1. CRPS Images
Complex Regional Pain Syndromes (CRPS)

2. Definition of CRPS Type I
a syndrome
initiating noxious event
not limited to the distribution of a single peripheral nerve
disproportionate to the inciting event
associated with edema, vasomotor, sudomotor, allodynia, and hyperalgesia in the region of pain
Affects about 2% of the population and that’s about it.

3. Causes
Trauma
sprain, strain, dislocation, fracture, laceration, contusion, crush injury, surgery, manipulation, tight cast, occupational repetitive trauma
Disease
intracerebral, intraspinal, nerve roots, ami, infection( joint, skin, periarticular), peripheral vascular
Idiopathic ( about 1/3rd of all the cases)

4. Epidemiology Onset 9 – 85 years of age Median 42 years
Women 3x > men
Veldman PH, Reynen HM, Arntz IE: Signs and symptoms of reflex sympathetic dystrophy: prospective study of 829 patients. Lancet 1993 Oct 23; 342(8878):

5. Modified from Blumberg, J. Auton. Nerv. Sys. 1983
All of the vaso-sudo motor responses are reactions. Causes wrong signals sent to and from the brain, causing abnormal motor responses which then feeds back distorted information sent to the brain via the spinal cord, etc. All are under sympathetic control – if blood flow is disrputed then there can be swelling and chronic atrophy, trophic changes. Result is an atrophic limb.
Modified from Blumberg, J. Auton. Nerv. Sys. 1983

6. Pathophysiology sympatholytic therapy abolishes pain and hyperalgesia
Sympathetically maintained pain
sympatholytic therapy abolishes pain and hyperalgesia
sympatholytic blockade followed by administration of adrenoceptor agonists, rekindles pain
distal electrical stimulation of a freshly cut sympathetic nerve induced pain in a patient with sympathetically maintained pain
Sympathetic system was once blocked by surgery – cut the affected ganglion nerve and thus no sx…and also no pain, sweating, feeling, etc. Pretty drastic stuff.

7. Pathophysiology(continued)
Ghostine et al - ephaptic transmission
erosion of nerve insulation -> abnormal internerve communication
short circuiting between somatic afferents and sympathetic efferents
Bennett (NIH) - sprouting of damaged nerves
sensitive to norepinephrine
will discharge upon exposure to norepinephrine
sympathetic fibers as a source of norepinephrine
produce norepinephrine receptors at damaged ends
nociceptors in intact nerves fire more in response to norepinephrine

8. Pathophysiology(continued)
Schwartzman et al. - autoimmune etiology
tissue injury -> nerve growth factor release -> activation of sympathetic neurons -> recruitment of neutrophils/monocytes -> complement activation -> interleukin 2
Roberts - sensitization of intraspinal wide dynamic range (WDR) neurons
C fiber nociception
A fiber mechanoreceptor
sympathetic efferents
C fiber blockade fails alleviation of SMP
mechanoreceptor response to sympathetic activity
A fibers are the supertransmitters. C fibers are ?
These are theories, none proven.

9. Thalamus
Sympathetics
WDR Neurons
A Fiber Receptor
C Fiber Receptor

10. Pathophysiology(continued)
Sympathetic postganglionic neuron/afferent neuron coupling
direct noradrenergic coupling
within traumatized nerve
within dorsal root ganglion
via microvascular bed
indirect noradrenergic coupling
ephaptic coupling
? Abnormal inflammatory response

11. CLINICAL HISTORY ANTECEDENT TRAUMA WHEN WHERE TYPE SEVERITY
NERVE INVOLVEMENT

12. CLINICAL HISTORY (CONTINUED)
PAIN
BURNING, ACHING, THROBBING, STINGING, CONTINUOS WITH EXACERBATIONS, “EXCRUTIATING”, “UNBEARABLE”
SYMPATHETIC PAIN: CONSTANT, SPONTANEOUS, WORSE AT NIGHT, WORSE WITH MOVEMENT, TACTILE AND THERMAL STIMULI
IMMEDIATE OR DELAYED ONSET(WEEKS), GRADUAL INCREASE IN INTENSITY
PROPENSITY TO DIFFUSE, IPSILATERAL/CONTRALATERAL LIMB INVOLVEMENT
Usually starts on the same side then progresses to opposite side as condition continues.

