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Biomedical subjects

M Swash

Publications and source records attributed to M Swash.

At least 55 records · Page 3Linked to original sources

Sacral reflexes: physiology and clinical application.

Sacral reflexes consist of motor responses in the pelvic floor and sphincter muscles evoked by stimulation of sensory receptors in pelvic skin, anus, rectum, or pelvic viscera. These responses may be elicited by physical or electrical stimuli. They have been used in research studies of the pathophysiology of pelvic floor and anorectal disorders and many have been recommended for diagnostic use. These reflexes are described and discussed in this review. More rigorous evaluation of their value in the clinical assessment and care of patients with pelvic floor and sphincter disorders is required. Currently direct comparisons of the value of particular responses are generally not available, and few of these reflexes have proven validity for use in clinical diagnosis.

Anal Canal↗

Riluzole has no acute effect on motor unit parameters in ALS.

Riluzole is the only drug to have been approved for the treatment of amyotrophic lateral sclerosis (ALS/MND). Its mechanism of action is complex and includes actions on NMDA and kainate receptors and modulation of voltage gated Na channels. In ALS, its effects on measurable parameters of the motor units utilising current neurophysiological techniques are unknown. In an acute randomized, double-blind, placebo-controlled, cross-over experiment, we serially assessed the effects of riluzole on motor units in muscles affected by ALS/MND using EMG. We discuss the results of our observations in the light of previous clinical trials, and their implications.

Amyotrophic Lateral Sclerosis↗

The European Amyotrophic Lateral Sclerosis Health Profile Study. ALS-HPS Steering Group.

Quality of life is increasingly regarded as an important outcome measure in the evaluation of treatment regimes. The last decade has seen an enormous growth in the application of measures designed to assess quality of life in a vast array of medical specialities. However, the use of such measures in neurology has been limited and is virtually non-existent in amyotrophic lateral sclerosis (ALS). The European ALS Health Profile Study is a longitudinal survey of patients diagnosed with ALS or motor neurone disease in which patients are asked to complete questionnaires concerning their subjective health status. Data from clinical assessments is also collected. It is intended that the information collected will provide more systematic and detailed evidence of the impact of the disease from the perspective of the patient. This paper outlines the purpose and methodology of the project.

Amyotrophic Lateral Sclerosis↗

Early diagnosis of ALS/MND.

With the advent of a specific biological therapy for ALS there is an increasing imperative for early diagnosis. As new, more effective therapies become available, this will become more important. It is intuitively probable that early therapy will prevent disability in this otherwise relentlessly progressive disorder. The difficulties posed by this need for early diagnosis in clinical practice are discussed.

Amyotrophic Lateral Sclerosis↗

Faecal incontinence in myotonic dystrophy.

Two siblings with myotonic dystrophy presented for treatment of faecal incontinence. The pathophysiology of this functional disorder is described with the results of anorectal manometry, EMG, and biopsy of smooth and striated muscle of the anorectal sphincters. Both medical and surgical management of the incontinence was unsatisfactory in the long term. Involvement of gastrointestinal musculature is a characteristic feature the disease.

Adult↗

Quality of life in multiple sclerosis in France, Germany, and the United Kingdom. Cost of Multiple Sclerosis Study Group.

OBJECTIVE: To assess the quality of life (QoL) of patients with multiple sclerosis in France, Germany, and the United Kingdom with a cross sectional study. METHODS: Patients were classified into three severity groups according to the expanded disability severity scale (EDSS); stage I, II, and III, corresponding to mild (EDSS 1.0-3.5), moderate (EDSS 4.0-6.0), or severe (EDSS 6.5-8.0) multiple sclerosis respectively. Ninety patients with multiple sclerosis and 30 control patients without multiple sclerosis were recruited in each country. Control patients were matched to the patients with multiple sclerosis according to age and sex. Quality of life was assessed using the functional status questionnaire (FSQ). RESULTS: The aspects of QoL that were mostly affected in the three countries under study were physical function and general wellbeing. Social role function decreased with increased severity of disease in France and in particular in Germany. Multiple sclerosis did not seem to have an impact on psychological function. The QoL of control patients was systematically higher than that of patients with multiple sclerosis. CONCLUSIONS: Use of such a generic scale showed that progression of multiple sclerosis is accompanied by a decrease in QoL and suggested that this could be a relevant measurement in assessing the effect of treatment and progression of disease. Variation between countries, however, may be important.

