dejerine-Roussy Syndrome, Post Stroke Syndrome, Central Sensitization)

WHAT IS CPS? (CPSF Official Definition)

  • Central pain syndrome (CPS) is a neurological disorder consisting of agonizing pain signals of many differing types at once: burning, freezing, shocking, aching, crushing, tearing, and spasticity.
  • It has been characterized as the worst pain known to man.
  • It is caused by damage to or injury of the Central Nervous System (CNS), which includes the brain, brainstem and spinal cord; its causes include but are not limited to, stroke, MS, epilepsy, head & spinal cord injuries, aneurysms, tumors and infections.
  • It is estimated that millions of people in the U.S. suffer from CPS.
  • It is aggressive, never ending and disabling, making normal life immensely painful and incredibly difficult.
  • The medications and treatments used to fight it are very limited and highly ineffective.
  • There is presently no cure.

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Central Pain Syndrome (CPS) is known by many names, including thalamic pain syndrome, post-stroke syndrome, Dejerine-Roussy syndrome and Central Sensitization. CPS is not an ailment that develops in and of itself. Instead, it requires a precursory condition that first injures the Central Nervous System (CNS). This includes the brain, brain stem and spinal cord. Once thought an uncommon neurological curiosity, CPS is now said to be an important and underrecognized condition.

Central type pain has been documented since the early 1800’s. In 1891 it was formally described in a published paper by Dr. Edinger, a physician working in Frankfurt am Main Germany. After a stroke patient of his committed suicide from this pain, he hypothesized that central pain could be released independently of  peripheral nociceptive stimulation. In 1906 Dejerine and Roussy described the so called “thalamic syndrome” that was said to be caused by and associated with a variety of thalamic infarctions and hemorrhages. Now, over 100 years later, CPS has been given numerous other titles and is known to be caused by CNS damage of varying types.

A lesion somewhere along the spinothalamic tract is the direct cause of CPS, but the cause of the lesion can come from many differing sources. In major infarcts and hemorrhages, such as strokes, and other medical conditions where damage is detectable, a diagnosis of CPS is sometimes easily obtained. However, lesions as small as the size of a pinprick, occurring anywhere along the spinothalamic tract can cause the very same excruciating pain as the larger, more easily detectable lesions. The smaller lesions are often entirely unseen via today’s scanning procedures. Therefore, many CPS sufferers are treated with inadequate medical care, due to a lack of clinical findings.

Some practitioners feel that patients are drug seeking or have emotional/mental issues that are the underlying cause of their pain. We hope to provide clinicians with the necessary knowledge to treat all CPS sufferers properly and in a timely manner. It is imperative to do so, since a delay in treatment can actually lead to worsening CPS pain.

Once the central nervous system is damaged, it can take days, weeks, months or even years for CPS to appear.

WHAT ARE SOME OF THE CAUSES OF CPS?

Some common causes are stroke, spinal cord or traumatic brain injury, cancer, Parkinson’s disease, multiple sclerosis and Epilepsy. Less common causes of CPS are:

  • AIDS, especially end-stage
  • Aneurysm
  • Arachnoiditis
  • Arteriovenous malformation
  • Cauda equina syndrome
  • Cervical myelopathy
  • Charcot Marie Tooth
  • Chemical toxicity
  • Cluster headaches (some think this might be a form of central pain)
  • Gunshot wounds
  • Infection (bacterial or viral; such as Shingles and encephalitis)
  • Lead neuropathy
  • Meralgia paresthetica
  • Mercury toxicity
  • Myelomalacia
  • Neurofibromatosis
  • Posterior myelitis
  • Post-polio syndrome
  • Prion disorders
  • Radiation exposure
  • Reflex sympathetic dystrophy syndrome
  • Spinal cord infarction
  • Surgical accidents
  • Syringomyelia
  • Tethered cord syndrome
  • Transverse myelitis
  • Vascular malformation
  • Vitamin B-12 deficiency
  • Any condition that causes nerve demyelination or other nerve or brain damage 

WHAT ARE THE SYMPTOMS OF CPS?

