Complex Regional Pain Syndrome Type Research Articles

Diagnosis and Management of Knee Arthrofibrosis and Associated Pain-Related Fear Using Multidimensional Clinical Reasoning and Exposure In Vivo Concept: A Case Report

BACKGROUND: A plethora of treatment approaches are used to manage persistent pain, disability, and fear associated with restricted knee range of motion following knee surgery. We used the pain and disability driver model (PDDM) and exposure in vivo (EIV) concepts to manage pain-related fear and disability. CASE PRESENTATION: A 68-year-old male who underwent arthroscopic partial meniscectomy presented to physical therapy with a medical diagnosis of Complex Regional Pain Syndrome Type I (CRPS-I) and knee range of motion restriction. Despite extensive medical care and physical therapy for three years (202 visits) he continued to experience debilitating knee pain and demonstrated a high level of fear. To ambulate, he wore a knee brace and used a trekking pole. OUTCOME AND FOLLOW UP: Interventions to address the knee stiffness included combined tibiofemoral joint mobilization and high velocity low amplitude thrust manipulation (HVLAT) targeting tibial internal rotation and low load long duration sustained capsular stretching using total end range time (TERT) principle for extension. His fear was managed using techniques inspired by in vivo exposure concepts. Total knee range of motion improved 110 degrees and the patient was able to ambulate for 30 minutes (0.5 mile) without an assistive device with <2/10 pain intensity. DISCUSSION: Outside-the-box clinical reasoning suggested poor outcomes with previous interventions were likely due to hypervigilance and pain-related fear. This case delineates the value of non-reductionist clinical reasoning in diagnosis and management of musculoskeletal conditions. It also outlines how the exposure in vivo approach helped the patient overcome his long-established avoidance behaviors.

Quality of life, functioning and participation of adult patients with an amputation following complex regional pain syndrome I or brachial plexus injury: a scoping review protocol

IntroductionThis planned scoping review aims to provide insight into current literature regarding perceived quality of life (QoL), functioning and participation of patients with upper limb amputations (ULA) because of therapy-resistant...

Mechanisms of complex regional pain syndrome.

Complex Regional Pain Syndrome (CRPS) is a chronic pain disorder characterized by a diverse array of symptoms, including pain that is disproportionate to the initial triggering event, accompanied by autonomic, sensory, motor, and sudomotor disturbances. The primary pathology of both types of CRPS (Type I, also known as reflex sympathetic dystrophy, RSD; Type II, also known as causalgia) is featured by allodynia, edema, changes in skin color and temperature, and dystrophy, predominantly affecting extremities. Recent studies started to unravel the complex pathogenic mechanisms of CRPS, particularly from an autoimmune and neuroimmune interaction perspective. CRPS is now recognized as a systemic disease that stems from a complex interplay of inflammatory, immunologic, neurogenic, genetic, and psychologic factors. The relative contributions of these factors may vary among patients and even within a single patient over time. Key mechanisms underlying clinical manifestations include peripheral and central sensitization, sympathetic dysregulation, and alterations in somatosensory processing. Enhanced understanding of the mechanisms of CRPS is crucial for the development of effective therapeutic interventions. While our mechanistic understanding of CRPS remains incomplete, this article updates recent research advancements and sheds light on the etiology, pathogenesis, and molecular underpinnings of CRPS.

Permanent Peripheral Nerve Stimulator for Complex Regional Pain Syndrome of the Forearm and Hand

Summary: A 51-year-old woman with diabetes and depression was referred to the anesthesia pain clinic with a 15-year history of complex regional pain syndrome type I of the right forearm and hand in the radial sensory nerve distribution. There was no recognized antecedent trauma and she had failed both medical treatment and radial sensory nerve decompression surgery. An ultrasound-guided local anesthetic block of the radial nerve in the upper arm resulted in partial and temporary improvement in pain. She subsequently underwent trial of a peripheral nerve stimulator (PNS) followed by permanent PNS implant placement over the radial nerve proper proximal to the elbow. Within the first month of use, she endorsed substantially improved pain and strength in her right hand. These improvements were sustained for more than 1 year. Applications of PNS technology for treatment of extremity complex regional pain syndrome and neuropathic extremity pain in general are reviewed.

