Complex Regional Pain Syndrome (CRPS): Difference between revisions

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<div class="noeditbox">Welcome to [[PPA Pain Project]]. This page is being reviewed and updated by participants of a project to populate the Pain section of Physiopedia. &nbsp;The project is supervised and co-ordinated by the [[The Physiotherapy Pain Association]].
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'''Original Editors '''- Katelyn&nbsp;Koeninger &amp; Kristen Storrie&nbsp;&nbsp;[[Pathophysiology of Complex Patient Problems|from Bellarmine University's&nbsp;Pathophysiology of Complex Patient Problems project.]]  
*If you would like to get involved in this project and earn accreditation for your contributions, [mailto:[email protected] please get in touch]!
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'''Original Editors ''' - [[User:Yves Hubar|Yves Hubar]]  


'''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} &nbsp;  
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== Search Strategy  ==
Literature was found on pubmed and the vub v-spaces system.<br>


== Definition/Description  ==
== Definition/Description  ==


Complex regional pain syndrome (CRPS) is a term for a variety of clinical conditions characterized by chronic persistent pain. It is a disease that may develop after a limb trauma. <ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref> This appears mostly in 1 or more limbs, usually in the arms or legs. We can say a CRPS is a regional posttraumatic neuropathic pain problem. <ref>RHO, R. e.a., Concise Review for Clinicians: Complex Regional Pain Syndrome. Mayo Foundation for Medical Education and Research, 2002. (level of evidence 3A)</ref> Neuropathic pain disorders are a disproportionate consequence of painful trauma or nerve lesion. <ref>WASNER, G., e.a., Vascular abnormalities in reflex sympathetic dystrophy (CRPS I): mechanisms and diagnostic value. Oxford University Press, 2001. (level of evidence 3A)</ref><br> CRPS is subdivided into type I and type II CRPS.
Complex regional pain syndrome (CRPS) is a term for a variety of clinical conditions characterized by chronic persistent pain. It is a disease that may develop after a limb trauma.&nbsp;<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref> This appears mostly in 1 or more limbs, usually in the arms or legs. We can say a CRPS is a regional posttraumatic neuropathic pain problem.<ref>RHO, R. e.a., Concise Review for Clinicians: Complex Regional Pain Syndrome. Mayo Foundation for Medical Education and Research, 2002. (level of evidence 3A)</ref> Neuropathic pain disorders are a disproportionate consequence of painful trauma or nerve lesion. <ref>WASNER, G., e.a., Vascular abnormalities in reflex sympathetic dystrophy (CRPS I): mechanisms and diagnostic value. Oxford University Press, 2001. (level of evidence 3A)</ref>


In the literature, there are a lot of names used to describe this syndrome such as ‘‘Reflex Sympathetic Dystrophy’’, ‘‘causalgia’’, ‘‘algodystrophy’’, ‘‘Sudeck’s atrophy’’, ‘‘neurodystrophy’’, and ‘‘post-traumatic dystrophy’’. To standardize the nomenclature, the name ‘complex regional pain syndrome’ was adopted in 1995 by the ‘International Association for the Study of Pain’ (IASP).<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><br>
CRPS is subdivided into type I and type II CRPS.


== Clinically Relevant Anatomy  ==
In the literature, there are a lot of names used to describe this syndrome such as ‘‘Reflex Sympathetic Dystrophy’’, ‘‘causalgia’’, ‘‘algodystrophy’’, ‘‘Sudeck’s atrophy’’, ‘‘neurodystrophy’’, and ‘‘post-traumatic dystrophy’’. To standardize the nomenclature, the name ‘complex regional pain syndrome’ was adopted in 1995 by the ‘International Association for the Study of Pain’ (IASP). <ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><br><br>


CRPS can take place in any body part, but often in the extremities. The wrist is most frequently affected after [http://www.physio-pedia.com/Distal_Radial_Fractures distal radial fractures].<ref>JELLAD, A., Complex Regional Pain Syndrome Type I: Incidence and Risk Factors in Patients With Fracture of the Distal Radius. Archives of Physical Medicine and Rehabilitation, 2014. (level of evidence 2B)</ref> It is a disorder with assorted clinical appearances and gravity of disease. Central and peripheral nervous system are connected through neural and chemical pathways, there is a direct control over the autonomic system. It is for this reason that there can be changes in the vasomotor and sudomotor without any impairment in the peripheral nervous system. Pain, heath and swelling are usually not located at the site of inciting injury and there may be no clear damage. [http://www.physio-pedia.com/Central_sensitisation Central sensitisation] is seen as a main cause for developing CRPS.<ref>BARON, R., Complex regional pain syndromes. Curr Pain Headache Rep., 2001. (level of evidence 2A)</ref><ref>SHIPTON, E., Complex regional pain syndrome – Mechanisms, diagnosis, and management. Current Anaesthesia &amp;amp;amp; Critical Care, 2009. (level of evidence 2A)</ref><br>
== Prevalence  ==


[[Image:Teasdall et al-2.jpg]]<br>  
CPRS affects approximately 26 out of every 100,000 people. It is more common in females than males, with a ratio of 3.5:1.<ref name="goebel" /> CRPS can affect people of all ages, including children as young as&nbsp;three years old and adults as old as 75 years old, but typically is most prevalent beginning in the mid-thirties. CRPS type I occurs after five percent of all traumatic injuries.<ref name="Patho Book" /> Ninety-one percent of all CRPS cases occur after surgery.<ref name="turner">Turner-Stokes L, Goebel A. Complex regional pain syndrome in adults: concise guidance. Clinical Med 2011; 11(6):596-600.</ref>  


Fig.1: Nociceptive (painful) information is relayed through the dorsal horn of the spinal cord for processing and modulation before cortical evaluation.<ref>TEASDALL, R., Complex Regional Pain Syndrome (reflex sympathetic dystrophy). Clin Sports Med, 2004. (level of evidence 2A)</ref><br>
== Characteristics/Clinical Presentation  ==


== Epidemiology /Etiology  ==
<u>Signs and Symptoms of CRPS:<br></u>


Complex regional pain syndrome can develop after different types of inciting injuries.<br>A few examples are:<br>● sprains and strains,<br>● post-surgical, <br>● fractures,<br>● contusions,<br>● crush injuries,<br>● nerve lesions,<br>● stroke.<br>Sometimes the inciting injury can occur spontaneously or can not be determined. <ref>ROSS A.H. et al., The Theoretical Basis for and Treatment of Complex Regional Pain Syndrome with Prolotherapy. Journal of Prolotherapy, 2010. (level of evidence 2C)</ref><ref>BARON, et al., Complex regional pain syndrome: mystery explained? The Lancet Neurology, 2003. (level of evidence 3B)</ref><ref>ALLEN, G., et al., Epidemiology of complex regional pain syndrome: a retrospective chart review of 134 patients. Pain,1999. (level of evidence 2B)</ref><ref>SUMITANI M., et al., Complex regional pain syndrome. Rheumatology, 2014. (level of evidence 2C)</ref><ref>JUOTTONEN, K., et al., Altered central sensorimotor processing in patients with complex regional pain syndrome. Pain, 2002. (level of evidence 5)</ref><ref>ATALA,  N.S., et al., Prednisolone in Complex Regional Pain Syndrome. Pain Physician, 2014. (level of evidence 2B)</ref> <br> Fifty-six percent of patients reported CRPS was due to an ‘on-the-job’ injury. The most common type of work-related injury occurred in service employments, such as in restaurants, bakeries and police offices.<ref>ALLEN, G., et al., Epidemiology of complex regional pain syndrome: a retrospective chart review of 134 patients. Pain,1999. (level of evidence 2B)</ref>  
*pain (pain may not be present in 7%of CRPS sufferers)<ref name="Patho Book" /><ref name="goebel">Goebel A. Complex regional pain syndrome in adults. Rheumatology 2011;50(10):1739-50.</ref><ref name="Hooshmand" />  
*swelling<ref name="Patho Book" /><ref name="goebel" /><ref name="Hooshmand" />  
*tremor<ref name="Patho Book" />  
*trouble initiating movements<ref name="Patho Book" />  
*muscle spasms (may be present in the cervical and lumbar spine regions in advanced cases)<ref name="Patho Book" /><ref name="Hooshmand" />  
*muscle atrophy<ref name="Patho Book" />  
*temperature changes<ref name="Patho Book" /><ref name="goebel" />  
*color changes (red, blue)<ref name="Patho Book" />  
*thick, brittle, or rigid nails<ref name="Patho Book" />  
*weakness<ref name="Patho Book" /><ref name="goebel" /><ref name="Hooshmand" />  
*thin, shiny, clammy skin<ref name="goebel" />  
*stiffness or decreased joint motion<ref name="goebel" />  
*painful or decreased sensation on skin (some patients report intolerance to air moving over skin)<ref name="goebel" />  
*strange, disfigured, or dislocated feelings in limbs<ref name="goebel" />


The location of the CPRS varies from person to person. It often occurs in the extremities, a little bit more in the lower extremities (+/- 60%) than in the upper extremities (+/- 40%). It can also appear on the left and the right side and in both extremities.<ref>ROSS A.H. et al., The Theoretical Basis for and Treatment of Complex Regional Pain Syndrome with Prolotherapy. Journal of Prolotherapy, 2010. (level of evidence 2C)</ref><ref>ALLEN, G., et al., Epidemiology of complex regional pain syndrome: a retrospective chart review of 134 patients. Pain,1999. (level of evidence 2B)</ref><ref>SUMITANI M., et al., Complex regional pain syndrome. Rheumatology, 2014. (level of evidence 2C)</ref>
<br>  
 
Complex regional pain syndrome occurs regularly in young adults. It is more frequent in females than males.<ref>SRINIVASA N. et al., Complex Regional Pain Syndrome I (Reflex Sympathetic Dystrophy). American Society of Anesthesiologists, 2002. (level of evidence 3B)</ref><ref>SANDRONI, P., et al., Complex regional pain syndrome type I: incidence and prevalence in Olmsted county, a population-based study. Pain, 2003. (level of evidence 2B)</ref>  


The onset is mostly associated with a trauma in history, immobilization, injections or surgery. But there is no relation between the grade of severity of the initial injury and the following syndrome. A stressful life and other psychological factors may be potential risk factors that impact the severity of symptoms in CPRS.<ref>SRINIVASA N. et al., Complex Regional Pain Syndrome I (Reflex Sympathetic Dystrophy). American Society of Anesthesiologists, 2002. (level of evidence 3B)</ref><br>
<u></u><u>Characteristics:<br></u>  


== Characteristics/Clinical Presentation<br>  ==
*<u></u>sensory impairments<ref name="Patho Book" />  
 