13. CLINICAL HISTORY (CONTINUED)
INITIAL DESCRIPTION OF PAIN
ADEQUACY OF TREATMENT
CHANGE IN CHARACTER/INTENSITY
IMMOBILIZATION
HOW LONG, TO WHAT EXTENT
HAS THE PRECIPITATING FACTOR RESOLVED?
VASOMOTOR CHANGES?
SUDOMOTOR CHANGES?
Vascular = swelling/sweating. Will rsvp to sympathetic manipulation like needling.
Sudomotor = pain upon movement. Produces atrophy of the muscles.

14. CLINICAL HISTORY(CONTINUED)
TROPHIC CHANGES?
PSYCHOLOGICAL COMPONENT?
LITIGATION?
PAST MEDICAL HISTORY
SYMPATHOLYTC MEDICATIONS
FACTORS LIMITING PHYSICAL ACTIVITY
NICOTINE, CAFFEINE
Psychological component – ideation, or depression due to long term pain. Nicotine and caffeine will trigger pain rsvp more.

15. PHYSICAL EXAMINATION COMPLETE GENERAL EXAM CARDIOPULMONARY VASCULAR
NEUROLOGIC
MUSCULOSKELETAL
GENERAL APPEARANCE
AFFECT, MOOD
APPREHENSION, PROTECTIVE AND PAIN BEHAVIORS
Do all vitals, reflexes, gait, cranial nerves, cerebellar signs, then flexion/extension/tonicity. General appearance is how do they look overall. Protective refers to protecting the affected area.

16. PHYSICAL EXAMINATION SYMMETRICAL VISUAL INSPECTION PALPATION
AFFECTED LIMB
SYMMETRICAL VISUAL INSPECTION
PALPATION
MOTOR/SENSORY EXAM
Affected will be hotter or colder. Motor/sensory = heat, cool, touch, etc., including proprioception.

17. PHYSICAL EXAMINATION OF THE AFFECTED LIMB
VISUAL INSPECTION
SWELLING
DISCOLORATION (ERYTHEMA, PALLOR, BLUISH MOTTLING, BRAWNY EDEMA)
HYPERHIDROSIS
MUSCLE WASTING
POSTURING
JOINT ABNORMALITY
EVIDENCE OF TRAUMA
Brawny edema – may brown with long term edema. Posture refers to how they hold the body and whether you can see the pain there.

18. PHYSICAL EXAMINATION OF THE AFFECTED LIMB
skin thickening, wrinkling, flaking
skin thinning, smoothing, tightening, shining
hair coarsening, lengthening, increase in distribution
nail thickening, ridging, weakening with accelerated growth, growth asymmetry
arthritic appearing joints
Long term joint disuse may appear arthritic.

19. Physical Examination: Palpation
Affected Limb
allodynia
hyperesthesia
hyperalgesia
warmth
coolness
sweaty
coarse skin
Hyperesthesia = hypersensitive. Hyperalgesia = excess pain response.

20. Physical Examination: Motor & Sensory Exam
Affected Limb
weakness
tremor
fine motor movement
decreased AROM/PROM
allodynia
hyperesthesia
hyperalgesia
Unaffected Areas
neck/shoulder stiffness
trapezial spasm with shoulder elevation and loss of motion
altered gait with subsequent hip and back pain
Fine motor movement can decrease. Stiffness and spasms even in unaffected areas, can’t shrug well. As you can tell, affects all body parts.

21. Diagnostic Tests
Sensory
Von Frey hairs, brush hairs, feather
Sudomotor
ninhydrine sweat test, skin conductance response, cobalt blue test
Swelling
tape measure
water displacement
Joint mobility
goniometer

22. organic vs. nonorganic patient
Diagnostic Tests
Psychological
External Motor Behavior (ADL, disability)
Visual Analogue Scale
McGill pain questionnaire
Minnesota Multiphasic Personality Inventory (MMPI)
chronic pain profile
organic vs. nonorganic patient
See the McGill Pain Questionnaire, also downloadable from the internet.

