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Purpose: To investigate experiences and reflections on challenges in everyday life of people living with limb-girdle muscular dystrophy (LGMD) and chronic pain in order to improve rehabilitation services., Materials and Methods: The design for this study was qualitative using the Interpretive Description methodology and the salutogenic theory of Sense of Coherence as the theoretical framework. Four semi-structured focus group interviews were conducted with 19 adults with LGMD from April to May 2021. The interviews were conducted online due to COVID-19., Results: Living with chronic pain and LGMD affected everyday life in terms of the participants' overall Sense of Coherence. Beneficial or unfavorable coping strategies were identified within four interrelated categorical themes: pain management, normality comprehension, affected emotional sentiment and altered identity ., Conclusion: Healthcare professionals should acknowledge possible chronic pain secondary to LGMD. Chronic pain appears to be a prevalent problem in people with LGMD with negative impact on everyday life, yet patients with LGMD did not receive sufficient information and necessary tools from health professionals to cope with chronic pain. Thus, adequate pain management appeared to be a difficult and self-taught process. Educating health professionals on how to support patients with LGMD and chronic pain is needed.IMPLICATIONS FOR REHABILITATIONHealth professionals should acknowledge and address the possibility of chronic pain secondary to limb-girdle muscular dystrophy (LGMD) and educate patients in pain management.Physiotherapy, energy management and engagement in meaningful activities may help patients gain some control of pain and limit the consequences of pain on everyday life.Supporting patients to accept pain and to shift focus towards their current capabilities may potentially improve pain management.Educating health professionals on how to support patients with LGMD and chronic pain is needed.

Purpose: The aim was to investigate the prevalence, characteristics, predictors, and consequences of chronic pain in a national cohort of patients with limb-girdle muscular dystrophy (LGMD)., Materials and Methods: Questionnaires were sent to all Danish LGMD patients (≥18 years of age) registered with the National Rehabilitation Center for Neuromuscular Diseases., Results: Of 209 patients, 121 responded. 44.7% of the patients experienced persistent (daily or constant) chronic pain lasting more than 3 months. 21.0% of patients experienced chronic pain that was not daily. Most pain patients experienced three or more pain problems, primarily in the lower back, neck, shoulders, hips, and legs. Symptoms suggestive of neuropathic pain were sometimes present. Patients with persistent chronic pain reported moderate pain interference with daily activities, greater psychological distress, and lower quality of life compared to patients without pain but did not differ regarding physical functioning. Sex, age, LGMD duration, LGMD type, mechanical ventilation use, mobility, arm function, or performance on activities of daily living did not predict chronic pain., Conclusion: Chronic pain is common in patients with LGMD. Chronic pain should be considered an important component of LGMD and addressed in the clinic and rehabilitation setting from a biopsychosocial perspective.Implication for rehabilitationChronic pain is highly prevalent in patients with limb-girdle muscular dystrophy.Health professionals need to systematically ask patients about pain and the influence of pain on everyday life irrespective of LGMD-duration and extent of muscle wastage.Chronic pain and psychological distress need to be addressed in the clinic and rehabilitation setting as an additional disabling component of LGMD and this should be done within a biopsychosocial framework.

The purpose was to investigate the impact of the COVID-19 pandemic on biopsychosocial health, daily activities, and quality of life among children and adults with neuromuscular diseases, and to assess the prevalence of COVID-19 infection and the impact of this in patients with neuromuscular diseases. The study was a national questionnaire survey. Responses were obtained from 811 adults (29%) and 67 parents of children (27%) with neuromuscular diseases. Many patients reported decreased health or physical functioning, and changes in access to physiotherapy or healthcare due to the pandemic. Participants generally perceived themselves or their child to be at high risk of severe illness from COVID-19, but only 15 patients had suffered from COVID-19 and experienced mild flu-like symptoms. 25.3% of adults and 46.6% of parents experienced anxiety. 20.4% of adults and 27.6% of parents experienced symptoms of depression. In general, the pandemic contributed to anxiety, a depressed mood as well as to fewer leisure activities, less social contact, isolation from work/school and a reduced quality of life, in particular for patients who perceived themselves to be at high risk of severe illness. The results demonstrate that the pandemic has had a negative impact on biopsychosocial health and quality of life of patients with neuromuscular diseases., Competing Interests: The authors have declared that no competing interests exist.