*movement disorders, typically in contralateral extremity<ref name="Patho Book" /><ref name="Hooshmand" />  
We can subdivide CRPS in type I and type II. The difference between type I and II is based on a consensus between clinicians and scientists. The definition of type II (see below) can be largely interpreted. For example post-traumatic neuralgia has different syndromes with different underlying mechanisms but it can be included in type II. This means that the definition of both types need to be improved in specificity and sensitivity in the future. In both cases we assume that long-term central changes have occurred. Somatosensory changes, changes in the sympathetic nervous system, changes in the peripheral, changes in the somatomotor system and changes in the neuroendocrine systems cause changes in the central nervous system. All symptoms of CRPS II show many similarities to those of CRPS I.<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><ref>WASNER, G., e.a., Vascular abnormalities in reflex sympathetic dystrophy (CRPS I): mechanisms and diagnostic value. Oxford University Press, 2001. (level of evidence 3A)</ref><ref>BARON, R., Complex regional pain syndromes. Curr Pain Headache Rep., 2001. (level of evidence 2A)</ref><ref>JANIG, W., et al., Complex regional pain syndrome is a disease of the central nervous system. Clinical Autonomic Research, 2002. (level of evidence 5)</ref>
*ANS dysfunction<ref name="Patho Book" />  
 
*dystrophy<ref name="Patho Book" />  
CRPS is clinically characterized by sensory, autonomic and motor disturbances. The table below shows an overview of the different characteristics of CRPS I and II:<ref>WASNER, G., e.a., Vascular abnormalities in reflex sympathetic dystrophy (CRPS I): mechanisms and diagnostic value. Oxford University Press, 2001. (level of evidence 3A)</ref><ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><ref>BARON, R., Complex regional pain syndromes. Curr Pain Headache Rep., 2001. (level of evidence 2A)</ref><ref>JUOTTONEN, K., et al., Altered central sensorimotor processing in patients with complex regional pain syndrome. Pain, 2002. (level of evidence 5)</ref><ref>JANIG, W., et al., Complex regional pain syndrome is a disease of the central nervous system. Clinical Autonomic Research, 2002. (level of evidence 5)</ref><ref>GALER, B. et al., Course of Symptoms and Quality of Life Measurement in Complex Regional Pain Syndrome: A Pilot Survey. Journal of Pain and Symptom Management, 2000. (level of evidence 2C)</ref><br>
*atrophy<ref name="Patho Book" />  
 
*spreads proximally, to other extremities, and possibly the entire body<ref name="Patho Book" />  
{| width="757" height="957" cellspacing="1" cellpadding="1" border="1"
*similar presentation to osteoporosis on radiographic images<ref name="Patho Book" />
|-
| Type<br>
| Type 1 <br>
| Type 2&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp; <br>
|-
| Definition<br>
|
= formerly reflex sympathetic dystrophy
 
After an injury with minor or without nerve damage, after a trauma remote from the affected extremity<br>
 
|
= formerly causalgia
 
After injury to a major peripheral nerve<br>
 
|-
| Etiology<br>
| minor, soft tissue trauma (sprains, bruises and skin lesions);<br>bone fracture or surgery;<br>frostbite or burns;<br>stroke, [http://www.physio-pedia.com/Myocardial_Infarction myocardial infarction] or lesion of the central nervous system;<br>immobilisation<br><br>
| <br>
|-
| Sensory disturbances<br>
| ● Allodynia and hyperalgesia<br>● Hypoesthesia and hypoalgesia<br><br>
|
<br>


● Allodynia and hyperalgesia<br>● Hypersensitivity of the skin to light mechanical stimulation.<br>
Patients typically progress through three stages as CRPS develops. CRPS in children does not always follow the same stage patterns and may at times become stagnate or even slowly improve.<ref name="Hooshmand" /><br>  


{| width="591" border="1" cellspacing="1" cellpadding="1"
|-
|-
| Autonomic disorders + inflammatory symptoms<br>
! scope="col" | Stage
| ● Swelling and edema<br>● Changes of sweating (especially hyperhidrosis)<br>● Abnormal skin blood flow<br>● Color changes (redness or pale)<br>● Temperature changes<br><br>
! scope="col" | Time Period
| ● Limb is cold and sweaty<br>● Distal extremity swelling<br>● Changes of sweating<br>● Abnormal skin blood flow<br>● Temperature changes<br><br>
! scope="col" | Classic Signs and Symptoms<ref name="Patho Book" />
|-
|-
| Trophic changes<br>
| '''Stage I''': acute inflammation: denervation and sympathetic hypoactivity
| ● Abnormal nail growth<br>● Increased or decreased hair growth<br>● Fibrosis<br>● Thin glossy skin<br>● Osteoporosis (chronic stage)<br><br>
| Begins 10 days post injury;<br>Lasts 3-6 months<br>  
| ● Smoothness and mottling of the skin<br>● Acute arthritis<br><br>
| '''Pain''': more severe than expected; burning or aching; increased with dependent position, physical condition, or emotional disturbances<br>Hyperalgesia, allodynia, hyperpathia: lower pain threshold, increased sensitivity, all stimuli are perceived as painful, increased pain threshold then increased sensation intensity (faster and greater pain)<br>'''Edema''': soft and localized<br>'''Vasomotor/Thermal Changes''': warmer<br>'''Skin''': hyperthermia, dryness<br>'''Other''': increased hair and nail growth<br>
|-
|-
| Motor dysfunction<br>
| '''Stage II''': dystrophic: paradoxic sympathetic hyperactivity
| ● Weakness of all muscles<br>● inability to move the extremity<br>● Stiffness<br>● Tremor<br>● Reduced range of motion<br>● Severe impairment of complex movements<br>● Atrophy<br><br>
| Begins 3-6 months after onset of pain;<br>Lasts about 6 months<br>  
| ● Weakness of all muscles<br>● Inability to move the extremity<br>● Stiffness<br>● Tremor <br>● [http://www.physio-pedia.com/Focal_dystonia Dystonia]<br>● Reduced range of motion<br>● Severe impairment of complex movements<br><br>
| '''Pain''': worsens, constant, burning, aching<br>Hyperalgesia, allodynia, hyperpathia: present<br>'''Edema''': hard, causes joint stiffness<br>'''Vasomotor/Thermal Changes''': none<br>'''Skin''': thin, glossy, cool due to vasoconstriction, sweaty<br>'''Other''': thin &amp; rigid nails, osteoporosis and subchondral bone erosion noted on x-rays<br>
|-
|-
| Pain (sympathetic nervous system)<br>
| '''Stage III''': atrophic
| ● Burning and spontaneous<br>● Disproportionable in intensity to the inciting event<br>● Increase when the extremity is in a dependent position<br>● Elicited by movement and pressure at the joints<br>● Not in all cases<br>● Deep, unpleasant, sensitive, surface, dull<br><br>
| Begins 6-12 months after onset of pain;<br>Lasts for years, or may resolve and reappear<br>  
| ● Ongoing<br>● Neuropathic<br>● Spontaneous<br>● Triggered by movement, loud noises or strong emotions<br>● Deep, unpleasant, sensitive, surface, dull<br><br>
| '''Pain''': spreads proximally and occasionally to entire body, may plateau<br>'''Edema''': hardening<br>'''Vasomotor/Thermal Changes''': decreased SNS regulation, cooler<br>'''Skin''': thin, shiny, cyanotic, dry<br>'''Other''': fingertips and toes are atrophic, thick fascia, possible contractures, demineralization and ankylosis seen on x-rays<br>
|}
|}


<br>Symptoms can spread beyond the territory of the lesioned nerve in type II. Ongoing neurogenic inflammation, vasomotor dysfunction, central sensitization and maladaptive neuroplasticity contribute to the clinical phenotype of CRPS. Genetic and psychological factors can influence the vulnerability to CRPS and also affect the mechanisms that maintain CRPS. Peripheral and central changes can be irreversible. The sympathetic nervous system plays a key role in maintaining pain and autonomic dysfunction in the affected extremity.<ref>WASNER, G., e.a., Vascular abnormalities in reflex sympathetic dystrophy (CRPS I): mechanisms and diagnostic value. Oxford University Press, 2001. (level of evidence 3A)</ref><ref>SUMITANI M., et al., Complex regional pain syndrome. Rheumatology, 2014. (level of evidence 2C)</ref><br>
<br>


== Differential Diagnosis ==
== <ref>Cristiana Kahl Collins. Physical Therapy Management of Complex Regional Pain Syndrome I in a 14-Year-Old Patient Using Strain Counterstrain: A Case Report. J Man Manip Ther. 2007; 15(1): 25–41.</ref>[[Image:Criteria.JPG|left]]<br> ==


The differential diagnosis includes the direct effects of <ref>TURNER-STOKES, L., e.a., Complex regional pain syndrome in adults: concise guidance. Clinical Med, 2011. (level of evidence 5)</ref><ref>MCBRIDE, A., e.a., Complex Regional Pain Syndrome, Current Orthopaedics, 2005. (level of evidence 3A)</ref>
== Associated Co-morbidities  ==


:<br>● Bony or soft tissue injury<br>● Peripheral neuropathy, nerve lesions<br>● Arthritis<br>● Infection<br>● [http://www.physio-pedia.com/Compartment_Syndrome Compartment syndrome]<br>● Arterial insufficiency<br>● Raynaud’s Disease<br>● Lymphatic or venous obstruction<br>● [http://www.physio-pedia.com/Thoracic_Outlet_Syndrome Thoracic outlet syndrome]<br>● Gardner-Diamond Syndrome<br>● Erythromelalgia<br>● Self-harm or malingering<br>● Cellulitis<br>● Undiagnostic fracture
CRPS may also be associated with:  


<br>  
*Arterial Insufficiency<ref name="MD Guide">MD Guidelines, Medical DIsability Advisor. Complex Regional Pain Syndrome: Comorbid Conditions. http://www.mdguidelines.com/complex-regional-pain-syndrome/comorbid-conditions (accessed March 28, 2012).</ref>
*Asthma<ref name="goebel" />
*Bone Fractures<ref name="Hooshmand" />
*Cellulitis<ref name="MD Guide" />
*Central Pain Syndromes<ref name="MD Guide" />
*Conversion Disorder<ref name="MD Guide" />
*Depression/Anxiety
*Factitious Disorder&nbsp;<ref name="MD Guide" />
*Lymphedema<ref name="MD Guide" />
*Malignancy<ref name="MD Guide" />
*Migraines<ref name="goebel" />
*Nerve Entrapment Syndromes<ref name="MD Guide" />
*Osteomyelitis<ref name="MD Guide" />
*Osteoporosis<ref name="goebel" /><ref name="Hooshmand" />
*Pain Disorder<ref name="MD Guide" />
*Peripheral Neuropathies<ref name="MD Guide" />
*Rheumatoid Arthritis<ref name="MD Guide" />
*Scleroderma<ref name="MD Guide" />
*Septic arthritis<ref name="MD Guide" />
*Systemic Lupus Erythematosus<ref name="MD Guide" />
*Tenosynovitis<ref name="MD Guide" />
*Thrombophlebitis<ref name="MD Guide" /><br>


'''Diagnostic Procedures'''
== Medications  ==


----
Possible treatments for CRPS include:


