Watch a seminar with Dr. David L. Shulman CFCP, DAAPM
RSD is now known as Complex Regional Pain Syndrome (CRPS) type 1. CRPS type 2 is causalgia which is identical to type 1 except that it is caused by a nerve lesion. Evidence suggests that CRPS1 is also from nerve damage.("Evidence of Nerve Damage in CRPS1" on WHAT'S NEW? page.)
Newer evidence also suggests that type 1 and 2 are no longer relevant since four subtypes (four kinds) have been discovered. Research by Stephen Breuhl PhD and Ralf Baron MD PhD confirm these findings.
The four subtypes are: acute, chronic, warm and cold.
CRPS has existed since the American Civil War (1861-5) where Dr. Silas Weir Mitchell documented cases of causalgia in Civil War soldiers. He was a Philadelphia neurologist who wrote "Gunshot Wounds and Other Injuries " (1864).(Available through Google.)
CRPS is a multi-system syndrome with diverse symptoms characterized by chronic pain. It affects the immune system, central nervous system (brain and spinal cord) autonomic and vascular system (hot/cold).
Recent studies (Lewis J PhD Eur J Pain 2012) have found changes in the brain. Body perception disorder is very common. For example, CRPS patients perceive their affected limb as 8% larger than what it is.The brain has a "GPS but cannot 'find' the limb that is affected." The brain must recognize the affected limb as part of the body. Graded motor imagery (GMI) and mirror therapy help "retrain" the brain.
Usually CRPS affects one or more extremities but it can affect any part of the body. Due to dilation or constriction of the blood vessels, the blood supply to the limb (hand, foot, hip, shoulder) is affected. The nerves, skin, muscle and bone are also involved.
CRPS is a debilitating disease which can impair the ability of the limb to function or function can be lost. Therefore, physiotherapy is very important. Physiotherapy e.g. sensitization, range of motion, graded motor imagery and mirror therapy help restore function.
How do you get CRPS? It is usually from an inciting event:
a trauma in 65% of cases e.g. injury, fall, fracture, sprain, etc.
new data says 44% of cases caused by fracture
surgery 19% of cases
spontaneous occurrence 10% of cases
other causes 6% (certain cervical spine or spinal cord disorders, infections, stroke, heart attack, repetitive motion disorder, or cumulative trauma e.g.. carpal tunnel.)
certain medications or venipuncture in rare cases
CURRENT BUDAPEST DIAGNOSTIC CRITERIA
(To patients: If you are reading this and someone suspect CRPS, underline/highlight the symptoms you have in the second column. Then take it to your doctor. It is the clinical criteria for diagnosing CRPS.)
(N. Harden 2010)
SYMPTOMS2/4 (patient reports)
SIGNS 2/4 (found by the doctor)
(increased sensitivity to pain)
Allodynia (pain to light touch)
Hyperalgesia (to pinprick)
Allodynia (pain to light touch) and/or temperature sensation and/or deep somatic pressure and/or joint movement
Skin color changes,
Skin color asymmetry
Temperature asymmetry >1°C
Skin color changes
Skin color asymmetry
Decreased range of motion
Motor Dysfunction (weakness, tremor,dystonia)
Trophic Changes (hair,nails,skin)
Decreased range of motion
Motor Dysfunction (tremor,weakness,dystonia)
Trophic changes (hair,nails,skin)
What are the possible symptoms? (Please note that people do not need to have every symptom.)
PAIN usually burning, severe, constant and in an area other than the primary injury site
SWELLING usually localized but can involve entire limb
SKIN CHANGES: TEMPERATURE or COLOUR: i.e. dystrophy, dryness, tissue atrophy e.g.. cold CRPS can be cool, pallid or mottled; or warm CRPS can be warm, red with increased sweating.
LIMITED AROM (active range of motion) in the affected part
INCREASE OF ABOVE COMPLAINTS after exercise
What other signs/symptoms are there?
motor dysfunction e.g. tremor, weakness, dystonia, spasms
dystrophy e.g. muscle wasting
limbic system dysfunction e.g. insomnia, agitation, anxiety, depression, memory loss, poor memory or judgment
hair and nail changes (hair grows more or less hair)
bone changes e.g.. osteoporosis
joint tenderness and swelling
Please note, patients do not have to display all of these symptoms.