Adult↗

Economic evaluation of multiple sclerosis in the UK, Germany and France.

A cross-sectional cost-of-care study was performed to assess the economic burden of multiple sclerosis (MS) in France, Germany and the UK. Patients were stratified into 3 groups according to the Expanded Disability Severity Scale (EDSS): stages I, II and III, corresponding to mild (EDSS 1.0 to 3.5), moderate (EDSS 4.0 to 6.0) and severe (EDSS 6.5 to 8.0) MS, respectively. 90 patients with MS and 30 non-MS control patients were recruited in each country. Control patients were matched to the patients with MS on the basis of age and gender. Demographic, clinical and economic data during the 3-month period prior to entry were collected in patient interviews. Total costs included actual expenditures, such as direct medical and non-medical costs, as well as indirect costs. From the societal perspective, the total cost of MS for 3 months was estimated at 1,928 US dollars, 3,941 US dollars and 5,678 US dollars in France, 2,772 US dollars, 2,056 dollars and 5701dollars in Germany, and 5,125 US dollars, 6,751 US dollars and 14, 622 US dollars in the UK, for stage I, II and III patients, respectively. The major medical cost driver in the UK was outpatient consultations, whereas hospitalisations were the major component in Germany and France. The major cost in the UK arose from the dependence of patients with MS on caregivers, which caused high non-medical, societal costs compared with France and Germany. From both the societal and health insurance perspectives in each country, costs for control patients were lower than those for stage I MS patients. MS represents a major financial burden on the individual, the family, health services and society, and these costs increase with MS progression.

Adult↗

Randomised trial of oral and intravenous methylprednisolone in acute relapses of multiple sclerosis.

BACKGROUND: An intravenous rather than oral course of methylprednisolone is often prescribed for treating acute relapses in multiple sclerosis (MS) despite the lack of evidence to support this route of administration. Our double-blind placebo-controlled randomised trial was designed to compare the efficacy of commonly used intravenous and oral steroid regimens in promoting recovery from acute relapses in MS. METHODS: 42 patients with clinically definite relapse in MS received oral, and 38 intravenous, methylprednisolone. Clinical measurements at entry and at 1 week, 4 weeks, 12 weeks, and 24 weeks included Kurtzke's expanded disability status scale (EDSS), Hauser's Ambulatory Index, and an arm-function index. The primary outcome criterion was a difference between the two treatment groups of one or more EDSS grades at 4 weeks. FINDINGS: There were no significant differences between the two groups at any stage of the study in any measurement taken: the mean difference in EDSS at 4 weeks (adjusted for baseline level) was 0.07 grades more in those taking oral steroids (95% CI -0.46 to 0.60). The most optimistic outcome for intravenous therapy is an average benefit of less than half a grade improvement on EDSS over oral treatment. INTERPRETATION: Since our study did not show any clear advantage of the intravenous regime we conclude that it is preferable to prescribe oral rather than intravenous steroids for acute relapses in MS for reasons of patient convenience, safety, and cost.

Administration, Oral↗

Circulating tumor necrosis factor-alpha correlates with electrodiagnostic abnormalities in Guillain-Barré syndrome.