The diagnosis of CRPS requires a complicated process due to the absence of a golden standard test for diagnosis.<ref>PEREZ, R., et al., Diagnostic criteria for CRPS I: Differences between patient profiles using three different diagnostic sets. European Journal of Pain, 2007. (level of evidence 2A)</ref><ref>ALBAZAZ, R. N., et al., Complex Regional Pain Syndrome: A review. Annals of Vascular Surgery Inc., 2008. (level of evidence 2A)</ref><ref>HARDEN, R. N., et al., Validation of proposed diagnostic criteria (the ‘‘Budapest Criteria”) for Complex Regional Pain Syndrome. International Association for the Study of Pain, 2010. (level of evidence 1C)</ref><ref>CHOI, E., et al., Interexaminer reliability of infrared thermography for the diagnosis of complex regional pain syndrome. Skin Research and Technology, 2013. (level of evidence 2B)</ref> There are some criteria for diagnosis of CRPS, known as the Budapest criteria, and until there is a golden standard these criteria must suffice.<ref>HARDEN, R. N., Commentary: The Diagnosis of CRPS: Are we there yet?. International Association for the Study of Pain, 2012. (level of evidence 1B)</ref> The Budapest criteria, also called the IASP clinical diagnostic criteria for complex regional pain syndrome are<ref>PEREZ, R., et al., Diagnostic criteria for CRPS I: Differences between patient profiles using three different diagnostic sets. European Journal of Pain, 2007. (level of evidence 2A)</ref><ref>ALBAZAZ, R. N., et al., Complex Regional Pain Syndrome: A review. Annals of Vascular Surgery Inc., 2008. (level of evidence 2A)</ref><ref>HARDEN, R. N., et al., Validation of proposed diagnostic criteria (the ‘‘Budapest Criteria”) for Complex Regional Pain Syndrome. International Association for the Study of Pain, 2010. (level of evidence 1C)</ref><ref>HARDEN, R. N., Commentary: The Diagnosis of CRPS: Are we there yet?. International Association for the Study of Pain, 2012. (level of evidence 1B)</ref><ref>WASNER, G., et al., Complex regional pain syndrome - diagnostic, mechanisms, CNS involvement and therapy. International Spinal Cord Society, 2003. (level of evidence 2A)</ref><ref>HODGE, S., et al., Complex Regional Pain Syndrome: The Anatomy Of A Controversy. Anatomy For Litigators: complex regional pain syndrome, p. 163-167. (level of evidence 2A)</ref>:<br>● Constant pain, higher than the normally perceived pain <br>● Minimum one symptom in three of the following four symptom categories must be reported:<br>○ Vasomotor: temperature asymmetry and/or skin color changes/asymmetry<br>○ Sensory: hyperalgesia and/or allodynia<br>○ Sudomotor/edema: changes in sweating <br>○ Motor/trophic: smaller range of motion,motor dysfunction and/or changes in hair, nails and skin<br>● In addition to these symptoms the patient must also show signs that he will develop symptoms in at least two symptom categories<br>● No other illness could explain the set of symptoms the patient is showing. <br> CRPS diagnosis is mainly based on history, clinical examination, and some supportive investigations, just like:<br> <br>1. Infrared thermography<br>Infrared thermography (IRT) is an effective mechanism to find significant asymmetry in temperature between both limbs by determining if the affected side of the body shows vasomotor differences in comparison to the other side. It is reported having a sensitivity of 93% and a specificity of 89%.<ref>ALBAZAZ, R. N., et al., Complex Regional Pain Syndrome: A review. Annals of Vascular Surgery Inc., 2008. (level of evidence 2A)</ref> This test is hard to obtain so it is not often used for diagnosis of CRPS.<ref>PEREZ, R., et al., Diagnostic criteria for CRPS I: Differences between patient profiles using three different diagnostic sets. European Journal of Pain, 2007. (level of evidence 2A)</ref><ref>ALBAZAZ, R. N., et al., Complex Regional Pain Syndrome: A review. Annals of Vascular Surgery Inc., 2008. (level of evidence 2A)</ref><ref>CHOI, E., et al., Interexaminer reliability of infrared thermography for the diagnosis of complex regional pain syndrome. Skin Research and Technology, 2013. (level of evidence 2B)</ref><br> <br>2. Sweat Testing<br>To determine if the patient sweats abnormally the amount of sweat that he produces can be measured. Q-sweat is an adequate instrument to measure sweat production. The sweat samples should be taken from both sides of the body at the same time.<ref>BARON, R., Complex regional pain syndromes. Curr Pain Headache Rep., 2001. (level of evidence 2A)</ref><ref>ALBAZAZ, R. N., et al., Complex Regional Pain Syndrome: A review. Annals of Vascular Surgery Inc., 2008. (level of evidence 2A)</ref><ref>CHEMALI, K., et al., α-adrenergic supersensitivity of the sudomotor nerve in complex regional pain syndrome. Annals of Neurology, 2001. (level of evidence 2B)</ref><br> <br>3. Radiographic Testing<br>Irregularities in the bone structure of the affected side of the body can become visible with the use of [http://www.physio-pedia.com/X-Rays X-rays]. If the X-ray shows no sign of osteoporosis, CRPS can be excluded if the patient is an adult.<ref>BARON, R., Complex regional pain syndromes. Curr Pain Headache Rep., 2001. (level of evidence 2A)</ref><ref>ALBAZAZ, R. N., et al., Complex Regional Pain Syndrome: A review. Annals of Vascular Surgery Inc., 2008. (level of evidence 2A)</ref><ref>HODGE, S., et al., Complex Regional Pain Syndrome: The Anatomy Of A Controversy. Anatomy For Litigators: complex regional pain syndrome, p. 163-167. (level of evidence 2A)</ref> 4. Three-phase bone scan<br>With the use of technetium Tc 99m-labeled bisophosphonates increase in bone metabolism can be shown. Higher uptake of the substance means increased bone metabolism which means the body part could be affected.<ref>ALBAZAZ, R. N., et al., Complex Regional Pain Syndrome: A review. Annals of Vascular Surgery Inc., 2008. (level of evidence 2A)</ref><ref>HODGE, S., et al., Complex Regional Pain Syndrome: The Anatomy Of A Controversy. Anatomy For Litigators: complex regional pain syndrome, p. 163-167. (level of evidence 2A)</ref> <br> <br>5. Bone densitometry<br>An affected limb often shows less bone mineral density and a change in the content of the bone mineral. During treatment of the CPRS the state of the bone mineral will improve. So this test can also be used to determine if the patient’s treatment is effective.<ref>ALBAZAZ, R. N., et al., Complex Regional Pain Syndrome: A review. Annals of Vascular Surgery Inc., 2008. (level of evidence 2A)</ref><br> <br>6. [http://www.physio-pedia.com/MRI_Scans Magnetic resonance imaging (MRI)]<br>MRIs are useful to detect periarticular marrow edema, soft tissue swelling and joint effusions. And in a later stage atrophy and fibrosis of periarticular structures can be detected. But these symptoms are not exclusively signs of CRPS.<ref>BARON, R., Complex regional pain syndromes. Curr Pain Headache Rep., 2001. (level of evidence 2A)</ref><ref>ALBAZAZ, R. N., et al., Complex Regional Pain Syndrome: A review. Annals of Vascular Surgery Inc., 2008. (level of evidence 2A)</ref><ref>HODGE, S., et al., Complex Regional Pain Syndrome: The Anatomy Of A Controversy. Anatomy For Litigators: complex regional pain syndrome, p. 163-167. (level of evidence 2A)</ref><br> <br>7. Sympathetic Blocks<br>If the patient shows vasomotor or sudomotor dysfunction and severe pain, blocking the sympathetic nerves proves to be an effective technique to evaluate if the sympathetic nervous system is causing the pain to remain. This technique requires local anesthetic or ablation and is successful if at least 50% of the pain is reduced.<ref>ALBAZAZ, R. N., et al., Complex Regional Pain Syndrome: A review. Annals of Vascular Surgery Inc., 2008. (level of evidence 2A)</ref>
*Oral pain-relieving medications including corticosteroids and NSAIDs, as well as acupuncture provide effective pain relief in approximately 20% of those with CRPS, but this is supported by weak evidence.<ref name="Patho Book" />  
 
*Treatments may be geared to helping patients manage symptoms. Amitriptyline relieves depression and acts as a sleeping aid. Calcium channel blockers can help to improve circulation through SNS effect. Intrathecal baclofen, among other measures, improves motor dystonia.<ref name="Patho Book" />  
== Outcome Measures  ==
*Pain intensity and perception of pain is sometimes relieved through use of an implanted transcutaneous electrical nerve stimulation (TENS) unit.<ref name="Patho Book" />  
 
*A randomized double dummy controlled, double blind trial compared the effectiveness of Dimethylsulfoxide 50% (DMSO) and N-acetylcysteine (NAC) in treating CRPS type I. There were no significant differences between the two treatments, but are both successful treating CRPS type I. This study showed that DMSO-treatment is more favorable for warm CRPS whereas NAC is more favorable for cold CRPS<ref name="Perez">Perez R, Zuurmond W, Bezemer P, Kuik D, vanLoenen A, deLange J, et al. The treatment of complex regional pain syndrome type I with free radical scavengers: a randomized controlled study. Pain 2003;102(3):297-307.</ref>  
The diagnosis is based on the clinical presentation described above. Procedures should start with taking a detailed medical history, considering an initiating trauma and any history of sensory, autonomic, and motor disturbances. Also the development, time period, distribution and characteristics of pain should be asked. A general examination is necessary. Detection of any swelling, sweating, trophic, temperature, and motor abnormality in the disturbed area is important. Skin temperature differences may be helpful for diagnosis of CRPS.<br>There are several methods to evaluate CRPS. Each symptom has another test to evaluate its severity. Below is a list of the different types of instruments with their evaluated symptoms. [for references see below]<br>
*Low doses of ketamine infusion has been shown to decrease pain in patients with CRPS type I who had been unsuccessful with other conservative methods of management. Ketamine blocks central sensitization by effecting the N-methyl-D-aspartate receptor which has been shown to be effected in CRPS.<ref name="Goldberg">Goldberg M, Domsky R, Scaringe D, Hirsh R, Dotson J, Sharaf I, et al. Multi-Day Low Dose Ketamine Infusion for the Treatment of Complex Regional Pain Syndrome. Pain Physician 2005;8:175-179.</ref>  
 
*Antidepressants may be utilized to treat associated depression.<ref name="Hooshmand" />
<br>
 
{| width="511" cellspacing="1" cellpadding="1" border="1"
|-
| Test<br>
| Evaluated items<br>
| References<br>
|-
| Impairment sum score<br>
|
<br>  


