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Complex Regional Pain Syndrome Type II

Complex regional pain syndrome is a condition characterized by onset of chronic pain that affects an arm or a leg. It is a rare form of disease and the type II variety often results from nerve injury.


Presentation

The following are the various signs and symptoms experienced by individuals affected by complex regional pain syndrome type II [8].

  • Continuous throbbing pain in the affected limb, which may also be accompanied by burning sensation
  • The affected area is sensitive to touch and cold
  • Development of swelling in the affected area
  • Significant changes in the nail and hair growth of the affected limb
  • Skin can become tender and atrophic
  • Stiffness in joint can set in, accompanied by development of swelling with consequent damage
  • Reduced ability to move the affected joint
  • Weakness and muscle spasms
  • Change in color of the skin 
  • Change in the temperature of the affected skin – skin can sometimes sweat or even turn cold
Limb Pain
  • TACTILE DISCRIMINATION TRAINING Another approach to CRPS is based on a treatment called sensory discrimination training, which was used for phantom limb pain.[mipmpc.com]
  • pain and discrimination, mirror and imaginary training in patients with phantom limb pain and complex regional pain syndrome . de Treten bei komplexem regionalem Schmerzsyndrom (CRPS) komorbide psychische Störungen, Störungen der Schmerzverarbeitung[de.glosbe.com]
  • Mirror therapy has been reported to benefit patients with CRPS type 1 due to phantom limb pain or stroke. Patients straddle a large mirror between their legs.[merckmanuals.com]
  • Pain is continuous and may be heightened by emotional stress. Moving or touching the limb is often intolerable. If untreated, the joints may become stiff.[davidlnelson.md]
  • The features are limb pain, allodynia, hypersensitivity, hyperalgesia, abnormalities of the vasomotor, sudomotor and motor systems, and trophic changes, with reduced use of the affected limb. The diagnosis is clinical and one of exclusion.[nps.org.au]
Arm Pain
  • Three months after, he presented to an outpatient pain management clinic right arm pain and was diagnosed with complex regional pain syndrome type II (CRPS II).[ncbi.nlm.nih.gov]
  • Thinking about movement hurts: The effect of motor imagery on pain and swelling in people with chronic arm pain.[bodyinmind.org]
Hand Pain
  • Two patients received mirror therapy with the painful hand hidden behind the mirror while the non-painful hand was positioned so that, from the perspective of the patient, the reflection of this hand was "superimposed" on the painful hand.[ncbi.nlm.nih.gov]
Leg Swelling
  • As unilateral leg swelling usually implies a local mechanical or inflammatory process, our differential diagnosis in this case included soft tissue or ligamentous injury of the foot/ankle, fracture, DVT, and venous or lymphatic insufficiency.[academic.oup.com]
Malnutrition
  • Post-surgery, patients may discontinue chronic follow-up and nutritional supplementation leading to malnutrition and PPND 31,37-41. PPND is well recognized in metabolic syndrome (obesity, diabetes, hypertension, and hyperlipidemia) 42.[bmj.com]
Venous Insufficiency
  • Undiagnosed fracture, venous insufficiency, and DVT were excluded by plain X-rays and venous ultrasonography. MRI of the left foot and ankle ruled out soft tissue injury.[academic.oup.com]
Muscular Atrophy
  • Duchenne's muscular dystrophy ( Duchenne type muscular dystrophy ) The childhood type of muscular dystrophy. myotonic dystrophy a rare, slowly progressive, hereditary disease, marked by myotonia followed by muscular atrophy (especially of the face and[medical-dictionary.thefreedictionary.com]
Shoulder Pain
  • "Shoulder pain in hemiplegia". Physical Medicine and Rehabilitation Clinics of North America. 15 (3): vi–vii, 683–97. doi : 10.1016/S1047-9651(03)00130-X. PMID 15219895. Pawelka S, Fialka V, Ernst E (January 1993).[en.wikipedia.org]
Severe Osteoporosis
  • Severe osteoporosis. Thickening of joints and atrophy of muscles. Stage Three: Irreversible of skin and bones. Unyielding pain. Major atrophy of muscles. Flexor tendon contractions. Limitations upon movement of affected areas. Limb displacement.[midwestcompassion.org]
Long Leg
  • Review Topic QID: 1155 1 Long leg cast immobilization for 3 months 2 Walking boot with non weight bearing for three months 3 Exchange nailing to stimulate healing response to the limb 4 Epidural spinal cord stimulator 5 Surgical sympathectomy of the affected[orthobullets.com]
Distractibility
  • […] clinical suspicion and treatment - late CRPS is highly refractory to treatment and results in permanent disability - early mobilization - it is first essential to address any direct cause or associated cause of the pain; - examples include: - an over-distracted[wheelessonline.com]
  • It is worth noting, however, that in complex polytrauma patients, distracting injuries can effectively delay the onset of symptoms [9].[academic.oup.com]
Amenorrhea
  • I conclude that gabapentin has the potential to cause amenorrhea with return of menses occurring after discontinuation of the drug.[ncbi.nlm.nih.gov]
Dysautonomia
  • Bruehl’s comment should assist physicians with the differential diagnosis and evaluation of patients with painful peripheral neuropathy/dysautonomia (PPND). These address physical issues only.[bmj.com]
  • These studies determined that sensitivity of the criteria was high (0.98), but specificity was poor (0.36); there was a tendency to over-diagnosis; a positive diagnosis could be correct in as few as 40% of cases; and the criteria for dysautonomia were[medlink.com]
  • […] related pain (acute) (chronic) G89.4 Chronic pain syndrome G90 Disorders of autonomic nervous system G90.0 Idiopathic peripheral autonomic neuropathy G90.01 Carotid sinus syncope G90.09 Other idiopathic peripheral autonomic neuropathy G90.1 Familial dysautonomia[icd10data.com]
  • […] be triggered by accumulation of metabolites and uraemia Reye's syndrome cerebral oedema and death (in 50% of cases, usually children), provoked by aspirin therapy; aspirin is proscribed for children less than 16 years old Riley–Day syndrome; familial dysautonomia[medical-dictionary.thefreedictionary.com]
Cervical Radiculopathy
  • Review Topic QID: 3356 4 Cervical radiculopathy from herniated nucleus pulposis ML 1 Select Answer to see Preferred Response PREFERRED RESPONSE 2 (OBQ13.176) Vitamin C has been shown to decrease the likelihood of which of the following complications following[orthobullets.com]

