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.

INTRODUCTION: This 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 debilitating complex regional pain syndrome type I (CRPS-I) or brachial plexus injury (BPI). It is important to gain insight into these outcomes, so we can properly inform and select patients eligible for amputation. METHODS AND ANALYSIS: Joanna Briggs Institute methodology for scoping reviews, Systematic Reviews and Meta-Analyses Scoping Reviews guidelines and Arksey and O'Malley's framework will be used. Studies regarding adult patients with either BPI or CRPS-I who underwent ULA will be considered for inclusion. Studies should include one or more of the following topics: QoL, functioning or participation and should be written in English, German or Dutch. Searches will be conducted in the Cochrane database, PubMed, EMBASE and Google Scholar. Search strings will be provided by a licenced librarian. All relevant literatures will be considered for inclusion, regardless of published date, in order to give a full scope of available literature. Studies will be selected first by title, then abstract and finally by full article by two reviewers who will discuss after every round. A third reviewer will make final decisions to reach consensus if needed. Data will be presented as brief summaries and in tables using a modified data extraction table. ETHICS AND DISSEMINATION: No ethical approval is required since no original data will be collected. Results will be disseminated through publication in a peer-reviewed journal and presentations at (inter)national conferences.

Generalization of exposure in vivo in Complex Regional Pain Syndrome type I.

Exposure in vivo has been found successful in reducing pain-related fear, disability, and experienced pain in chronic pain patients. Despite the success of exposure treatment, experimental studies show that extinction learning is fragile, raising doubts whether extinction of pain-related fear generalizes to new threatening activities after treatment. This study examined whether a particular exposure treatment, in which patients are exposed to a variety of activities (Multiple Exposure condition), promotes generalization of extinction to new threatening situations, compared to an exposure treatment in which subjects are repeatedly exposed to the same set of activities (Repeated Exposure condition). Generalization tests were combined with randomized replicated single case experimental designs (N = 8). Included were patients with Complex Regional Pain Syndrome type I reporting elevated levels of pain-related fear. The Multiple Exposure treatment condition consisted of at least 15 activities to which patients were exposed once. The Repeated Exposure treatment condition exposed patients to only three activities during five sessions each. Generalization was tested by exposing patients to new fearful activities post-treatment and 6-months follow-up. Patients from both conditions performed equally well at both generalization tests. Daily measures showed that the Multiple Exposure condition is preferred to reduce fear of movement/(re)injury, pain catastrophizing and pain experience.

Clinical Features of Pediatric Complex Regional Pain Syndrome: A 5-Year Retrospective Chart Review.

OBJECTIVES: Complex regional pain syndrome (CRPS) is a painful condition of a limb characterized by a constellation of symptoms. Little is known about the clinical features of pediatric CRPS, with fewer than a dozen studies published to date. The aim of this study was to explore the clinical course of pediatric CRPS, with emphasis on clinical features and disease outcomes. A secondary aim was to discern differences in clinical features of pediatric CRPS with and without related movement disorders, and between children who had a favorable and unfavorable outcome. MATERIALS AND METHODS: We carried out a retrospective chart review of children with CRPS who presented to a pediatric Chronic Pain Clinic in Canada over a 5-year period (2012 to 2016). RESULTS: The study identified 59 children with CRPS (mean age: 12.7±2.5; 74.6% female; 72.9% lower extremity). In total, 87% (n=48) of children experienced complete resolution or significant improvement of CRPS, with a relapse rate of 15%. Overall, 25% (n=15) had a CRPS-related movement disorder. There were no differences in the clinical features of pediatric CRPS with or without related movement disorders. Children who experienced a favorable outcome had a significantly shorter symptom duration at the initial visit in comparison with children who experienced an unfavorable outcome. DISCUSSION: In this cohort, pediatric CRPS was most common in girls around the age of 12, usually in the lower extremity, and most experienced a favorable outcome. Further research is needed to better understand the prognosis and relapse rate of pediatric CRPS.

Prognostic Factors in Complex Regional Pain Syndrome Type 1 Occurring in the Korean Army.