Pain ([http://www.physio-pedia.com/Visual_Analogue_Scale VAS] and McGill)<br>Skin temperature<br>Volume<br>ROM<br>
== Diagnostic Tests/Lab Tests/Lab Values  ==


| <ref>SCHASFOORT, F.C., et al., Outcome measures for complex regional pain syndrome type I: an overview in the context of the international classification of impairments disabilities and handicaps. Disability and Rehabilitation, 2000. (level of evidence 2C)</ref><ref>PACKHAM, T., A Systematic Review of Psychometric outcome Assessment in Complex Regional Pain Syndrome. Disability and Rehabilitation, 2012. (level of evidence 2A)</ref><ref>HARDEN, R.N., et al., Development of a Severity Score for CRPS. Pain, 2010. (level of evidence 1C)</ref><ref>OERLEMANS, H.O., et al., Impairment Level Sumscore in Reflex Sympathetic Dystrophy of One Upper Extremity. Archives of Physical Medicine and Rehabilitation, 1998. (level of evidence 2B)</ref> <ref>GROENEWEG, J.G., et al., Increased endothelin-1 and diminished nitric oxide levels in blister fluids of patients with intermediate cold type complex regional pain syndrome type 1. BMC Musculoskeletal Disorders, 2006. (level of evidence 1B)</ref><ref>ATALA,  N.S., et al., Prednisolone in Complex Regional Pain Syndrome. Pain Physician, 2014. (level of evidence 2B)</ref><ref>FISCHER, S.G., et al., Intravenous magnesium for chronic complex regional pain syndrome type 1 (CRPS-1). Pain Medicine, 2013. (level of evidence 1B)</ref><br>
Diagnosis of CRPS is based solely on examination and patient history.<ref name="Patho Book" /> A triple-phase bone scan is the best method to rule out type I CPRS.<ref name="Cappello">Cappello Z, Kasdan M, Louis D. Meta-analysis of imaging techniques for the diagnosis of complex regional pain syndrome type I. JHS 2012;37A:288-296.</ref> According to Cappello, the triple-phase bone scan has the best sensitivity, NPV, and PPV compared to MRI and plain film radiographs.<ref name="Cappello" /> Radiographic examinations, laser Doppler flowmetry, and thermographic studies may be utilized to assess the secondary issues and symptoms of CRPS.<ref name="Patho Book" /><br>  
|-
| Grip Strength<br>
| Dynamometry<br>Full fist grip, pinch grip<br><br>
| <ref>HOTTA, J., et al., Patients with complex regional pain syndrome overestimate applied force in observed hand action. European Journal of Pain, 2015. (level of evidence 3B)</ref><ref>ATALA,  N.S., et al., Prednisolone in Complex Regional Pain Syndrome. Pain Physician, 2014. (level of evidence 2B)</ref><br>
|-
| Foot function (Radboud skills test)<br>
| Movements of the foot<br>
| <ref>VAN GIJN, J.C., et al., Pain exposure physical therapy may be a safe and effective treatment for longstanding complex regional pain syndrome type 1: a case series. Sage Journals, 2015. (level of evidence 1C)</ref><ref>FISCHER, S.G., et al., Intravenous magnesium for chronic complex regional pain syndrome type 1 (CRPS-1). Pain Medicine, 2013. (level of evidence 1B)</ref><br>
|-
| Walking stairs<br>
| Time, effort, need for assistance<br>
| <ref>PEREZ R. et al., Measuring perceived activity limitations in lower extremity Complex Regional Pain Syndrome type 1 (CRPS I): test-retest reliability of two questionnaires. Clinical Rehabilitation, 2002. (level of evidence 1B)</ref><ref>PACKHAM, S. Assessment of CRPS. 2015. (level of evidence 5)</ref><ref>FISCHER, S.G., et al., Intravenous magnesium for chronic complex regional pain syndrome type 1 (CRPS-1). Pain Medicine, 2013. (level of evidence 1B)</ref><br>
|-
| Rising and sitting<br>
| Getting in and out of car, bed, toilet<br>
| <ref>PEREZ R. et al., Measuring perceived activity limitations in lower extremity Complex Regional Pain Syndrome type 1 (CRPS I): test-retest reliability of two questionnaires. Clinical Rehabilitation, 2002. (level of evidence 1B)</ref><ref>FISCHER, S.G., et al., Intravenous magnesium for chronic complex regional pain syndrome type 1 (CRPS-1). Pain Medicine, 2013. (level of evidence 1B)</ref><br>
|-
| Trend (trauma related neuronal dysfunction) symptom inventory<br>
| 164 items in 10 subscales (sensory, trophic, autonomic, motor, visceral symptoms)<br>
| <ref>SCHASFOORT, F.C., et al., Outcome measures for complex regional pain syndrome type I: an overview in the context of the international classification of impairments disabilities and handicaps. Disability and Rehabilitation, 2000. (level of evidence 2C)</ref><ref>GROENEWEG, J.G., et al., Increased endothelin-1 and diminished nitric oxide levels in blister fluids of patients with intermediate cold type complex regional pain syndrome type 1. BMC Musculoskeletal Disorders, 2006. (level of evidence 1B)</ref><ref>COLLINS, S., et al., Development of a symptoms questionnaire for complex regional pain syndrome and potentially related illnesses: the Trauma Related Neuronal Dysfunction Symptoms Inventory. Archives of Physical Medicine and Rehabilitation, 2008. (level of evidence 3B)</ref><br>
|-
| [http://www.physio-pedia.com/Neuropathic_pain Neuropathic pain ]questionnaire<br>
| 12 items (see figure below)<br>
| <ref>BOUHASSIRAA, D., et al., Comparison of pain syndromes associated with nervous or somatic lesions and development of a new neuropathic pain diagnostic questionnaire (DN4). Pain, 2004. (level of evidence 1B)</ref><ref>BACKONJA, M., et al., Neuropathic Pain Questionnaire - Short Form. The Clinical Journal of Pain, 2003. (level of evidence 2B)</ref><br>
|-
| Brush allodynia<br>
| Brush evoked allodynia<br>
| <ref>VAN EIJS, F., et al., Brush-evoked allodynia predicts outcome of spinal cord stimulation in CRPS. European Journal of Pain, 2010. (level of evidence 1B)</ref><br>
|}


<br>  
<br>  


There is also a CPRS Severity Score (CSS). It includes signs and symptoms that reflect the sensory, vasomotor, sudomotor/edema and motor/trophic disorders of CPRS.<br>This scoring list evaluates CRPS symptoms separately in order to develop a self-monitoring tool for patients. It is anticipated that this tool will help to facilitate disease management and improve dialogue between patients and their physicians. <br>Eight symptoms are evaluated at the baseline visit with self-reported ratings of worst pain in the affected area, disability, depression, and quality of life (SF-36).<ref>KIRSLING, A., et al., The Complex Regional Pain Syndrome Symptom Severity Score (SSS): toward the development of a patient-administered screening tool. Rehabilitation Institute of Chicago. (level of evidence 3B)</ref><ref>HARDEN, R.N., et al., Development of a Severity Score for CRPS. Pain, 2010. (level of evidence 1C)</ref><ref>ATALA,  N.S., et al., Prednisolone in Complex Regional Pain Syndrome. Pain Physician, 2014. (level of evidence 2B)</ref><ref>FISCHER, S.G., et al., Intravenous magnesium for chronic complex regional pain syndrome type 1 (CRPS-1). Pain Medicine, 2013. (level of evidence 1B)</ref><br>
<u>'''The Budapest Criteria'''</u>  


== Examination  ==
The Budapest Criteria have been used to diagnose CRPS as well.&nbsp; This method has high specificity and sensitivity according to Goebel.<ref name="goebel" />


The original International Association for the Study of Pain criteria required only history and subjective symptoms for a diagnosis of CRPS. But recent consensus guidelines developed by experts have argued for the inclusion of objective findings. <ref>PENTLAND, B., e.a., Parkinsonism and dystonia. Neurological Rehabilitation, 1997. (level of evidence 2C)</ref> <br> <br>The examination of the affected limb should be done from the neck downwards. The examination should be carried out at rest, during activity, and during ambulation. <ref>SANDEEP, J., Complex Regional Pain Syndrome. Indian Journal of Plastic Surgery, 2011. (level of evidence 2A )</ref> <br> <br>During the physical examination, it is very important to investigate the sensory, motor and autonomic dysfunctions. <ref>PENTLAND, B., e.a., Parkinsonism and dystonia. Neurological Rehabilitation, 1997. (level of evidence 2C)</ref><ref>ROMMEL, e.a., Quantitative sensory testing, neurophysiological and psychological examination in patients with complex regional pain syndrome and hemisensory deficits. Ruhr-University, 2000. (level of evidence 1B)</ref><ref>SANDEEP, J., Complex Regional Pain Syndrome. Indian Journal of Plastic Surgery, 2011. (level of evidence 2A )</ref> <br> <br>1. Autonomic dysfunction<br>The majority of patients with CRPS have side-to-side differences in temperature of the limbs. The skin temperature depends of the chronicity of the disease. The temperature will increase in acute stages, this is most of the time in combination with a white or reddish skin with more swelling. The temperature will decrease in chronic stages. This is associated with bluish skin and more atrophy. <ref>FRONTERA, W, e.a., Essentials of Physical Medicine and Rehabilitation - Musculoskeletal disorders, pain and rehabilitation. Saunders Elsevier, 2002. (level of evidence 5)</ref> <br> <br>2. Motor dysfunction<br>Studies have shown that approximately 70% of the patients with CRPS show muscle weakness in the affected limb, exaggerated tendon reflexes or tremor, irregular myoclonic jerks, and dystonic muscle contractions. Muscle dysfunction often coincides with a loss of range of motion in the distal joints. <ref>SANDEEP, J., Complex Regional Pain Syndrome. Indian Journal of Plastic Surgery, 2011. (level of evidence 2A )</ref><br> <br>3. Sensory dysfunction<br>The distal ends of the extremities require more attention when examining a patient with CRPS. However, common findings of regional neuropathic and motor dysfunction have shown us that it is important to broaden the examination both proximally and contra laterally.<ref>FRONTERA, W, e.a., Essentials of Physical Medicine and Rehabilitation - Musculoskeletal disorders, pain and rehabilitation. Saunders Elsevier, 2002. (level of evidence 5)</ref><br> <br>Light touch, pinprick, temperature and vibration sensation should be assessed to have a correct examination of CRPS.<ref>FRONTERA, W, e.a., Essentials of Physical Medicine and Rehabilitation - Musculoskeletal disorders, pain and rehabilitation. Saunders Elsevier, 2002. (level of evidence 5)</ref> Most assessments are linked with each other. This means that when for example vibration sensation is very positive, light touch should be positive too.<ref>FRONTERA, W, e.a., Essentials of Physical Medicine and Rehabilitation - Musculoskeletal disorders, pain and rehabilitation. Saunders Elsevier, 2002. (level of evidence 5)</ref><br> <br>To help distinguish the findings of a sensory dysfunction, you have to compare the affected area with an unaffected area. The findings should be clear and reliable.<ref>FRONTERA, W, e.a., Essentials of Physical Medicine and Rehabilitation - Musculoskeletal disorders, pain and rehabilitation. Saunders Elsevier, 2002. (level of evidence 5)</ref><ref>SANDEEP, J., Complex Regional Pain Syndrome. Indian Journal of Plastic Surgery, 2011. (level of evidence 2A )</ref><br>
To make the clinical diagnosis, the following criteria must be met:<br>1. Continuing pain, which is disproportionate to any inciting event<br>2. Must report at least one symptom in three of the four following categories:


<br>
*Sensory: Reports of hyperesthesia and/or allodynia
*Vasomotor: Reports of temperature asymmetry and/or skin color changes and/or skin color asymmetry
*Sudomotor/Edema: Reports of edema and/or sweating changes and/or sweating asymmetry
*Motor/Trophic: Reports of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)


== Medical Management <br>  ==
3. Must display at least one sign at time of evaluation in two or more of the following categories:


*Sensory: Evidence of hyperalgesia (to pinprick) and/or allodynia (to light touch and/or temperature sensation and/or deep somatic pressure and/or joint movement)
*Vasomotor: Evidence of temperature asymmetry (&gt;1 °C) and/or skin color changes and/or asymmetry
*Sudomotor/Edema: Evidence of edema and/or sweating changes and/or sweating asymmetry
*Motor/Trophic: Evidence of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)