Workup

A preliminary physical examination, coupled with past medical history would be taken initially. This would be followed by several diagnostic methods to accurately judge the condition.

  • Bone scan: Such a kind of procedure helps in detection of changes in the bone.
  • Tests for sympathetic nervous system: In this, various tests are employed for detecting the functioning of sympathetic nervous system. These tests include measuring the temperature of the affected skin and blood flow through thermography. In addition, the amount of sweat produced in both the limbs would also be done. If there is significant difference in the amount of sweat production, it suggests development of complex regional pain syndrome.
  • Imaging studies: MRI would be carried out, which would provide insight about the changes that have taken place in the tissues. X-rays of the bone should also be done which would reveal mineral density of the bone [9].
HLA-DQ1
  • HLA-DQ1 associated with reflex sympathetic dystrophy. Neurology . 1999 Oct 12. 53(6):1350-1. [Medline] . Van Hilten JJ, Van De Beek WJ, Roep BO.[emedicine.medscape.com]

Treatment

If treatment is initiated within first few months of onset of symptoms, then the condition can be effectively managed with medications and therapies. The following methods are administered for treatment of complex regional pain syndrome type II.

Medications

Various pain relieving medications are employed for helping the patients deal with pain. Other class of medications to manage other symptoms is also employed. These include anticonvulsants, corticosteroids, intravenous ketamine and sympathetic nerve blocking medications. Research has shown tha, ketamine given intravenously helps in relieving the pain [10].

Therapies

Various types of therapies that involve physical therapy, biofeedback and transcutaneous electrical nerve stimulation are beneficial. In addition to these, analgesics to be applied on the area are also provided. A new mode of therapy has been introduced known as the spinal cord stimulation therapy. In this, tiny electrodes are inserted along the spinal cord in order to provide relief [11].

Prognosis

If treatment has been initiated within 3 months of first appearance of the signs and symptoms then a good prognosis can be expected. However, if there is delay in initiation of treatment, the disease can rapidly affect the entire limb causing irreversible damage to the bones, nerves and muscles. In addition, affected individuals can suffer from atrophy of muscles and in many cases, limb amputation may be required [7].

Etiology

Complex regional pain syndrome type II majorly occurs due to nerve injury that can results from various factors. These include crush injuries, surgery, forceful trauma to the arm and leg, heart attack, sprained ankle and infections. In many cases, emotional stress also significantly participates in the development of complex regional pain syndrome type II. All these factors are known to cause injury to the nerve of the peripheral nervous system [3].

