complex regional pain syndrome is most commonly misdiagnosed, leading to improper medical treatment that is ineffective for the disease causing devastating morbidity and eventually mortality. remember pain is what the patient says it is and its subjective from patient to patient. Thus any history of trauma to a particular region of the body can be a sufficient enough for you to suspect CRPS. Early detection of complex regional pain syndrome with good medical management and physiotherapy reduces progression of the disease.
2. Table of content
• Definition
• Epidemiology
• Etiology
• Signs and symptoms
• Pathophysiology
• Types of CRPS (Stages)
• Diagnosis
• Investigations
• Treatment Modalities
3. Definition
The term complex regional pain syndrome (CRPS)
describes a variety of painful conditions following injury.
• minor trauma (fractures, sprain etc), elective surgeries,
stroke.
• exaggerated in both magnitude and duration of the
expected clinical course
• often result in significant impairment of motor function,
and showing variable progression over time.
• Complex : sensory sx, local autonomic instability, trophic
changes, motor abnormalities.
4. Epidemiology
• Females > males 2-4:1
• 5.5 – 26.2 cases per 100 000
persons/year.
• Across all ages, peak at 40
years (range 50-70 yrs).
• Arm > legs (in adults)
• Preceding event: minor
trauma, fracture, surgery.
• No relation between
severity of primary trauma
and presentation.
Risk Factors
Immobilization of injured
limb
Use of ACE inhibitors
Asthma
Familial
5. Etiology
• Most common triggers:
- Fractures
- Sprains
- Soft tissue injuries – burns, cuts, bruises.
- Limb immobilization (cast)
- Surgery and medical conditions (stroke) or procedures
(needle prick)
6. S&S
• Key symptom is prolonged severe pain that may be
constant. (burning, pins & needles, squeezing)
• Radiate
• Allodynia - ↑ sensitivity in affected area.
• Skin: color, temperature, swelling of the limb, skin
texture (shiny & thin), abnormal sweating patterns.
• Changes in Nails and hair growth patterns.
• Stiffness in the joints.
• Muscle incoordination and Dystonia/ tremors
7.
8. Pathophysiology
Exact mechanism is still poorly understood - both
peripheral & central factors involved.
• Aberant inflammatory mechanisms.
• Nociceptive sensitization.
• Vasomotor dysfunction.
• Maladaptive neuroplasticity.
9. Inflammatory
mechanisms
• Activation of cutaneous nociceptors following injury →
Retrograde depolarization of primary afferents → Release
of neuropeptides (substance P, CGRP)
• Increase Interleukins and TNFα
• ? Autoimmune mechanisms
10. Vasomotor dysfunction
• A common problem in CRPS
• Warm (4 months), intermediate (4-15 months) and cold
type
• Inhibition of cutaneous sympathetic vasoconstrictor
activity
• Increased sensitivity to circulating catecholamines (SMP)
• Endothelial dysfunction
11. Central nervous system
• Central sensitization
• Structural changes to emotional centers
- Distortion of mental image of affected area
• Impaired motor control – dystonia
• Cortical re-organisation
12.
13. Types
• CRPS type I (reflex sympathetic dystrophy)
- minor injuries/ fractures
• CRPS type II (causalgia)
- develops after injury to a major peripheral nerve.
These chronic pain syndromes comprise
different clinical features, including
spontaneous pain, allodynia, hyperalgesia,
edema, autonomic abnormalities, and
trophic signs.
14.
15.
16. CRPS Stage 1 (Acute)
Immediately after injury (within weeks of injury)
MOST LIKELY TO BE REVERSED AND CURED
• SKIN: Red, warm, swollen, dry, inflamed.
• DISTRIBUTION: Pain is not compatible with a single
peripheral nerve, trunk, or root lesion.
• SYMPATHETIC:
- VASOMOTOR: Disturbances occur with variable intensity,
producing altered color and temperature
– SUDOMOTOR: Hyperhydrosis or diaphoresis
• MOTOR: Decreased ROM, weakness
• X-RAYS: Normal
• BONE SCAN: Increased uptake
23. Diagnosis
• The diagnosis of CRPS is based solely on clinical signs
and symptoms (BUDAPEST CRITERIA 2012).