Autoimmune damage to peripheral nerves, mediated by activated T lymphocytes and macrophages, underlies the pathogenesis of inflammatory demyelination in Guillain-Barré syndrome. Both T lymphocytes and macrophages secrete tumor necrosis factor-alpha, a cytokine that exerts toxic effects on myelin, Schwann cells, and endothelial cells. The reportedly high serum levels of this cytokine in patients with Guillain-Barré syndrome may reflect the degree of immune activation rather than a direct pathogenic effect. We compared serum levels of tumor necrosis factor-alpha, interleukin-1 beta, and soluble interleukin-2 receptor with well-established electrodiagnostic criteria for primary demyelination in 23 patients with Guillain-Barré syndrome, to assess the relationship between these cytokines and peripheral myelin damage. High serum levels of tumor necrosis factor-alpha were associated with prolonged distal motor latencies and slowed motor conduction velocities, prolonged or absent F-wave responses, and reduced amplitude of distal compound muscle action potentials. No significant correlation was observed between electrodiagnostic criteria for primary demyelination and serum levels of interleukin-1 beta or soluble interleukin-2 receptor. These findings suggest a putative role of tumor necrosis factor-alpha in the pathogenesis of peripheral nerve demyelination in Guillain-Barré syndrome.

Adult↗

Health outcome and quality-of-life measurements in amyotrophic lateral sclerosis.

Quality of life has been used as a primary outcome measure in the treatment of cancer and cardiovascular disease, and as a secondary outcome measure in therapy of Parkinson's disease. However, it has been relatively neglected in studies of amyotrophic lateral sclerosis (ALS). Although there is need for the development of an ALS-specific quality-of-life measure, it will be necessary, nonetheless, to continue to use generic measures in order to ensure comparability of measurement between disease states. An argument is put forward for the use of quality-of-life measures as a primary end-point in future clinical trials in ALS. A distinction is drawn between the demonstration of biological efficacy and clinically useful benefit. The most likely instruments to prove useful are briefly discussed.

Amyotrophic Lateral Sclerosis↗

Fasciculations: what do we know of their significance?

Fasciculations are observed in patients with neurogenic disorders and in healthy individuals. Depending on the associated clinical symptoms and signs, they may signify the presence of a variety of disorders of the lower motor neuron. Divergent and occasionally conflicting opinions prevail regarding the aetiology, pathogenesis, clinical significance, neurophysiological characteristics and the physiological site of origin of fasciculations. In this review we examine the published literature and attempt to clarify these issues.

Amyotrophic Lateral Sclerosis↗

Hypokalaemic myopathy in alcoholism.

We describe the clinical and pathological features of a patient with an acute painless proximal myopathy due to hypokalaemia associated with alcoholism. There was an excellent response to treatment with potassium supplements. The importance of recognition of low potassium states in alcohol-dependent patients with muscular weakness is emphasized.

Adult↗

HTLV-1 polymyositis.

The case is described of an HTLV-1 seropositive Jamaican woman who presented with signs and symptoms of polymyositis and myelopathy. A muscle biopsy showed features of myositis with a mononuclear inflammatory infiltrate, variation in fibre size and evidence of regeneration. Immunocytochemistry showed the mononuclear cells were composed of macrophages and T-lymphocytes suggesting a cell-mediated response. Multiplex PCR demonstrated the presence of the HTLV-1 tax gene within the muscle.

Biopsy↗

Paradoxical puborectalis contraction is a feature of constipation in patients with multiple sclerosis.

OBJECTIVE: To study the disturbed anorectal physiology associated with constipation in multiple sclerosis. METHODS: Anorectal function in 10 patients with clinically definite multiple sclerosis and constipation has been compared with 10 normal persons and 11 patients with idiopathic constipation, without multiple sclerosis. RESULTS: All 10 constipated patients with multiple sclerosis had difficulty evacuating barium paste during defaecography. In four of these there was complete failure of puborectalis relaxation when straining to defaecate, and in another four there was incomplete puborectalis relaxation. There was no evidence of lower motor neuron involvement of pelvic floor muscles in the multiple sclerosis group. CONCLUSIONS: Paradoxical puborectalis contraction is common in patients with multiple sclerosis in whom constipation is a symptom. This may be a feature of the disturbed voluntary sphincter control mechanism, analogous to detrusor sphincter dyssnergia in the bladder.