Please refer to the Budapest criteria for "exact diagnostic criteria."
There is a skin biopsy test with a 3mm sample which can detect distal nerve endings which are damaged. As of now, the biopsy is being used only in research. Germany currently does 3mm biopsies for research.
Tests are not being done in Canada yet.
In the meantime, doctors can:
do a thorough medical history
do an examination or refer to a qualified expert physician using the BUDAPEST CRITERIA (listed above)
may use thermography which measures the heat emitted from the body and senses skin temperature differences as noted in Budapest criteria(vasomotor).
MRI, CT scan x-ray or bone scans do NOT diagnoise CRPS. (The exception is that a bone scan in a patient < 1 year may be positive. In this case it is a piece of the puzzle.) Other doctors disregard bone scans and use theBudapest criteria only.).
BACKGROUND: CRPS Criteria was developed by the International Association for the Study of Pain (IASP) in 1994 and modified in 2004. Dr. Norman Harden revised it in 2010. Now it is renamed the Budapest Criteria. "The criteria are under continuous review to improve their specificity while maintaining adequate sensitivity." Efforts to add the body perception disorder are underway.
JULY 2017: There is a Dutch doctor working hard to develop a blood test for CRPS. If more information comes in we will post it here.
DO YOU SUSPECT CRPS/RSD?
"If the pain is out of proportion to the injury, there is stiffness and inflammation following a seemingly minor trauma (e.g. twisted ankle, dropping item on foot, sprain, suspect CRPS.) The pain is described as burning, shooting, stabbing or a "hot poker". If the pain persists longer than the expected healing time of the injury, suspect CRPS."
( Hooshmand, H MD CRPS: Diagnosis and Management Pain Digest Spring Verlager 1999).
TREATMENT IS CRUCIAL WITHIN THE 0--6 MONTHS when the disease responds best. It is essential that the person be referred to the proper specialist for treatment as soon as possible.
EARLY CRPS (0-3 months) IS CONSIDERED AN EMERGENCY.
A specialist has an interest in CRPS (not a specialty). He/she could be a rheumatologist, neurologist, physiatrist, anesthesiologist or pain management doctor. After 6 months you can still receive treatment however, avoid delays and see a specialist as soon as possible for best results.
DELAY IN TREATMENT COULD MEAN A LIFETIME OF CHRONIC PAIN for the patient. Without treatment, the disease could eventually become resistant to treatment.
Who can get CRPS?
Anyone can get CRPS, but it is more prevalent among women 3-1 to men; typically it is women ages 40-60 who are most affected. Upper limb is more common than lower limb. However, children and teenagers can have CRPS as well. For them, the prognosis is more favorable than adults.
What can I do?
"Early diagnosis brings the best prognosis" In CRPS the best response to treatment is within the first 0-6 months of the disease. If after reading this, you suspect that you or a friend may have CRPS, get a definitive diagnosis and treatment as soon as possible. For doctor referrals in your area, please contact us.
The following is a list of treatments for/CRPS:
Drug therapy: a) local or systemic corticosteroids b) muscle relaxants c) alpha-adrenergic and beta blockers d) analgesics e) anti-inflammatories f) tricyclics and related compounds g) tranquillizers h) calcium channel blockers i) membrane stabilizers j) opioids
Blocks: a) focal b) sympathetic blockade c) intravenous regional blocks d) epidural e)plexus catheter blocks
Physical therapy: land PT or aqua therapy, graded motor imagery(GMI) and mirror visual feedback (MVF or mirror therapy). Others are laser therapy, vitamin/mineral supplements.
Transcutaneous electrical nerve stimulator (TENS)
Sympathectomy: a) surgical b) chemical in rare cases (many doctors no longer do this surgery due to complications).
