A rare but challenging ,pain disease-CRPS

1. Complex Regional Pain Syndrome
Lejay Jacob
All Saints University of Medicine
Psychiatry Clerkship, MD 9
Preceptor:
Dr. Daniel B. Martinez
Presented:June 11, 2015

2. Overview
DSM 5
Types
Etiology &
Pathophys
Epidemiology
Symptoms
Dx
Related Diseases
Txt
Prevention &
Prognosis
References

3. DSM 5
• Classified as a Somatic Symptom Disorder
-All Pain Disorders in DSM4 are omitted and now
fit under this category , has a specified clause
- ICD 10 code F45.1, G56.4, G57.7 , M89.(0-9)
Terms
Somatic- of/relating to body , as opposed to mind
Soma( Gk)= Body
Algesia=Pain
HyperAlgesia- excess pain to normal stimuli (eg. Pinprick)
Allodynia- pain from non harmful stimuli (eg. Feather stroke- dynamic mechanical allodynia)
Hyperpathia- increased painful response to repeated stimuli

4. Types
• The International Association for Study of Pain
(IASP) proposed 3 types -
• Complex Regional Pain Syndrome Type 1
-formerly know as Reflex Sympathetic Dystrophy (RSD)
-Sudeck’s Dystropy/ Atrophy/ Disease
-Reflex Neurovascular Dystrophy
-AlgoDystrophy/ AlgoNeuroDystrophy
-Shoulder Hand Syndrome
• CRPS Type 2
-formerly known as Causalgia
-from caus=heat and algia= pain
- Significant nerve injure Seen
• CRPS –NOS (Not Otherwise Specified)

5. Image Source- www.occupational-therapy.advanceweb.com
Nerve injury evidentNo nerve injury seen

6. Etiology and PathoPhysiology
• Etiology unknown
• Pathophysiology – prospective models but not
proven for all CRPS cases.
-modest rather than extensive abrasion of
perineurium is likely to cause CRPS 1
-inflammatory cytokines in affected and unaffected
limb in blood are not significant. More factors
others than inflammation might be involved in
chronic CRPS’s changing inflammatory profile.2
1-Needlestick injury caused CRPS- Hand Surgery, 2014
2-inflammation in CRPS, American academy of Neurology 2013

7. Pathophysiology (contd)
• CRPS in p/t s with dystonic symptoms have been shown to have link to
gene markers HLA –DR 13, 15 and DQ1. This may show predisposition to
Autoimmune diseases 1.
• High levels of TNF alpha in Acute CRPS. Overproduction of cytokines in
keratinocytes , proliferation , more mast cells in affected skin Acute CRPS .
In chronic CRPS, cytokines normalize over a course, indicating varying
pathophysiology 1
• In chronic CRPS , there is a notable decrease in grey matter in areas that
are responsible for stress processing such as right insular cortex, nucleus
accumbens , ventromedial prefrontal cortex. 1
• Other models include 2-
1- CRPS –optimistic, Neurology. 2015. 2- CRPS . DynaMed database 2015
Inflammation
SNS dysfunction
Central N.S. dysfunction
AutoImmune condition
Rheumatology
Limb Ischemia or Ischemia Reperfusion Injury
Cortical reorganization – correlation between
amount of reorganization and pain intensity
with limb alienation
Nerve damage( small fiber Neuropathy)
Neurogenic Inflammation

9. Xrays of CRPS cases
Source- radiopedia.org
Findings- severely patchy Osteopenia particularly in the peri-articular region
-Soft tissue swelling, with eventual soft tissue atrophy
-Subperiosteal bone absorbtion
-Preservation of Joint spaces

10. Epidemiology
• Only 2 studies have been performed over a large scale ,
A US study found incidence of 5.46 cases per 100,000
per year(1999) and a Netherlands study showed 26.2
cases per 100,000 per year in 2007. The prevalence of
the 1999 study was 20.57 per 100,000 cases.
• In both studies the female to male ratio was between
3.4 to 4 per 1 male.
• The peak age range was 50 to 70*.
• 3-5% of all distal radial fractures subsequently develop
CRPS 2
*CRPS . BMJ 2014 2- Moseley et.al. J. Pain 2014 :15:16-23

11. Symptoms

12. Symptoms
• Warm CRPS(85%) vs Cold CRPS( worse
prognosis*)
- See Columns to rate severity of CRPS in Dx
section of presentation. All may be present
based on severity.
*CRPS BMJ 2014

13. Dx
Image source- CRPS BMJ 2014

14. Image Source- CRPS –optimistic. Neurology 2015

15. Mis diagnosed,
Similar presenting diseases
• Malingering or somatising. Psychologic profile
and pre-existing depression or anxiety do NOT
predispose individual to CRPS.1
• Limb pain due to-
1-BMJ 2014
Non-union of fracture
Tendonitis
Diabetic Peripheral Neuropathy
Oteomyelitis
Cellulites
Poly Neuropathy
Radiculopathy
DVT
Raynaud Phenomenon

16. Txt
• Exercise,
Short videos in reference slide
• Mirror Therapy, Visual feedback
• and Graded Motor Imagery
• Desensitisation
• Psychotherapy. There are no studies shown
improvement in CRPS but since it is used in other
neuropathic pain management it should not be
neglected. Often helps CRPS caused Anxiety and
depression . CBT has been proven to help chronic pain
patients 2
Image- CRPS treatment- NHS.uk 2-NHS choices. Web.