4. There is no other diagnosis that better explains the signs and symptoms<br>


Treatment of complex regional pain syndrome should be immediate, and most importantly directed toward restoration of full function of the extremity. There are several options to treat CRPS.
== Etiology/Causes  ==
<ref>WASNER, G., et al., Complex regional pain syndrome - diagnostic, mechanisms, CNS involvement and therapy. International Spinal Cord Society, 2003. (level of evidence 2A)</ref>


The cause of CRPS remains unknown, however there are explanations for the presentation of the condition.&nbsp; After trauma or external stresses placed on the body, the nervous system has a heightened response to stimuli.<ref name="Medline Plus">Medline Plus, the U.S. National Library of Medicine and the National Institutes of Health. Medical Encyclopedia: Complex Regional Pain Syndrome. http://www.nlm.nih.gov/medlineplus/ency/article/007184.htm (accessed 29 March 2012).</ref>&nbsp; The inflammatory process of the body overreact leading to red hot extremities due to exaggerated blood supply to the area,&nbsp;or blue, cold extremities&nbsp;due to blood vessel constriction.<ref name="Hooshmand" />


Bone demineralization is not unusual in CRPS patients, so treatment with calcitonin and biphosphonates is suitable. Calcitonin has received considerable interest in the management of CRPS because of its analgesic properties through release of ß-endorphin as well as its inhibition of bone resorption.<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref> Biphosphonates are potent inhibitors of bone resorption; <br>- Alendronate: less pain and swelling, more range of motion.<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><ref>WASNER, G., et al., Complex regional pain syndrome - diagnostic, mechanisms, CNS involvement and therapy. International Spinal Cord Society, 2003. (level of evidence 2A)</ref><ref>ADAMI, S., et al., Bisphosphonate therapy of reflex sympathetic dystrophy syndrome. Ann Rheum Dis,1997. (level of evidence 2B)</ref><br>- Clodronate: less pain, improved global assessment (evaluated by investigator), and higher perceived efficacy (evaluated by patients).<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><ref>WASNER, G., et al., Complex regional pain syndrome - diagnostic, mechanisms, CNS involvement and therapy. International Spinal Cord Society, 2003. (level of evidence 2A)</ref><ref>VARENNA, M., et al., Intravenous clodronate in the treatment of reflex sympathetic dystrophy syndrome. A randomized, double blind, placebo controlled study. Journal of Rheumatology, 2000. (level of evidence 2B)</ref><br>- Palmidronate: less pain, higher overall improvement (patient’s assessment), and higher functional assessment scores.<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><ref>ROBINSON, J.N., et al., Efficacy of pamidronate in complex regional pain syndrome type I. Pain Medicine, 2004. (level of evidence 2B)</ref><br>Generally biphosphonates have been shown to decrease pain and swelling as well as to increase range of motion for patients with CRPS<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref>
[[Image:Cycle of CRPS.jpg|291x217px|The cycle of CRPS]]&nbsp;<ref name="Aurora">Aurora Health Care. Health Information: Complex Regional Pain Syndrome. http://www.aurorahealthcare.org/yourhealth/healthgate/getcontent.asp?URLhealthgate=%2296853.html%22 (accessed 28 March 2012).</ref>  


An excessive inflammatory reaction can lead to the overproduction of free radicals, resulting in the destruction of healthy tissue and possibly leading to CRPS. Thus, free radical scavengers have been proposed to curtail the disease process. To date, three free radical scavengers, like dimethyl sulfoxide (DMSO), have been investigated for the treatment of CRPS.<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><br>A scorelist (based on pain, daily activities, edema, color, and ROM) showed greater improvement by dimethyl sulfoxide treatment. DMSO seems to provide a mild improvement in range of motion and vasomotor instability in patients with CRPS.<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref>
== Systemic Involvement  ==


Biopsy studies showing tissue inflammation in CRPS caused many researchers to use steroids.These steroids showed great improvement in pain and edema. They showed a better post treatment score.<ref>WASNER, G., et al., Complex regional pain syndrome - diagnostic, mechanisms, CNS involvement and therapy. International Spinal Cord Society, 2003. (level of evidence 2A)</ref><ref>CHRISTENSEN, K., et al., The reflex dystrophy syndrome response to treatment with systemic corticosteroids. Acta Chir Scand 1982. (level of evidence 2B)</ref><ref>BRAUS, D.F., et al., The shoulder-hand syndrome after stroke: a prospective clinical trial. Annals of Neurology, 1994. (level of evidence 1B)</ref>
CRPS primarily affects the vascular system, but over time it can spread to other systems including the nervous, endocrine,&nbsp;cardiovascular systems.&nbsp;&nbsp;Problems develop such as headache, dizziness, tinnitus, urgency/frequency of urination, erection disturbances, renal&nbsp;bleeding,&nbsp;hypertension, nose bleeds,&nbsp;syncope, abdominal pain, peptic ulcers, nausea, vomiting, weight loss, diarrhea, chest pain, abnormal heart beat, tachycardia, heart attack, and&nbsp;falls.<ref name="Hooshmand" />  


Intravenous immunoglobulin can reduce pain in refractory CRPS.The average pain intensity was 1.55 units lower after IVIG treatment.<ref>GOEBEL, A. et al., Intravenous Immunoglobulin Treatment of the Complex Regional Pain Syndrome: A Randomized Trial, Annals of internal medicine, 2010. (level of evidence 1B)</ref>
<br>  


Hyperbaric oxygen therapy is an effective and well tolerated method for decreasing pain, allodynia, oedema and increasing the range of motion in CRPS. Also, the skin colour returns to normal.<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><ref>KIRALP, M.Z. et al., Effectiveness of Hyperbaric Oxygen Therapy in the Treatment of Complex Regional Pain Syndrome, The Journal of International Medical Research, 2004. (level of evidence 1B)</ref>
== Medical Management (current best evidence) ==


In the majority of patients with chronic reflex sympathetic dystrophy, electrical stimulation of the spinal cord can reduce pain intensity and improve health-related quality of life.<ref>KEMLER M. et al., Spinal Cord Stimulation in Patients with Chronic Reflex Sympathetic Dystrophy. The New England Journal of Medicine, 2000. (level of evidence 2B)</ref><ref>FOROUZANFAR T. et al., Spinal cord stimulation in complex regional pain syndrome: cervical and lumbar devices are comparably effective. British Journal of Anaesthesia, 2004. (level of evidence 2B)</ref><br>
*Spinal cord stimulation was more effective than conventional medical management in reducing pain in patients with CRPS type I<ref name="Simpson">Simpson E, Duenas A, Holmes M, Papaloannou D, Chilcott J. Spinal cord stimulation for chronic pain of neuropathic or ischaemic origin: systematic review and economic evaluation. Health Technology Assessment 2009;13(17):1-179.</ref>
*Spinal cord stimulation was shown to be effective in completely eliminating pain in adolescent females 2-6 weeks after stimulation<ref name="Olson">Olson GL, Meyerson BA, Linderoth B. Spinal cord stimulation in adolescents with complex regional pain syndrome type I. EUR J PAIN 2008;12(1):53-59.</ref>
*Use of surgical and chemical sympathectomy show moderate improvement in pain scores in patients with CRPS. There were no significant differences found between the surgical and chemical groups when comparing pain scores from day one to four months. More high quality research needs to be done before recommending this as a first line of defense.<ref name="Straube">Straube S, Derry S, Moore RA, McQuay HJ. Cervico-thoracic or lumbar sympathectomy for neuropathic pain and complex regional pain syndrome. Cochrane Database of Systematic Reviews 2010;7:1-14.</ref>
*High frequency repetitive transcranial magnetic stimulation on the motor cortex in addition to pharmacological management was effective in reducing pain. This was demonstrated by the scores on the McGill Pain Questionnaire and Short Form-36 which include different aspects of pain such as sensory-discriminative and emotional-affective.<ref name="Picarelli">Picarelli H, Teixeira M, deAndrade D, Myzkowski M, Luvisotto T, Yeng L, et al. Repetitive Transcranial Magnetic Stimulation Is Efficacious as an Add-On to Pharmacological Therapy in Complex Regional Pain Syndrome Type I. J Pain 2010;11(11):1203-10.</ref>  
*Surgery, casts, and ice should be avoided when treating CRPS because they further aggravate the nervous system. Surgery leads to further stress, inflammation, and immune system disturbances.<ref name="Hooshmand">Hooshmand H, Phillips E. Spread of complex regional pain syndrome. Vero Beach, Florida. Neurological Associates Pain Management Center.</ref>
*A stellate ganglion block, or sympathectomy, blocks the nerve pathways causing pain. This may be most beneficial in the early stages of CRPS.<ref name="Patho Book" /><ref name="goebel" />


<br>
{{#ev:youtube|izOYrLUuNd8}}<ref>Arizona Pain. Stellate Ganglion Block. Available from: http://www.youtube.com/watch?v=izOYrLUuNd8 [last accessed 3/29/12]</ref>  


== Physical Therapy Management <br> ==
<br>  


== Physical Therapy Management (current best evidence)  ==


It has been found that physical therapy and occupational therapy are effective in reducing pain and increasing function in patients who have had CRPS for less than 1 year<ref name="goebel" />. Physical therapy should focus on patient education of CRPS and functional activities.&nbsp; The typical preferred practice patterns for CRPS are as follows: 4A, 4D, 5G, and 7B.<ref name="Patho Book" />&nbsp;


It is difficult to manage CRPS because there is lack of understanding of the pathophysiologic abnormalities and lack of specific diagnostic criteria. In literature there is very low quality evidence to treat CRPS.
Physical therapy intervention could include any of the following:
<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref>
<br>The main goals of treating the patient are reduction of pain, preservation of limb function and return to work. Don’t forget to check the comorbidities such as depression, sleep disturbance and anxiety. Those have to be treated concurrently.<br>The basic rule is treating the patient in a multidisciplinary approach. This multidisciplinary team should include neurologists, anesthesiologists, orthopedics, physiotherapists and psychologists.


*TENS: Somers, et al found that high frequency TENS contralateral to the nerve injury reduces mechanical allodynia, while low frequency reduces thermal allodynia in rats.<ref name="Patho Book" /><ref name="Somers">Somers D, Clemente F. Transcutaneous Electrical Nerve Stimulation for the Management of Neuropathic Pain: The Effects of Frequency and Electrode Position on Prevention of Allodynia in a Rat Model of Complex Regional Pain Syndrome Type II. Phys Ther 2006;86:698-709.</ref>
*aquatics:&nbsp;Aquatic&nbsp;therapy allows activities to be performed with decreased weight bearing on the lower extremities.<ref name="Patho Book" />
*mirror therapy
*desensitization<ref name="goebel" />
*gradual weight bearing<ref name="goebel" />
*stretching<ref name="goebel" />
*fine motor control<ref name="goebel" />


It is important for physical therapists to recognize that CRPS typically follows blood vessel pathways, and therefore symptoms may not always follow neural patterns.&nbsp; Also, due to the spread pattern, CRPS treatment should be provided bilaterally, due to the contralateral connections present between the extremities.<ref name="Hooshmand" /><br>


The treatment should be based on basic principles of pain management (pain and symptom relief, supportive care, rehabilitation). Due to the lack of evidence in treatment of CRPS we have to rely on treatments of other neuropathic pain syndromes.<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><ref>BARON, R., Complex regional pain syndromes. Curr Pain Headache Rep., 2001. (level of evidence 2A)</ref>
== Differential Diagnosis  ==


CRPS needs to be differentiated from the following diagnosis<ref name="turner" />:


*Bony or soft tissue injury
*Neuropathic pain
*Arthritis
*Infection
*Compartment syndrome
*Arterial insufficiency
*Raynaud’s Disease
*Lymphatic or venous obstruction
*Thoracic outlet syndrome
*Gardner-Diamond Syndrome
*Erythromelalgia
*Self-harm or malingering<br>


'''Acute phase'''<br>Immobilization and contralateral therapy should be the treatment in the acute phase. Intensive active therapy in the acute phase can lead to deterioration.<ref>BARON, R., Complex regional pain syndromes. Curr Pain Headache Rep., 2001. (level of evidence 2A)</ref>
== Clinical Guidelines  ==


[http://www.rcplondon.ac.uk/sites/default/files/complex-regional-pain-full-guideline.pdf Complex regional pain syndrome in adults&nbsp;UK guidelines for diagnosis, referral and management in primary and secondary care.] Royal College of Physicians, May 2012.