OBJECTIVES: In Korea, patients diagnosed with complex regional pain syndrome (CRPS) in the army are typically discharged from the army; however, the course of the disease after discharge is not known. The purpose of this study was to investigate the course of CRPS that occurred in the army and to identify the influences of various clinical and psychosocial factors immediately before discharge on the disease course. METHODS: Clinical profiles and psychosocial status were collected from the medical records of 16 patients with CRPS type 1 who were discharged during the period between March 2017 and April 2018. The degree of improvement after discharge was assessed by follow-up evaluation through telephone contact. Cox proportional hazards regression was performed to identify clinical and psychosocial predictors for the rate of recovery. RESULTS: The median time to recovery after discharge was 39 weeks (95% confidence interval [CI] = 8.8-69.2 weeks). The sole predictor for time to recovery after discharge was the time period from the onset of pain to discharge. Each one-week increase in the duration of pain experienced in the military was associated with a 18.2% (95% CI = 5.3%-29.5%) reduction in the rate of recovery after discharge (P = 0.007). CONCLUSIONS: Patients who experienced a short period of pain in the military demonstrated a relatively good prognosis after discharge. This may show how prolonged pain in the army could affect the experience of pain from a social point of view, in that it shows the disease course after a change in the social environment.

Investigate the Effect of Psychological Factors in Development of Complex Regional Pain Syndrome Type I in Patients with Fracture of the Distal Radius: A Prospective Study.

BACKGROUND: To investigate the role of psychological factors (anxiety and depression) and pain measured on a visual analogue scale (VAS) in the development of complex regional pain syndrome type I (CRPS I) following the distal radius fracture (DRF). METHODS: A consecutive sample of patients (N=60) with a distal radius fracture was measured for signs of CRPS by Budapest criteria weekly till 8 weeks and then another follow-up one year after injury to determine the incidence and predictors of developing CRPS I in a prospective cohort study and also to discover whether there is difference between pain, depression, and anxiety level in the patients with and without CRPS I. The most of the patients were treated by Pin stabilization. The Beck depression inventoryshort form (BDI), State-Trait Anxiety Inventory, and Numerical pain rating scale were used to determine the patients' psychological features and pain intensity at the base line and 8 weeks after the fracture. RESULTS: CRPS I developed in 15 (25%) patients after eight weeks and just last in 10 (16.67%) patients after one year. No difference was found between the two groups (CRPS and non-CRPS group) in terms of state (STAI-I) and trait (STAI-II) anxiety, and BDI score. Pain at the base line was the most important risk factor in developing CRPS (odds ratio [OR] =1.52; 95% CI). CONCLUSIONS: After fracturing the distal radius, patients who have high pain intensity have a higher risk of developing CRPS I. To following these patients closely for the development of CRPS I may be advantageous for early preventative and therapeutic interventions.

Complex regional pain syndrome type 1 in the medico-legal setting: High rates of somatoform disorders, opiate use and diagnostic uncertainty.

Objective The aim of this study was to review demographic and clinical characteristics of patients with complex regional pain syndrome type 1 (CRPS) seen in a UK medico-legal setting - particularly the relationship between CRPS and somatoform disorders. Methods Fifty consecutive cases of CRPS (interviewed 2005-2016) undergoing psychiatric assessment were reviewed. A systematic assessment of mental states was conducted via interview and examination of medical/psychiatric records. Thirty patients also completed the Brief Illness Perception Questionnaire (BIPQ). Results Sixty per cent of patients ( n = 30) were female, and the mean age was 43 years. Twenty-two per cent ( n = 11) were employed, and 60% ( n = 30) received disability benefits. Symptoms were reported in the upper limb (62%; n = 31), lower limb (30%; n = 15), both (6%; n = 3) or elsewhere (2%; n = 1). Eighty-four per cent ( n = 42) satisfied DSM-5 criteria for current somatoform disorder. A history of more than two pain-related functional somatic syndromes (e.g. non-cardiac chest pain) was found in 42% ( n = 21) and functional neurological symptoms (e.g. 'claw-hand') in 42% ( n = 21). BIPQ scores resembled those associated with somatoform disorders and disorders mediated by psychological factors (e.g. irritable bowel syndrome). In 38% ( n = 19), the CRPS diagnosis was disputed among experts. A history of depression was noted in 60% ( n = 30), panic attacks in 20% ( n = 10) and alcohol/substance misuse in 18% ( n = 9). Opiates were prescribed to 64% ( n = 32). Conclusions Patients diagnosed with CRPS involved in litigation have high rates of prior psychopathology (mainly somatoform disorders) and pain-related disability for which opiate use is common. They risk an adverse reaction to limb pain 'shaped' by maladaptive illness beliefs. The CRPS diagnosis lacks reliability in medico-legal settings and may cause iatrogenic harm.