17. Txt• Encourage p.t. to use affected extremities. Increase in pain on exacerbation does
not indicate detioration. However painful external intervention by the health care
team or invasive procedure suggestions , especially against the will of the p.t.
should be avoided
• Physical therapy. Two different approaches of PT have been observed to be used in
CRPS p/ts- PEPT( Pain Exposure PT) and GExP.( Graded Exposure PT). Studies show
that which form did not show difference in recovery but regaining limb usefullness
should be key goal in all CRPS txt.
• Pacing , goal setting and relaxation techniques should be implemented
• General medications for neuropathic pain management include TCAs , SSRI, SNRI,
anticonvulsants like gabapentin and pregabalin, topical lidocaine.1
• Bisphosphonates
• Regional Anesthetic blocks
• Opiates like morphine and codeine
• Topical Dimethylsulfoxide(DMSO 50%) for mainly warm CRPS p/t.s
• If non invasive procedures fail, Invasive procedures such as blocking relavant SNS
fibres[casestudy next slide] or Spinal Cord Stimulation implants. The Implants have
been particularily shown to be useful in lower limb CRPS cases. 2
• Amputation
1- CRPS BMJ 2- CRPS optimistic – Neurology . 2015

18. Case Study results
-Invasive procedure
RF ablation of stellate ganglion in CRPS.
Saudi Journal of Anesthesia. 2014

19. Prevention and Prognosis
• A multispecialty CRPS care team would include the following
healthcare specialists –psychotherapist, occupational therapist, pain
relief specialist, psychologist, neurologist, social worker,
employment advisor.1
• Vitamin C has been shown to be preventive in progression to
Chronic CRPS and in prevention of beginning of Acute CRPS , after a
minor neurologic trigger. 2
• Prognosis and recovery varies based on multiple factors. Studies of
improvement in acute CRPS of more than 50% in pain all the way to
complete remission of pain, for 55 out of 74 , have been
documented 3.
• Although CRPS presents as a incrementing condition , after the first
year ,the majority present only with a moderate increase in disease
severity 4.
1- NHS Choices, web 2- CRPS . Dynamed database 2015.
3 CRPS BMJ 2014 4- CRPS needlestick injury. HandSurgery 2014

20. References
• Video- exercises – Youtube.com. Last accessed on June 2015.
• Image- title page. Bennett, T. (2012, September 10). How Can Complex Regional Pain Syndrome
(CRPS) Be Treated? (Part 2). Retrieved June 10, 2015, from
http://workerscompensationwatch.com/2012/09/10/how-can-complex-regional-pain-syndrome-
crps-be-treated-part-2/
• Image, slide 2- Stickmancommunications.co.uk. Retrieved June 10, 2015.
• Image Slide 4- Complex regional pain syndrome. (n.d.). Retrieved June 10, 2015, from
http://en.wikipedia.org/wiki/Complex_regional_pain_syndrome
• Image slide 5- IIS7. (n.d.). Retrieved June 10, 2015, from http://www.occupational-
therapy.advanceweb.com
• Image slide 9- Complex regional pain syndrome | Radiology Reference Article | Radiopaedia.org.
(n.d.). Retrieved June 10, 2015, from http://radiopaedia.org/articles/complex-regional-pain-
syndrome
• Image Slide 11- CRPS Ppt. (n.d.). Retrieved June 10, 2015, from
http://www.authorstream.com/Presentation/Anowak757-1451250-crps-ppt/
• Image Slide 16- Complex regional pain syndrome - Treatment . (n.d.). Retrieved June 10, 2015, from
http://www.nhs.uk/Conditions/Complex-Regional-Pain-Syndrome/Pages/Treatment.aspx
• Image Slide 24- Dermatome (anatomy). (n.d.). Retrieved June 10, 2015, from
http://en.wikipedia.org/wiki/Dermatome_(anatomy)

21. References
• Diagnostic and statistical manual of mental disorders: DSM-5. (5th ed.). (2013). Washington,
D.C.: American Psychiatric Association.
• Hirata, H. (2014). How big of a threat is Needlestick- injury-induced Complex Regional Pain
Syndrome? A scientific perspective. Hand Surgery, 19(2), 151-162.
• Parkitny, L., McAuley, J. H., Pietro, F. D., Stanton, T. R., O'Connell, N. E., Marinus, J., . . . Van
Hilten, J. J. (2013). Inflammation in Complex Regional Pain Syndrome. A systematic review
and meta- analysis. Neurology, 80, 106-117
• Birklein, F., O'Neill, D., & Schlereth, T. (2015). Complex Regional Pain Syndrome. An optimistic
perspective. Neurology, 84, 89-96.
• DynaMed. (updated 2014, November 19). Complex Regional Pain Syndrome. Ipswich, MA:
EBSCO Information Services. Retrieved June 5, 2015, from
http://search.ebscohost.com/login.aspx?direct=true&db=dme&AN=113862&site=dynamed-
live&scope=site
• Fukushima, F. B., Bezerra, D. M., Villas, P. J., Valle, A. P., & Vidal, E. I. (2014). Complex Regional
Pain Syndrome. British Medical Journal (BMJ), 348. doi:10.1136
• Case Study 1-Roy, C., & Chatterjee, N. (2014). Radiofrequency ablation of stellate ganglion in
a patient with Complex Regional Pain Syndrome. Saudi Journal of Anesthesia, 8(3), 408-411.
• Case Study 2- Breivak, H. (2013). European pain management discussion forum. Case
study. Journal of Pain and Palliative care Pharmacotherapy, 27, 190-191.
doi:10.3109/15360288.2013.788607
• Le, T., Bhushan, V., & Singh, B. H. (2010). First aid for the USMLE step 2 CK. New York:
McGraw-Hill Medical.
• Moore, K. L., Dalley, A. F., & Agur, A. M. (2010). Clinically Oriented Anatomy. 6th ed.LWW

22. Images- Clinical Oriented Anatomy, 6th ed. 2010

23. Images- Wikimedia commons- web

24. Thank you!