Komplexes regionales Schmerzsyndrom

Complex regional pain syndrome (CRPS) may develop following fractures, limb trauma, or lesions of the peripheral or central nervous system. The clinical picture consists of a triad of symptoms including autonomic, sensory, and motor dysfunction. Diagnosis is based on clinical signs and symptoms according to the Budapest criteria. Therapy is based on an individual and multidisciplinary approach. Distinct methods of physical therapy and pharmacological strategies are the mainstay of therapy. Pharmacotherapy is based on individual symptoms and includes steroids, free radical scavengers, treatment of neuropathic pain, and agents interfering with bone metabolism. In some cases invasive methods may be considered.     

Complex regional pain syndromes

Complex regional pain syndromes (CRPS) (formerly reflex sympathetic dystrophy and causalgia) are neuropathic pain conditions that are initiated by an extremity trauma or peripheral nerve lesion. Clinical definition and scientific understanding of CRPS are still evolving; however, both the clinical picture and therapeutic options are significantly influenced by a dysfunction of the sympathetic nervous system. Recent investigations suggest functional central abnormalities and a peripheral inflammatory component in the pathophysiology of CRPS. Interdisciplinary treatment includes physical, pharmacologic, and invasive interventional therapy, as well as stimulation techniques.     

Komplexes regionales Schmerzsyndrom

Complex regional pain syndrome (CRPS) may develop following fractures, limb trauma, or lesions of the peripheral or central nervous system. The clinical picture consists of a triad of symptoms including autonomic, sensory, and motor dysfunction. Diagnosis is based on clinical signs and symptoms according to the Budapest criteria. Therapy is based on an individual and multidisciplinary approach. Distinct methods of physical therapy and pharmacological strategies are the mainstay of therapy. Pharmacotherapy is based on individual symptoms and includes steroids, free radical scavengers, treatment of neuropathic pain, and agents interfering with bone metabolism. In some cases invasive methods may be considered.     

Ausgeprägte Symptomverschlechterung bei CRPS Typ II nach einmaliger Applikation eines hochprozentigen Capsaicinpflasters

Topical 8 % capsaicin is an established therapeutic option for the treatment of peripheral neuropathic pain. In accordance with the internationally accepted definition, complex regional pain syndrome (CRPS) type II is a form of neuropathic pain so that capsaicin plasters represent a treatment option. However, for the treatment of CRPS it is recommended that painful stimuli should be avoided but capsaicin induces a strong nociceptive stimulation and so its use is at present controversial. We report on the course of such an application in a patient who developed CRPS type II with intractable neuropathic pain after hallux surgery. As a result of a single treatment with capsaicin a pronounced recurrence developed with central nervous symptoms.     

Complex regional pain syndrome: a review of diagnostics, pathophysiologic mechanisms, and treatment implications for certified registered nurse anesthetists

The pathophysiologic mechanisms for complex regional pain syndrome (CRPS) are complex and elusive. The proposed etiologic mechanisms for CRPS include inflammatory responses, peripheral or central sensitization, and sympathetic dysfunction. Anesthesia care of patients with CRPS is challenging. Treatments including physiotherapy, peripheral vasodilators, sympathetic blockade, analgesics, and other systemic medications can help optimize mobility, perfusion, and pain relief for affected patients.     

Despite clinical similarities there are significant differences between acute limb trauma and complex regional pain syndrome I (CRPS I).

In order to analyze the pathophysiology behind the clinical similarity acutely after limb trauma and in acute stages of complex regional pain syndrome (CRPS), 20 patients with external fixation after distal radius fracture (3.5 days after surgery) without signs of CRPS and 24 patients suffering from acute CRPS I (without nerve lesion; duration, 5 weeks) were investigated. Hyperalgesia to heat was tested by a feedback-controlled thermode, and to mechanical stimuli by an impact stimulator. The sympathetic nervous system was examined by measuring skin temperature (infra-red thermography), testing different sympathetic vasoconstrictor reflexes (laser-Doppler flowmetry) and quantitative sudometry after thermal load (thermoregulatory sweat test). We found hyperalgesia to heat after trauma (P<0.001), but not in CRPS, whereas mechanical hyperalgesia was present in both patient groups (trauma: P<0.001; CRPS: P<0.005). Skin temperature was significantly increased on the affected side in both patient groups (acute trauma: P<0.001; CRPS: P<0.005). However, sympathetic failure, as indicated by impairment of sympathetic vasoconstrictor reflexes (P<0.02) and hyperhidrosis (P<0.01), was found exclusively in CRPS patients. Our results indicate that pain and vasomotor disturbances may be generated by different mechanisms acutely after trauma and in acute CRPS. Despite the clinical similarity, additional changes in the peripheral or central nervous system are required for CRPS. In the light of our observations, it seems unlikely that CRPS is a simple exaggeration of post-traumatic inflammation.     

