Complex Regional Pain Syndrome (CRPS): Difference between revisions

m (Text replace - ''''Lead Editors'''' to ''''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}}')
m (Text replace - ''''Lead Editors'''' to ''''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}}')
Line 1: Line 1:
<div class="noeditbox">Welcome to [[Vrije Universiteit Brussel Evidence-based Practice Project|Vrije Universiteit Brussel's Evidence-based Practice project]]. This space was created by and for the students in the Rehabilitation Sciences and Physiotherapy program of the Vrije Universiteit Brussel, Brussels, Belgium. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!</div> <div class="editorbox">
<div class="editorbox">
'''Original Editors ''' - [[User:Yves Hubar|Yves Hubar]]
'''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.]]  


'''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}}  &nbsp;   
'''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}}  &nbsp;   
</div>  
</div>  
== Search Strategy ==
== Definition/Description  ==
 
Complex Regional Pain Syndrome (CRPS) is also known as reflex sympathetic dystrophy, causalgia, Sudeck's atrophy, algoneurodystrophy, among other names. It is a disease causing severe pain, disproportional to the expected amount of pain from a stimulus.<ref name="Patho Book">Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. Saint Louis: Saunders Elsevier, 2009.</ref> It is typically confined in one limb, but may spread to other limbs or even to the entire body. A person with CRPS will experience sensory, motor, autonomic, and skin/bone changes.<ref name="goebel">Goebel A. Complex regional pain syndrome in adults. Rheumatology. 2011;50;288-6.</ref><br>There are two types of CRPS. CRPS type I occurs after any type of trauma. CRPS type II may also occur after trauma, but has neuronal involvement. CRPS most commonly occurs after surgery (including arthroscopies), upper and lower motor neuron injuries, traumatic brain injury, cerebrovascular accident, central nervous system lesion, neuropathies, or nerve entrapments.<ref name="Patho Book" /><br>
 
== Prevalence  ==
 
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>
 
== Characteristics/Clinical Presentation ==


Literature was found on pubmed and the vub v-spaces system.<br>
<u>Signs and Symptoms of CRPS:<br></u>  


== Definition/Description  ==
*pain (pain may not be present in 7%of CRPS sufferers)<ref name="Patho Book" /> <ref name="goebel" /><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" />
 
<br>


The international association for the study of pain defines CRPS as a collection of locally occurring painful conditions, usually following traumatic injury, which tends to express itself distally and exceeds the expected pain of the original trauma and usually results in significant motor deficit. <ref name="reu1">L.Verbruggen. Reumatologie. Dienst Uitgaven VUB 2011</ref><br>
<u></u><u>Characteristics:<br></u>  


CRPS is subdivided into type I and type II CRPS. <br>Type I CRPS signifies that no peripheral nerve injury can be linked to the condition, while type II signifies that the condition results from a peripheral nerve injury. <ref name="art1">Groeneweg G, Huygen FJ, Coderre TJ, Zijlstra FJ. Regulation of peripheral blood flow in Complex Regional Pain Syndrome: clinical implication for symptomatic relief and pain management. BMC Musculoskelet Disord. 2009 Sep 23;10:116. (Level A1)</ref><br>
*<u></u>sensory impairments<ref name="Patho Book" />
*movement disorders, typically in contralateral extremity<ref name="Patho Book" /><ref name="Hooshmand" />
*ANS dysfunction<ref name="Patho Book" />
*dystrophy<ref name="Patho Book" />
*atrophy<ref name="Patho Book" />  
*spreads proximally, to other extremities, and possibly the entire body<ref name="Patho Book" />  
*similar presentation to osteoporosis on radiographic images<ref name="Patho Book" />


== Clinically Relevant Anatomy  ==
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>


CRPS can take place in any body part, but the wrist is most frequently affected after fractures.
{| border="1" cellspacing="1" cellpadding="1" width="591"
|-
! scope="col" | Stage
! scope="col" | Time Period
! scope="col" | Classic Signs and Symptoms<ref name="Patho Book" />
|-
| '''Stage I''': acute inflammation: denervation and sympathetic hypoactivity
| Begins 10 days post injury;<br>Lasts 3-6 months<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>
|-
| '''Stage II''': dystrophic: paradoxic sympathetic hyperactivity
| Begins 3-6 months after onset of pain;<br>Lasts about 6 months<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>
|-
| '''Stage III''': atrophic
| Begins 6-12 months after onset of pain;<br>Lasts for years, or may resolve and reappear<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>
|}