23. Diagnostic Tests Illness Behavior Questionnaire general hypochondria
Psychological
Illness Behavior Questionnaire
general hypochondria
illness conviction
psychological/somatic perception
emotional inhibition
dysphoria
rejection
irritability
Depression and Anxiety Tests

24. Treatment Prevention Early Diagnosis Physical Therapeutics
Overview
Prevention
Early Diagnosis
Physical Therapeutics
Pharmacological Therapeutics
Psychological Therapy
Prevention of Late Complications
Outcome Measurement

25. Treatment: Prevention
high risk patient
trauma
cva
nerve injury
early mobilization
AROM/PROM
Braus
patents with stroke and hemiplegia
early PT
27% to 8% incidence of CRPS Type I

26. Treatment: Early Diagnosis
improved outcome
high degree of suspicion
early treatment

27. Treatment: Physical Therapeutics
elevation
compression
heat/cold
tens/ultrasound
stretching/AROM/PROM
stress loading
exercise(active/passive)

28. Treatment: Pharmacological Therapeutics
Components of Pain
inflammatory
neuropathic
sympathetic
central nervous system

29. Treatment: Pharmacological Therapeutics
Inflammatory Component
NSAIDS
central effect of prostaglandins
IM/IV RB toradol - one study with good effect
early phase intervention
Prednisone -
efficacy comparable to sympatholytics
1 mg/kg (up to 100 mg/day), 2 week taper
membrane stabilizing effects
binding to lamina III and VII

30. Treatment: Pharmacological Therapeutics
Neuropathic Component
anticonconvulsants - disappointing
tricyclics - paucity of trials
gabapentin - at least one study: highly effective
CNS Component
opioids
TCAs
anticonvulsants
NSAIDs, steroids
Tricyclics are good antidepressants and also raise pain thresholds, but have nasty side fx. Amitryptaline is an example.
Opioids, usually codeine, is extremely addicting.

31. Treatment: Pharmacological Therapeutics
Calcitonin
? mechanism of action in CRPS I
moderate efficacy in some studies

32. Treatment: Surgical Intervention
Chemical Sympathectomy
phenol, alcohol
longer than sympathetic blockade
pain recurs
Radiofrequency Sympathectomy
Endoscopic-guided Sympathectomy
Open Surgical Sympathectomy
Results: 12-90% efficacy % recurrence
Complications: sympathalgia in 7-44% of patients

33. Treatment: Prevention of Late Complications
muscle atrophy/weakness
osteoporosis
contractures
pain

34. A 29-year-old woman with reflex sympathetic dystrophy in the right foot demonstrates discoloration of the skin and marked allodynia.
Right foot is more swollen, color differentiations. Vascular blockage.

35. This photo shows the same patient as in the above image, following a right lumbar sympathetic block. Marked increase in the temperature of the right foot is noted, with more than 50% pain relief.

36. A 68-year-old woman with complex regional pain syndrome type II (causalgia).
Nerve involvment on the left foot.

37. A 36-year-old woman with right arm reflex sympathetic dystrophy and dystonic posture (movement disorder).
Note right hand – can’t straighten fingers. Dystonic posture too.

38. Normal laser Doppler study of the upper extremities
Normal laser Doppler study of the upper extremities. When the patient performs inspiratory gasp repeatedly during laser Doppler image acquisition, the transient capillary flow decreases are displayed easily and dramatically (as dark bands) in the pseudocolor image.

39. Laser Doppler study of the upper extremities in a patient with right hand reflex sympathetic dystrophy.

40. Laser Doppler study of the lower extremities in a 25-year-old woman with reflex sympathetic dystrophy in the right foot.
Better supply to the left foot – the darker blue indicates less inervation and less flow.