complex regional pain syndrome - dr. ramani
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Complex Regional Pain Syndrome(CRPS)
Ramani VijayanUniversity Malaya Medical Centre
Kuala LumpurMalaysia
CRPS
Is a debilitating painful condition in a limb Associated with sensory, motor, autonomic, skin and
bone abnormalities
PAIN is the leading symptom Often associated with limb dysfunction Psychological Distress
It commonly arises after injury to a limb (sometimes trivial)
CRPS
Type 2Type 1
Absence of a major nerve lesion
Presence of a major nerve lesion
(Reflex sympathetic dystrophy Sudeck’s Atrophy )
(Causalgia)
Historical Background First described by Weir Mitchell after the American
Civil War in 1872 When he encountered soldiers who were injured by gun-shot
wounds exhibiting “bizarre” symptoms and coined the term “Causalgia”
Early 20th century Peter Sudeck Described features of pain, swelling, atrophy etc. following
minor injury to limbs – hence this phenomenon came to be called “Sudeck’s atrophy”
A few years on – its association with the sympathetic nervous system was recognized and hence the term “reflex sympathetic dystrophy” was increasingly used
IASP Consensus group - in 1994
The presence of an initiating noxious event, or a cause for immobilization
Continuing pain and allodynia which is disproportionate to any inciting event
Evidence at some time of oedema, changes in skin blood flow, abnormal sudomotor activity in the region of pain
The diagnosis is excluded by the existence of other conditions that can account for the degree of pain and dysfunction
Criteria 2-4 must be satisfied
Continuing pain, allodynia, or hyperalgesia after nerve injury, not necessarily limited to the distribution of the injured nerve
Evidence at some time of oedema, changes in skin blood flow, abnormal sudomotor activity in the region of pain
The diagnosis is excluded by the existence of other conditions that can account for the degree of pain and dysfunction
All 3 Criteria must be satisfied
Complex Regional Pain SyndromeTYPE I TYPE II
1994 IASP Criteria
Proved to be extremely sensitive
Insufficiently specific Over diagnoses of the
syndrome Difficult to validate
In 2003, a workshop was held in Budapest
Published in 2007 Modified diagnostic
criteria Better discrimination
between CRPS and Non-CRPS neuropathic pain
IASP revised criteria – “Budapest criteria”
Continuing pain that is disproportionate to any inciting event
At least one symptom reported in at least 3 of the following categories Sensory: Hyperesthesia or allodynia Vasomotor: Temperature asymmetry, skin colour changes,
skin colour asymmetry Sudomotor/ Oedema, sweating changes or sweating
oedema asymmetry
Motor / Trophic Decreased range of motion, motor dysfunction (E.g. weakness, tremor, dystonia), or trophic changes (e.g. hair, skin, nails)
Criteria continued ……………
At least one sign at time of evaluation in at least two of the following categories Sensory: Evidence of hyperalgesia (to pinprick),
allodynia (to light touch), temperature
sensation, deep somatic pressure or joint movement Vasomotor: Evidence of temperature asymmetry (>1°C),
skin colour changes or symmetry
Sudomotor/ Evidence or oedema, sweating changes, or Oedema sweating asymmetry
Motor/Trophic Evidence of deceased range of movement, motor dysfunction
(E.g. weakness, tremor, dystonia), trophic changes (E.g. nail, skin, hair)
No other diagnosis explaining the signs and symptoms
More stringent criteria in a research setting: to increase specificity
At least one SYMPTOM in Three ‘SYMPTOM’ categories
At least one SIGN in Three ‘SIGN’ categories
For diagnosis of CRPS in a Research Setting
Epidemiology A population study from the Netherlands showed
Incidence of 26 in 100,000; Female : Male ratio of 3.5:1 Peak incidence in the age group of 55-70 years Upper limb more common than the lower Fracture was the most common precipitating factor CRPS I more common than CRPS II
(de Mos M et al. Pain 2007; 129:12-20)
This was 4 times that of a previous population study in the US – 5.5 / 100,000
Pathophysiology Not well understood for many years Renewed research interest
Revised diagnostic criteria Availability of animal models of CRPS
Pain 2015; 156: S94-S103
Inciting event – Trivial injury to the limb or injury to a peripheral nerve
Inflammation is exaggerated for yet unclear reasons inflammatory mediators leading to swelling, changes in
colour, increased skin temperature Pain and hyperalgesia Hyperhidrosis / hypohidrosis due to mediators such as
neuropeptides (Substance P, CGRP, bradykinin) Trophic changes due to cytokines Motor function is also impaired by peripheral inflammation
Increased levels of TNF-α, with a decrease in anti-inflammatory cytokines
Neuropeptides – released by sensitized peptidergic nociceptors ( neurogenic inflammation)
Endothelin-1 – potent vasoconstrictor (hyperalgesia) Autoantibodies on surface structures of β2-adregeric