== Associated Co-morbidities  ==


CRPS may also be associated with:


An important aspect of the disease is the occurance of vascular disturbances. Mostly affected are primary small vessels, causing an impact on microcirculation, skin temperature and clinical appearance of the limb.<br>A paper described the changes in microcirculation as an increase in the number of capillaries, endothelial swelling and changes in the vessel luminal wall.<ref name="art1" /><br>According to a review, the acute stage features inhibited sympathetic vasoconstriction and exaggerated neurogenic inflammation, whereas the cold stage features vasoconstriction and endothelial disfunction or vascular hyperreactivity while neurogenic inflammation is less severe.<ref name="art2">Wasner G. Vasomotor Disturbances in Complex Regional Pain Syndrome—A Review. Pain Med. 2010 Aug;11(8):1267-73. (Level C)</ref><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>


== Epidemiology /Etiology ==
== Medications ==


CRPS is found to result:<ref name="reu1" /><br>- After traumatic injury (65%)<br>
Possible treatments for CRPS include:  


*1-2% of all fractures result in CRPS  
*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" />
*Largest risk of CRPS for fractures of the wrist
*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" />
*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>
*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" />


- After surgical intervention (19%)<br>- Infection (4%)<br>- Prior inflammation (2%)<br>- No clear cause (10%)
== Diagnostic Tests/Lab Tests/Lab Values  ==


A review stated that women are predominantly affected, by a factor of 3,5 and a genetic predisposition has also been theorized.<br>The disease affects all ages, though most cases are between 50 and 70 years old, and it is generally believed to occur mainly in caucasian and Japanese people.<ref name="art4">de Mos M, Sturkenboom MC, Huygen FJ. Current Understandings on Complex Regional Pain Syndrome. Pain Pract. 2009 Mar-Apr;9(2):86-99. Epub 2008 Feb 9. (Level A1)</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>  


== Characteristics/Clinical Presentation  ==
<br>


The following symptoms have been found in literature:<ref name="art3">Maihöfner C, Seifert F, Markovic K. Complex regional pain syndromes: new pathophysiological concepts and therapies. Eur J Neurol. 2010 May;17(5):649-60. Epub 2010 Feb 18. (Level A1)</ref><br>- Autonomic and trophic disorders:
<u>'''The Budapest Criteria'''</u>  


*Distal Edema in 80% of the patients
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" />
*Skin temperature changes at the affected body part in 80% of the patients, initially warmer and in 40% of patients gradually cools down until colder in comparison to the rest of the body as the disease progresses. Another review mentioned that 30% of the patients start off from the primarily cold stage.3
*In 40% of the patients skin at the affected body part starts showing redness, but becomes pale or livid in later stages
*In 55% altered sweating takes place, with hyperhydrosis being more common than hypohydrosis.  
*Hair and nail growth possibly increase in early stages
*Atrophy of skin and muscles in later stages, as well as contractures may severely restrict movement


- Sensory disturbances (90%) typically in a glove or stocking-like distribution
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:


*Spontaneous pain occurs in 75%, usually burning dragging or stinging
*Sensory: Reports of hyperesthesia and/or allodynia
<blockquote>
*Vasomotor: Reports of temperature asymmetry and/or skin color changes and/or skin color asymmetry
*68% felt in deep structures
*Sudomotor/Edema: Reports of edema and/or sweating changes and/or sweating asymmetry
*32% felt in skin  
*Motor/Trophic: Reports of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)
*In 77% pain shows fluctuating intensity, lesser proportion shows shooting pain
*Pain can be increased by orthostasis, anxiety, exercise or temperature changes.
*In many cases, pain is more pronounced at night
</blockquote>
*Sensory gain (Mechanical hyperalgesia, allodynia, ...) or sensory loss (hypaesthesia, hypalgesia, …) may be present.


