Complex Regional Pain Syndrome

Lejay Jacob All Saints University of Medicine

Psychiatry Clerkship, MD 9 Preceptor:

Dr. Daniel B. Martinez Presented:June 11, 2015

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

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

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)

Image Source- www.occupational-therapy.advanceweb.com

Nerve injury evident No nerve injury seen

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

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

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

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

Symptoms

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

Dx

Image source- CRPS BMJ 2014

Image Source- CRPS –optimistic. Neurology 2015

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

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.

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

Case Study results -Invasive procedure

RF ablation of stellate ganglion in CRPS. Saudi Journal of Anesthesia. 2014

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

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)

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

Images- Clinical Oriented Anatomy, 6th ed. 2010

Images- Wikimedia commons- web

Thank you!