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Complex Regional
 Pain Syndrome
   Aaron J. Mascarenhas
       080201022
Background
• Recognized in during the civil war.

• The syndromes appearing under this
  include Sudeck’s atrophy, Sympathetic
  dystrophy, algodystophy, shoulder-hand
  syndrome, causalgia.
common?

•   Pain, OUT OF PROPORTION to the inciting
    cause.
•   Vasomotor instability
•   Trophic skin changes
•   Regional osteoporosis
•   Functional impairment
Consesus Expert panel recommended: CRPS
Definition?
“CRPS is a multi-symptom, multi-system,
syndrome usually affecting one or more
extremities, but may affect virtually any part of
the body.”
-International Research Foundation For RSD/CRPS.
Etiology
A number of precipitating factors have been
associated with RSD / CRPS including:
•Trauma (often minor)
•Ischemic heart disease and myocardial infarction
•Spinal cord disorders
•Cerebral lesions
•Infections
•Surgery
•However, in some patients a definite precipitating
event can not be identified
Pathophysiology
•   Sympathetic pain results from tonic activity in myelinated
    mechanoreceptor afferents. Input causes tonic firing in neurons
    that are part of a nociceptive pathway.
•   Injury to central or peripheral neural tissue.
•   Elevated levels of soluble tumor necrosis factor receptor 1
    (sTNF-R1) and enhanced tumor necrosis factor-alpha activity in
    patients with polyneuropathy with allodynia
•   Distal degeneration of small-diameter peripheral axons may
    be responsible for the pain, vasomotor instability, edema,
    osteopenia, and skin hypersensitivity of CRPS-1.
•   Cortical changes, suggesting a possible role in pathophysiology
Clinical features
1.PAIN
 a. occurring in one or more extremities is
    described as severe, constant, burning and/or
    deep aching pain
 b.All tactile stimulation of the skin (e.g. wearing
    clothing, a light breeze) may be perceived as
    painful (allodynia).
 c. Paroxysmal dysaesthesias and lancinating pains
2. SKIN CHANGES
  a. Skin may appear shiny (dystrophy-atrophy), dry or scaly
  b. Hair may initially grow coarse and then thin. Nails in the
      affected extremity may be more brittle, grow faster and
      then slower.
   c. Rashes, Ulcers and Pustules
   d. Abnormal sympathetic (vasomotor changes) activity may be
      associated with skin that is either warm or cold to touch.
   e. Increased sweating (sudomotor changes) or increased
      chilling of the skin with goose flesh (pilomotor changes)
3.SWELLING
a.Localised, initially pitting and later Brawny
b.Edema may be sharply demarcated along
  a line on the skin surface.*
4.MOVEMENT DISORDER
 a.May develop dystonia
 b.Tremors and involuntary jerking of
    extremities may be present.
 c.Disuse atrophy sets in natural history.
1.SPREADING SYMPTOMS
 a. A "continuity type" of spread where the symptoms
     spread upward from the initial site, e.g. from the hand to
     the shoulder.
  b. A "mirror-image type" where the spread was to the
     opposite limb.
  c. An "independent type" where symptoms spread to a
     separate, distant region of the body. This type of spread
     may be spontaneous or related to a second trauma.
  d. *Total body RSD
When to make a
                    diagnosis?
CRPS Type 1:
1. Initiating noxious event or a cause of immobilization
2. Continuing pain, allodynia, or hyperalgesia disproportionate to the inciting event
3. Evidence at some time of edema, changes in skin blood flow, or abnormal
sudomotor activity in the area of pain
4. The diagnosis is excluded by the existence of any condition that would
otherwise account for the degree of pain and dysfunction.
  CRPS Type 2:
• Continuing pain, allodynia, or hyperalgesia after a nerve injury, not necessarily
limited to the distribution of the injured nerve
1. Evidence at some time of edema, changes in skin blood flow, or abnormal
sudomotor activity in the region of pain
2. The diagnosis is excluded by the existence of any condition that would
otherwise account for the degree of pain and dysfunction.
Stage I
1.Onset of severe, pain limited to the site of injury
2.Hyperesthesia
3.Localized swelling
4.Muscle cramps
5.Stiffness and limited mobility
6.At onset, skin is usually warm, red and dry and then it
  may change to a blue (cyanotic) and become cold and
  sweaty.
7. Hyperhydrosis
8. Lasts a few weeks, then subsides spontaneously or
  responds rapidly to treatment.
Stage II
1.Pain becomes even more severe and more diffuse
2.Swelling tends to spread and it may change from a soft to hard
 (brawny) type