receptors and m2-cholinergic receptors (which provides a link to the sympathetic nervous system)
In a significant subset of patients, CRPS gradually “centralizes” – (time frame varies with individuals) Mechanical hyperalgesia Non-dermatomal sensory deficits Body perception disturbances Movements disorders
Conceptual model of CRPS -
• Enhanced anti-dromic secretion of Neuropeptides
• Enhanced release of Immune mediators
• Surface binding auto-antibodies
• Contributes to changes in sensory nerve function and axonal degeneration
• Viscous cycle is set in motion
Electrical Stimulation induces Plasma Protein Extravasation in CRPS
Pathophysiology which includes central components
Clinical Features are a continuum Stage One (acute stage – 6-8 weeks after injury)
Warmth, coolness, burning pain, oedema, increased sensitivity to touch, increased pain with hyperalgesia, accelerated hair / nail growth, tenderness or stiffness of joints, spasm, bone changes on X-ray
Decreased sympathetic activity
Clinical Features of CRPS Stage Two: Dystrophic phase ( can last several
months) Pain is constant – throbbing, burning, aching, exaggerated by
stimuli Affected limb may still have oedema, cool, mottled appearance Nails – brittle and ridged Pain and stiffness of joints persist Muscles – tremors, signs of wasting
Psychological distress sets in (mainly from lack of pain relief)
Changes in body perception (limbs) Increased sympathetic activity
Stage 3 – Atrophic phase (unlimited amount of time)
Typically the patient has had CRPS for 3+ years Pain is still constant (varies in degree depending on
the patient) Skin is cool, thin and shiny Atrophy of limb – with contractures of joints Muscle wasting Increase in osteoporosis Unlikely for sympathetic blocks to be effective
Central changes CRPS features can extend beyond the original region
Diagnosis Clinical – There are no specific tests Differential diagnosis needs to be considered and
excluded
• 53 year old male patient• Clinical features of pain and allodynia• Limited to the fourth and fifth fingers• Bluish and significantly cooler
• Thermography showed only the innervation of the ulnar nerve was colder
• Traumatic ulnar paresis
• Diagnosis : Posttraumatic neuralgia
Having a Check-List will help with the Diagnosis
Item 4. No other diagnosis explaining the signs and symptoms
Management
Early Recognition is the key to Management
• Patients may be first seen by a host of different specialists
• It is important to create awareness about CRPS in these groups
• Early recognition and referral for appropriate therapy can improve chances for better management
Budapest diagnostic criteria (A–D must apply).
Andreas Goebel Rheumatology 2011;rheumatology.ker202
© The Author 2011. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com
CHECK LIST
The 4 Pillars of Management
Pain Relief
Corticosteroids Calcium-regulating drugs
Calcitonin, Alendronate Opioids Anti Neuropathic drugs Ketamine Sodium channel blockers
Lignocaine ClonidineVery little consistent information is available for these pharmacological agents
In early CRPS – may be useful Has been tried
When others fail to provide pain relief TCA, gabapentin, pregabalin Ketamine ( Low dose infusions have
been tried with mixed results)
Pain relief – so that patients are able to comply with physical therapy, which is the KEY to management
Medications
Procedures Intravenous Regional
Techniques Local anaesthetics Guanethidine
Selective Sympathetic ganglion blocks Useful is some cases of
sympathetically maintained pain
Spinal Cord Stimulation and Neuromodulation
Physical and Vocational therapy
Patient education Stretching General exercise and
Strengthening Desensitization Gait re-education TENS Postural control Oedema control
strategies Sleep hygiene
Graded Motor Imagery Mirror Visual Feedback
Patient viewing non‐reflective surface with painful limb hidden.
C. S. McCabe et al. Rheumatology 2003;42:97-101
© British Society for Rheumatology
Patient viewing non‐painful limb in mirror with painful limb hidden.
C. S. McCabe et al. Rheumatology 2003;42:97-101
© British Society for Rheumatology
Psychological Therapy
Patient education and support Goal setting Relaxation techniques Pacing techniques Coping skills Facilitating self-management of condition
Watch for Yellow Flags
In Conclusion CRPS is a chronic debilitating painful condition There has been significant advances in our
understanding of its Pathophysiology Early diagnosis and management – is essential to
help patients and reduce suffering The Budapest Criteria should help while excluding others
A Multidisciplinary Approach to Management has been shown to be beneficial With particular emphasis on Patient Education and Support
https://www.rcplondon.ac.uk/sites/default/files/documents/complex-regional-pain-full-guideline.pdf
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