What is RSD / CRPS?

DO YOU HAVE BURNING PAIN?

  • Is the pain from an injury that has not healed?
  • Is the pain out of proportion to the severity of the injury?
  • Has it lasted past the expected healing time?
  • If your answer is yes to any of these questions, read on....
What is RSD? Suspect RSD? Treatment Problems Ways to Help your Doctor


COMPLEX REGIONAL PAIN SYNDROME (CRPS)

aka REFLEX SYMPATHETIC DYSTROPHY (RSDS)

RSD is also 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. Recent evidence suggests that CRPS1 is also from nerve damage.(Please see "Evidence of Nerve Damage in CRPS1" on WHAT'S NEW? page.

Other names:

  • causalgia
  • algodystrophy
  • postraumatic dystrophy
  • Sudeck's atrophy
  • shoulder-hand syndrome
  • Morbus Sudeck


BACKGROUND:

RSD/CRPS has existed since the American Civil War (1861-5) where Dr. Silas Weir Mitchell documented cases of causalgia in Civil War soldiers.

RSD/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. Mirror therapy helps "retrain" the brain.

Usually CRPS/RSD 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.
RSD is a debilitating disease which can impair the ability of the limb to function or function can be lost. Physiotherapy and mirror therapy restore function.

How do you get RSD/CRPS?

  • a soft tissue injury due to minor trauma in 65% of cases e.g. sprain, twisted ankle etc.
  • fracture
  • surgery
  • 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

What are the symptoms?

  • 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: ie. dystrophy, dryness, tissue atrophy e.g.. cold RSD can be cool, pallid or mottled; or warm RSD 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 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, poor memory or judgment
  • hair and nail changes
  • bone changes e.g.. osteoporosis
  • joint tenderness and swelling

How is it diagnosed?

RECENT DEVELOPMENTS: After many many years of not having a definitive test, there are new markers to test for CRPS. From Boston, we have a skin biopsy test with a 3mm sample which can detect distal nerve endings which are damaged. From Haifa, Israel, we have a saliva test for high levels of LDH (lactate dehydrogenase). There is also an albumin test.

Tests are not being done in Canada yet.

In the meantime, doctors who are qualified :

  • do a thorough medical history noting sign and symptoms
  • give an examination by a qualified expert physician using the IASP criteria
  • may use thermography which measures the heat emitted from the body and senses skin temperature differences (may help)

CURRENT IASP DIAGNOSTIC CRITERIA (Moscovitz 2010)

DIAGNOSTIC CRITERIA

 

CLINICAL CRITERIA

 

PAIN

 

 

continuous pain which is disproportionate to any inciting event

 

SYMPTOMS

4 CATEGORIES: by report:

Sensory: hyperaesthesia and /or allodynia

Vasomotor: temperature and or color asymmetry and/or change

Sudomotor: edema, and/or sweating asymmetry and/or change

Motor/Trophic: less ROM and/or motor dysfunction, and/or skin/hair/nail changes

 

one in 3 or more of 4 categories

 

SIGNS:

4 Categories by observation at the time of diagnosis: As above

 

one in 2 or more of 4 categories

 

There is no other diagnosis that better explains the signs and symptoms above.

 

Criteria was developed by the International Association for the Study of Pain (IASP) in 1994 and modified in 2004. "The criteria are under continuous review to improve their specificity while maintaining adequate sensitivity."

To read entire study: Moscovitz.pdf

 

DO YOU SUSPECT 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 RSD.) 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 RSD."

( Hooshmand, H MD CRPS: Diagnosis and Management Pain Digest Spring Verlager 1999).


TREATMENT IS CRUCIAL WITHIN THE 0-3 or 3-6 MONTHS when the disease responds best. It is essential that the person be referred to the proper specialist for treatment as soon as possible. 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 RSD/CRPS?

Anyone can get RSD, but it is more prevalent among women 3-1 to men; typically it is women ages 40-60 who are most affected. However, children and teenagers can have RSD as well. For them, the prognosis is more favourable.

What can I do?

"Early diagnosis brings the best prognosis" In RSD/CRPS the best response to treatment is within the first zero to six months of the disease. If after reading this, you suspect that you or a friend may have RSD, get a definiive diagnosis and treatment as soon as possible. For doctor referrals in your area, please contact us.

TREATMENT

The following is a list of treatments for RSD/CRPS:

  1. 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
  2. Blocks: a) focal b) sympathetic blockade c) intravenous regional blocks d) epidural e)plexus catheter blocks
  3. 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
  4. Transcutaneous electrical nerve stimulator (TENS)
  5. Sympathectomy: a) surgical b) chemical in rare cases (many doctors no longer do this surgery due to complications).
  6. Implantable devices: a) dorsal column stimulator b) peripheral nerve stimulator c) morphine infusion pump (intrathecal drug delivery e.g. baclofen)
  7. Ketamine: a) low dose, b) 4 hour IV infusion c)ketamine coma (currently not available in Canada) or lidocaine infuson.

CURRENT RECOMMENDATIONS FOR TREATMENT

2015: 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:

  1. 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.)
  2. Aqua therapy is also recommended for lower extremity CRPS in 92-93 degree water. It is moist heat which is good for most RSD patients, it is non-weight bearing.It supports weak muscles, and the amount of resistance can be controlled. Warm water is also soothing.
  3. Psychological support is also recommended e.g. counselling, cognitive behaviour techniques, biofeedback, visualization, relaxation techniques, hypnosis. Any of these modalities can help.
  4. Medications that control pain are essential and will facilitate the progress made in physiotherapy.Adequate and early pain control helps the person cope 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.

 

PROBLEMS

If treatment is delayed, many RSD/CRPS patients can face the following:

  • a lifetime of chronic pain which is resitant to treatment.
  • not being believed or misdiagnosed
  • receiving inappropriate treatments
  • dealing with doctors who are inexperienced in diagnosing and treating RSD/CRPS

Unfortunately, these situations are all too common. Most of the time, reliable information about RSD/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 RSD" would also not occur.

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 so that he can better treat you will certainly promote a healthy doctor-patient relationship. Working together with your doctor is a win-win situation.


WAYS TO HELP YOUR DOCTOR

    1. 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.
    2. Keep a journal of your medications. e.g. effective and non-effective medications, side effects, etc.
    3. 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.
    4. Prepare a list of questions. Your spouse/friend can record the answers.
    5. Keep a record of your past treatments and medications. Record how they affected you.
    6. For new patients, bring your test results, scans, x-rays etc. Make a brief summary of your medical history thus far.

    OPQRST

    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?

    PAIN DIARY

    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:

  • 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/RSD?

2015: This urgent question remains in the minds of researchers.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 mounting evidence that CRPS may be an auto-immune disease. (Plese see PARC's Facebook page: RSDCANADA:PARC.)

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 the 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."

OTHER THEORIES

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 RSD. 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 RSD. In chronic 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 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 RSD. The causalgia theory stated that the burning sensations in RSD were caused by a nerve injury. However, not all RSD had an nerve injury as the cause. In many cases, fracture, or soft tissue injury was the trigger for 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 RSD 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 RSD/CRPS patients in Holland. 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.

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 onf why we get CRPS, remains unknown.

 


Updated 2015.


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