Complex regional pain syndrom: practical diagnostic and treatment guidelines, 4th edition. Pain Medicine. 2013, 14: 180-229


'''Chronic phase'''<br>1. Passive physical therapy including manipulation, manual therapy<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref>, massage<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref> and mobilizations. Lymphatic drainage can be used to facilitate regression of [http://www.physio-pedia.com/Lymphatic_Obstruction_%28Lymphedema%29 edema].<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref> You can also threat tender points by the following rules: more severe tender points as identified by tenderness to palpation are treated before less severe tender points; more proximal or more medial tender points are addressed before more distally and laterally located points; the area of greatest accumulation of tender points is treated first; when tender points are located in a row, the one in the middle of the row is treated first.<ref>COLLIN, C., Physical Therapy Management of Complex Regional Pain Syndrome I in a 14-Year-Old Patient Using Strain Counterstrain: A Case Report. J Man Manip Ther., 2007. level of evidence 4)</ref> (level of evidence 4)
== &nbsp;&nbsp;Case Reports/ Case Studies  ==


<br>2. Therapeutic exercise including isometric strengthening therapy followed by active isotonic training in combination with sensory [http://www.physio-pedia.com/Desensitization desensitization] programs.<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref> 70<ref>BARON, R., Complex regional pain syndromes. Curr Pain Headache Rep., 2001. (level of evidence 2A)</ref> (level of evidence 3A) The strengthening training includes exercises for all four extremities and trunk. The exercises can be done with Theraband.<ref>STANTON-HICKS, M., et al., Complex Regional Pain Syndromes: Guidelines for Therapy. The Clinical Journal of Pain, 1998. (level of evidence 5)</ref> Desensitization programs contains giving stimuli of different fabrics, different pressure (light or deep), vibration, tapping, heat or cold. The exercises can be stress-loading (f.e. scrubbing, walking, carrying weights), endurance training, written instructions and functional training.<ref>STANTON-HICKS, M., et al., Complex Regional Pain Syndromes: Guidelines for Therapy. The Clinical Journal of Pain, 1998. (level of evidence 5)</ref> When CRPS occurs in the lower extremities, in that case including gait training in the therapy is recommended.<ref>COLLIN, C., Physical Therapy Management of Complex Regional Pain Syndrome I in a 14-Year-Old Patient Using Strain Counterstrain: A Case Report. J Man Manip Ther., 2007. level of evidence 4)</ref> (level of evidence 4). It is also useful to give the patient an home exercise program.<ref>STANTON-HICKS, M., et al., Complex Regional Pain Syndromes: Guidelines for Therapy. The Clinical Journal of Pain, 1998. (level of evidence 5)</ref><br>
*[http://web.ebscohost.com/ehost/pdfviewer/pdfviewer?vid=6&hid=107&sid=b31938a9-e11b-4a82-b517-b07f03b65ccb%40sessionmgr104 More Than Meets the Eye: Clinical Reflection and Evidence-Based Practice in an Unusual Case of Adolescent Chronic Ankle Sprain]
*[http://www.jospt.org/issues/articleID.447,type.2/article_detail.asp Thoracic Spine Dysfunction in Upper Extremity Complex Regional Pain Syndrome Type I]


<br>


{{#ev:youtube|jaTlI6bfF64}}<ref>CNN. CNN Report on Reflex Sympathetic Dystrophy. Available from: http://www.youtube.com/watch?v=jaTlI6bfF64 [last accessed 3/28/12]</ref>


3. [http://www.physio-pedia.com/Mirror_Therapy Mirror therapy] or mirror visual feedback<ref>SMITH, T., How effective is physiotherapy in the treatment of complex regional pain syndrome type I? A review of the literature. Musculoskeletal care, 2005. (level of evidence 3A)</ref> (level of evidence 3A)<br>Mirror therapy contains placing both hands into a box with a mirror separating the two compartments and, while moving both hands, watching the reflection of the unaffected hand in the mirror<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><br>Evidence:<br>-reducing pain intensity and improve function in post-stroke CRPS<ref>MCGABE, C.S., et al., Mirror visual feedback for the treatment of complex regional pain syndrome (Type 1). Current Pain and Headache Reports, 2008. (level of evidence 2A)</ref> (level of evidence 2A)<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><ref>BOWERING, K. J., The effects of graded motor imagery and its components on chronic pain: a systematic review and meta-analysis. The American Pain Society, 2013. (level of evidence 1A)</ref><br>-a significant improvement in pain with mirror therapy in a study where they compare a group with mirror therapy doing the same exercises as a group without mirror<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref> (level of evidence 2C)<br>-improve function (low quality evidence)<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><br>-no conclusions could be drawn regarding the efficacy of mirror therapy (Rothgangel)<ref>ROTHGANGEL, A.S., et al., The clinical aspects of mirror therapy in rehabilitation: a systematic review of the literature. International Journal of Rehabilitation Research,  2011. (level of evidence 3A)</ref><br> <br>4. Graded motor imagery/learning<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><ref>MOSELEY, G.L., Graded motor imagery is effective for long standing complex regional pain syndrome: a randomised controlled trial. Pain, 2004.  (level of evidence 1B)</ref><ref>SMITH, T., How effective is physiotherapy in the treatment of complex regional pain syndrome type I? A review of the literature. Musculoskeletal care, 2005. (level of evidence 3A)</ref> (level of evidence 1B) <br>Graded motor imagery consists of recognition of hand laterality (= pictures were presented of right and left hands and then you have to identify the correct size) and imagined hand movement (= pictures of a hand in different positions are presented and then the patient has to move his hand in that position).<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><br>Evidence:<br>-Further trials are required<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref> (level of evidence 1B)<br>-GMI plus medical management more effective than medical management plus physiotherapy<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref> (level of evidence 3A)<br>-GMI may reduce pain and improve function<ref>MOSELEY, G.L., Graded motor imagery is effective for long standing complex regional pain syndrome: a randomised controlled trial. Pain, 2004.  (level of evidence 1B)</ref> (level of evidence 1B)<br> <br>5. Relaxation<ref>BRUEHL, S., Psychological interventions. In: Harden RN editor(s). Complex regional pain syndrome: treatment guidelines. Reflex Sympathetic Dystrophy Syndrome Association, 2006 (level of evidence 1C)</ref><ref>SMITH, T., How effective is physiotherapy in the treatment of complex regional pain syndrome type I? A review of the literature. Musculoskeletal care, 2005. (level of evidence 3A)</ref> (level of evidence 1C)<br>[http://www.physio-pedia.com/Cognitive_Behavioural_Therapy Cognitive-behavioral therapy]<ref>LEE, B., et al., Physical therapy and cognitive-behavioral treatment for complex regional pain syndromes. Pain Treatment Service, 2002. (level of evidence 2B)</ref> (level of evidence 2B) contains relaxation training, deep breathing exercises, biofeedback. 4 Relaxation therapy falls under the responsibility of the psychologist.<br> <br>6. Neuromodulation or invasive stimulation techniques<br>Neuromodulation contains peripheral nerve stimulation with implanted electrodes, epidural spinal cord stimulation, deep brain stimulation and electrotherapy.<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><br>[http://www.physio-pedia.com/Current_Concepts_in_Electrotherapy Electrotherapy] includes transcutaneous electric nerve stimulation [http://www.physio-pedia.com/Transcutaneous_Electrical_Nerve_Stimulation_%28TENS%29 TENS]<ref>OAKLANDER, A., et al., Evidence of focal small-fiber axonal degeneration in complex
<br>  
regional pain syndrome-I (reflex sympathetic dystrophy). Pain, 2006. (level of evidence 1B)</ref><ref>SMITH, T., How effective is physiotherapy in the treatment of complex regional pain syndrome type I? A review of the literature. Musculoskeletal care, 2005. (level of evidence 3A)</ref> (level of evidence 3A), Spinal cord stimulation SCS<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><ref>ALBAZAZ, R. N., et al., Complex Regional Pain Syndrome: A review. Annals of Vascular Surgery Inc., 2008. (level of evidence 2A)</ref><ref>KENLER, M., et al., The effect of Spinal Cord Stimulation in Patients with Chronic Reflex Sympathetic Dystrophy: Two Years’ Follow-up of the Randomized Controlled Trial. Annals of Neurology, 2004. (level of evidence 1B)</ref> (level of evidence 1B),non-invasive brain stimulation (repetitive transcranial magnetic stimulation).<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><br>Evidence:<br>-to reduce pain<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><br>-further trials are required<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref>
 
Examples of spinal cord stimulation in the treatment of CRPS in the lower extremities.<ref>COLLIN, C., Physical Therapy Management of Complex Regional Pain Syndrome I in a 14-Year-Old Patient Using Strain Counterstrain: A Case Report. J Man Manip Ther., 2007. level of evidence 4)</ref> (level of evidence 4)
 
<br>7. Other treatments:<br>-Whirlpool bath/ contrast baths<ref>DEVRIMSEL, G., et al., The effects of whirlpool bath and neuromuscular electrical stimulation on complex regional pain syndrome. J. Phys. Ther. Sci., 2015. (level of evidence 3B)</ref> (level of evidence 3B)<br>-Vocational and recreational rehabilitation<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><br>-Psychological therapies: cognitive-behavioral therapy (CBT), operant conditioning (OC), counselling, pain education and relaxation techniques<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><ref>BRUEHL, S., Psychological interventions. In: Harden RN editor(s). Complex regional pain syndrome: treatment guidelines. Reflex Sympathetic Dystrophy Syndrome Association, 2006 (level of evidence 1C)</ref><br>-[http://www.physio-pedia.com/Acupuncture Acupuncture], electroacupuncture<ref>SMITH, T., How effective is physiotherapy in the treatment of complex regional pain syndrome type I? A review of the literature. Musculoskeletal care, 2005. (level of evidence 3A)</ref> (level of evidence 3A)<br>-Tactile sensory discrimination training<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><ref>BOWERING, K. J., The effects of graded motor imagery and its components on chronic pain: a systematic review and meta-analysis. The American Pain Society, 2013. (level of evidence 1A)</ref><br>-Weight bearing<ref>TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)</ref><br>-[http://www.physio-pedia.com/Ultrasound_therapy Ultrasound therapy]<br>-Kinesiotaping<ref>ANANDKUMAR, S.; MANIVASAGAM, M., Multimodal physical therapy management of a 48-year-old female with post-stroke complex regional pain syndrome. Physiother Theory Pract, 2014. (level of evidence 3B)</ref> (level of evidence 3B)
 