- Because poorly understood pathophysiological mechanisms
of CRPS, mechanism based diagnosis is not yet feasible
24.
25. Investigations
• Objective testing (thermography, triple phase bone scan,
quantitative sudomotor axon reflex test) is not necessary to
make the diagnosis, but in some cases may be used to support
a clinical diagnosis
• There is no specific diagnostic test available for CRPS
• Main purpose: to exclude other diagnoses
– Lab: FBC,ESR,CRP to exclude infection/rheumatologic
– Duplex / Ultrasound: exclude peripheral vascular disease
– Nerve conduction velocity studies: exclude peripheral neuropathic
disease, painful
• Imaging: may demonstrate osteoporosis in affected limb (but
no diagnostic value)
27. Management
• Patient information and education
• Pain relief and inflammatory control
• Physical Rehabilitation
• Psychological and Vocational rehabilitation
28. Treatment
• Two types of treatment modalities:
1. Conservative treatment
• Most important: early active mobilization physical therapy
combined with pharmacotherapy, psychological therapy
2. Interventional management
• Sympathetic block
• Spinal cord stimulator
34. Interventional magement
• Sympathetic block (stellate/lumbar block)
– Break the vicious cycle of pain.
– Permit more vigorous physical activity.
• Neurolytic blocks - use alcohol
– Give excellent results when performed early.
– Diagnostic blocks using LA should be done prior to neurolytic block
• Spinal cord stimulation
-alters neuro-chemistry in dorsal horns, suppressing the
hyperexcitability, increase GABA
• Peripheral nerve stimulation
• Intrathecal drug delivery system
- Used for patients with dystonia , failed neuro-stimulation, long
standing disease, multi limb involvement or need palliative care.
35. Complications
• cause of significant morbidity and loss of quality of life.
• It affects:
- Anxiety, depression, stressed
- cognition,
- sleep (falling asleep and staying asleep),
- bladder and bowel function,
- -self-esteem, job, family
• includes side effects of medications (such as dry mouth
and constipation
36. Take Home Message
• High index of suspicion for the clinician.
• Treatment should be immediate after diagnosis -
physiotherapy.
• Multidisciplinary approach
• Aim treatment:
– toward restoration of full function of the affected area.
• Intensity of therapy (in particular physiotherapy) should be
adapted to the severity of the disease.
– Gentle and below pain threshold and must not exacerbate the pain
since every painful stimulus may worsen the syndrome
38. References
1. Knobler, R., (2019). Hypervigilance in the Detection of Early Complex Regional
Pain Syndrome, Type 1/Reflex Sympathetic Dystrophy— An Underutilized Tool in
Treatment. Knobler Institute of Neurologic Disease, Fort Washington, PA, USA.
2. "Complex Regional Pain Syndrome Fact Sheet", NINDS, Publication date January
2017. NIH Publication No. 17-4173
https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-
Sheets/Complex-Regional-Pain-Syndrome-Fact-
Sheet#:~:text=Complex%20regional%20pain%20syndrome%20(CRPS,peripheral%
20and%20central%20nervous%20systems.
3. Poree L, Krames E, Pope J, Deer TR, Levy R, Schultz L. Spinal cord stimulation
as treatment for complex regional pain syndrome should be considered earlier than
last resort therapy. Neuromodulation. 2013 Mar-Apr;16(2):125-41
https://www.neuromodulation.com/complex-regional-pain-syndrome-definition
Editor's Notes
Primary trigger.
the syndrome is complex because the patients affected may experience sensory symptoms i.e pain, paraesthesia (pins & needles), local autonomic instability such as oedema, temperature instabilty (hot and cold) skin color changes, trophic changes i.e changes in skin texture, nails and hair distribution with poor skin healing and motor abnormality.
Relatively rare in the elderly.
Very few children under 10.
Almost no children under 5 years are affected.
Unclear as to why some individuals develop CRPS and others with similar trauma do not.
However in more than 90% of the cases, there is a clear history of trauma or injury.
Radiate: may or may not radiate to the entire limb even if the injury might have only involved the finger or toe. Radiate to the contralateral limb in rare cases.