CPS patients deal with PERSISTENT pain. It is characterized by a mix of pain sensations, the most common of which is constant burning, which may be unrelenting and occur over large areas of the body. CPS may also include other pain sensations such as freezing, aching, ballooning, stretching, crushing, spasticity, shocking/electrical type jolts & stabbing.

Depending on the location & amount of CNS damage, the pain can occur anywhere from head to toe or just in certain areas. It can be totally one sided or may cover the entire body. Often, as CPS progresses, it transforms, covering new areas and varying in pain intensity and character.

Other CPS symptoms include intolerance to temperature change, fatigue, a vague numb sensation (like coming out of dental anesthesia), hyperalgesia (abnormally excessive sensitivity to pain), allodynia (pain elicited by stimuli that normally doesn’t cause pain), allachesthesia ( a touch in one area resulting in pain in a neighboring area), visceral pain, bowel and bladder pain and emotional lability.

CPS becomes worse with normal sensory input, such as light, sound, movement, vibrations, emotions, stress and even thinking. A light touch or hug, a breeze, sunshine or even clothing touching the skin may cause intense, burning pain. Touching an item that is hot or cold, such as a door handle, coffee mug, water faucet or steering wheel, can cause pain not only to the hand that touches it, but in a range of different areas in the body. The pain makes no sense. Barometric pressure changes, such as storms rolling in or out, and altitude can wreak havoc on a CPS sufferer. Preparing for and getting to a doctor’s appointment can be a major challenge with CPS.  Showering, dressing, walking outside into the daylight, sitting on a hot or cold car seat, touching the warm or cool steering wheel and riding in a car that vibrates down the road can add to the pain a CPS patient feels.

For many with CPS, the most symptom free time of day is just after waking up, before the senses are bombarded with the input of normal everyday life. The sleep cycle can, in some, calm the pain and many CPS sufferers will attest to having their best hour(s) of the day shortly after waking. But, once sensory stimuli starts ( i.e. moving, getting dressed, walking to the bathroom, seeing daylight, talking, eating etc.) the pain begins to ramp up. Things that used to be easily accomplished are often avoided because of the extreme pain they evoke. Isolation and disability can occur due to the enormous amount of discomfort that normal ADL’s (activities of daily living) cause. CPS sufferers may have a lack of patience from tolerating constant neurological pain as well as emotional lability.

HOW INJURY OR DISEASE AFFECTS THE SPINOTHALAMIC TRACT IN CPS

Central pain is by definition “bizarre” and is the result of a diseased pain pathway. Research reports the most severe central pain is found only in those with some remaining function, however miniscule, of the spinothalamic tract. Loss of motor function does not correlate with degree of central pain. In general, the most severe central pain can be expected where significant retention of spinothalamic tract is present. Such pain typically appears when the CNS injury is resolving and is revealed to be incomplete. In a healthy spinothalamic tract, radiostimulation causes no sensation. In patients with CPS it recreates the sensations of central pain and acts as if the entire pain system is like a nerve ending. When these ungated pain signals reach the cortex, they may cause unbearable suffering.

INJURED MOTOR NERVES VS. INJURED PAIN NERVES

An injured motor nerve simply carries less current than an uninjured motor nerve. Injured pain nerves, however, do exactly the opposite, increasing their signal. But, it is not just a simple increase. They eventually gain power to influence uninjured neighbor neurons, which begin to autonomously fire. (Devor’s work in The Axon, ed. Waxman, Oxford Univ. Press, 1995).

The process can become so violent that the thalamus, the pain center of the brain, records “bursts” of impulses from these injured nerves. CPS apparently occurs at this point. 

