complex regional pain syndrome

1. Complex Regional Pain Syndrome
(CRPS)
Presenter :Dr pankaj bhosale
Moderator : Dr Jesto

2. 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

3. MC GILL PAIN INDEX AND CRPS

4.  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)

5. 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

6. 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 Syndrome
TYPE I TYPE II

7. 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

8. 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)

9. 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

10. 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

11. 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

12. Pathophysiology
 Not well understood for many years
 Renewed research interest
 Revised diagnostic criteria
 Availability of animal models of CRPS
Pain 2015; 156: S94-S103

13. 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

14.  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

15. Pathophysiology summarised…

16. 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

17. Speculative model of crps

18. Electrical Stimulation induces Plasma Protein Extravasation in
CRPS

19. 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

20. 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

21. 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

22. 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

23. Having a Check-List
will help with the
Diagnosis

24. Item 4. No other diagnosis explaining the signs and symptoms

25. Management

26. 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

27. 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

28. The 4 Pillars of Management

29. Pain Relief
 Corticosteroids
 Calcium-regulating drugs
 Calcitonin, Alendronate
 Opioids
 Anti Neuropathic drugs
 Ketamine
 Sodium channel blockers
 Lignocaine
 Clonidine
Very little consistent information is
available for these pharmacological
agents
 In early CRPS – may be useful
 Has been tried
 Intravenous immunoglobulins 0.5 g/kg
 When others fail to provide pain relief
 TCA, gabapentin, pregabalin
 Ketamine ( Low dose infusions have been
tried with mixed results)
 Dimethyl sulfoxide and n-acetyl cystiene
 Pain relief – so that patients are able to
comply with physical therapy, which is the
KEY to management
Medications

30. CREATE 1 TRIAL – CRPS TREATMENT EVALUATION 1 STUDY

31. Procedures
 Intravenous Regional Techniques
 Local anaesthetics
 Guanethidine
 Selective Sympathetic ganglion
blocks
 Useful is some cases of
sympathetically maintained pain
 Spinal Cord Stimulation and
Neuromodulation

32. Figure 2 Pathophysiology and mechanism-based treatment options in CRPS
Gierthmühlen, J. et al. (2014) Mechanism-based treatment in complex regional pain syndromes
Nat. Rev. Neurol. doi:10.1038/nrneurol.2014.140

33. 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

34. Psychological Therapy
 Patient education and support
 Goal setting
 Relaxation techniques
 Pacing techniques
 Coping skills
 Facilitating self-management of condition

35. Watch for Yellow Flags

36. Rsdsa foundation

37. World orange day

38. 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

39. THANK YOU