Purpose: Although autonomic features are part of the diagnostic criteria for complex regional pain syndrome (CRPS), the role of the autonomic nervous system in CRPS pathophysiology has been downplayed in recent years. The purpose of this review is to redress this imbalance., Methods: We focus in this review on the contribution of the autonomic nervous system to CRPS pathophysiology. In particular, we discuss regional sympathetic and systemic autonomic disturbances in CRPS and the mechanisms which may underlie them, and consider links between these mechanisms, immune disturbances and pain., Results: The focused literature research revealed that immune reactions, alterations in receptor populations (e.g., upregulation of adrenoceptors and reduced cutaneous nerve fiber density) and central changes in autonomic drive seem to contribute to regional and systemic disturbances in sympathetic activity and to sympathetically maintained pain in CRPS., Conclusions: We conclude that alterations in the sympathetic nervous system contribute to CRPS pathology. Understanding these alterations may be an important step towards providing appropriate treatments for CRPS.

The aim of this study was to determine whether upregulated cutaneous expression of α1-adrenoceptors (α1-AR) is a source of pain in patients with complex regional pain syndrome (CRPS). Immunohistochemistry was used to identify α1-AR on nerve fibres and other targets in the affected and contralateral skin of 90 patients, and in skin samples from 38 pain-free controls. The distribution of α1-AR was compared between patients and controls, and among subgroups of patients defined by CRPS duration, limb temperature asymmetry, and diagnostic subtype (CRPS I vs CRPS II). In addition, α1-AR expression was investigated in relation to pain and pinprick hyperalgesia evoked by intradermal injection of the α1-AR agonist phenylephrine. Expression of α1-AR on nerve bundles in the CRPS-affected limb was greater in patients who reported prolonged pain and pinprick hyperalgesia around the phenylephrine injection site than in patients with transient pain after the injection. In addition, α1-AR expression in nerve bundles was greater in patients with CRPS II than CRPS I, and was greater in acute than more long-standing CRPS. Although less clearly associated with the nociceptive effects of phenylephrine, α1-AR expression was greater on dermal nerve fibres in the painful than contralateral limb. Together, these findings are consistent with nociceptive involvement of cutaneous α1-AR in CRPS. This involvement may be greater in acute than chronic CRPS, and in CRPS II than CRPS I.

Objective: Findings regarding small nerve fiber damage in complex regional pain syndrome type I (CRPS-I) are not uniform, and studies have not included a matched healthy control group. The aim was to assess intraepidermal nerve fiber density (IENFD) in relation to thermal sensitivity of the same skin areas in CRPS-I patients and a gender- and age-matched healthy control group., Methods: IENFD was investigated in skin biopsies from the CRPS-affected and contralateral limbs of eight CRPS-I patients and from an equivalent site in eight gender- and age-matched healthy controls (HCs). Thermal thresholds (cold/warm detection, cold- and heat-pain detection) were assessed on the affected limb, the matching contralateral limb, and on the equivalent limbs of HCs, and participants rated the intensity of cold/heat and pain to static thermal stimuli (5 °C and 40 °C)., Results: IENFD was significantly lower in both the affected and contralateral limbs of CRPS-I patients than HCs, but IENFD did not differ between the affected and contralateral limbs of patients. The heat pain threshold was lower in the affected CRPS-I limb than in HCs, but all other thermal thresholds were similar in both groups. CRPS-I patients rated the cold stimulus as colder and more painful in the affected limb, and the warm stimulus as hotter, bilaterally, than the HCs., Conclusions: CRPS-I may be associated with bilateral small fiber damage, and perhaps small fiber neuropathy and bilateral disturbances in thermo-sensory perception. These disturbances could stem from a systemic response to injury or might increase the risk of developing CRPS-I after physical trauma.