Epidemiology

The incidence of complex regional pain syndrome type II has been calculated to be 0.8 per 100,000 individuals. The prevalence of the condition is estimated to strike every 4 individuals per 100,000. Statistics have also revealed that, after peripheral nerve injury, the incidence of complex regional pain syndrome II occurs in 1 – 5% of cases [4] [5]. Females are more prone to develop the disease condition than males.

Sex distribution
Age distribution

Pathophysiology

The exact pathophysiology of complex regional pain syndrome type II is not completely understood. Scientists believe that sensitization of the peripheral and central nerves that occur due to response to tissue injury give rise to the severe pain in the affected arm or leg.

Sensitization of the central nervous system and increase in pain gradually are some of the key points in the onset of complex regional pain syndrome. In addition to this, there is significant evidence of involvement of N-methy-D-aspartate in the sensitization process of the central nervous system. Such sequence of events leads to development of mechanical hyperalgesia, which is characterized by severe pain in the affected limb [6].

Prevention

Various steps can be taken for preventing the development of complex regional pain syndrome. Individuals who have suffered wrist fracture are advised to take vitamin C supplements in order to prevent developing the syndrome later in life. It is also necessary that patients who have suffered at attack of stroke should practice early mobilization.

Summary

Complex regional pain syndrome type II in the past was also known as causalgia [1]. Significant trauma to a nerve of the peripheral nervous system gives rise to severe pain along with burning sensation in the affected arm or leg. It is a chronic disease condition, which gradually gets more severe as the disease progresses. So far, there is no known cure to the condition; it can however be managed with medications [2].

Patient Information

  • Definition: Complex regional pain syndrome type II, is characterized by onset of pain in an arm or leg due to injury to the nerve. The condition is more common in females than males.
  • Cause: Injuries, stroke, heart attack, sprained ankle and minor injuries can favor distinct nerve injury causing development of complex regional pain syndrome.
  • Symptoms: Some of the common symptoms include pain in the affected limb along with burning sensation. The affected area may also undergo change in color as well as temperature. In addition, there is joint stiffness, muscle spasms, and reduced ability to move the part that is affected.
  • Diagnosis: Physical examination, radiographs, MRI and bone scan are employed for diagnosing the condition.
  • Treatment: Affected individuals are given medications and various therapies for relieving the pain and management of other symptoms. Neurostimulation of the spinal cord is also done for relieving the intense pain.

References

Article

  • Bonica JJ. Causalgia and other reflex sympathetic dystrophies. Postgrad Med 1973; 53:143.
  • Harden RN, Bruehl S, Galer BS, et al. Complex regional pain syndrome: are the IASP diagnostic criteria valid and sufficiently comprehensive?. Pain. Nov 1999;83(2):211-9.
  • Schinkel C, Gaertner A, Zaspel J, et al. Inflammatory mediators are altered in the acute phase of posttraumatic complex regional pain syndrome. Clin J Pain 2006; 22:235.
  • Allen G, Galer BS, Schwartz L. Epidemiology of complex regional pain syndrome: a retrospective chart review of 134 patients. Pain. Apr 1999;80(3):539-44
  • Baron R, Binder A. Pappagallo M (ed). Complex regional pain syndromes, in The neurological basis of pain. New York: McGraw-Hill; 2005:359-378.
  • Baron R, Wasner G, Borgestedt R, et al. Effects of sympathetic activity on capsaicin-evoked pain, hyperalgesia and vasodilation Neurology. 1999;52:923-932.
  • Pleger B, Tegenthoff M, Ragert P, et al. Sensorimotor retuning [corrected] in complex regional pain syndrome parallels pain reduction. Ann Neurol 2005; 57:425.
  • Janig W, Baron R. Complex regional pain syndrome is a disease of the central nervous system. Clin Auton Res. Jun 2002;12(3):150-64
  • Todorovic-Tirnanic M, Obradovic V, Han R, Goldner B, Stankovic D, Sekulic D. Diagnostic approach to reflex sympathetic dystrophy after fracture: radiography or bone scintigraphy?. Eur J Nucl Med. Oct 1995;22(10):1187-93.
  • Christensen K, Jensen EM, Noer I. The reflex dystrophy syndrome response to treatment with systemic corticosteroids. Acta Chir Scand. 1982;148(8):653-5. 
  • Davis KD, Treede RD, Raja SN, Meyer RA, Campbell JN. Topical application of clonidine relieves hyperalgesia in patients with sympathetically maintained pain. Pain. Dec 1991;47(3):309-17

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Last updated: 2019-07-11 19:58