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


*Motor weakness
*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)
*Severe impairment of complex movements
*Vasomotor: Evidence of temperature asymmetry (&gt;1 °C) and/or skin color changes and/or asymmetry
*Impairment of range of motion, initially by concomitant edema, later by contractures and fibroses
*Sudomotor/Edema: Evidence of edema and/or sweating changes and/or sweating asymmetry
*Neglect like symptoms have been found in some patiënts, described as the body part in question feeling foreign.
*Motor/Trophic: Evidence of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)
*Enhanced physiological tremor in around 50%
*Myoclonus or dystonia, especially in type II CRPS<br>


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


The differential diagnostic consists of:<ref name="art2" /><br>
== Etiology/Causes  ==


*[[Rheumatology|Rheumatic conditions]]
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" />
*Inflammatory conditions (infections following bone surgery, neuritides)
*Thromboembolic conditions
*[[Compartment_Syndrome|Compartment syndrome]]<br>  
*Peripheral neuropathy (mainly for type II CRPS)


== Diagnostic Procedures  ==
[[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>


add text here related to medical diagnostic procedures
== Systemic Involvement  ==


== Outcome Measures  ==
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" />


add links to outcome measures here (also see [[Outcome Measures|Outcome Measures Database]])
<br>


== Examination ==
== Medical Management (current best evidence) ==


No golden standard has been developed yet, but included here are the Budapest criteria.<ref name="art2" />
*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" />


The following must be met<br>- Continuing pain, which is disproportionate to any inciting event<br>- Must report at least one symptom in three of the four following categories:<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>  


*Sensory: reports of hypaesthesia and/or allodynia
<br>  
*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 <br>
*Motor/trophic: reports of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)


- Must display at least one sign at time of evaluation in two or<br> more of the following categories:<br>
== Physical Therapy Management (current best evidence)  ==


*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)
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;
*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)


- There is no other diagnosis that better explains the signs and symptoms<br>
Physical therapy intervention could include any of the following:


== Medical Management <br> ==
*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" />


Concerning pharmacogenic treatment:
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>


- Pathophysiologically oriented pharmacogenic treatment include application of glucocorticoids, tnf-alpha antibodies, free radical scavengers and sympathic blockade.<br>- Symptomatically oriented pharmacogens include opioids, gabapentin, NSAIDs and baclofen.<br>- To inhibit osteoclastic activity calcitonin, bisphosphonates and mannitol and vasodilating drugs may be given.
== Alternative/Holistic Management (current best evidence)  ==


<br>  
All patients with CRPS should receive a full&nbsp;psychological evaluation.&nbsp; They should receive cognitive-behavioral pain management treatment.&nbsp; Patients with CRPS who also suffer from other psychological disorders should also receive general cognitive behavioral therapy.<ref name="Bruehl">Bruehl S, Chung OY. Psychological and behavioral aspects of complex regional pain syndrome management. Clin J Pain 2006;22(5):430-7.</ref>  


Definite reports on the efficacy of sympathectomy are currently lacking and there is a risk of developing post sympathectomy pain syndrome.<ref name="art2" />
Alternative or holistic means of management may involve:


A review has been found, describing the positive effects of Spinal Cord Stimulation and several theories regarding its effectiveness.<ref name="art5">Prager JP. What Does the Mechanism of Spinal Cord Stimulation Tell Us about Complex Regional Pain Syndrome? Pain Med. 2010 Aug;11(8):1278-83. (Level C)</ref><br>
*Accupuncture<ref name="Sprague">Sprague M, Chang J. Integreative approach focusing on acupuncture in the treatment of chronic complex regional pain syndrome. J of Alternative and Complementary Medicine 2011;17(1):67-70.</ref>
*Pilates<ref name="pilates">Maier K, Livestrong.com. Why is pilates good for RSD?. http://www.livestrong.com/article/323975-why-is-pilates-good-for-rsd/ (accessed 28 March 2012).</ref>  
*Tai Chi<ref name="pilates" />
*Yoga<ref name="pilates" />


== Physical Therapy Management <br> ==
== Differential Diagnosis ==


The following interventions were found in literature: <ref name="art2" />  
CRPS needs to be differentiated from the following diagnosis<ref name="turner" />:


*Lymphatic drainage to facilitate regression of edema
*Bony or soft tissue injury
*[[Mirror Therapy]]
*Neuropathic pain
*Graded motor learning
*Arthritis
*TENS (Unless patient cannot tolerate the therapy due to allodynia or hyperalgesia)
*Infection
*Compartment syndrome
*Arterial insufficiency
*Raynaud’s Disease
*Lymphatic or venous obstruction
*Thoracic outlet syndrome
*Gardner-Diamond Syndrome
*Erythromelalgia
*Self-harm or malingering<br>


A paper on movement disorders in CRPS stated that splints or plaster casts are often ineffective and might even worsen dystonic postures related to CRPS. <ref name="art6">de Mos M, Sturkenboom MC, Huygen FJ. Movement Disorders in Complex Regional Pain Syndrome. Pain Pract. 2009 Mar-Apr;9(2):86-99. Epub 2008 Feb 9. (Level D)</ref>
== Clinical Guidelines ==


== Key Research  ==
[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.


