11 resultados para Spinal muscular atrophy
em Brock University, Canada
Resumo:
The current classification system for spinal cord injury (SCI) considers only somatic information and neglects autonomic damage after injiuy. Heart rate variability (HRV) has the potential to be a valuable measure of cardiac autonomic control after (SCI). Five individuals with tetraplegia and four able-bodied controls underwent 1 min continuous ECG recordings during rest, after Metoprolol administration (max dose=3x5mg) and after Atropine administration (0.02mg/kg) in both supine and 40° head-up tilt. After Metoprolol administration there was a 61.8% decrease in the LF:HF ratio in the SCI participants suggesting that the LF:HF ratio is a reflection of cardiac sympathetic outflow. After Atropine administration there was a 99.1% decrease in the HF power in the SCI participants suggesting that HF power is highly representative of cardiac parasympathetic outflow. There were no significant differences between the SCI and able-bodied participants. Thus, HRV measures are a valid index of cardiac autonomic control after SCI.
Resumo:
The newt, Notopthalmus viridescens is one of the few tet rapod vertebrates capable of extensive regeneration of the central nervous system, however, the factors involved in this process are still unknown. Chemokine signalling through the receptor CXCR4, has been found to be involved in the development of the central nervous system of mammals and more recently in epimorphic fin regeneration in zebrafish. We have hypothesized that the CXCR4 signalling pathway is involved in spinal cord and tail regeneration in the adul t newt , possibly as a downstream target of retinoic acid signalling. We found that CXCR4 mRNA expression was observed in the brain, spinal cord, heart, gut, liver and regenerating tail blastemas. CXCR4 expression increased over the f i rst 12 days of tail regeneration and returned to basal expression levels at day 21 of regeneration. Inhibition of CXCR4 wi th AMD3100, a specific receptor antagonist, led to a decrease in CXCR4 mRNA in the regenerating tail 14 days post amputation. Histological analysis suggests a delay in the early stages of tail and spinal cord regeneration. Spinal cord explants t reated wi th CXCL12, the ligand to CXCR4, displayed enhanced neurite outgrowth in vitro. Explants t reated wi th AMD3100 abolished any retinoic acid enhanced neurite outgrowth effects suggesting a link between these signalling pathways.
Resumo:
The purpose of this study was to investigate the effects of a 12-week FES-ambulation program on locomotor function and quality of life after incomplete spinal cord injury. Six individuals with incomplete SCI participated in the study. Over-ground walking endurance (6MWT), speed (10MWT), independence (WISCI II) and body-weight support were assessed. Quality of life was assessed via the SF-36, WHOQOL-BREF, Perceived Stress Scale, Center of Epidemiological Studies for Depression scale, and task self-efficacy. Participants experienced significant improvements in walking endurance (223.6±141.5m to 297.3±164.5m; p=0.03), body-weight support (55.3±12.6% to 14.7±23.2%; p= 0.005) and four of the six participants showed improvements on the WISCI II scale (1-4 points). In addition, there was a significant reduction in reported bodily pain (6.5±1.2 to 5.0±1.7; p=0.04). Therefore, FES-ambulation is an effective means for enhancing over-ground locomotor function in individuals with incomplete SCI. It may also be an effective method for reducing pain in individuals with SCI.
Resumo:
The human neuromuscular system is susceptible to changes within the thermal environment. Cold extrinsic temperatures can significantly reduce muscle and nervous system function and communication, which can have consequences for motor performance. A repeated measures design protocol exposed participants to a 12°C cold water immersion (CWI) up to the ankle, knee, and hip to determine the effect that reduced skin and muscle temperature had on balance and strength task execution. Although a linear reduction in the ability to perform balance tasks was seen from the control condition through to the hip CWI, results from the study indicated a significant reduction in dynamic balance (Star Excursion Balance Test reach distance) performance from only the hip CWI (P<0.05). This reduced performance could have been due to an increase in joint stiffness, increased agonist-antagonist co-contraction, and/or reduced isokinetic muscular strength. Reduced physical performance due to cold temperature could negatively impact outdoor recreational athletics.
