J KMA Special Issue Diagnosis and Treatment of Complex Regional Pain Syndrome Dong Eon Moon, MD Department of Anesthesiology and Pain Medicine, Catholic University College of Medicine Email : demoon@catholic.ac.kr J Korean Med Assoc 2006; 49(8): 688-700 Abstract The complex regional pain syndromes (CRPS I and CRPS II), also known as reflex sympathetic dystrophy and causalgia, have been recognized for the past 2,500 years. Despite its long history, the diagnosis and treatment of CRPS are still challenging. These syndromes can be characterized by discrete sensory, motor, and autonomic findings, but many patients with CRPS continue to suffer for years without the diagnosis. Although much progress has been made in the understanding of CRPS, many questions still remain unanswered. CRPS is probably a disease of the central nervous system. Yet, peripheral inflammatory processes, abnormal sympatheticafferent coupling, and adrenoreceptor pathology may also be part of the picture. A close multidisciplinary approach amongst the pain medicine consultants, psychologist, physical and occupational therapists, and neurologist is necessary to achieve the maximum treatment outcomes. If conventional treatment (e.g. pharmacotherapy) fails to show a significant response within 12 weeks, an interventional technique such as spinal cord stimulation (SCS) needs to be tried. The current concepts of CRPS could be replaced by a new mechanismbased term or group of terms in the near future leading to improved clinical guidelines. This article reviews the different aspects of CRPS including its definition, classification, epidemiology and natural history, clinical presentation, pathophysiology and management. Keywords : Complex regional pain syndrome; Reflex sympathetic dystrophy; Causalgia; Multidisciplinary approach; Spinal cord stimulation 688
Diagnosis and Treatment of Complex Regional Pain Syndrome International Association for the Study of Pain (IASP) Diagnostic Criteria for CRPS and CRPS CRPS (Reflex Sympathetic Dystrophy) 1. The presence of an initiating noxious event, or a cause of immobilization. 2. Continuous pain, allodynia, or hyperalgesia with which the pain is disproportionate to any inciting event. 3. Evidence at some time of oedema, changes in skin blood flow, or abnormal sudomotor activity in the region of the pain. 4. The diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction. Note : Criteria 24 must be satisfied CRPS (Causalgia) 1. The presence of continuing pain, allodynia, or hyperalgesia after a nerve injury, not necessarily limited to the distribution of the injured nerve. 2. Evidence at some time of oedema, changes in skin blood flow, or abnormal sudomotor activity in the region of the pain. 3. This diagnosis is excluded by the existence of a condition that would otherwise account for the degree of pain and dysfunction. Note : All three criteria must be satisfied 689
Moon DE Proposed Experimental Revision of CRPS Diagnostic Criteria 1. Continuing pain which is disproportionate to inciting event 2. Must report at least one symptom in each of the four following categories Sensory : reports of hyperesthesia 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 in two or more of the follwing categories: Sensory : evidence of hyperalgesia (to pinprick) and / or allodynia (to light touch) Vasomotor : evidence of temperature asymmetry 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) 690
Diagnosis and Treatment of Complex Regional Pain Syndrome Revised Diagnostic Criteria for CRPS Categories of clinical signs or symptoms Positive sensory abnormalities : Oedema, sweating abnormalities Spontaneous pain Swelling Mechanical hyperalgesia Hyperhidrosis Thermal hyperalgesia Hyporhidrosis Deep somatic hyperalgesia Motor (M) or trophic (T) changes Vascular abnormalities Motor weakness (M) Vesodilation Tremor (M) Vasoconstriction Dystonia (M) Skintemperature asymmetries Coordination deficits (M) Skincolour changes Nail or hair chages (T) Skin atrophy (T) Joint stiffness (T) Softtissue changes (T) Interpretation Clinical use 1 symptoms of 3 categories each AND 1 signs of 2 categories each Sensitivity 0.85, specifcity 0.60 Research use 1 symptoms in each of the 4 categories AND 1 signs of 2 categories each Sensitivity 0.70, specifidity 0.96 691
Moon DE Differential diagnosis of CRPS and CRPS Etiology Localization Spreading of Sx Spontaneous pain Mechanical allodynia Autonomic Sx Motor Sx Sensory Sx Sxsymptom CRPS (RSD) Any kind of lesion Distal part of extremity Independent from site of lesion Obligatory Common Mostly deep & superficial Orthostatic component Most of patients with spreading tendency Distally generalized with spreading tendency Distally generalized with spreading tendency Distally generalized with spreading tendency CRPS (Causalgia) Partial nerve lesion Mostly confined to the territory of affected nerve Rare Obligatory Predominately superficial No orthostatic component Obligatory in nerve territory Related to nerve lesion Related to nerve lesion Related to nerve lesion 692
Diagnosis and Treatment of Complex Regional Pain Syndrome TCA, tricyclic antidepressants; noradrenaline reuptake inhibitors. *Pain relieving effect of topical lidocaine has been shown in patients with allodynia. Treatment algorithm. Proposed algorithm for the treatment of peripheral neuropathic pain 693
Moon DE Treatment paradigm for CRPS. A paradigm should be layered (inparallel) in a interdisciplinary approach with the goal being functional recovery. 694
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