Complex regional pain syndrome: diagnostic challenges and favorable response to prednisolone

Complex regional pain syndrome (CRPS), characterized by severe and disproportionate pain, is a rare and debilitating condition. Due to its rarity, evidence-based treatment guidelines remain limited, creating a challenge for clinicians. We present the case of a 20-year-old female with CRPS type 1 of the right hand. Her pain, initially triggered by a minor trauma, had persisted for three months. The patient demonstrated severe pain, swelling, hyperesthesia, and restricted range of motion. Despite multiple hospital visits, her symptoms did not improve until she was diagnosed with CRPS and treated with oral prednisolone. A dosage of 40 mg daily led to a dramatic response within 10 days. Our report emphasizes the importance of recognizing CRPS and highlights the potential of prednisolone as a treatment option, particularly in resource-limited settings, where more specialized interventions may be unavailable. Further research is essential to establish a stronger evidence base for the use of steroids in CRPS management.

Factors predicting complications following open reduction and internal fixation of intra-articular distal radius fracture.

This study aimed to determine the incidence and predictors of the complications after open reduction and internal fixation (ORIF) of intra-articular distal radius fracture (IADRF) with a minimum follow-up of 12 months. Medical records and outpatient follow-up records were retrospectively reviewed to collect medical, surgical, and complication data on consecutive patients who had undergone an ORIF procedure for an IADRF between January 2019 and June 2022. Data included demographics, comorbidities, injury, surgical characteristics, and laboratory findings on admission. A multivariate logistic regression model was constructed to identify the significant predictors, with a composite of any complications occurring within 12 months after the operation as the outcome variable and potentially a range of clinical data as the independent variables. The magnitude of the relationship was indicated by the odds ratio (OR) and the 95% confidence interval (CI). During the study period, 474 patients were included, and 64 had documented complications (n = 73), representing an accumulated rate of 13.5%. Among them, carpal tunnel syndrome was the most common, followed by tenosynovitis caused by tendon irritation/rupture, superficial or deep wound infection, complex regional pain syndrome (CRPS) type 1, radial shortening (≥4 mm), plate/screw problems, and others. The multivariate results showed the following factors significantly associated with increased risk of complications: experience of DRF surgery with <30 cases (OR: 2.2, 95% CI: 1.6-3.5), AO type C fracture (OR: 1.7, 95% CI: 1.2-2.9), initial lunate facet collapse of ≥5 mm (OR: 4.2, 95% CI: 1.4-8.9), and use of temporary external fixation before index surgery (OR: 2.4, 95% CI: 1.5-4.3). These findings may aid in patient counseling and quality improvement initiatives, and IADRF should be directed by an experienced surgeon.

Teriparatide and clodronate combination as a potential treatment for complex regional pain syndrome type I in delayed consolidation after foot surgery: a case report and review of the literature

BackgroundComplex regional pain syndrome type I is a pathological condition characterized by an exaggerated response of tissues to low or moderate pain stimuli. The exact pathogenesis and optimal medical treatment for complex regional pain syndrome type I are still not fully understood, although bisphosphonates have shown positive effects in reducing pain. Foot surgery can be complicated by the development of complex regional pain syndrome type I, leading to functional decline and difficulties in weight-bearing.Case presentationThe authors present a clinical case involving complex regional pain syndrome type I that developed after surgical foot arthrodesis. The patient, a 42-year-old Caucasian male, did not respond to clodronate treatment but experienced successful outcomes upon the addition of teriparatide, which effectively stimulated the healing of arthrodesis.ConclusionTeriparatide cannot be considered the primary treatment for complex regional pain syndrome due to insufficient solid clinical data. However, when complex regional pain syndrome is associated with or caused by delayed union, teriparatide can be used to address the underlying cause of complex regional pain syndrome.

Intramuscular botulinum toxin-A in complex regional pain syndrome resistant to standard treatment: acase report.

To present apatient with complex regional pain syndrome type1 (CRPS-I) and improvement of contracture of hand muscles and grip strength after successful treatment with botulinum neurotoxin‑A (BoNT-A). A53-year-old woman with CRPS‑I experienced severe allodynia, swelling and autonomic changes in the left hand after a distal radius fracture. Over the succeeding months, she developed contracture of the left hand muscles which was treated with injection of BoNT‑A into the hand muscles (10points). In the patient treatment with BoNT‑A an improvement was seen in the hand range of motion (ROM) and grip strength. Successful results can be obtained with BoNT‑A injection in treatment-resistant CRPS‑I cases which may develop joint contracture.