Resumo:
The phenomenon of over-recovery consists of a participant’s maximal force levels returning to values above initial levels. The present study examined the presence and causes of over-recovery following local muscular fatigue. Fourteen males completed two fatigue protocols consisting of maximal isometric dorsiflexion contractions. Upon completion of the fatigue protocol participants’ force was monitored over a 15 minute recovery period. Dorsiflexion force and surface electromyography (sEMG) from the tibialis anterior and soleus were monitored concurrently. Following the two fatigue conditions (10 and 20% force decrement) force recovered to 100.5 and 99.5% of initial levels for each condition, respectively. Surface EMG root-mean-square amplitude and MPF exhibited changes consistent with a warm-up effect. It was concluded that over-recovery was not present in the tibialis anterior following a local muscular fatigue. However, the return in force to initial values, rather than a persistent decrement as normally observed, was mediated by the warm-up effect.
Resumo:
Sexuality after spinal cord injury (SCI) is a complex issue that is influenced by a number of social, psychological and physiological factors, one of which is urinary incontinence (UI). Using a phenomenological approach, seven mixed methods interviews combining both the interview guide and standardized open-ended approaches were conducted to examine the experience of sexuality for women who are concerned about UI following SCI. Sexual function was one of the top priorities for the women after SCI, and UI was one of the main concerns the women had regarding sexuality. The findings of this study demonstrate that various dimensions of intimacy and the sexual experience as a whole were affected by UI, and the women discussed both physical and psychological concerns. The main issues regarding sexuality included concerns related to relationships, frustrations with limited sexual activities and the difficulty of being sexually satisfied, the number of unanswered questions and concerns, and a fear of being hurt or injured while participating in sexual activities. The main concerns regarding UI were embarrassment, the work and inconvenience involved with the clean-up of UI, bladder infections, the lack of accessible washrooms, and the negative effects of UI medications. When examining sexuality and UI together, the major issues were the constant comparison to the way things were before SCI, as well as the new concerns that the women did not have to worry about previously, worrying about how their partner would react if UI were to occur during sexual activity, and the impact of their own feelings toward UI on sexuality, a connection between pleasurable sexual sensations and UI as well as difficulty differentiating between the sensation of UI with the sensation of UI, dealing with infected urine during sexual activity, having to discuss UI with a new potential sexual partner, and a fear of rejection. Other identified issues included those related to body image, a lack of resources, Doctors who were inadequately educated regarding SCI, and issues related to both having and raising children. There is a significant shortage of information available for women with SCI to use as a resource regarding sexual function in general, and sexual function as it relates to UI. It is necessary that future work focus on creating resources to assist in this area, and that the dissemination of those resources becomes both appropriate and effective. Addressing sexual function and UI which are among the top concerns for this population has the opportunity to greatly improve quality of life (QOL) for these individuals.
Resumo:
Using modified constructivist grounded theory, the purpose of the present study was to explore body-related experiences, specifically body image, in people with spinal cord injury. A total of nine participants (five women, four men) who had a broad range of body image experiences (from very negative to very positive) were interviewed. Most participants explained experiencing a fluctuating body image that varied from day-to-day. Negative body image experiences were represented by appearance, weight concerns, and function with all body image experiences encompassed by self-presentational concerns and tactics (an unanticipated finding). Positive body image was represented by acceptance, appreciation and gratitude of the body. Interestingly, negative body image experiences were not found to be represented by the opposite of positive body image experiences as they were each distinct. These findings have direct implications for medical professionals in hospital and rehabilitation settings to understand the importance of body image after spinal cord injury.