3.Hair may become coarse then scant, nails may grow faster
 then grow slower and become brittle, cracked and heavily
 grooved

4.Spotty wasting of bone (osteoporosis) occurs early but may
 become severe and diffuse

5.Muscle wasting begins
Stage III
1.Marked wasting of tissue (atrophic)
 eventually become irreversible.

2.For many patients the pain becomes
 intractable and may involve the entire limb.

3.A small percentage of patients have
 developed generalized RSD affecting the
 entire body.
Medical Management
For constant pain associated with inflammation
      Nonsteroidal anti-inflammatory agents (e.g. aspirin, ibuprofen, naproxen, indomethacin, etc).
For constant pain not caused by inflammation:
      Agents acting on the central nervous system by an atypical mechanism (e.g. tramadol)
For constant pain or spontaneous (paroxysmal) jabs and sleep disturbances;
      Anti-depressants (e.g. amitriptyline, doxepin, nortriptyline, trazodone, etc)
      Oral lidocaine (mexilitine - some what experimental)
For spontaneous (paroxysmal) jabs
Anti-convulsants (e.g. carbamazepine, gabapentin may relieve constant pain as well)
For widespread, severe RSD / CRPS pain, refractory to less aggressive therapies
      Oral opiods tried.
For the treatment of sympathetically maintained pain (SMP)
     Clonidine Patch.
For muscle cramps (spasms and dystonia)
     Klonopin (clonazepam)
     Baclofen
Surgical

1.Sympathetic Blockade
2.Regional Block (Guanethidine)
3.Spinal cord stimulation
4.Morphine pump

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Complex Regional Pain Syndrome (CRPS)/ Causalgia