Patients may also experience increased sensitivity in the affected area where by any normal contact or touch of the skin affected is experienced as very painful. This is termed as allodynia.
CGRP - Calcitonin gene-related peptide
There seem to be an aspect of an autoimmune mechanism as high levels of cytokines have been found in the tissues of people with CRPS. These also contributes to the redness, swelling and warth reported by pts.
CRPS is common in pts with inflammatory and autoimune conditions such as asthma.
Clinical experience has shown that CRPS1 is less likely to respond to treatment in its more advanced disease phases. For this reason, it is pertinent that clinicians are able to recognize the early, variable signs of CRPS1 which can provide the opportunity to initiate treatment early and facilitate resolution of this disorder, prior to the development of sequelae and spread.
Despite the utility of the validated Budapest Criteria for diagnosing CRPS, the issues regarding early diagnosis and spread of CRPS, were not addressed in these criteria. Because the early features frequently are labile, the diagnosis may be delayed until they are more consistently displayed. This diagnostic uncertainty may contribute to a delay in the onset of treatment.
Type 1 is further subdividd into phases.
No definitive sequence of stages occurs in all patients.
Diffuse patchy osteoporosis (Sudeck’s Atrophy
For this reason, clinicians should be able to recognize the early, variable signs of CRPS1 which can provide the opportunity to initiate treatment early and facilitate resolution of this disorder, prior to the development of sequelae and spread.
Despite the utility of the validated Budapest Criteria for diagnosing CRPS, the issues regarding early diagnosis and spread of CRPS, were not addressed in these criteria. Because the early features frequently are labile, the diagnosis may be delayed until they are more consistently displayed. This diagnostic uncertainty may contribute to a delay in the onset of treatment.
There are four major categories in the Budapest Criteria utilized in order to diagnose CRPS. These criteria are widely accepted at this point. They have been under study for over 25 years and have been experimentally validated.
In order to obtain information relevant to the clinical diagnosis of CRPS, the following nine diagnostic questions are proposed:
1. Is the pain out of proportion to the injury?
2. Is there a burning or aching quality to the pain?
3. Is there associated allodynia (pain from a non-painful stimulus),
hyperpathia (exaggerated/prolonged pain to a painful stimulus), or
hyperalgesia (increased sensitivity to a painful stimulus)?
4. Is there swelling (edema)? Is there sweating?
5. Are there associated color or skin temperature changes?
6. Is the skin smooth, shiny, or are there pronounced hair follicles?
7. Is there increased or decreased hair growth?
8. Are the nails cracked and grooved?
9. Are there abnormal movements, tone, posture (dystonia), reflexes
or weakness?
It deserves repeating that symptoms and signs will commonly vary with activity, posture, ambient temperature and/or humidity and with stress.
These issues are not typically addressed in the conduct of a standard neurologic examination, but deserve consideration in the context of CRPS.
Diagnoses readily confused with CRPS1/RSD could include those with fairly symmetrical symptoms, such as Raynaud’s syndrome and scleroderma, and possibly diabetic polyneuropathy. Alternatively, conditions with asymmetrical presentations could resemble CRPS, including diabetic neuropathy and post-herpetic neuralgia amongst others. However, these conditions tend to have distinct abnormalities on blood testing and do not have a specific traumatic origin preceding onset.
Within the first week of symptoms, a short course of methylprednisolone, which affords 6 days of tapering therapy can be beneficial to jump-start treatment. This provides 24 mg, 20 mg, 16 mg, 12 mg, 8 mg, and 4 mg of methylprednisolone by mouth
If untreated, or even incompletely treated, the severity of the pain from this disorder has driven people to suicidal ideation, and some to suicide.
This problem is compounded by difficulties in obtaining adequate pain-relieving. Malingering.
The natural history and pathophysiology of CRPS1/RSD is generally acknowledged as highly variable from individual to individual and from time to time within any given individual. This disorder can be the cause of significant morbidity and loss of quality of life. It affects mood, cognition,sleep (falling asleep and staying asleep), bladder and bowel function, self-esteem, job, family, home and includes side effects of medications (such as dry mouth and constipation). There do not appear to be psychological or psychiatric predisposing factors to the development of CRPS1/RSD, although there frequently are consequences of depression and anxiety following its onset.