Graded motor imagery for women at risk for developing type I CRPS following closed treatment of distal radius fractures: a randomized comparative effectiveness trial protocol.

BACKGROUND: Distal radius fractures (DRF) account for nearly one-fifth of all fractures in older adults, and women experience them 5× as often as men. Most DRF occur with low impact injuries to the wrist with an outstretched hand, and are often managed via closed treatment and cast immobilization. Women sustaining a DRF are at risk for upper limb immobility, sensorimotor changes, edema and type I complex regional pain syndrome (CRPS). Since CRPS onset is likely influenced by alterations in the brain's somatosensory region, a rehabilitation intervention, Graded Motor Imagery (GMI), aims to restore cortical representation, including sensory and motor function, of the affected limb. To date, there are no studies on the use of GMI in reducing risk of or preventing the onset of type I CRPS in women with DRF treated with cast immobilization. Due to a higher likelihood of women with this injury developing type I CRPS, it is important to early intervention is needed. METHODS/DESIGN: This article describes a six-week randomized comparative effectiveness trial, where the outcomes of a modified GMI program (mGMI) + standard of care (SOC) group (n = 33) are compared to a SOC only control group (n = 33). Immediately following cast immobilization, both groups participate in four 1-h clinic-based sessions, and a home program for 10 min three times daily until cast removal. Blinded assessments occur within 1 week of cast immobilization (baseline), at three weeks post cast immbolization, cast removal, and at three months post cast removal. The primary outcomes are patient reported wrist/hand function and symptomology on the Patient Rated Wristand Hand Evaluation, McGill Pain Questionnaire, and Budapest CRPS Criteria. The secondary outcomes are grip strength, active range of motion as per goniometry, circumferential edema measurements, and joint position sense. DISCUSSION: This study will investigate the early effects of mGMI + SOC hand therapy compared to SOC alone. We intend to investigate whether an intervention, specifically mGMI, used to treat preexisiting pain and motor dysfunction might also be used to mitigate these problems prior to their onset. If positive effects are observed, mGMI + SOC may be considered for incorporation into early rehabilitation program. TRIAL REGISTRATION: This trial is registered at ClinicalTrials.gov with identifier NCT02957240 (Approval date: April 20, 2017).

Approach to the Patient with Disproportionate Pain.

Faced with a patient who presents with unexplained disproportionate pain, a surgeon may be tempted to diagnose a low pain threshold, malingering, poor coping, anxiety, or other emotional condition. However, a variety of conditions must be ruled out before the orthopedist can prescribe watchful waiting. Computed tomography and magnetic resonance imaging can detect occult fractures, acute spinal conditions or vascular occlusions, but early on are inadequate to diagnose a compartment syndrome, necrotizing fasciitis, or reflex sympathetic dystrophy (RSD). These diagnoses underpin a pain-out-of-proportion situation whereby the patient presents with disproportionate pain following a sometimes minor trauma with normal imaging studies and otherwise nonspecific presentations. Though these conditions are well described in the literature, investigations of malpractice data reveal a non-negligible prevalence of missed diagnoses for each of these entities. Determining that a patient exhibits otherwise unexplained pain-out-of-proportion situation is the first step in making a timely diagnosis.

Mental load during cognitive performance in complex regional pain syndrome I.