  • 1. Complex Regional Pain Syndrome Aaron J. Mascarenhas 080201022
  • 2. Background • Recognized in during the civil war. • The syndromes appearing under this include Sudeck’s atrophy, Sympathetic dystrophy, algodystophy, shoulder-hand syndrome, causalgia.
  • 3. common? • Pain, OUT OF PROPORTION to the inciting cause. • Vasomotor instability • Trophic skin changes • Regional osteoporosis • Functional impairment Consesus Expert panel recommended: CRPS
  • 4. Definition? “CRPS is a multi-symptom, multi-system, syndrome usually affecting one or more extremities, but may affect virtually any part of the body.” -International Research Foundation For RSD/CRPS.
  • 5. Etiology A number of precipitating factors have been associated with RSD / CRPS including: •Trauma (often minor) •Ischemic heart disease and myocardial infarction •Spinal cord disorders •Cerebral lesions •Infections •Surgery •However, in some patients a definite precipitating event can not be identified
  • 6. Pathophysiology • Sympathetic pain results from tonic activity in myelinated mechanoreceptor afferents. Input causes tonic firing in neurons that are part of a nociceptive pathway. • Injury to central or peripheral neural tissue. • Elevated levels of soluble tumor necrosis factor receptor 1 (sTNF-R1) and enhanced tumor necrosis factor-alpha activity in patients with polyneuropathy with allodynia • Distal degeneration of small-diameter peripheral axons may be responsible for the pain, vasomotor instability, edema, osteopenia, and skin hypersensitivity of CRPS-1. • Cortical changes, suggesting a possible role in pathophysiology
  • 7.
  • 8. Clinical features 1.PAIN a. occurring in one or more extremities is described as severe, constant, burning and/or deep aching pain b.All tactile stimulation of the skin (e.g. wearing clothing, a light breeze) may be perceived as painful (allodynia). c. Paroxysmal dysaesthesias and lancinating pains
  • 9. 2. SKIN CHANGES a. Skin may appear shiny (dystrophy-atrophy), dry or scaly b. Hair may initially grow coarse and then thin. Nails in the affected extremity may be more brittle, grow faster and then slower. c. Rashes, Ulcers and Pustules d. Abnormal sympathetic (vasomotor changes) activity may be associated with skin that is either warm or cold to touch. e. Increased sweating (sudomotor changes) or increased chilling of the skin with goose flesh (pilomotor changes)
  • 10.
  • 11.
  • 12. 3.SWELLING a.Localised, initially pitting and later Brawny b.Edema may be sharply demarcated along a line on the skin surface.*
  • 13.
  • 14. 4.MOVEMENT DISORDER a.May develop dystonia b.Tremors and involuntary jerking of extremities may be present. c.Disuse atrophy sets in natural history.
  • 15. 1.SPREADING SYMPTOMS a. A "continuity type" of spread where the symptoms spread upward from the initial site, e.g. from the hand to the shoulder. b. A "mirror-image type" where the spread was to the opposite limb. c. An "independent type" where symptoms spread to a separate, distant region of the body. This type of spread may be spontaneous or related to a second trauma. d. *Total body RSD
  • 16. When to make a diagnosis? CRPS Type 1: 1. Initiating noxious event or a cause of immobilization 2. Continuing pain, allodynia, or hyperalgesia disproportionate to the inciting event 3. Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the area of pain 4. The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction. CRPS Type 2: • Continuing pain, allodynia, or hyperalgesia after a nerve injury, not necessarily limited to the distribution of the injured nerve 1. Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the region of pain 2. The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.
  • 17. Stage I 1.Onset of severe, pain limited to the site of injury 2.Hyperesthesia 3.Localized swelling 4.Muscle cramps 5.Stiffness and limited mobility 6.At onset, skin is usually warm, red and dry and then it may change to a blue (cyanotic) and become cold and sweaty. 7. Hyperhydrosis 8. Lasts a few weeks, then subsides spontaneously or responds rapidly to treatment.
  • 18. Stage II 1.Pain becomes even more severe and more diffuse 2.Swelling tends to spread and it may change from a soft to hard (brawny) type 3.Hair may become coarse then scant, nails may grow faster then grow slower and become brittle, cracked and heavily grooved 4.Spotty wasting of bone (osteoporosis) occurs early but may become severe and diffuse 5.Muscle wasting begins
  • 19. Stage III 1.Marked wasting of tissue (atrophic) eventually become irreversible. 2.For many patients the pain becomes intractable and may involve the entire limb. 3.A small percentage of patients have developed generalized RSD affecting the entire body.
  • 20. Medical Management For constant pain associated with inflammation Nonsteroidal anti-inflammatory agents (e.g. aspirin, ibuprofen, naproxen, indomethacin, etc). For constant pain not caused by inflammation: Agents acting on the central nervous system by an atypical mechanism (e.g. tramadol) For constant pain or spontaneous (paroxysmal) jabs and sleep disturbances; Anti-depressants (e.g. amitriptyline, doxepin, nortriptyline, trazodone, etc) Oral lidocaine (mexilitine - some what experimental) For spontaneous (paroxysmal) jabs Anti-convulsants (e.g. carbamazepine, gabapentin may relieve constant pain as well) For widespread, severe RSD / CRPS pain, refractory to less aggressive therapies Oral opiods tried. For the treatment of sympathetically maintained pain (SMP) Clonidine Patch. For muscle cramps (spasms and dystonia) Klonopin (clonazepam) Baclofen
  • 21. Surgical 1.Sympathetic Blockade 2.Regional Block (Guanethidine) 3.Spinal cord stimulation 4.Morphine pump