Implantable devices: a) spinal column stimulator b) peripheral nerve stimulator c) morphine infusion pump (intrathecal drug delivery e.g. baclofen)
Ketamine: a) low dose, b) 4 hour IV infusion c) ketamine coma (currently not available in Canada) or lidocaine infusion.Some sites in Canada have IV ketamine and lidocaine infusion. (Ask us).
Deep brain stimulation.This is still largely experimental in Canada.
A seminar with Andrea Hebert, BSc. OT
CURRENT RECOMMENDATIONS FOR TREATMENT
Contact PARC for current treatments available in Canada.
The approach to treating CRPS is to treat the whole patient using the biosocialpsychological model. Most CRPS treatment centres agree on the following:
Physiotherapy is the treatment modality which will return function to the limb, range of motion, flexibility and strength. It is imperative that graded motor imagery and mirror visual feedback (mirror therapy) be part of the treatment program. (Please see: McCabe, C PhD RGN et al "Mirror Visual Feedback Therapy: A Practical Approach" Journal of Hand Therapy Elsevier 2011.)
Aqua therapy is also recommended for lower extremity CRPS in 92-93 degree water. It is moist heat which is good for most CRPS patients and it is non-weight bearing.It supports weak muscles, and the amount of resistance can be controlled. Warm water is also soothing.
Psychological support is also recommended e.g. counselling, cognitive behaviour techniques, biofeedback, visualization, relaxation techniques, hypnosis. Any of these modalities can help.
Medications that control pain are essential and will facilitate the progress made in physiotherapy. Adequate and early pain control helps the person cope and manage better.
Harden, N MD et al. CRPS:Practical Diagnostic and Treatment Guidelines 4th Edition Pain Medicine 2013 contains updates on treatment which include evidence for graded motor imagery and mirror therapy.
If treatment is delayed, many CRPS patients can face the following:
a lifetime of chronic pain which is resistant to treatment.
not being believed or misdiagnosed
receiving inappropriate treatments
dealing with doctors who are inexperienced in diagnosing and treating CRPS
Unfortunately, these situations are all too common. Most of the time, reliable information about CRPS is the key to being believed and diagnosed properly. Receiving inappropriate treatment would not happen if the patient and doctor were better informed. Unnecessary surgery to "correct CRPS" would also not occur.
Surgery after getting CRPS is not recommended. It usually makes the pain worse. There are some rare exceptions.
As the patient, it is possible to take on the responsibility of informing the doctor. Perhaps then, some of these problems could be avoided. Giving the doctor as much information as you can. A doctor can better help you with this Budapest Criteria. It will certainly promote a healthy doctor-patient relationship. Working together with your doctor is a win-win situation.
WAYS TO HELP YOUR DOCTOR
If you are experiencing pain and are having difficulty describing it, this is a device that doctors use to diagnose pain. It could help prepare you for your next doctor’s visit. Answers should be as clear and brief as possible:
• ONSET: When did the pain start?
• PROVOKES: What makes it better and/or worse?
• QUALITY: What does the pain feel like? e.g. burning, throbbing
• RADIATES: Does the pain travel or stay in one place?
• SITE: Where do you feel the pain?
• TIMING: When do you get the pain? (e.g. morning, evening,) and how long does it last?
Keep a pain diary with recorded pain levels three times per day. Divide the seven days into three parts and record levels by assigning number to your pain:
Prioritize a list of your complaints. Whatever is bothering you the most, list it first. e.g.. be specific about where your pain is. e.g.. Before and after the visit, record pain levels throughout the day to help determine the overall pattern of how the disease affects you.
Keep a journal of your medications. e.g. effective and non-effective medications, side effects, etc.
Be sure to take someone with you. Take notes so that you are clear as to what transpired. Another point of view is often helpful.
Prepare a list of questions. Your spouse/friend can record the answers.
Keep a record of your past treatments and medications. Record how they affected you.
For new patients, bring your test results, scans, x-rays etc. Make a brief summary of your medical history thus far.
0-3 = mild pain
4-7 = moderate pain
7-9 = severe pain
10= worst pain you ever had
Take this pain diary along with your other materials to your doctor.
WHY DO WE GET CRPS?
This urgent question remains in the minds of doctors and researchersThere are still various theories about the cause.