Successful therapy of complex regional pain syndrome after hip arthroscopy for femoroacetabular impingement syndrome: a case report

BackgroundComplex regional pain syndrome is a neuropathic pain disorder associated with ongoing pain that persists beyond the usual expected tissue healing time and that is disproportionate to the degree of tissue injury present. Complex regional pain syndrome after hip arthroscopy has not been reported before. Hip arthroscopy is a fast-growing domain that could lead to an increasing number of complex regional pain syndrome cases, probably owing to the high traction forces that are necessary.Case presentationWe report the case of a 30-year-old German female semiprofessional tennis player who presented with complex regional pain syndrome type I in the lower leg 3 weeks after hip arthroscopy for femoroacetabular impingement syndrome with suture anchor labral repair and femoroplasty. After 2 months of immediate multimodal conservative therapy including administration of gabapentin, prompt full weight-bearing, and intensified physiotherapy, complete recovery was achieved.ConclusionComplex regional pain syndrome does occur after elective hip arthroscopy. Disproportionate postoperative pain or other symptoms raising suspicion of complex regional pain syndrome should be promptly evaluated and treated through a multimodal approach. Postless hip arthroscopy may be advantageous.

Contact-Heat Evoked Potentials: Insights into Pain Processing in CRPS Type I.

The pathophysiological mechanisms underlying the development of chronic pain in complex regional pain syndrome (CRPS) are diverse and involve both peripheral and central changes in pain processing, such as sensitization of the nociceptive system. The aim of this study was to objectively distinguish the specific changes occurring at both peripheral and central levels in nociceptive processing in individuals with chronic CRPS type I. Nineteen individuals with chronic CRPS type I and 16 age- and sex-matched healthy controls (HC) were recruited. All individuals underwent a clinical examination and pain assessment in the most painful limb, the contralateral limb, and a pain-free control area to distinguish between peripheral and central mechanisms. Contact-heat evoked potentials (CHEPs) were recorded after heat stimulation of the three different areas and amplitudes and latencies were analyzed. Additionally, quantitative sensory testing (QST) was performed in all three areas. Compared to HC, CHEP amplitudes in CRPS were only increased after stimulation of the painful area (p=0.025), while no increases were observed for the pain-free control area (p=0.14). None of the CHEP latencies were different between the two cohorts (all p>0.23). Furthermore, individuals with CRPS showed higher pain ratings after stimulation of the painful limb compared to their contralateral limb (p=0.013). Lastly, compared to HC, mechanical (p=0.012) and thermal (p=0.046) sensitivity was higher in the painful area of the CRPS cohort. This study provides neurophysiological evidence supporting an intact thermo-nociceptive pathway with signs of peripheral sensitization, such as hyperexcitable primary afferent nociceptors, in individuals with CRPS type I. This is further supported by the observation of mechanical and thermal gain of sensation only in the painful limb. Additionally, the increased CHEP amplitudes might be related to fear-induced alterations of nociceptive processing.

Utility of Ultrasound-Guided Continuous Lower Limb Perineural Catheters for Treatment of Pediatric Complex Regional Pain Syndrome Type I: A Primary Case Report from India

Abstract Complex regional pain syndrome (CRPS) is a chronic localized pain condition affecting all age groups with more propensity toward adolescent girls. This disease has a multifactorial pathophysiology involving the sympathetic and central nervous system, along with psychosocial, immune, and genetic factors. We reported a case of CRPS Type I in an adolescent boy who was successfully managed by continuous lower limb nerve blocks through perineural catheters along with the continuation of pharmacological management.

Incidence and Prevalence of Pain Medication Prescriptions in Pathologies with a Potential for Chronic Pain.