Sensory disturbances often spread beyond the site of injury in complex regional pain syndrome (CRPS) but whether this applies equally to CRPS I and II, or changes across the course of the disease, is unknown. Establishing this is important, because different symptom profiles in CRPS I and II, or in acute vs chronic CRPS, might infer different pathophysiology and treatment approaches. To explore these questions, sensory disturbances were assessed in the limbs and forehead of 71 patients with CRPS I and 33 patients with CRPS II. Pain had persisted up to 12 months in 32 patients, for 13 to 36 months in 29 patients, and for longer than this in 43 patients. Patients with CRPS I were more likely to be female, and pain was more likely to be present in an additional limb, than patients with CRPS II. Conversely, pain was more likely to be associated with sensory deficits and allodynia in patients with CRPS II than CRPS I. Nevertheless, heightened sensitivity, allodynia, and/or hyperalgesia to mechanical and thermal stimuli were detected in a hemisensory distribution ipsilateral to the affected limb in both forms of CRPS. Some of these hemisensory disturbances strengthened with chronicity of pain. These findings suggest that heightened excitability of nociceptive pathways in CRPS spreads to hemisensory convergence points in the brainstem or higher brain centres, possibly in association with compromised pain controls. The similarity of symptom profiles in chronic CRPS I and II implies shared mechanisms despite different triggers.

Background: Substantial literature documents that persistent postsurgical pain is a possible outcome of many common surgical procedures. As fracture-related surgery implies a risk of developing neuropathic pain and complex regional pain syndrome (CRPS), further studies investigating the prevalence and pain characteristics are required., Methods: All patients undergoing primary surgery because of ankle or wrist fracture at Hvidovre and Odense University Hospitals, Denmark, between April 15, 2013 and April 15, 2014, were identified from the Danish Fracture Database. A questionnaire regarding pain characteristics was sent to patients 1 yr after primary surgery., Results: Replies were received from 328 patients, of whom 18.9% experienced persistent postsurgical pain defined as pain daily or constantly at a level that interfered much or very much with daily activities, 42.8% reported symptoms suggestive of neuropathic pain, and 4.0% fulfilled the diagnostic patient-reported research criteria for CRPS., Conclusions: Persistent postsurgical pain 1 yr after wrist and ankle fracture surgery is frequent, and a large proportion of patients experience symptoms suggestive of neuropathic pain and CRPS. Patients should be informed about the substantial risk of developing persistent postsurgical pain. Future studies investigating risk factors for persistent postsurgical pain that include both surgically and conservatively treated fractures are required., (© The Author 2016. Published by Oxford University Press on behalf of the British Journal of Anaesthesia. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Background: Ambiguous visual stimuli increase limb pain in patients with complex regional pain syndrome (CRPS), possibly due to afferent sensory feedback conflicts. Conflicting sensory stimuli can also generate unpleasant sensations in healthy people such as during motion sickness. We wanted to investigate the mechanisms underlying the link between sensory conflicts and pain in CRPS using optokinetic stimulation (OKS) - a method known to induce motion sickness., Methods: Twenty-one CRPS patients underwent OKS and rated symptoms of motion sickness. Patients also rated limb pain and pain-related distress before, during and after OKS. In addition, pressure-pain and sharpness sensations were investigated on both sides of the forehead and in the affected and contralateral limb before and after OKS., Results: Limb pain and forehead hyperalgesia to pressure increased in parallel in response to OKS. In a subgroup of nauseated patients who withdrew early from OKS, hyperalgesia to pressure in the ipsilateral forehead persisted longer than in the remaining participants. Sharpness sensations remained constant at all sites., Conclusions: Sensory conflicts may facilitate pain in CRPS by activating the mechanisms of general facilitation of nociception and, during more severe sensory conflicts, also a facilitatory mechanism that operates mainly ipsilateral to the affected limb., (© 2014 European Pain Federation - EFIC®)

Electrical impedance measurements have been assessed as a method of detecting changes in striated muscle following vigorous exercise. Transverse and longitudinal resistivities of the calf and thigh have been measured before and after four subjects ran a half marathon (21 km). No changes were observed in longitudinal resistivity but transverse resistivity rose by an average of 7% following the race. These results are consistent with changes in the muscle fibre membranes or interstitial fluid content.