The conclusion we can make is that a few clinical studies could demonstrate a significant improvement of CRPS after physical therapy.<ref>BIRKLEIN, F., et al., Neurological findings in complex regional pain syndromes—analysis of 145 cases. Acta Neurol Scand, 2000. (level of evidence 2A)</ref><ref>SHERRY, D., et al., Short- and long-term outcomes of children with complex regional pain syndrome type I treated with exercise therapy. Clin J Pain, 1999. (Level of evidence 1B)</ref><ref>OERLEMANS, H., et al., Adjuvant Physical Therapy Versus Occupational Therapy in patients With Reflex Sympathetic Dystrophy/Complex Regional Pain Syndrome type I. Arch Phys Med Rehabil, 2000. (level of evidence 1B)</ref><ref>LEE, B., et al., Physical therapy and cognitive-behavioral treatment for complex regional pain syndromes. Pain Treatment Service, 2002. (level of evidence 2B)</ref><ref>OERLEMANS, H., et al., Pain and reduced mobility in complex regional pain syndrome I: outcome of a prospective randomised controlled clinical trial of adjuvant physical therapy versus occupational therapy. Pain, 1999. (level of evidence 1B)</ref> (level of evidence 1B) But further trials are required for an optimal treatment.<ref>PEREZ, R., Evidence based guidelines for complex regional pain syndrome type 1. BMC Neurology, 2010. (level of evidence 2C)</ref><br>
 
== Key Research  ==
 
add links and reviews of high quality evidence here (case studies should be added on new pages using the [[Template:Case Study|case study template]])<br>  


== Resources <br>  ==
== Resources <br>  ==


add appropriate resources here <br>
*[http://www.rsds.org Reflex Sympathetic Dystrophy Syndrome Association]&nbsp;
*[http://www.theacpa.org American Chronic Pain Association]
*[http://www.mayoclinic.com Mayo Clinic]
*[http://www.ninds.nih.gov National Institute of Neurological Disorders and Stroke]


== Clinical Bottom Line  ==
== &nbsp;Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])  ==
 
<div class="researchbox"><rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1fAD01Rzfn7B0ZRzIfL12TBhNQObAZ_2nJk-8802GMR8R-nYIR|charset=UTF-8|short|max=10</rss></div>  
Complex regional pain syndrome (CRPS) is a term for a variety of clinical conditions characterized by chronic persistent pain. There are two types of CRPS: The difference between type I and type II is based on a consensus between clinicians and scientists. All symptoms of CRPS II show many similarities to those of CRPS I.<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref><ref>WASNER, G., e.a., Vascular abnormalities in reflex sympathetic dystrophy (CRPS I): mechanisms and diagnostic value. Oxford University Press, 2001. (level of evidence 3A)</ref><ref>BARON, R., Complex regional pain syndromes. Curr Pain Headache Rep., 2001. (level of evidence 2A)</ref><ref>JANIG, W., et al., Complex regional pain syndrome is a disease of the central nervous system. Clinical Autonomic Research, 2002. (level of evidence 5)</ref> It is difficult to manage CRPS because there is lack of understanding of the pathophysiologic abnormalities and lack of specific diagnostic criteria. In literature there is very low quality evidence to treat CRPS.<ref>O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)</ref> The little amount of clinical studies that could demonstrate a significant improvement after physical therapy needs to become more complete by further trials. This is required for an optimal physical management.<br>
 
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])  ==
 
see tutorial on [[Adding PubMed Feed|Adding PubMed Feed]]
<div class="researchbox">
<rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1P7PR1QO6IS9xQ724YOoSHF8On2gkKwiE3UGU6x5C6gE847gBI|charset=UTF-8|short|max=10</rss>  
</div>  
== References  ==
== References  ==


see [[Adding References|adding references tutorial]].  
see [[Adding References|adding references tutorial]].  
 
<references /></div>  
<references />  
[[Category:Pain]] [[Category:PPA_Project]] [[Category:Bellarmine_Student_Project]] [[Category:Neurology]] [[Category:Medical]]
 
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Revision as of 19:10, 26 February 2016

Original Editors - Katelyn Koeninger & Kristen Storrie  from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Katelyn Koeninger, Kristen Storrie, Laura Ritchie, Yves Hubar, Laure-Anne Callewaert, Angeliki Chorti, Admin, Melissa Coetsee, Evan Thomas, Scott Cornish, Elaine Lonnemann, Kim Jackson, Rachael Lowe, Gayatri Jadav Upadhyay, Matthias Steenwerckx, Vidya Acharya, Lucinda hampton, Habibu Salisu Badamasi, Gwen Wyns, Claire Knott, Lauren Lopez, Jo Etherton, Maria Galve Villa, Michelle Lee, Richmond Stace, WikiSysop, Wendy Walker and Naomi O'Reilly  

Definition/Description[edit | edit source]

Complex regional pain syndrome (CRPS) is a term for a variety of clinical conditions characterized by chronic persistent pain. It is a disease that may develop after a limb trauma. [1] This appears mostly in 1 or more limbs, usually in the arms or legs. We can say a CRPS is a regional posttraumatic neuropathic pain problem.[2] Neuropathic pain disorders are a disproportionate consequence of painful trauma or nerve lesion. [3]

CRPS is subdivided into type I and type II CRPS.

In the literature, there are a lot of names used to describe this syndrome such as ‘‘Reflex Sympathetic Dystrophy’’, ‘‘causalgia’’, ‘‘algodystrophy’’, ‘‘Sudeck’s atrophy’’, ‘‘neurodystrophy’’, and ‘‘post-traumatic dystrophy’’. To standardize the nomenclature, the name ‘complex regional pain syndrome’ was adopted in 1995 by the ‘International Association for the Study of Pain’ (IASP). [4]

Prevalence[edit | edit source]

CPRS affects approximately 26 out of every 100,000 people. It is more common in females than males, with a ratio of 3.5:1.[5] CRPS can affect people of all ages, including children as young as three years old and adults as old as 75 years old, but typically is most prevalent beginning in the mid-thirties. CRPS type I occurs after five percent of all traumatic injuries.[6] Ninety-one percent of all CRPS cases occur after surgery.[7]

Characteristics/Clinical Presentation[edit | edit source]

Signs and Symptoms of CRPS:

  • pain (pain may not be present in 7%of CRPS sufferers)[6][5][8]
  • swelling[6][5][8]
  • tremor[6]
  • trouble initiating movements[6]
  • muscle spasms (may be present in the cervical and lumbar spine regions in advanced cases)[6][8]
  • muscle atrophy[6]
  • temperature changes[6][5]
  • color changes (red, blue)[6]
  • thick, brittle, or rigid nails[6]
  • weakness[6][5][8]
  • thin, shiny, clammy skin[5]
  • stiffness or decreased joint motion[5]
  • painful or decreased sensation on skin (some patients report intolerance to air moving over skin)[5]
  • strange, disfigured, or dislocated feelings in limbs[5]


Characteristics:

  • sensory impairments[6]
  • movement disorders, typically in contralateral extremity[6][8]
  • ANS dysfunction[6]
  • dystrophy[6]
  • atrophy[6]
  • spreads proximally, to other extremities, and possibly the entire body[6]
  • similar presentation to osteoporosis on radiographic images[6]

Patients typically progress through three stages as CRPS develops. CRPS in children does not always follow the same stage patterns and may at times become stagnate or even slowly improve.[8]

Stage Time Period Classic Signs and Symptoms[6]
Stage I: acute inflammation: denervation and sympathetic hypoactivity Begins 10 days post injury;
Lasts 3-6 months
 Pain: more severe than expected; burning or aching; increased with dependent position, physical condition, or emotional disturbances
Hyperalgesia, allodynia, hyperpathia: lower pain threshold, increased sensitivity, all stimuli are perceived as painful, increased pain threshold then increased sensation intensity (faster and greater pain)
Edema: soft and localized
Vasomotor/Thermal Changes: warmer
Skin: hyperthermia, dryness
Other: increased hair and nail growth
Stage II: dystrophic: paradoxic sympathetic hyperactivity Begins 3-6 months after onset of pain;
Lasts about 6 months
 Pain: worsens, constant, burning, aching
Hyperalgesia, allodynia, hyperpathia: present
Edema: hard, causes joint stiffness
Vasomotor/Thermal Changes: none
Skin: thin, glossy, cool due to vasoconstriction, sweaty
Other: thin & rigid nails, osteoporosis and subchondral bone erosion noted on x-rays
Stage III: atrophic Begins 6-12 months after onset of pain;
Lasts for years, or may resolve and reappear
 Pain: spreads proximally and occasionally to entire body, may plateau
Edema: hardening
Vasomotor/Thermal Changes: decreased SNS regulation, cooler
Skin: thin, shiny, cyanotic, dry
Other: fingertips and toes are atrophic, thick fascia, possible contractures, demineralization and ankylosis seen on x-rays


[9]
Criteria.JPG

[edit | edit source]

Associated Co-morbidities[edit | edit source]

CRPS may also be associated with:

  • Arterial Insufficiency[10]
  • Asthma[5]
  • Bone Fractures[8]
  • Cellulitis[10]
  • Central Pain Syndromes[10]
  • Conversion Disorder[10]
  • Depression/Anxiety
  • Factitious Disorder [10]
  • Lymphedema[10]
  • Malignancy[10]
  • Migraines[5]
  • Nerve Entrapment Syndromes[10]
  • Osteomyelitis[10]
  • Osteoporosis[5][8]
  • Pain Disorder[10]
  • Peripheral Neuropathies[10]
  • Rheumatoid Arthritis[10]
  • Scleroderma[10]
  • Septic arthritis[10]
  • Systemic Lupus Erythematosus[10]
  • Tenosynovitis[10]
  • Thrombophlebitis[10]

Medications[edit | edit source]

Possible treatments for CRPS include:

  • Oral pain-relieving medications including corticosteroids and NSAIDs, as well as acupuncture provide effective pain relief in approximately 20% of those with CRPS, but this is supported by weak evidence.[6]
  • Treatments may be geared to helping patients manage symptoms. Amitriptyline relieves depression and acts as a sleeping aid. Calcium channel blockers can help to improve circulation through SNS effect. Intrathecal baclofen, among other measures, improves motor dystonia.[6]
  • Pain intensity and perception of pain is sometimes relieved through use of an implanted transcutaneous electrical nerve stimulation (TENS) unit.[6]
  • A randomized double dummy controlled, double blind trial compared the effectiveness of Dimethylsulfoxide 50% (DMSO) and N-acetylcysteine (NAC) in treating CRPS type I. There were no significant differences between the two treatments, but are both successful treating CRPS type I. This study showed that DMSO-treatment is more favorable for warm CRPS whereas NAC is more favorable for cold CRPS[11]
  • Low doses of ketamine infusion has been shown to decrease pain in patients with CRPS type I who had been unsuccessful with other conservative methods of management. Ketamine blocks central sensitization by effecting the N-methyl-D-aspartate receptor which has been shown to be effected in CRPS.[12]
  • Antidepressants may be utilized to treat associated depression.[8]

Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]

Diagnosis of CRPS is based solely on examination and patient history.[6] A triple-phase bone scan is the best method to rule out type I CPRS.[13] According to Cappello, the triple-phase bone scan has the best sensitivity, NPV, and PPV compared to MRI and plain film radiographs.[13] Radiographic examinations, laser Doppler flowmetry, and thermographic studies may be utilized to assess the secondary issues and symptoms of CRPS.[6]


The Budapest Criteria

The Budapest Criteria have been used to diagnose CRPS as well.  This method has high specificity and sensitivity according to Goebel.[5]

To make the clinical diagnosis, the following criteria must be met:
1. Continuing pain, which is disproportionate to any inciting event
2. Must report at least one symptom in three of the four following categories:

  • Sensory: Reports of hyperesthesia and/or allodynia
  • Vasomotor: Reports of temperature asymmetry and/or skin color changes and/or skin color asymmetry
  • Sudomotor/Edema: Reports of edema and/or sweating changes and/or sweating asymmetry
  • Motor/Trophic: Reports of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)

3. Must display at least one sign at time of evaluation in two or more of the following categories:

  • Sensory: Evidence of hyperalgesia (to pinprick) and/or allodynia (to light touch and/or temperature sensation and/or deep somatic pressure and/or joint movement)
  • Vasomotor: Evidence of temperature asymmetry (>1 °C) and/or skin color changes and/or asymmetry
  • Sudomotor/Edema: Evidence of edema and/or sweating changes and/or sweating asymmetry
  • Motor/Trophic: Evidence of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)

4. There is no other diagnosis that better explains the signs and symptoms

Etiology/Causes[edit | edit source]

The cause of CRPS remains unknown, however there are explanations for the presentation of the condition.  After trauma or external stresses placed on the body, the nervous system has a heightened response to stimuli.[14]  The inflammatory process of the body overreact leading to red hot extremities due to exaggerated blood supply to the area, or blue, cold extremities due to blood vessel constriction.[8]

The cycle of CRPS [15]

Systemic Involvement[edit | edit source]

CRPS primarily affects the vascular system, but over time it can spread to other systems including the nervous, endocrine, cardiovascular systems.  Problems develop such as headache, dizziness, tinnitus, urgency/frequency of urination, erection disturbances, renal bleeding, hypertension, nose bleeds, syncope, abdominal pain, peptic ulcers, nausea, vomiting, weight loss, diarrhea, chest pain, abnormal heart beat, tachycardia, heart attack, and falls.[8]


Medical Management (current best evidence)[edit | edit source]

  • Spinal cord stimulation was more effective than conventional medical management in reducing pain in patients with CRPS type I[16]
  • Spinal cord stimulation was shown to be effective in completely eliminating pain in adolescent females 2-6 weeks after stimulation[17]
  • Use of surgical and chemical sympathectomy show moderate improvement in pain scores in patients with CRPS. There were no significant differences found between the surgical and chemical groups when comparing pain scores from day one to four months. More high quality research needs to be done before recommending this as a first line of defense.[18]
  • High frequency repetitive transcranial magnetic stimulation on the motor cortex in addition to pharmacological management was effective in reducing pain. This was demonstrated by the scores on the McGill Pain Questionnaire and Short Form-36 which include different aspects of pain such as sensory-discriminative and emotional-affective.[19]
  • Surgery, casts, and ice should be avoided when treating CRPS because they further aggravate the nervous system. Surgery leads to further stress, inflammation, and immune system disturbances.[8]
  • A stellate ganglion block, or sympathectomy, blocks the nerve pathways causing pain. This may be most beneficial in the early stages of CRPS.[6][5]

[20]


Physical Therapy Management (current best evidence)[edit | edit source]

It has been found that physical therapy and occupational therapy are effective in reducing pain and increasing function in patients who have had CRPS for less than 1 year[5]. Physical therapy should focus on patient education of CRPS and functional activities.  The typical preferred practice patterns for CRPS are as follows: 4A, 4D, 5G, and 7B.[6] 

Physical therapy intervention could include any of the following:

  • TENS: Somers, et al found that high frequency TENS contralateral to the nerve injury reduces mechanical allodynia, while low frequency reduces thermal allodynia in rats.[6][21]
  • aquatics: Aquatic therapy allows activities to be performed with decreased weight bearing on the lower extremities.[6]
  • mirror therapy
  • desensitization[5]
  • gradual weight bearing[5]
  • stretching[5]
  • fine motor control[5]

It is important for physical therapists to recognize that CRPS typically follows blood vessel pathways, and therefore symptoms may not always follow neural patterns.  Also, due to the spread pattern, CRPS treatment should be provided bilaterally, due to the contralateral connections present between the extremities.[8]

Differential Diagnosis[edit | edit source]

CRPS needs to be differentiated from the following diagnosis[7]:

  • Bony or soft tissue injury
  • Neuropathic pain
  • Arthritis
  • Infection
  • Compartment syndrome
  • Arterial insufficiency
  • Raynaud’s Disease
  • Lymphatic or venous obstruction
  • Thoracic outlet syndrome
  • Gardner-Diamond Syndrome
  • Erythromelalgia
  • Self-harm or malingering

Clinical Guidelines[edit | edit source]

Complex regional pain syndrome in adults UK guidelines for diagnosis, referral and management in primary and secondary care. Royal College of Physicians, May 2012.

Complex regional pain syndrom: practical diagnostic and treatment guidelines, 4th edition. Pain Medicine. 2013, 14: 180-229

  Case Reports/ Case Studies[edit | edit source]


[22]


Resources
[edit | edit source]

 Recent Related Research (from Pubmed)[edit | edit source]

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References[edit | edit source]

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  1. ↑ O’CONNEL, N.E., e.a., Interventions for treating pain and disability in adults with complex regional pain syndrome- an overview of systematic reviews (Review). The Cochrane Collaboration, 2013. (level of evidence 3A)
  2. ↑ RHO, R. e.a., Concise Review for Clinicians: Complex Regional Pain Syndrome. Mayo Foundation for Medical Education and Research, 2002. (level of evidence 3A)
  3. ↑ WASNER, G., e.a., Vascular abnormalities in reflex sympathetic dystrophy (CRPS I): mechanisms and diagnostic value. Oxford University Press, 2001. (level of evidence 3A)
  4. ↑ TRAN, Q., e.a., Treatment of complex regional pain syndrome: a review of the evidence. Canadian Anesthesiologists’ Society, 2010. (level of evidence 1A)
  5. ↑ 5.00 5.01 5.02 5.03 5.04 5.05 5.06 5.07 5.08 5.09 5.10 5.11 5.12 5.13 5.14 5.15 5.16 5.17 5.18 Goebel A. Complex regional pain syndrome in adults. Rheumatology 2011;50(10):1739-50.
  6. ↑ 6.00 6.01 6.02 6.03 6.04 6.05 6.06 6.07 6.08 6.09 6.10 6.11 6.12 6.13 6.14 6.15 6.16 6.17 6.18 6.19 6.20 6.21 6.22 6.23 6.24 6.25 6.26 6.27 Cite error: Invalid <ref> tag; no text was provided for refs named Patho Book
  7. ↑ 7.0 7.1 Turner-Stokes L, Goebel A. Complex regional pain syndrome in adults: concise guidance. Clinical Med 2011; 11(6):596-600.
  8. ↑ 8.00 8.01 8.02 8.03 8.04 8.05 8.06 8.07 8.08 8.09 8.10 8.11 8.12 Hooshmand H, Phillips E. Spread of complex regional pain syndrome. Vero Beach, Florida. Neurological Associates Pain Management Center.
  9. ↑ Cristiana Kahl Collins. Physical Therapy Management of Complex Regional Pain Syndrome I in a 14-Year-Old Patient Using Strain Counterstrain: A Case Report. J Man Manip Ther. 2007; 15(1): 25–41.
  10. ↑ 10.00 10.01 10.02 10.03 10.04 10.05 10.06 10.07 10.08 10.09 10.10 10.11 10.12 10.13 10.14 10.15 10.16 MD Guidelines, Medical DIsability Advisor. Complex Regional Pain Syndrome: Comorbid Conditions. http://www.mdguidelines.com/complex-regional-pain-syndrome/comorbid-conditions (accessed March 28, 2012).
  11. ↑ Perez R, Zuurmond W, Bezemer P, Kuik D, vanLoenen A, deLange J, et al. The treatment of complex regional pain syndrome type I with free radical scavengers: a randomized controlled study. Pain 2003;102(3):297-307.
  12. ↑ Goldberg M, Domsky R, Scaringe D, Hirsh R, Dotson J, Sharaf I, et al. Multi-Day Low Dose Ketamine Infusion for the Treatment of Complex Regional Pain Syndrome. Pain Physician 2005;8:175-179.
  13. ↑ 13.0 13.1 Cappello Z, Kasdan M, Louis D. Meta-analysis of imaging techniques for the diagnosis of complex regional pain syndrome type I. JHS 2012;37A:288-296.
  14. ↑ Medline Plus, the U.S. National Library of Medicine and the National Institutes of Health. Medical Encyclopedia: Complex Regional Pain Syndrome. http://www.nlm.nih.gov/medlineplus/ency/article/007184.htm (accessed 29 March 2012).
  15. ↑ Aurora Health Care. Health Information: Complex Regional Pain Syndrome. http://www.aurorahealthcare.org/yourhealth/healthgate/getcontent.asp?URLhealthgate=%2296853.html%22 (accessed 28 March 2012).
  16. ↑ Simpson E, Duenas A, Holmes M, Papaloannou D, Chilcott J. Spinal cord stimulation for chronic pain of neuropathic or ischaemic origin: systematic review and economic evaluation. Health Technology Assessment 2009;13(17):1-179.
  17. ↑ Olson GL, Meyerson BA, Linderoth B. Spinal cord stimulation in adolescents with complex regional pain syndrome type I. EUR J PAIN 2008;12(1):53-59.
  18. ↑ Straube S, Derry S, Moore RA, McQuay HJ. Cervico-thoracic or lumbar sympathectomy for neuropathic pain and complex regional pain syndrome. Cochrane Database of Systematic Reviews 2010;7:1-14.
  19. ↑ Picarelli H, Teixeira M, deAndrade D, Myzkowski M, Luvisotto T, Yeng L, et al. Repetitive Transcranial Magnetic Stimulation Is Efficacious as an Add-On to Pharmacological Therapy in Complex Regional Pain Syndrome Type I. J Pain 2010;11(11):1203-10.
  20. ↑ Arizona Pain. Stellate Ganglion Block. Available from: http://www.youtube.com/watch?v=izOYrLUuNd8 [last accessed 3/29/12]
  21. ↑ Somers D, Clemente F. Transcutaneous Electrical Nerve Stimulation for the Management of Neuropathic Pain: The Effects of Frequency and Electrode Position on Prevention of Allodynia in a Rat Model of Complex Regional Pain Syndrome Type II. Phys Ther 2006;86:698-709.
  22. ↑ CNN. CNN Report on Reflex Sympathetic Dystrophy. Available from: http://www.youtube.com/watch?v=jaTlI6bfF64 [last accessed 3/28/12]
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