Resumo:
The molecular events after spinal cord injury that lead to the establishment of a permissive environment and epimorphic regeneration remain unclear. Two molecular pathway regulators that may converge to create a spinal cord regeneration-permissive environment in the urodele are retinoic acid (RA) and microRNAs (miRNAs). Recent evidence suggests that RARβ-mediated signaling is necessary for tail and caudal spinal cord regeneration in the adult newt. MicroRNAs are attractive candidates as mediators of retinoid signaling during regeneration, as their pleiotropic effects are vital in situations where global changes in gene expression are required. Thus, the overall aim of this thesis was to determine if miRNAs are involved in tail and caudal spinal cord regeneration in the adult newt, and if they act as regulators and/or effectors of retinoid signaling during this process. I have demonstrated here, for the first time, that multiple miRNAs are dysregulated in response to spinal cord injury in the adult newt, as well as in response to inhibition of retinoid signaling. Two of these miRNAs, miR-133a and miR-1, appear to target RARβ2 transcripts both in vivo and in vitro. Inhibition of RA signaling via RARβ with a selective antagonist, LE135, alters the pattern of expression of these miRNAs, which leads to an inhibition of tail regeneration. These data are indicative of a negative feed back loop, albeit potentially an indirect one. I also aimed to examine which miRNAs are affected by inhibiting RA synthesis during regeneration, and provided a long list of miRNAs that are dysregulated. These data provide the foundation for future studies on the putative roles of these miRNAs, as well as their function in retinoid signaling. Overall, these studies provide the first evidence for a role for miRNAs as mediators of retinoid signaling during caudal spinal cord regeneration in any system.
Resumo:
Most research on the effects of endurance training has focused on endurance training's health-related benefits and metabolic effects in both children and adults. The purpose of this study was to examine the neuromuscular effects of endurance training and to investigate whether they differ in children (9.0-12.9 years) and adults (18.4-35.6 years). Maximal isometric torque, rate of torque development (RTD), rate of muscle activation (Q30), electromechanical delay (EMD), and time to peak torque and peak RTD were determined by isokinetic dynamometry and surface electromyography (EMG) in elbow and knee flexion and extension. The subjects were 12 endurance-trained and 16 untrained boys, and 15 endurance-trained and 20 untrained men. The adults displayed consistently higher peak torque, RTD, and Q30, in both absolute and normalized values, whereas the boys had longer EMD (64.7+/-17.1 vs. 56.6+/-15.4 ms) and time to peak RTD (98.5+/-32.1 vs. 80.4+/-15.0 ms for boys and men, respectively). Q30, normalized for peak EMG amplitude, was the only observed training effect (1.95+/-1.16 vs. 1.10+/-0.67 ms for trained and untrained men, respectively). This effect could not be shown in the boys. The findings show normalized muscle strength and rate of activation to be lower in children compared with adults, regardless of training status. Because the observed higher Q30 values were not matched by corresponding higher performance measures in the trained men, the functional and discriminatory significance of Q30 remains unclear. Endurance training does not appear to affect muscle strength or rate of force development in either men or boys.
Resumo:
Duchenne muscular dystrophy is a X-linked muscle disease, which leads to alterations in membrane phospholipid fatty acid (FA) composition and skeletal muscle damage. Increased membrane saturated FA in muscular dystrophy may suggest its association with increased susceptibility (as being the cause or consequence) to muscle damage. It was hypothesised that increased saturation is positively correlated to increased muscle damage. Correlations were hypothesized to be greater in extensor digitorum longus (EDL) at 20 weeks compared to soleus (SOL) at 10 weeks in dystrophin deficient (mdx) mice. Increased saturation was correlated to damage in EDL at both 10 and 20 weeks, with stronger correlations at 10 weeks. The results suggest that membrane PL FA composition may be associated with damage through two possible means. Increased saturation may be a cause or consequence of membrane damage. Association of membrane composition with eccentric induced damage has underscored the importance of saturated PL FA compositions in damage to dystrophic myofibres.