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>
== &nbsp;&nbsp;Case Reports/ Case Studies  ==


== Resources <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]


add appropriate resources here <br>  
<br>  


== Clinical Bottom Line  ==
{{#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>


add text here <br>  
<br>  


== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed]) ==
== Resources <br> ==


see tutorial on [[Adding PubMed Feed|Adding PubMed Feed]]  
*[http://www.rsds.org Reflex Sympathetic Dystrophy Syndrome Association]&nbsp;
<div class="researchbox">
*[http://www.theacpa.org American Chronic Pain Association]  
<rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1P7PR1QO6IS9xQ724YOoSHF8On2gkKwiE3UGU6x5C6gE847gBI|charset=UTF-8|short|max=10</rss>
*[http://www.mayoclinic.com Mayo Clinic]  
</div>
*[http://www.ninds.nih.gov National Institute of Neurological Disorders and Stroke]


== &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>
== References  ==
== References  ==


Line 151: Line 227:
<references />  
<references />  


[[Category:Vrije_Universiteit_Brussel_Project|Template:VUB]]
[[Category:Bellarmine_Student_Project]][[Category:Neurology]][[Category:Medical]][[Category:Pain]]

Revision as of 16:41, 14 June 2013

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

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

Definition/Description[edit | edit source]

Complex Regional Pain Syndrome (CRPS) is also known as reflex sympathetic dystrophy, causalgia, Sudeck's atrophy, algoneurodystrophy, among other names. It is a disease causing severe pain, disproportional to the expected amount of pain from a stimulus.[1] It is typically confined in one limb, but may spread to other limbs or even to the entire body. A person with CRPS will experience sensory, motor, autonomic, and skin/bone changes.[2]
There are two types of CRPS. CRPS type I occurs after any type of trauma. CRPS type II may also occur after trauma, but has neuronal involvement. CRPS most commonly occurs after surgery (including arthroscopies), upper and lower motor neuron injuries, traumatic brain injury, cerebrovascular accident, central nervous system lesion, neuropathies, or nerve entrapments.[1]

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.[2] 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.[1] Ninety-one percent of all CRPS cases occur after surgery.[3]

Characteristics/Clinical Presentation[edit | edit source]

Signs and Symptoms of CRPS:

  • pain (pain may not be present in 7%of CRPS sufferers)[1] [2][4]
  • swelling[1][2][4]
  • tremor[1]
  • trouble initiating movements[1]
  • muscle spasms (may be present in the cervical and lumbar spine regions in advanced cases)[1][4]
  • muscle atrophy[1]
  • temperature changes[1][2]
  • color changes (red, blue)[1]
  • thick, brittle, or rigid nails[1]
  • weakness[1][2][4]
  • thin, shiny, clammy skin[2]
  • stiffness or decreased joint motion[2]
  • painful or decreased sensation on skin (some patients report intolerance to air moving over skin)[2]
  • strange, disfigured, or dislocated feelings in limbs[2]


Characteristics:

  • sensory impairments[1]
  • movement disorders, typically in contralateral extremity[1][4]
  • ANS dysfunction[1]
  • dystrophy[1]
  • atrophy[1]
  • spreads proximally, to other extremities, and possibly the entire body[1]
  • similar presentation to osteoporosis on radiographic images[1]

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.[4]

Stage Time Period Classic Signs and Symptoms[1]
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

Associated Co-morbidities[edit | edit source]

CRPS may also be associated with:

  • Arterial Insufficiency[5]
  • Asthma[2]
  • Bone Fractures[4]
  • Cellulitis[5]
  • Central Pain Syndromes[5]
  • Conversion Disorder[5]
  • Depression/Anxiety
  • Factitious Disorder [5]
  • Lymphedema[5]
  • Malignancy[5]
  • Migraines[2]
  • Nerve Entrapment Syndromes[5]
  • Osteomyelitis[5]
  • Osteoporosis[2][4]
  • Pain Disorder[5]
  • Peripheral Neuropathies[5]
  • Rheumatoid Arthritis[5]
  • Scleroderma[5]
  • Septic arthritis[5]
  • Systemic Lupus Erythematosus[5]
  • Tenosynovitis[5]
  • Thrombophlebitis[5]

