Bethlem myopathy: a slowly progressive congenital muscular dystrophy with contractures. (1/56)

Bethlem myopathy is an early-onset benign autosomal dominant myopathy with contractures caused by mutations in collagen type VI genes. It has been reported that onset occurs in early childhood. We investigated the natural course of Bethlem myopathy in five previously published kindreds and two novel pedigrees, with particular attention to the mode of onset in 23 children and the progression of weakness in 36 adult patients. Our analysis shows that nearly all children exhibit weakness or contractures during the first 2 years of life. Early features include diminished foetal movements, neonatal hypotonia and congenital contractures which are of a dynamic nature during childhood. The course of Bethlem myopathy in adult patients is less benign than previously thought. Due to slow but ongoing progression, more than two-thirds of patients over 50 years of age use a wheelchair.  (+info)

An electromyographic study of the hip abductor muscles as subjects with a hip prosthesis walked with different methods of using a cane and carrying a load. (2/56)

BACKGROUND AND PURPOSE: Certain methods of carrying handheld loads or using a cane can reduce the demands placed on the hip abductor (HA) muscles and the loads on the underlying prosthetic hip. In certain conditions, unusually large forces from the HA muscles may contribute to premature loosening of a prosthetic hip. The purpose of this study was to examine HA use by measuring the amplitude of the electromyographic (EMG) signal from the HA muscles as subjects carried a load and simultaneously used a cane. SUBJECTS: Twenty-four active subjects (mean age = 63.3 years, SD = 10.7, range = 40-86) with a unilateral prosthetic hip were tested. METHODS: The HA muscle surface EMG activity was analyzed as subjects carried loads weighing 5%, 10%, or 15% of body weight held by either their contralateral or ipsilateral arm relative to their prosthetic hip. They simultaneously used a cane with their free hand. RESULTS: The contralateral cane and ipsilateral load conditions produced HA muscle EMG activity that was approximately 40% less than the EMG activity produced while walking without carrying a load or using a cane. CONCLUSION AND DISCUSSION: People who are in danger of premature loosening of their prosthetic hip should, if possible, avoid carrying loads. If a load must be carried, however, then the contralateral cane and ipsilateral load condition appears to minimize the loads placed on the prosthetic hip due to HA muscle activity.  (+info)

Health impact of pain in the hip region with and without radiographic evidence of osteoarthritis: a study of new attenders to primary care. The PCR Hip Study Group. (3/56)

OBJECTIVES: To assess the health impact of hip pain at the time of first presentation to primary care, and the influence on this of radiographic evidence of osteoarthritis. SUBJECTS AND METHODS: Cross sectional survey of 195 patients (63 male, 132 female), aged 40 years and over, presenting with a new episode of hip pain, recruited from 35 general practices across the UK. Health status at presentation was determined by a structured questionnaire on symptoms, healthcare use, and health related quality of life (SF-36). Pelvic radiographs were assessed blindly for hip osteoarthritis using standard scoring systems. RESULTS: The overall impact on health was substantial. Before their first consultation, three quarters of patients needed analgesics, half used topical creams or ointments, and one in eight used a walking stick. Most of these impact measures were, however, unrelated to the degree of radiographic change, though use of a walking stick was increased in those with the most severe damage. Health status, as judged by the SF-36, was also impaired for measures of physical function and pain, but the impact on the "mental health", "general health", and "vitality" dimensions was small. There was a weak relation between the SF-36 scores and radiographic change, with many domains unrelated to the severity of radiographic damage. CONCLUSIONS: This study is the first to show the therapeutic impact and pattern of impairment in health status resulting from hip pain at the time of first presentation to the healthcare services. Unlike many regional pain syndromes seen in primary care, such as back pain, hip pain does not impact on wider aspects of quality of life, such as general health status, mental health, or vitality. Furthermore, any impact of hip pain in this group is not markedly influenced by the degree of structural damage. Further follow up is required to determine whether such damage influences the persistence of any adverse impact.  (+info)

Nutritional supplementation of elderly hip fracture patients. A randomized, double-blind, placebo-controlled trial. (4/56)

BACKGROUND: undernourishment is common in elderly hip fracture patients and has been linked to poorer recovery and increased post-operative complications. OBJECTIVE: to determine whether a nutritional supplement may (i) help elderly patients return to pre-fracture functional levels 6 months post-fracture and (ii) decrease fracture-related complications and mortality. DESIGN: a double-blind, randomized, placebo-controlled clinical trial. SETTING: a county hospital near Barcelona. SUBJECTS: 171 patients, aged 70 and older, hospitalized for hip fracture between July 1994 and July 1996. METHODS: we randomized patients to intervention (n = 85) or control (n = 86) group. Patients received a nutritional supplement containing 20 g of protein and 800 mg of calcium or placebo for 60 days. We determined functional levels by the Barthel index, the mobility index and by the use of walking aids. We performed assessments during hospitalization and at 2 and 6 months post-fracture. FINDINGS: the two groups were comparable at study entry. We observed no differences in return to functional status 6 months post-fracture (61% intervention group vs 55% in control group) nor in fracture-related mortality (13% in intervention group vs 10% in control group). The intervention group suffered fewer in-hospital [odds ratio 1.88 (95% CI 1.01 - 3.53), P = 0.05] and total complications [odds ratio 1.94 (95% CI 1.02-3.7), P = 0.04] than the control group. CONCLUSION: based on our results, we cannot recommend routine nutritional supplementation of all elderly hip fracture patients. While nutritional supplementation may be useful in decreasing complications, this reduction does not result in improvement in functional recovery and nor does it decrease fracture-related mortality. Selected patients may, however, benefit from nutritional supplementation.  (+info)