BACKGROUND: Complex regional pain syndrome (CRPS) is associated with deficits in limb recognition. The purpose of our study was to determine whether mental load during this task affected performance, sympathetic nervous system activity or pain in CRPS patients. METHODS: We investigated twenty CRPS-I patients with pain in the upper extremity and twenty age- and sex-matched healthy controls. Each participant completed a limb recognition task. To experimentally manipulate mental load, the presentation time for each picture varied from 2 s (greatest mental load), 4, 6 to 10 s (least mental load). Before and after each run, pain intensity was assessed. Skin conductance was recorded continuously. RESULTS: Patients with CRPS did not differ from controls in terms of limb recognition and skin conductance reactivity. However, patients with CRPS reported an increase in pain during the task, particularly during high mental load and during the latter stages of the task. Interestingly, state anxiety and depressive symptoms were also associated with increases in pain intensity during high mental load. CONCLUSIONS: These findings indicate that high mental load intensifies pain in CRPS. The increase of pain in association with anxiety and depression indicates a detrimental effect of negative affective states in situations of high stress and mental load in CRPS. SIGNIFICANCE: The effects of mental load need to be considered when patients with CRPS-I are investigated for diagnostic or therapeutic reasons.

Complex regional pain syndrome associated with hyperattention rather than neglect for the healthy side: A comprehensive case study.

Complex regional pain syndrome (CRPS) is a dehabilitating chronic condition occurring with peripheral lesions. There is growing consensus for a central contribution to CRPS. Although the nature of this central body representation disorder is increasingly debated, it has been repeatedly argued that CRPS results in motor neglect of the affected side. The present article describes a comprehensive and quantitative case report demonstrating that: (1) not all patients with chronic CRPS exhibit decreased spatial attention for the affected side and (2) patients may actually exhibit a substantial, broad and reliable attentional bias toward the painful side, akin to spatial neglect for the healthy side. This unexpected result agrees with the idea that patients can be hyper-attentive toward their pathological side as a manifestation of lowered pain threshold, allodynia and kinesiophobia.

Factors Associated With Disability and Sick Leave in Early Complex Regional Pain Syndrome Type-1.

OBJECTIVE: Factors influencing disability and work absence in complex regional pain syndrome type-1 (CRPS)-1 have not been thoroughly described in the literature. We sought to determine whether demographic variables, work-related factors, CRPS clinical severity ratings, pain scores, or psychological variables were associated with disability and sick leave in early CRPS-1. METHODS: A total of 66 CRPS-1 patients were recruited within 12 weeks of CRPS onset. Patients completed measures of pain, depression, anxiety, stress, pain catastrophizing, and pain-related fear. A physical examination was conducted to assess signs and symptoms of CRPS and to calculate a CRPS severity score. Demographic details, clinical details, treatments, work type, and work status were recorded. RESULTS: In multivariate analyses, the following factors were associated with greater disability: higher pain scores, more restricted ankle or wrist extension, and higher levels of depression. Among the 49 who were either working or studying before developing CRPS, 28 had stopped work or study at the time of assessment. Multivariate analyses showed that sick leave was more likely among those whose CRPS was triggered by more severe injuries, whose work was more physically demanding, among those with higher disability scores, and there was also a significant effect of depression on sick leave, which was mediated by disability. DISCUSSION: Although the study was cross-sectional and so cannot differentiate cause from effect, results suggest that even in the early stages of CRPS, a cycle of pain, disability, depression, and work absence can emerge. Treatments aimed to prevent this cycle may help prevent adverse long-term outcomes.

Do psychological factors influence recovery from complex regional pain syndrome type 1? A prospective study.

Previous studies have shown that the outcomes of complex regional pain syndrome (CRPS) vary significantly between patients, but few studies have identified prognostic indicators. The aim of this study was to determine whether psychological factors are associated with recovery from recently onset CRPS amongst patients followed prospectively for 1 year. Sixty-six patients with CRPS (type 1) were recruited within 12 weeks of symptom onset and assessed immediately and at 6 and 12 months, during which time they received treatment as usual. At each assessment, the following were measured: signs and symptoms of CRPS, pain, disability, depression, anxiety, stress, pain-related fear, pain catastrophising, laterality task performance, body perception disturbance, and perceived ownership of the limb. Mixed-effects models for repeated measures were conducted to identify baseline variables associated with CRPS severity, pain, and disability over the 12 months. Results showed that scores for all 3 outcome variables improved over the study period. Males and those with lower levels of baseline pain and disability experienced the lowest CRPS severity scores over 12 months. Those with lower baseline anxiety and disability had the lowest pain intensity over the study period, and those with lower baseline pain and pain-related fear experienced the least disability over the 12 months. This suggests that anxiety, pain-related fear, and disability are associated with poorer outcomes in CRPS and could be considered as target variables for early treatment. The findings support the theory that CRPS represents an aberrant protective response to perceived threat of tissue injury.