Heightened risks of dependence, addiction, anxiolytic effects, or prescription overdose death due to long-term use of pain medication have increased awareness about extended pain medication use in chronic pain populations. The goal of this study was to evaluate the incidence and prevalence of pain medication prescriptions from 2012 to 2022 in common pathologies with a potential for chronic pain. A retrospective cohort study was conducted using electronic health records from TriNetX (Cambridge, Massachusetts) Global Collaborative Network. For 10 distinct cohorts (total n = 9,357,584 patients), pain medication prescriptions were extracted for five classes, namely nonsteroidal anti-inflammatory drug (NSAIDs) and acetaminophen, opioids, gabapentinoids, neuropathic mood agents, and muscle relaxants. Annual incidence and prevalence of each class of medication were evaluated for the past 11 yr. From 2012 to 2022, there was a significant increase in prescriptions of NSAIDs, except for patients with fibromyalgia, and persistent spinal pain syndrome (PSPS) type 2. Interestingly, over time, prescriptions of opioids in patients with complex regional pain syndrome, endometriosis, osteoarthritis, and PSPS type 2 increased, as did prescriptions of muscle relaxants for all cohorts except those with fibromyalgia. Incidence of prescriptions of neuropathic mood agents is high for patients with complex regional pain syndrome (both types) and PSPS type 2. Only for benzodiazepines did there seem to be a decline over the years, with a significantly decreased time trend in patients with complex regional pain syndrome type 1, fibromyalgia, and PSPS type 2. During the last 11 yr, an increase in incidence of NSAIDs and acetaminophen, opioids, neuropathic agents, and muscle relaxants was observed. Only prescriptions of benzodiazepines significantly decreased over time in specific cohorts. Overall, patients with PSPS type 2 and complex regional pain syndrome (both types) consume a broad variety of pain medication classes.

Subtypes of complex regional pain syndrome-a systematic review of the literature.

To systematically identify and summarize possible subtypes of complex regional pain syndrome (CRPS), we searched MEDLINE, Embase, Cochrane, Scopus, and Web of Science for original studies reporting or investigating at least one subtype within a group of patients with CRPS. The search retrieved 4239 potentially relevant references. Twenty-five studies met our inclusion criteria and were included in the analysis. Complex regional pain syndrome phenotypes were investigated based on the following variables: clinical presentation/sensory disturbances, dystonia, skin temperature, disease duration, onset type, CRPS outcome, and neuropsychological test performance. Support was found for the following CRPS subtypes: CRPS type I, CRPS type II, acute CRPS, chronic CRPS, centralized CRPS, cold CRPS, warm CRPS, inflammatory CRPS, dystonic CRPS, nondystonic CRPS, familial CRPS, and nonfamilial CRPS. It is unclear whether these are distinct or overlapping subtypes. The results of this comprehensive review can facilitate the formulation of well-defined CRPS subtypes based on presumed underlying mechanisms. Our findings provide a foundation for establishing and defining clinically meaningful CRPS subtypes, with the ultimate goal of developing targeted and enhanced treatments for CRPS.

Alpha-lipoic acid reduces nociception by reducing oxidative stress and neuroinflammation in a model of complex regional pain syndrome type I in mice

Complex regional pain syndrome type I (CRPS-I) is a disabling pain condition without adequate treatment. Chronic post-ischemia pain injury (CPIP) is a model of CRPS-I that causes allodynia, spontaneous pain, inflammation, vascular injury, and oxidative stress formation. Antioxidants, such as alpha lipoic acid (ALA), have shown a therapeutic potential for CRPS-I pain control. Thus, we aim to evaluate if ALA repeated treatment modulates neuroinflammation in a model of CRPS-I in mice. We used male C57BL/6 mice to induce the CPIP model (O-ring torniquet for 2 h in the hindlimb). For the treatment with ALA or vehicle (Veh) mice were randomly separated in four groups and received 100 mg/kg orally once daily for 15 days (CPIP-ALA, CPIP-Veh, Control-ALA, and Control-Veh). We evaluated different behavioral tests including von Frey (mechanical stimulus), acetone (cold thermal stimulus), rotarod, open field, hind paw edema determination, and nest-building (spontaneous pain behavior). Also, hydrogen peroxide (H2O2) levels, NADPH oxidase and superoxide dismutase (SOD) activity in the sciatic nerve and spinal cord, and Iba1, Nrf2, and Gfap in spinal cord were evaluated at 16 days after CPIP or sham induction. Repeated ALA treatment reduced CPIP-induced mechanical and cold allodynia and restored nest-building capacity without causing locomotor or body weight alteration. ALA treatment reduced SOD and NADPH oxidase activity, and H2O2 production in the spinal cord and sciatic nerve. CPIP-induced neuroinflammation in the spinal cord was associated with astrocyte activation and elevated Nfr2, which were reduced by ALA. ALA repeated treatment prevents nociception by reducing oxidative stress and neuroinflammation in a model of CRPS-I in mice.

Complex Regional Pain Syndrome Type I: Evidence for the CB1 and CB2 Receptors Immunocontent and Beneficial Effect of Local Administration of Cannabidiol in Mice.