Pathogenesis of complex regional pain syndrome (CRPS)

Neuropathic pain results from injury to neural structures within the peripheral or central nervous systems. Such injury promotes spontaneous and ectopic firing of nerves as well as reorganization of the nervous system. Neuropathic pain persists chronically. Patients who suffer from neuropathic pain exhibit persistent or paroxysmal pain without apparent immediate cause or pain hypersensitivity after tissue damage. This hypersensitivity is manifest as hyperalgesia and allodynia. Complex regional pain syndrome, CRPS is a category of neuropathic pain and is further divided into type I(reflex sympathetic dystrophy: RSD) and type II(causalgia). CRPS is characterized by localized autonomic dysregulation in the affected area with vasomotor and/or sudomotor changes, edema, colour difference, sweating abnormality, and atrophy.     

Complex regional pain syndrome type I associated with amyotrophic lateral sclerosis

BACKGROUND: (CRPS I [formerly called reflex sympathetic dystrophy]) is a syndrome with pain and signs of autonomic dysfunction after trauma or immobilization; the pathophysiologic mechanisms of CRPS I, however, remain unknown. DESIGN: The authors present a case of CRPS I associated with amyotrophic lateral sclerosis. Along with the motor paresis due to amyotrophic lateral sclerosis, pain, swelling, and signs of autonomic disturbance occurred. CONCLUSIONS: This case supports the hypothesis that immobilization is one of the major contributing factors for CRPS I.     

Pharmacologic treatment of complex regional pain syndrome I: a conceptual framework

Pain may be a leading symptom in complex regional pain syndrome type I (CRPS I) and may hinder functional recovery. In this case, a pharmacotherapeutic approach to pain should be part of the individually tailored interdisciplinary treatment regimen. However, operational criteria for determining which patient may profit from what therapeutic intervention are lacking. This article discusses a conceptual framework in which the rapid progress made in basic pain research may contribute to the clinical management of pain in CRPS I. First, recent insights in the pathophysiologic mechanisms underlying CRPS I are reviewed. CRPS I is considered a neuropathic pain syndrome with a mixed and time-dependent profile of a regional inflammation, sensitization of primary somatosensory afferents (peripheral sensitization), and sensitization of spinal neurons (central sensitization). The dominant mechanisms may vary across individual patients with different time profiles. Second, a model was constructed in which signs and symptoms in an individual patient are related to these mechanisms. Finally, relating the clinical picture to the underlying pathophysiology may help determine the pharmacotherapeutic approach for an individual patient. Pharmacologic options are discussed in this context. The presented framework does not aim to provide an evidence-based treatment algorithm, ready to be used in daily clinical practice; rather it offers a crude, first step toward a mechanism-based pharmacotherapy in CRPS I, in an effort to shift from a mainly empirical treatment paradigm toward theory-driven treatment procedures.     

Metallothionein deficiency in the injured peripheral nerves of complex regional pain syndrome as revealed by proteomics

Complex regional pain syndrome (CRPS) is characterized by persistent and severe pain after trauma or surgery; however, its molecular mechanisms in the peripheral nervous system are poorly understood. Using proteomics, we investigated whether injured peripheral nerves of CRPS patients have altered protein profiles compared with control nerves. We obtained nerve samples from 3 patients with CRPS-2 who underwent resection of part of an injured peripheral nerve. Sural nerves from fresh cadavers with no history of trauma or neuropathic pain served as controls. Proteomic analysis showed that the number and functional distribution of proteins expressed in CRPS and control nerves was similar. Interestingly, metallothionein was absent in the injured nerves of CRPS-2, although it was readily detected in control nerves. Western blotting further confirmed the absence of metallothionein in CRPS-2 nerves, and immunohistochemistry corroborated the deficiency of metallothionein expression in injured nerves from 5 of 5 CRPS patients and 2 of 2 patients with painful neuromas. In contrast, all control nerves, including 5 sural nerves from fresh cadavers and 41 nerves obtained from surgically resected tumors, expressed MT. Furthermore, expression of S100 as a marker for Schwann cells, and neurofilament M as a marker of axons was comparable in both CRPS-2 and controls. Metallothioneins are zinc-binding proteins that are probably involved in protection against injury and subsequent regeneration after CNS damage. Their absence from the injured peripheral nerves of patients with CRPS-2 suggests a potential pathogenic role in generating pain in the damaged peripheral nerves.     