Thoracic sympathetic block for the treatment of complex regional pain syndrome type I: a double-blind randomized controlled study.

Pain relief in complex regional pain syndrome (CRPS) remains a major challenge, in part due to the lack of evidence-based treatment trials specific for this condition. We performed a long-term randomized, double-blinded active-control study to evaluate the efficacy of thoracic sympathetic block (TSB) for upper limb type I CRPS. The study objective was to evaluate the analgesic effect of TSB in CRPS. Patients with CRPS type I were treated with standardized pharmacological and physical therapy and were randomized to either TSB or control procedure as an add-on treatment. Clinical data, pain intensity, and interference (Brief Pain Inventory), pain dimensions (McGill Pain Questionnaire [MPQ]), neuropathic characteristics (Neuropathic Pain Symptom Inventory [NPSI]), mood, upper limb function (Disabilities of Arm, Shoulder and Hand), and quality of life were assessed before, and at 1 month and 12 months after the procedure. Thirty-six patients (19 female, 44.7 ± 11.1 years of age) underwent the procedure (17 in the TSB group). Average pain intensity at 1 month was not significantly different after TSB (3.5 ± 3.2) compared to control procedure (4.8 ± 2.7; P=0.249). At 12 months, however, the average pain item was significantly lower in the TSB group (3.47 ± 3.5) compared to the control group (5.86 ± 2.9; P=0.046). Scores from the MPQ, evoked-pain symptoms subscores (NPSI), and depression scores (Hospital Anxiety and Depression Scale) were significantly lower in the TSB group compared to the control group at 1 and at 12 months. Other measurements were not influenced by the treatment. Quality of life was only slightly improved by TSB. No major adverse events occurred. Larger, multicentric trials should be performed to confirm these original findings.

Health-related quality of life in 975 patients with complex regional pain syndrome type 1.

There are limited data available on health-related quality of life (QoL) in patients with complex regional pain syndrome (CRPS). In the present study we examined QoL in 975 CRPS patients attending 6 different clinics in the Netherlands. QoL was assessed using the MOS 36-Item Short-Form Health Survey (SF-36) with the Mental Health Summary Score (MHS) and the Physical Health Summary Score (PHS) as dependent variables. The influences of gender, type of affected limb, disease duration, pain scores, CRPS severity and set of diagnostic criteria used were investigated. We found the lowest scores of QoL in the physical domains of the SF-36, with lower-limb CRPS patients reporting poorer results than patients with an affected upper limb. Influence of gender on QoL was not observed, and correlations of QoL with disease duration and the CRPS severity score were weak. Pain correlated moderately with QoL. In addition, patients fulfilling stricter diagnostic criteria (ie, the Budapest criteria) had lower QoL scores than patients fulfilling less strict criteria (ie, the Orlando criteria). We conclude that loss of QoL in CRPS patients is due mainly to reduced physical health. A comparison with data available from the literature shows that CRPS patients generally report poorer QoL than patients with other chronic pain conditions, particularly in the physical domains. Pain correlated moderately with QoL and therefore deserves ongoing attention by physicians. Finally, patients meeting the diagnostic Budapest criteria have lower QoL scores than patients meeting the Orlando criteria, highlighting the impact of different sets of criteria on population characteristics.

Multimodal physical therapy management of a 48-year-old female with post-stroke complex regional pain syndrome.

This case report describes a 48-year-old female who presented with complaints of right shoulder pain, hyperesthesias and swelling of the hand along with added symptoms of pain centralization following a cerebrovascular accident. On clinical evaluation, the patient satisfied the Budapest diagnostic criteria for Complex Regional Pain Syndrome (CRPS) type-1. Physical therapy management (1st three sessions) was initially focused on pain neurophysiology education with an aim to reduce kinesiophobia and reconceptualise her pain perception. The patient had an immediate significant improvement in her pain and functional status. Following this, pain modulation in the form of transcutaneous electrical nerve stimulation, kinesio tape application, "pain exposure" physical therapy and exercise therapy was carried out for a period of 7 weeks. The patient had complete resolution of her symptoms which was maintained at a six-month follow-up.

