Motor disturbances are frequently reported in Complex Regional Pain Syndrome (CRPS) and may involve muscle weakness, problems with initiation and execution of movements and abnormal postures. The... Show moreMotor disturbances are frequently reported in Complex Regional Pain Syndrome (CRPS) and may involve muscle weakness, problems with initiation and execution of movements and abnormal postures. The general aim of this thesis was to investigate the contribution of sensory dysfunction to motor deficits in CRPS patients and to improve quantification of motor dysfunction. Comprehensive quantitative sensory testing techniques showed that a lower pressure pain threshold, measured over muscles, was the most prominent sensory abnormality in affected body parts of CRPS patients with and without dystonia, compared to healthy controls. Moreover, an association was found between sensory and motor dysfunction. A reduced pressure pain threshold was also the most deviant abnormality found in unaffected extremities of CRPS patients, indicating that muscle hyperalgesia is a widespread characteristic of CRPS. Furthermore, there were indications that impaired processing of proprioceptive information related to force production is disturbed in CRPS patients. Reliability and validity of the Range of Motion Scale (ROMS), a new clinical rating scale developed to assess the severity of fixed abnormal postures, was demonstrated. The phenomenology of movement disorders after peripheral trauma was systematically reviewed and potential mechanisms that may explain the underlying pathophysiology were discussed. Show less
In complex regional pain syndrome (CRPS), motor disturbances are common and cause significant disability. Although motor dysfunction is a well known and often described characteristic of CRPS, the... Show moreIn complex regional pain syndrome (CRPS), motor disturbances are common and cause significant disability. Although motor dysfunction is a well known and often described characteristic of CRPS, the underlying mechanisms are poorly understood. This thesis aimed to obtain a better understanding of the pathophysiology underpinning the motor dysfunction in CRPS. First, a quantitative analysis of the motor disturbances was performed, and subsequently its relation to pain intensity was examined. Next, we evaluated the effects of botulinum toxin in muscles of a subgroup of CRPS patients suffering from abnormal posturing. Also, circadian temperature regulation and its variation during long-term registration in affected and unaffected extremities of chronic CRPS patients was examined. Furthermore, a literature review was performed to research the origin and change in meaning over time of akinesia, bradykinesia and hypokinesia, terms that are frequently used to describe disturbances of motor function in movement disorders in general. Show less
Bank, P.J.M.; Peper, C.E.; Marinus, J.; Beek, P.J.; Hilten, J.J. van 2014
In the chronic stage of Complex Regional Pain Syndrome (CRPS), motor disturbances are common and cause significant disability. The motor dysfunction of CRPS is a poorly understood phenomenon that... Show moreIn the chronic stage of Complex Regional Pain Syndrome (CRPS), motor disturbances are common and cause significant disability. The motor dysfunction of CRPS is a poorly understood phenomenon that is characterized predominantly by a decrease or loss of voluntary muscle control. This thesis aims to obtain a better understanding of the pathophysiology underpinning the motor dysfunction of CRPS by examining the potential roles of decreased inhibition of the motor system, changes in sensory processing and problems in sensory-motor integration. In specific, characteristics of muscle activity recordings are scrutinized in order to determine whether the loss of voluntary motor control and abnormal postures in CRPS exhibit characteristics of dystonia that are associated with reduced inhibition of the motor system (i.e., excessive muscle activation and enhanced mirror activity). The potential role of impaired processing of proprioceptive information related to wrist orientation and force production is examined, as well as the involuntary and voluntary (sensory-)motor interactions between the affected and unaffected arm. Furthermore, a systematic review of the literature on the motor consequences of experimental pain in healthy humans is presented in order to gain insight into the potential role of pain-related processes in the motor and sensory and motor disturbances of CRPS. Show less
Dit proefschrift beschrijft de resultaten van aan aantal studies naar bewegingsstoornissen en pijn bij pati_nten met complex regionaal pijnsyndroom (CRPS). Ten eerste werd het effect onderzocht van... Show moreDit proefschrift beschrijft de resultaten van aan aantal studies naar bewegingsstoornissen en pijn bij pati_nten met complex regionaal pijnsyndroom (CRPS). Ten eerste werd het effect onderzocht van intrathecaal baclofen op verschillende pijnkwaliteiten bij CRPS-pati_nten, de invloed bestudeerd van verschillende infusiesnelheden op dystonie bij CRPS en het optreden van chorea na baclofen geanalyseerd bij enkele CRPS-pati_nten. Daarnaast werd het effect bestudeerd van intrathecaal glycine op CRPS-gerelateerde dystonie en pijn. Vervolgens werd aan de hand van een metabolomics benadering het metabole profiel in urine en hersenvocht van CRPS-pati_nten onderzocht. Tenslotte werd het effect bestudeerd van intramusculair magnesiumsulfaat op CRPS-gerelateerde pijn en dystonie Show less