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.[1]
  • 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.[1]
  • Pain intensity and perception of pain is sometimes relieved through use of an implanted transcutaneous electrical nerve stimulation (TENS) unit.[1]
  • 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[6]
  • 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.[7]
  • Antidepressants may be utilized to treat associated depression.[4]

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

Diagnosis of CRPS is based solely on examination and patient history.[1] A triple-phase bone scan is the best method to rule out type I CPRS.[8] According to Cappello, the triple-phase bone scan has the best sensitivity, NPV, and PPV compared to MRI and plain film radiographs.[8] Radiographic examinations, laser Doppler flowmetry, and thermographic studies may be utilized to assess the secondary issues and symptoms of CRPS.[1]


The Budapest Criteria

The Budapest Criteria have been used to diagnose CRPS as well.  This method has high specificity and sensitivity according to Goebel.[2]

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.[9]  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.[4]

The cycle of CRPS [10]

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.[4]


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[11]
  • Spinal cord stimulation was shown to be effective in completely eliminating pain in adolescent females 2-6 weeks after stimulation[12]
  • 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.[13]
  • 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.[14]
  • 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.[4]
  • A stellate ganglion block, or sympathectomy, blocks the nerve pathways causing pain. This may be most beneficial in the early stages of CRPS.[1][2]

[15]


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[2]. 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.[1] 

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.[1][16]
  • aquatics: Aquatic therapy allows activities to be performed with decreased weight bearing on the lower extremities.[1]
  • mirror therapy
  • desensitization[2]
  • gradual weight bearing[2]
  • stretching[2]
  • fine motor control[2]

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.[4]

Alternative/Holistic Management (current best evidence)[edit | edit source]

All patients with CRPS should receive a full psychological evaluation.  They should receive cognitive-behavioral pain management treatment.  Patients with CRPS who also suffer from other psychological disorders should also receive general cognitive behavioral therapy.[17]

Alternative or holistic means of management may involve:

Differential Diagnosis[edit | edit source]

CRPS needs to be differentiated from the following diagnosis[3]:

  • 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.

  Case Reports/ Case Studies[edit | edit source]


[20]


Resources
[edit | edit source]

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

Failed to load RSS feed from http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1fAD01Rzfn7B0ZRzIfL12TBhNQObAZ_2nJk-8802GMR8R-nYIR|charset=UTF-8|short|max=10: Error parsing XML for RSS

References[edit | edit source]

see adding references tutorial.

  1. ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 1.15 1.16 1.17 1.18 1.19 1.20 1.21 1.22 1.23 1.24 1.25 1.26 1.27 1.28 1.29 Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. Saint Louis: Saunders Elsevier, 2009.
  2. ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 2.14 2.15 2.16 2.17 2.18 2.19 Goebel A. Complex regional pain syndrome in adults. Rheumatology. 2011;50;288-6.
  3. ↑ 3.0 3.1 Turner-Stokes L, Goebel A. Complex regional pain syndrome in adults: concise guidance. Clinical Med 2011; 11(6):596-600.
  4. ↑ 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 4.11 4.12 Hooshmand H, Phillips E. Spread of complex regional pain syndrome. Vero Beach, Florida. Neurological Associates Pain Management Center.
  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 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).
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ 8.0 8.1 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.
  9. ↑ 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).
  10. ↑ 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).
  11. ↑ 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.
  12. ↑ 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.
  13. ↑ 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.
  14. ↑ 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.
  15. ↑ Arizona Pain. Stellate Ganglion Block. Available from: http://www.youtube.com/watch?v=izOYrLUuNd8 [last accessed 3/29/12]
  16. ↑ 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.
  17. ↑ Bruehl S, Chung OY. Psychological and behavioral aspects of complex regional pain syndrome management. Clin J Pain 2006;22(5):430-7.
  18. ↑ Sprague M, Chang J. Integreative approach focusing on acupuncture in the treatment of chronic complex regional pain syndrome. J of Alternative and Complementary Medicine 2011;17(1):67-70.
  19. ↑ 19.0 19.1 19.2 Maier K, Livestrong.com. Why is pilates good for RSD?. http://www.livestrong.com/article/323975-why-is-pilates-good-for-rsd/ (accessed 28 March 2012).
  20. ↑ CNN. CNN Report on Reflex Sympathetic Dystrophy. Available from: http://www.youtube.com/watch?v=jaTlI6bfF64 [last accessed 3/28/12]
Retrieved from "https://www.physio-pedia.com/index.php?title=Complex_Regional_Pain_Syndrome_(CRPS)&oldid=73327"