The longer that pain pathways relay pain messages, the more efficient those pathways become, causing greater pain to be transmitted, the way a stream carves a path through land, so that over time, it flows more quickly and turns into a river…..progressively deeper levels of pain cells in the spinal cord are activated with prolonged injury. Also it may be that descending pathways from higher brain centres which normally dampen ascending pain (as during the placebo effect) are weakened.

“Physical pain changes the body in the same way that emotional loss watermarks the soul. The body’s pain system is not hardwired, but soft-wired (what neuroscientists call ‘plastic’), and it can be maladaptively molded by pain to increase its pain sensitivity……But in the case of persistent pain, neuroplasticity is negative. The nerves in the spinal cord become hyperexcitable and begin spontaneously firing and recruiting other nerves in their service, and the whole system revs up to be increasingly responsive to pain, in a phenomenon discovered by the pain researcher Clifford Woolf and termed…..central sensitization (when hypersensitivity occurs within the central nervous system). ” from ‘The Pain Chronicles’ by Melanie Therstrom

FINDING THE RIGHT MEDICAL CARE

It can be extremely difficult, time consuming and exhausting to find a doctor who is familiar with CPS. One of our main goals at CPSF is to educate the medical community about the causes of this menacing condition and how to effectively diagnose and treat patients who have it.

Because this type of pain can worsen with time, it is IMPERATIVE to diagnose and begin treatment of CPS as quickly as possible, before deeper damage is incurred. Although there is currently no cure for CPS, certain medications and treatments have been found to be more effective than others.

TREATMENTS AND MEDICATIONS

There are many medications used in the treatment of CPS, although none are completely effective in alleviating the symptoms. Through trial and error, patients and their doctors eventually find the medication(s) that work best for each specific case. It is essential to find the regime that allows for maximum quality of life for each patient. The most common medications for CPS are listed here:

Anticonvulsants, antidepressants, antispasmodics/anticholinergics, benzodiazipines, CNS stimulants, local anesthetics (transdermal cream and patches), medical marijuana (inhaled, ingested or topically applied), muscle relaxants, narcotic pain medications, neuroleptics and sedatives/sleep aids.

Medications will vary based on the precursory condition that led to CPS and any other medical issues a patient may be receiving treatment for.

Since chronic pain can worsen with vitamin D deficiency, ensure that vitamin D levels are within normal limits. Fatigue and depression, which are common in CPS may also be improved by adding a vitamin D supplement if needed.

Aside from medications, there are currently no treatments known to alleviate CPS symptoms. However, stress reduction, biofeedback and avoiding certain triggers help some patients keep their pain at more tolerable levels.

WHAT IS THE PROGNOSIS? 

In past references, CPS is listed as a non-fatal condition. But, as pain spikes to unthinkable levels, so can blood pressure. In certain patients, such as those prone to stroke, an increase in blood pressure can deliver fatal consequences. Also, there is a high incidence of suicide in the CPS community because this insidious pain NEVER goes away, often gets worse over time and is incredibly difficult to effectively treat.

* THIS WEBSITE DOES NOT PROVIDE MEDICAL ADVICE. PLEASE CONSULT YOUR DOCTOR OR A LICENSED MEDICAL PRACTITIONER  FOR INFORMATION, TREATMENT OPTIONS AND/OR DIAGNOSIS OF CENTRAL PAIN SYNDROME*  (See Legal Notice)


78 Comments

  1. I have CPS following a stroke 18 months ago. It is on the entire right side of my body. I have tried tons of meds (nothing worked) and acupuncture. Lately I have been using self hypnosis recordings for pain relief before I fall asleep at night. These have made a difference. The pain is not gone by any means, but a bit more tolerable. We all know just taking the edge off is the best we can hope for most days, and I am thankful. Good luck to everyone in their day to day struggle with this horrible condition.