1. Current theory is that CRPS is an interaction between the peripheral nervous system (limbs)and the central nervous system (brain, spinal cord).
Glia cells in the spinal cord activate after an injury and cause inflammation in the body. The inflammation causes pain.
2. Dr Andreas Goebel of the University of Liverpool has been carefully studying the auto-immune component of CRPS. His work is available on the University of Liverpool website.There is some evidence that CRPS may be an auto-immune disease. Since the discovery of the sIL2R receptor this year, this may still be a possibility.
However, the German and Dutch researchers disagree. Their theory is that the cause is inflammation.
Dr Anne Louise Oaklander has offered a rational explanation:
"RSD/CRPS THE END OF THE BEGINNING"
Oaklander AL MD PhD Pain 139 (2008) 239-40
" 'De Mos et al. have identified the clinical links to CRPS and validated findings that implicate inflammation, vasodysregulation and axonal injury in CRPS pathogenesis.'
The challenge now is to learn how these interact, because each can lead to the other, like the chicken and the egg. For instance distal axons are exquisitely vulnerable to energy deprivation, so vasodysregulation (blood flow problems) or inflammation can trigger axonopathy (nerve damage). "My working hypothesis is that although specific patients may enter the CRPS rotary from different points, malfunctioning small fiber axons are central."
There are several theories as to why we get CRPS. Most of the current theories cannot fully explain all the processes going on in the body e.g.. signs and symptoms vary from patient to patient and can be inflammatory e.g. pain, edema, skin and temperature changes and neurological e.g. hyperesthesia, hyperpathy or motor dysfunction.
The psychosocial theory states that various predisposing factors such as emotional instability, nervousness, depression, anxiety and life events contribute to a person developing CRPS. There is no evidence thus far, in the literature to support this theory.
Inactivity has also been suggested to contribute to edema and muscle atrophy in CRPS. In chronic CRPS/RSD, atrophy and edema are present. However, inactivity cannot explain the skin discoloration, altered temperature or pain. Quite popular even still, is the sympathetic theory which states that an hyperactive sympathetic nervous system is responsible for CRPS/RSD. Since sweating and vasomotor instability are present and a sympathectomy reduces pain, this theory was thought to hold water. However, it did not explain why some sympathectomies did not work. None fully explained the enigma of CRPS/RSD. The causalgia theory stated that the burning sensations in RSD were caused by a nerve injury. However, not all CRPS/RSD had an nerve injury as the cause. In many cases, fracture, or soft tissue injury was the trigger for CRPS/RSD and no definable nerve lesion could be found.
Based on Sudeck's exaggerated inflammatory response theory, the signs and symptoms of inflammation and acute CRPS have similarities: "rubor (redness), calor (warmth), dolor (pain), tumor (swelling) and functio laesa (limited function)". Based on Sudeck's theory Dr. Veldman, Dr L van der Laan and numerous other Dutch doctors have done extensive studies with CRPS patients in The Netherlands. They hypothesize that after an injury or surgery there is an exaggerated inflammatory reaction process which begins in the body. Based on a 1993 study of 829 patients over 8 years, Dr Veldman found that the most common signs and symptoms of pain (92%), swelling (86%), difference in skin temperature (98%), difference in skin colour (97%), limited active range of motion (90%), increase of complaints after exercise (89%) were inflammatory in nature. There is evidence that free radicals are also involved in this inflammatory process since they can damage tissue or membranes in the body. Further evidence, is that free radical scavengers like DMSO (dimethyl sulfoxide), N-Acetyl-cysteine,or IV Mannitol are used successfully in treatment of early cases in Holland.
From the IASP European conference (Zurich:CRPS Special Interest Group 2015), the experts now agree that CRPS begins with inflammation however, they do not agree on the cause.
ELEMENTS OF CRPS: Inflammation, blood flow problems, nerve damage and maladaptive plasticity (brain changes i.e. cortical reorganization, grey matter atrophy, body perception disorder) are thought to be the puzzle pieces in CRPS. Yet the question of why we get CRPS, remains unknown.