Watch a seminar with Dr. David L. Shulman CFCP, DAAPM
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. 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 have been discovered.
Research by Stephen Breuhl PhD and Ralf Baron MD PhD confirm these
findings. For diagnosis, the Budapest criteria
RSD/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.)
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. Graded motor imagery (GMI) and mirror therapy
help "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.
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 RSD/CRPS?It is usually from an inciting
a trauma in 65% of cases e.g. injury, fall, fracture, sprain,
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
(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
bone changes e.g.. osteoporosis
joint tenderness and swelling
Please note, patients do not have to display all of these symptoms.
Please refer to Budapest criteria for exact 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).
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.
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
EARLY CRPS 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 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.
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 RSD/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
The following is a list of treatments for RSD/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
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
Deep brain stimulation.This is still largely experimental
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:
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.)
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
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 RSD/CRPS patients can face the
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
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 CRPS" would also not
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
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
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:
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
WHY DO WE GET CRPS/RSD?
There are still various theroies about the cause. Current theor
is that CRPS is an interaction between the peripheral nervous
system (limbs)and the cventral nervous system (brain, spinal cord).
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 some evidence that CRPS
may be an auto-immune disease. However, the German and Dutch researchers
disagree. Their theory is 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
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 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 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.