Introduction: Complex regional pain syndrome type I (CRPS-I) is a debilitating neuropathic painful condition associated with allodynia, hyperalgesia, sudomotor and/or vasomotor dysfunctions, turning investigation of its pathophysiology and new therapeutic strategies into an essential topic. We aim to investigate the impact of ischemia/reperfusion injury on the immunocontent of CB1 and CB2 cannabinoid receptor isoforms in the paws of mice submitted to a chronic postischemia pain (CPIP) model and the effects of local administration of cannabidiol (CBD) on mechanical hyperalgesia. Methods: Female Swiss mice, 30-35 g, were submitted to the CPIP model on the right hind paw. Skin and muscle samples were removed at different periods for western blot analysis. Results: No changes in the immunocontent of CB1 and CB2 receptors in paw muscle tissues after ischemia-reperfusion were observed. CBD promoted an antihyperalgesic effect in both phases. AM281 reversed the effect of CBD, whereas ruthenium red abolished the late phase. Conclusion: Our results point to the possible beneficial effects of local administration of CBD in modulating CRPS-I in humans. As possible targets for CBD antihyperalgesia in this model, the contribution of cannabinoid receptor CB1, in addition to TRPM8 is suggested.

Somatotopic disruption of the functional connectivity of the primary sensorimotor cortex in complex regional pain syndrome type 1.

In complex regional pain syndrome (CRPS), the representation area of the affected limb in the primary sensorimotor cortex (SM1) reacts abnormally during sensory stimulation and motor actions. We recorded 3T functional magnetic resonance imaging resting-state data from 17 upper-limb CRPS type 1 patients and 19 healthy control subjects to identify alterations of patients' SM1 function during spontaneous pain and to find out how the spatial distribution of these alterations were related to peripheral symptoms. Seed-based correlations and independent component analyses indicated that patients' upper-limb SM1 representation areas display (i) reduced interhemispheric connectivity, associated with the combined effect of intensity and spatial extent of limb pain, (ii) increased connectivity with the right anterior insula that positively correlated with the duration of CRPS, (iii) increased connectivity with periaqueductal gray matter, and (iv) disengagement from the other parts of the SM1 network. These findings, now reported for the first time in CRPS, parallel the alterations found in patients suffering from other chronic pain conditions or from limb denervation; they also agree with findings in healthy persons who are exposed to experimental pain or have used their limbs asymmetrically. Our results suggest that CRPS is associated with a sustained and somatotopically specific alteration of SM1 function, that has correspondence to the spatial distribution of the peripheral manifestations and to the duration of the syndrome.

Evidence of a genetic background predisposing to complex regional pain syndrome type 1

BackgroundComplex regional pain syndrome type 1 (CRPS-1) is a rare, disabling and sometimes chronic disorder usually arising after a trauma. This exploratory study examined whether patients with chronic CRPS-1 have...

Clinical Application of Ultrasound Elastic Imaging in Assessing Poststroke Complex Regional Pain Syndrome (CRPS).

This study aimed to explore the characteristics and clinical application of ultrasonic elastography in peripheral soft tissue in patients with poststroke complex regional pain syndrome (CRPS). Complex regional pain syndrome (CRPS) type I is also known as shoulder hand syndrome (SHS). Its main symptoms include shoulder pain, limited activity, upper arm, wrist, and knuckle joint pain. Ultrasonic elastic imaging technology is gradually being applied to musculoskeletal system evaluation, primarily for the elastic examination of superficial tissue, as a result of the continual advancements in ultrasound technology. To make up for the absence of conventional imaging, functional state evaluation of the motor system can offer conventional ultrasonic tissue elasticity and hardness data. The purpose of this study was to objectively quantify the soft tissue surrounding the shoulder joint of stroke patients with CRPS using ultrasonic elastic imaging and to determine the diagnostic usefulness of ultrasonic elastic imaging for CRPS in stroke in order to promote its usage in clinical practice. Patients diagnosed with CRPS following a stroke and admitted to the rehabilitation unit at Shanxi Bethune Hospital between January, 2021 to June, 2021 were included in the analysis. The control group consisted of people without pain in their shoulder joints. Each group consisted of 30 patients. A high-frequency wire array probe (frequency = 8-16 Hz) was employed in conjunction with an ultrasonic diagnostic apparatus. A quantitative analytic system determined Young's modulus of the tissue, while the tracking of the shear wave provided an elastin map in real-time. An excitation pulse of acoustic radiation force was used to cause shear waves in the tissue. The Young's modulus of supraspinatus muscle in the study and control groups was 289.16±22.07 Kpa and 231.99±23.61 Kpa, respectively (P <0.01). Young's modulus values of the study group's subscapular biceps were compared to those of the control group (P > 0.05). The supraspinatus shear wave elastographic (SWE) imaging value was 10.01±0.49 m/s in the study group and 7.92±0.50 in the control group (P <0.05). The study and control groups had subscapular muscle SWE values of 15.99±1.95 and 8.64±0.56 m/s, respectively (P <0.05). The average biceps tendon SWE value in the study and control groups was 6.39±0.42 and 4.69±0.36 m/s, respectively (P <0.05). In conclusion, the SWE assessed by ultrasound elastography is useful for early diagnosis and evaluation of the superior shoulder tendon, subscapular tendon, and biceps tendon of CRPS following stroke.