Shoulder-hand syndrome after stroke. A complex regional pain syndrome

Complex regional pain syndrome (CRPS) types I and II are neuropathic pain disorders that develop as an exaggerated response to a traumatic lesion or nerve damage, that generally affects the extremities, or as the consequence of a distant process such as a stroke, spinal lesion or myocardial infarction. It rarely appears without an apparent cause. CRPS of upper limbs after stroke is frequently today called shoulder-hand syndrome (SHS). The onset and severity of SHS appears to be related with the aetiology of the stroke, the severity and recovery of motor deficit, spasticity and sensory disturbances. Another important aetiological factor is glenohumeral subluxation. The physiopathology of the disease is still not known. In CRPS, there is an exaggerated inflammatory response and some chemical mediators have been identified and are present in the inflammatory soup around the primary afferent fibres that, through different processes, can induce hyper-excitability of the afferent fibres (peripheral sensitization). It is hypothesized that a localized neurogenic inflammation is at the basis of oedema, vasodilation and hyperhidrosis that are present in the initial phases of CRPS. The repeated discharge of the C fibres causes an increased medullary excitability (central sensitization). Another important factor is the reorganisation of the central nervous system, and in particular this appears to affect the primary somatosensory cortex. The central role of the sympathetic nerve is presently in doubt. However, it is thought that a sub-group of CRPS patients exists in whom a predominant factor is the hyper-activity of the sympathetic nervous system, and that it responds positively to sympathetic block. Diagnosis is clinical and there are no specific tests, nor pathognomic symptoms to identify this disease with certainty. Diagnosis of CRPS after stroke appears more complex than in other pathological situations: the paretic upper arm frequently appears painful, oedematose, with altered heat and tactile sensations and slightly dystrophic skin within a non-use syndrome. Some investigations can aid differential diagnosis with other diseases. Treatment may be non-pharmacological, pharmacological, with psychotherapy, regional anaesthesia, neuromodulation and sympathectomy. In any case there is little evidence that supports the efficacy of the interventions normally used to treat or prevent CRPS-SHS. The key to effective treatment undoubtedly lies in a an expert multidisciplinary team that is co-ordinated and motivated and that treats the disorder with individualised therapy.     

Anesthésie locorégionale et anesthésie locorégionale intraveineuse dans la prise en charge du syndrome douloureux régional complexe (algoneurodystrophie) chez l’adulte

Physiopathology of the complex regional pain syndrome (CRPS) seems to be more due to the vascular and neuronal abnormalities. A maladaptive response of the central, more than peripheral, nervous system seems to be more involved. Proposed diagnostic criteria of CRPS were recently updated. Reviews and meta-analysis recommended physiotherapy as a cornerstone of the treatment. All analgesic procedures that allow rehabilitation must be used. Currently, intravenous regional anesthesia is not recommended; instead, prolonged regional anesthesia seems to be more useful.     

Complex regional pain syndrome and chiropractic

OBJECTIVE: Complex regional pain syndromes (CRPS) represent curious and difficult syndromes for both patient and clinician. CRPS presents as a triad of signs and symptoms, usually after a seemingly trivial injury to a peripheral joint or appendage. The clinical triad includes severe pain, vasomotor changes in and around the affected area, and trophic changes in the affected limb. Many of the acute symptoms are similar to those seen after many acute injuries, which makes an early diagnosis often times difficult. Current treatment protocols revolve around aggressive physical therapy plus pharmacologic interventions aimed at limiting sympathetic nervous system activity. OBJECTIVE: To review the literature on CRPS regarding symptoms, diagnosis, treatment, and causal mechanisms and to discuss alternative treatment approaches and the possible role of chiropractic care in patient rehabilitation. Data Sources: Texts, review articles, and randomized clinical trials investigating treatments, causes, and epidemiology. CONCLUSIONS: Recent research calls into question the predominant theories that view excessive sympathetic nervous system activity as the cause of CRPS. No evidence of an increase in sympathetic nervous system activity has been found, and new theories suggest that an increase in the sensitivity of neurotransmitter receptors may be the cause of CRPS. Alternatively, other research has suggested that a local inflammatory process may in fact cause CRPS. Although no research has been completed examining the role of chiropractic care in the treatment of CRPS, there is reason to believe that spinal manipulation may be beneficial to patients with CRPS.     

Komplexes regionales Schmerzsyndrom bei Nervenwurzelkompression und nach Wirbelsäulenoperation

Complex regional pain syndrome (CRPS) is an extremely painful and partially disabling disease. It often occurs secondary to trauma, but also spontaneously. The emergence of CRPS has been reported following nerve root compression and/or spinal surgery, but its incidence is unknown. In this article, the present knowledge about the incidence of CRPS in the context of nerve root compression and spine surgery is reviewed and therapeutic and diagnostic consequences are discussed.     