Bank, P.J.M.; Peper, C.E.; Marinus, J.; Beek, P.J.; Hilten, J.J. van 2013
Complex regional pain syndrome (CRPS) may occur after trauma, usually to one limb, and is characterised by pain and disturbed blood flow, temperature regulation and motor control. Knowledge on CRPS... Show moreComplex regional pain syndrome (CRPS) may occur after trauma, usually to one limb, and is characterised by pain and disturbed blood flow, temperature regulation and motor control. Knowledge on CRPS and its movement disorders is scarce. Dysfunction in small nerve fiber processing was found in CRPS patients with dystonia. Furthermore, a dominant pattern of fixed dystonia was observed in two thirds of arms and legs: finger flexion, wrist flexion, elbow flexion and/or shoulder adduction; and ankle plantar flexion/inversion, toe flexion, knee flexion and/or hip internal rotation. A modelling study demonstrated that aberrant force feedback from Golgi tendon organs may be a factor. Cerebrospinal fluid findings did not support a role of inflammatory mediators in chronic CRPS-related dystonia. Characteristics of CRPS-related myoclonus were diverse. One intrathecal administration of methylprednisolone was not efficacious in chronic CRPS. Also, continuous intrathecal glycine showed no efficacy in CRPS-related dystonia. However, intrathecal baclofen reduced severity of CRPS-related dystonia, improved quality of life and remained efficacious over a period of one year. The findings lend support to the role of GABA (gamma aminobutyric acid)-ergic mechanisms in this cause of dystonia. The findings stimulate new directions of research on this topic. Show less
Munts, A.G.; Rijn, M.A. van; Geraedts, E.J.; Hilten, J.J. van; Dijk, J.G. van; Marinus, J. 2011
The clinical characteristics of Complex Regional Pain Syndrome (CRPS) are defined by pain and various combinations of sensory disturbances, autonomic features, and sudomotor and trophic changes.... Show moreThe clinical characteristics of Complex Regional Pain Syndrome (CRPS) are defined by pain and various combinations of sensory disturbances, autonomic features, and sudomotor and trophic changes. Furthermore, patients with CRPS may suffer from movement disorders, of which dystonia is the most prevalent. Dystonia of CRPS can affect multiple extremities, is often resistant to treatment and seems to have a poor prognosis. Reliable information on the nature, chronology and clinical determinants of dystonia in CRPS patients is lacking but could provide better insight in the underlying pathophysiological mechanism. The results of our studies on clinical and neurophysiologic aspects of patients with multiple CRPS and MDs in CRPS provided data that suggest disturbances at multiple levels of the CNS. Maladaptive neural plasticity likely is an important mechanism underpinning the MDs that may occur in CRPS. The presence of disturbances at multiple levels of the central nervous system parallels recent hypotheses on the underlying mechanisms suggested for other types of dystonia. Finally, the study on intrathecal baclofen administration in patients with CRPS and dystonia showed marked improvement of dystonia and pain scores after one year. Show less
Complex Regional Pain Syndrome (CRPS) is a painful disorder affecting one or more extremities. CRPS is characterized by various combinations of sensory, autonomic and motor disturbances. Genetic... Show moreComplex Regional Pain Syndrome (CRPS) is a painful disorder affecting one or more extremities. CRPS is characterized by various combinations of sensory, autonomic and motor disturbances. Genetic factors are suggested to play a role in CRPS, but this has not been extensively studied. Therefore the aims of this thesis were to study the contribution and the size of the contribution of genetic factors in CRPS and to identify possible susceptibility and causative genes for CRPS. In our studies we have shown that CRPS may develop without a noxious event and it has been suggested that such patients have a larger genetic predisposition. Furthermore we have found that CRPS may occur in a familial form, but did not observe a clear inheritance pattern. We calculated a measure of familial aggregation. This study yielded no indications for a large overall increased risk of developing CRPS for siblings of CRPS patients. Significant associations were found with CRPS and dystonia and two HLA-B62 and HLA-DQ8. We also evaluated the presence of causative mutations in known primary dystonia genes and causative mutations in the SCN9A gene. No such mutations were identified, indicating that these genes do not seem to play a major role in CRPS. Show less