Balance and mobility following stroke: effects of physical therapy interventions with and without biofeedback/forceplate training. (5/56)

BACKGROUND AND PURPOSE: Visual biofeedback/forceplate systems are often used for treatment of balance disorders. In this study, the researchers investigated whether the addition of visual biofeedback/forceplate training could enhance the effects of other physical therapy interventions on balance and mobility following stroke. SUBJECTS: The study included a sample of convenience of 13 outpatients with hemiplegia who ranged in age from 30 to 77 years (mean=60.4, SD=15.4) and were 15 to 538 days poststroke. METHODS: Subjects were assigned randomly to either an experimental group or a control group when the study began, and their cognitive and visual-perceptual skills were tested by a psychologist. Subjects were also assessed using the Berg Balance Scale and the Timed "Up & Go" Test before and after 4 weeks of physical therapy. Both groups received physical therapy interventions designed to improve balance and mobility 2 to 3 times per week. The experimental group trained on the NeuroCom Balance Master for 15 minutes of each 50-minute treatment session. The control group received other physical therapy for 50 minutes. RESULTS: Following intervention, both groups scored higher on the Berg Balance Scale and required less time to perform the Timed "Up & Go" Test. These improvements corresponded to increased independence of balance and mobility in the study population. However, a comparison of mean changes revealed no differences between groups. DISCUSSION AND CONCLUSION: Although both groups demonstrated improvement following 4 weeks of physical therapy interventions, no additional effects were found in the group that received visual biofeedback/forceplate training combined with other physical therapy.  (+info)

Full weight-bearing after cementless total hip arthroplasty. (6/56)

In a prospective study of cementless total hip arthroplasty, 19 hips in 17 patients (Group A) were allowed full weight-bearing immediately after the operation while 18 hips in 16 patients (Group B) were first allowed weight-bearing after 6 weeks. Patients were matched for sex, age at surgery, height, weight, and follow-up period and there were no significant differences in hip scores between the two groups. Rehabilitation to gain walking ability with a cane lasted 5.8 days for Group A and 44.8 days for Group B (P = 0.0001). The hospital stay after surgery was 30.1 days for Group A and 46.7 days for Group B (P = 0.006). All patients showed bone ingrowth radiographically. There were no complications in either group.  (+info)

Upper-limb pain in long-term poliomyelitis. (7/56)

BACKGROUND: Late functional deterioration is common in long-term poliomyelitis patients. While upper-limb pain in individual functional regions is common, its overall prevalence and pattern in long-term poliomyelitis is poorly documented. AIM: To assess the nature of upper-limb pain in these patients and examine its association with dependence on mobility aids (and therefore upper limb 'overuse'). DESIGN: Questionnaire-based survey of patients attending a specialist unit. METHODS: Questionnaires were sent to 139 patients with known long-term poliomyelitis (mean 49.8 years post polio), attending the Lane Fox Unit out-patient clinic at St Thomas' Hospital between January 1998 and December 1998. There were questions on the nature of the patient's acute illness, limb involvement at initial infection ('polio-affected' limbs), the site and onset of upper-limb pain, hand dominance, previous injuries and surgery, and the use of mobility aids. Limbs that had sustained an injury or undergone surgery, at or preceding onset of upper-limb pain, were excluded from analysis. RESULTS: Among 103 valid replies, the prevalence of upper limb pain was 64%. Mobility aids were used by 74%, and were associated with an increased risk of upper-limb pain, while 'polio-affected' limbs were at reduced risk. DISCUSSION: These data support 'overuse' due to greater mobility aid dependence as a cause of increasing upper-limb pain in long-term poliomyelitis.  (+info)

The effect of walking aids on balance and weight-bearing patterns of patients with hemiparesis in various stance positions. (8/56)

BACKGROUND AND PURPOSE: Standard and quad canes are often prescribed to patients with hemiparesis, yet their effect on postural control remains unclear. Thus, the objective of this study was to examine the effects of standard and quad canes on postural sway and on weight-bearing patterns in patients with hemiparesis. SUBJECTS: Thirty subjects with a diagnosis of unilateral hemiparesis following a stroke (patient group; mean age=71.2 years, SD=7.0) and 20 age-matched, community-dwelling volunteers without hemiparesis (comparison group; mean age=72.1 years, SD=5.2) participated in the study. METHODS: Postural sway and percentage of body weight (%BW) borne by each extremity were measured in 3 positions: with the heels aligned with each other (aligned position) and in staggered foot positions with either the affected or unaffected extremity placed forward (affected FW and unaffected FW positions). All subjects were tested in each position with no cane, a standard cane, and a quad cane. The order of tests was randomized, and analysis of data included use of an analysis of variance and adjusted Tukey-Kramer tests. RESULTS: In both the aligned and unaffected FW positions, postural sway was reduced only with the quad cane. Both types of canes reduced postural sway in the affected FW position; however, the quad cane had a greater effect. An asymmetrical weight distribution between the lower extremities did not change in the patient group across positions, even with walking aids. DISCUSSION AND CONCLUSION: A quad cane appears to be more effective than a standard cane in decreasing postural sway in patients with moderate impairment secondary to hemiparesis during stance. The greatest effect on postural sway occurred when the assistive device was contralateral to the foot placed forward. The use of a cane does not appear to adversely affect the asymmetrical weight-bearing pattern during stance that is characteristic of patients with hemiparesis, even when balance is challenged by decreasing the base of support.  (+info)