  2. Great info! Very simple and easy. Nobody can explain as absorbing as this. I acknowledge your time and accomplishment on authoritative things simple and calmly understandable. I accept bookmarked your site. Thanks

  3. Leonie Arnold says:

    I have just realised that I have had increasing CPS for 25 years and a very large cavernous haemangioma was discovered in my brain then. I have had epilepsy, worse by 45.
    I put the aching, burning and pins and needles and now shooting pains down to epilepsy and injury and arthritic degeneration.
    I had a very traumatic time and pain at 20 and was never fit again, I could never sit or stand for long and was irritable and tired ruining marriages and friendships.
    Now at 70 I have to avoid all stimulus that also brings on seizures or headaches. No doctor has ever thought that I have this and I am seeing my neurologist again soon because no doctor has ever listened to me.
    My current new doctor I feel may be getting an understanding and is trying new meds that may help.
    I avoid people as any stimulus sets me off and I have to endure burning all over and lie down and stay still lying on my side as I can’t lie on my back anymore.
    Winter is awful and Summer starting sets me off but I get used to the increase in sunshine. EVERYTHING described above I experience.
    This is unbearable but is me, Since going off codeine I think I may be a bit better. Codeine caused an increase in pain after 10 years of it.
    I now have bouts of virtually almost no pain ie bearable and bouts of frightening unbearable uncontrollable pain. I have recently been given amitriptyline but I am unsure how to take it……………..lets hope my neurologist can help? I take Lamotrigine and clonozapam for epilepsy and extra clonozapam does help with bouts. Clonidine patches tried, I was severely allergic, diazepam tried to no effect at all. I don’t think amitriptyline helps except to put me to sleep.
    I worry that I am losing fitness as all stimulus is too much. Luckily I was always happy with my life and being alone, no wonder I preferred it.

    . No-one could understand except my daughter who works in the med school as Professor of Anatomy and has Fibromyalgia herself.
    Just writing this I am burning all over now.

    • andy says:

      i had a hemorrhagic stroke in 1990 when i was 34 and have been left with paralysis down the right side of my body – still cant use my right arm or hand just slight movement in hand and arm and walk with a carbon fiber leg braise – i could cope with all that but the (CPSP “central post stroke pain” is very bad, and have tried all of the medication even opiods which only worked for a short time so now i am drug free!i’ve had 3 “deep brain stimulater operations 2 in oxford with professor tipu aziz and 1 in the london hospital without success and i tried ( TMS) in liverpool also without success – i keep as active as i can on my better days! so i go to the gym 2 or 3 days a week – without the pain life would be much better! and iv’e been following stem cell therapy for stroke for over 20 years and i am very hopeful for this treatment to show great possibility’s!.. hope you find relief-andy

  4. Xanthia says:

    May God Bless you! I’m so sorry that you have lived in such pain for so long. I also have CPS. No one can understand the feelings of a total body “steam Burn” sensation unless you’ve been in a fire or have CPS. Whenever I say I feel like I’m burning it is confused with me meaning I’m hot. No, it’s not that I’m hot, it’s that I feel as though Ive been BURNED all over my body. I feel as though there are tourniquets around every body part and that I’m not just being crushed but Gnashed forever by some invisible monster. I have a spinal stimulator, and take many drugs. The “crushed” feeling is improved on and off but nothing takes away the burn. It’s all day every day. The medical field has a responsibility to spread awareness of CPS.

    • Leonie Arnold says:

      Exactly the feeling. It is impossible to describe because as you say it is not ‘hot’ it is as if you are being twisted as you say. I have now been prescribed amitriptyline at night now, it hasn’t kicked in yet but I do get real sleep. It is definitely not a pain killer. I wasn’t taking it properly as I misunderstood what the doctor wanted as he didn’t tell me anything about it. My daughter who is in the medical profession says don’t get too hopeful nothing will work. We’ll see. The burn is at night if I move around there is other pain and cramps but I am unfit.

  5. Gray Appleton says:

    rTMS has shown some benefit recently.

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