Are pain-related fears mediators for reducing disability and pain in patients with complex regional pain syndrome type 1? An explorative analysis on pain exposure physical therapy.

OBJECTIVE: To investigate whether pain-related fears are mediators for reducing disability and pain in patients with Complex Regional Pain Syndrome type 1 when treating with Pain Exposure Physical Therapy. DESIGN: An explorative secondary analysis of a randomised controlled trial. PARTICIPANTS: Fifty-six patients with Complex Regional Pain Syndrome type 1. INTERVENTIONS: The experimental group received Pain Exposure Physical Therapy in a maximum of five treatment sessions; the control group received conventional treatment following the Dutch multidisciplinary guideline. OUTCOME MEASURES: Levels of disability, pain, and pain-related fears (fear-avoidance beliefs, pain catastrophizing, and kinesiophobia) were measured at baseline and after 3, 6, and 9 months follow-up. RESULTS: The experimental group had a significantly larger decrease in disability of 7.77 points (95% CI 1.09 to 14.45) and in pain of 1.83 points (95% CI 0.44 to 3.23) over nine months than the control group. The potential mediators pain-related fears decreased significantly in both groups, but there were no significant differences between groups, which indicated that there was no mediation. CONCLUSION: The reduction of pain-related fears was comparable in both groups. We found no indication that pain-related fears mediate the larger reduction of disability and pain in patients with Complex Regional Pain Syndrome type 1 treated with Pain Exposure Physical Therapy compared to conventional treatment. TRIAL REGISTRATION: International Clinical Trials Registry NCT00817128.

Algodystrophy in children and adolescents: a review.

Algodystrophy occurs in children significantly less frequently than in adults. Symptomatology, course, responsiveness to treatment and prognosis of the pediatric disease is also different from the "adult" form. This paper presents substantial peculiarities of pediatric algodystrophy: it occurs after relatively minor trauma, involves lower limb more frequently than upper limb and presents with pain, paleness and coldness of the skin in the affected part, as well as with serious functional impairment. Diagnosis of the condition is based on clinical grounds and no imaging is necessary to confirm the diagnosis. Psychological distress is suspected in the development of the disease in children and adolescents, but there is no definitive evidence supporting this view. Treatment of pediatric algodystrophy should be complex and consist of physical therapy, psychotherapy, pain therapy and, in selected cases, sympathetic and somatic blocks. The literature emphasizes very poor knowledge of algodystrophy in the pediatric community.

The rubber hand illusion in complex regional pain syndrome: preserved ability to integrate a rubber hand indicates intact multisensory integration.

In patients with complex regional pain syndrome (CRPS) type 1, processing of static tactile stimuli is impaired, whereas more complex sensory integration functions appear preserved. This study investigated higher order multisensory integration of body-relevant stimuli using the rubber hand illusion in CRPS patients. Subjective self-reports and skin conductance responses to watching the rubber hand being harmed were compared among CRPS patients (N=24), patients with upper limb pain of other origin (N=21, clinical control group), and healthy subjects (N=24). Additionally, the influence of body representation (body plasticity [Trinity Assessment of Body Plasticity], neglect-like severity symptoms), and clinical signs of illusion strength were investigated. For statistical analysis, 1-way analysis of variance, t test, Pearson correlation, with α=0.05 were used. CRPS patients did not differ from healthy subjects and the control group with regard to their illusion strength as assessed by subjective reports or skin conductance response values. Stronger left-sided rubber hand illusions were reported by healthy subjects and left-side-affected CRPS patients. Moreover, for this subgroup, illness duration and illusion strength were negatively correlated. Overall, severity of neglect-like symptoms and clinical signs were not related to illusion strength. However, patients with CRPS of the right hand reported significantly stronger neglect-like symptoms and significantly lower illusion strength of the affected hand than patients with CRPS of the left hand. The weaker illusion of CRPS patients with strong neglect-like symptoms on the affected hand supports the role of top-down processes modulating body ownership. Moreover, the intact ability to perceive illusory ownership confirms the notion that, despite impaired processing of proprioceptive or tactile input, higher order multisensory integration is unaffected in CRPS.