A retrospective review of the management and outcomes of patients diagnosed with complex regional pain syndrome type II using electrodiagnostic findings

ABSTRACT Objectives The objective of this study was to assess the outcomes of the use of electrodiagnosis in the diagnosis and management of discrete nerve injuries in patients with complex regional pain syndrome (CRPS). Design This study is a secondary retrospective cohort analysis of patients diagnosed with CRPS from a single outpatient physical medicine and rehabilitation clinic and included all patients who had abnormal electrodiagnostic findings, in addition to CRPS. Results Sixty patients of 248 diagnosed with CRPS underwent electrodiagnosis, 41 of whom had abnormal electrodiagnostic findings indicating a discrete nerve injury. Only 51% of the 41 referrals had indicated the suspicion of a nerve injury. Nearly all patients had undergone physiotherapy. Forty-one percent responded to treatment with oral prednisone alone, 54% had a functional improvement after a combination of treatments including corticosteroids, and 5% improved with treatments that did not involve corticosteroids. Surgical interventions for nerve injuries were required for 34% of patients in the cohort. All surgeries involved the median or ulnar nerve, with the exception of one fibular nerve. After treatment, 39 of 41 patients had functional recoveries or better. Conclusions Electrodiagnosis can inform diagnosis of nerve injury and direct intervention including the need for surgical intervention. Electrodiagnosis should be considered for patients with initial signs of concomitant discrete nerve injury or with CRPS who are not responding to treatments because a nerve injury may be underlying. What is Known Complex Regional Pain Syndrome (CRPS) is a poorly understood pain condition. CRPS has been divided into two subtypes, the second subtype involves a discrete nerve injury with pain that extends beyond the territory of the nerve injury. What is New We observed that nerve injuries that may require surgical intervention are diagnosed just over half of the time upon initial assessment in patients with suspected CRPS. We observed that nerve injuries frequently required specifically directed interventions in place of or in conjunction with CRPS treatments. We suggest that electrodiagnosis is an important part of the triage protocol for CRPS II to reveal discrete nerve injuries that may be hidden. We recommend that electrodiagnosis be considered for patients with initial signs of concomitant discrete nerve injury or for CRPS patients who do not improve with medical therapies.

Causalgia: a review of nerve resection, amputation, immunotherapy and amputated limb CRPS II pathology.

Causalgia and complex regional pain syndrome (CRPS) type II with nerve injury can be difficult to treat. Surgical peripheral nerve denervation for causalgia has been largely abandoned by pain clinicians because of a perception that this may aggravate a central component (anesthesia dolorosa). We selectively searched Pubmed, Cochrane, MEDLINE, EMBASE, CINAHL Plus, and Scopus from 1947 for articles, books, and book chapters for evidence of surgical treatments (nerve resection and amputation) and treatment related to autoimmunity and immune deficiency with CRPS. Reviews were found for the treatment of causalgia or CRPS type II (n = 6), causalgia relieved by nerve resection (n = 6), and causalgia and CRPS II treated by amputation (n = 8). Twelve reports were found of autoimmunity with CRPS, one paper of these on associated immune deficiency and autoimmunity, and two were chosen for discussion regarding treatment with immunoglobulin and one by plasma exchange. We document a report of a detailed and unique pathological examination of a CRPS type II affected amputated limb and related successful treatment with immunoglobulin. Nerve resection, with grafting, and relocation may relieve uncomplicated causalgia and CRPS type II in some patients in the long term. However, an unrecognized and treatable immunological condition may underly some CRPS II cases and can lead to the ultimate failure of surgical treatments.