Advances in Translational Neuropathic Research: Example of Enantioselective Pharmacokinetic–Pharmacodynamic Modeling of Ketamine-induced Pain Relief in Complex Regional Pain Syndrome

Historically, complex regional pain syndrome (CRPS) was poorly defined, which meant that scientists and clinicians faced much uncertainty in the study, diagnosis, and treatment of the syndrome. The problem could be attributed to a nonspecific diagnostic criteria, unknown pathophysiologic causes, and limited treatment options. The two forms of CRPS still are painful, debilitating disorders whose sufferers carry heavy emotional burdens. Current research has shown that CRPS I and CRPS II are distinctive processes, and the presence or absence of a partial nerve lesion distinguishes them apart. Ketamine has been the focus of various studies involving the treatment of CRPS; however, currently, there is incomplete data from evidence-based studies. The question as to why ketamine is effective in controlling the symptoms of a subset of patients with CRPS and not others remains to be answered. A possible explanation to this phenomenon is pharmacogenetic differences that may exist in different patient populations. This review summarizes important translational work recently published on the treatment of CRPS using ketamine.     

Autoantibodies in complex regional pain syndrome bind to a differentiation-dependent neuronal surface autoantigen

Complex regional pain syndrome, which is characterised by pain and trophic disturbances, develops frequently after peripheral limb trauma. There is an increasing evidence of an involvement of the immune system in CRPS, and recently we showed that CRPS patients have autoantibodies against nervous system structures [6]. Therefore we tested the sera of CRPS patients, neuropathy patients and healthy volunteers for surface-binding autoantibodies to primary cultures of autonomic neurons and differentiated neuroblastoma cell lines using flow cytometry. Thirteen of 30 CRPS patients, but none of 30 healthy controls and only one of the 20 neuropathy sera had specific surface binding to autonomic neurons (p < 0.001). The majority of the sera reacted with both sympathetic and myenteric plexus neurons. Interestingly, 6/30 CRPS sera showed binding to undifferentiated SH-SY5Y neuroblastoma cells. However, differentiation of SH-SY5Y into a cholinergic phenotype induced a surface antigen, which is recognised by 60% of CRPS sera (18/30), but not by controls (p < 0.001). Our data show that about 30–40% of CRPS patients have surface-binding autoantibodies against an inducible autonomic nervous system autoantigen. These data support an autoimmune hypothesis in CRPS patients. Further studies must elucidate origin and function of these autoantibodies in CRPS.     

Impaired self-perception of the hand in complex regional pain syndrome (CRPS)

To investigate neglect, extinction, and body-perception in patients suffering from complex regional pain syndrome (CRPS). So-called ‘neglect-like’ symptoms have been reported in CRPS, however no studies have yet analyzed this phenomenon which might substantiate the theory of the central nervous system involvement in the pathophysiology of CRPS. A total of 114 patients with CRPS of the upper limb underwent bedside neurological examination. ‘Neglect-like’ symptoms were determined by asking all patients what kind of feeling they had toward the affected hand (feeling of foreignness). Hemispatial neglect was tested with the line bisection task in 29 patients and sensory extinction to simultaneous stimulation in 40 patients. The ability to identify fingers after tactile stimulation was tested in 73 patients. Independently of the affected side and disease duration, 54.4% of the patients reported that their hand felt ‘foreign’ or ‘strange’. The ability to identify fingers was impaired in 48% on the affected hand and in 6.5% on the unaffected hand (X²=33.52, df=1, p<0.0001). These findings were related to pain intensity, illness duration and the extent of sensory deficits. No typical abnormalities indicating neglect were found in the line bisection test. Sensory extinction was normal in all patients. A large proportion of CRPS patients have disturbances of the self-perception of the hand, indicating an alteration of higher central nervous system processing. There are no indicators that classic neglect or extinction contribute to these findings. Physical therapy of such patients should take this observation into consideration.     

Current Understandings on Complex Regional Pain Syndrome

The mechanisms underlying complex regional pain syndrome (CRPS) have been increasingly studied over the past decade. Classically, this painful and disabling disorder was considered to emerge from pathology of the central nervous system. However, the involvement of additional peripheral disease mechanisms is likely, and recently these mechanisms have also attracted scientific attention. The present article provides an overview of the current understandings regarding pathology of the autonomic and somatic nervous system in CRPS, as well as the roles of neurogenic inflammation, hypoxia, and the contribution of psychological factors. Potential connections between the separate disease mechanisms will be discussed. Additionally, currently known risk factors for CRPS will be addressed. Insight into risk factors is of relevance as it facilitates early diagnosis and tailored treatment. Moreover, it may provide clues for further unraveling of the pathogenesis and etiology of CRPS.     

反射性交感神经营养不良

Background: Reflex sympathetic dystrophy (RSD),also known as complex regional pain syndrome (CRPS), is a nervous system disorder that often results in severe chronic and burning pain and other symptoms.