Adult↗

CT muscle imaging and the clinical assessment of neuromuscular disease.

Twenty patients with neurogenic disorders, polymyositis, or muscular dystrophies were assessed clinically and by CT imaging of limb, limb girdle, and trunk muscles, using a standard protocol. On each side of these patients 26 movements were graded by the MRC scale, and 20 muscles were assessed by CT imaging. The clinical and CT findings could be compared, in a blind evaluation, in 10 muscles on each side. A quantitative assessment of the CT muscle images were also made. The CT images showed striking abnormalities, even in many muscles of normal strength by clinical testing. Asymmetrical involvement of muscles was found in all the disorders studied, even when not suspected on clinical examination. Muscles in patients with muscular dystrophy were more abnormal than those in patients with neurogenic disorders. In polymyositis the attenuation values were intermediate to the other two groups. A "washed-out" appearance with very low attenuation values was very suggestive of muscular dystrophy. Involvement of paraspinal and rectus abdominis muscles was uncommon in neurogenic disorders. The gracilis muscle was relatively resistant to degeneration. CT imaging can enhance the clinical assessment of patients with neuromuscular disease, often revealing unexpected abnormalities.

Amyotrophic Lateral Sclerosis↗

Involvement of the external anal sphincter in amyotrophic lateral sclerosis.

In amyotrophic lateral sclerosis the striated pelvic floor sphincter muscles are functionally uninvolved, and pathological studies have confirmed the relative resistance of the Onuf nucleus motor neurons. We have evaluated the external anal sphincter (EAS) muscle in 16 patients with ALS using single fiber EMG, and compared the results with the findings in the semimembranosus-semitendinosus (SM-ST) muscles that have innervation from the L-5, S-1, and S-2 segments. The results were compared with a group of controls matched for age and sex. None of the patients or controls had symptomatic sphincter involvement and none of the 4 women studied were parous. Eight patients with ALS showed an increased fiber density in the EAS; 6 had an abnormal neuromuscular jitter. In 1 there was fibrillation in the EAS. In the SM-ST muscle 11 patients showed an increased fiber density, and 7 had an abnormal neuromuscular jitter. In 3 patients with ALS in whom there were abnormal findings in the EAS the bulbocavernosus reflex and pudendal nerve evoked potentials were normal. Neurogenic change was more marked in the SM-ST than in the EAS muscle. These findings show that the EAS is not normal in ALS. However, the relative resistance of the EAS to ALS is sufficient to prevent incontinence, even in the longer-surviving older patients.

Adult↗

Prevalence of bowel dysfunction in patients with multiple sclerosis and bladder dysfunction.

Urinary dysfunction is common in cases of multiple sclerosis (MS). The close proximity of those neural pathways which control the bladder to those which control anorectal function might be expected to lead to a high coexistence of bladder and bowel symptoms. Seventy-seven consecutive patients with clinically definite MS attending a uroneurology clinic were interviewed about their bowel function. All patients had clinical evidence of spinal cord disease with varying degrees of impaired mobility and sufficiently severe disturbance of bladder control to seek medical advice. Thirty-six per cent of these patients had constipation. Twenty per cent had "current incontinence", although another 30% had had at least one episode of faecal incontinence more than 3 months previously. Some patients had both constipation and faecal incontinence. A total of 52% currently had at least one bowel symptom. The pattern of bowel symptoms did not correlate with the pattern of urinary disturbance, or the duration of MS, or the degree of disability. Bowel symptoms are common in patients with MS, but even in those with urinary dysfunction are not universal. Whereas bladder dysfunction in MS is clearly related to spinal cord disease, the neurological basis for the bowel dysfunction is less clear.

Adult↗