SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Hyperkalemia is a commonly encountered electrolyte disturbance in patients with renal insufficiency. It develops very rapidly when potassium is supplemented while a patient is on a potassium-sparing diuretic. Most often it remains asymptomatic and manifests in the form of electrocardiographic changes. Muscle weakness and paralysis although described is seldom observed in clinical practice. We report one such case.
A thirteen-year-old girl with normokalaemic familial periodic paralysis (FPP) suffered life threatening upper airway obstruction secondary to tonsillopharyngitis resulting from infectious mononucleosis (IM). Emergency tonsillectomy was performed, but her postoperative course was complicated by persistent muscle weakness requiring a very prolonged period of artificial ventilation.
Limb lengthening by distraction osteogenesis has a high complication rate. Much of the response of muscle and nerve to distraction is still unknown. Thirteen children, mean age 12.6 yr (8.4-17.3) were surgically treated by the Ilizarov procedure for acquired and congenital femoral limb-length discrepancy. All children showed a decrease in muscle strength in the quadriceps, shortly after the operation, followed by an improvement before distraction started. After an elongation in the early phase of distraction (1 to 2 cm), muscle weakness was again observed and the muscle strength gradually increased after ending of distraction. To provide an explanation for this clinical observation, in one patient (limb lengthening of 4.1 cm) muscle strength measurements were extended with investigations of Hoffman (H) reflex of m. vastus medialis and determination of muscle-fiber conduction velocity of m. vastus lateralis by using the invasive method (IMFCV). The examinations were performed every two weeks during 20 weeks and 12 weeks after removing the cast. A severe decrease in muscle strength of the corrected limb was found after 1.2 cm of distraction with a recovery in muscle strength before lengthening was ended. EMG study showed the same tendency. Denervation was observed as evidence by positive sharp waves and reduced IMFCV findings. Evidence for reinnervation before lengthening was ended, was found by an increased range of velocities consisting of a combination of slow potentials and gradual increase of the velocity of reinnervated fibers (increased Fast/Slow ratio). The latencies of M waves and H-M interval from both legs separated as well after 2.25 cm of distraction. At the end of the follow-up period, the H-M interval reached the preoperative value. It is suggested that these neurogenic changes are an effect of axonal dysfunction and the local effect due to intraoperative trauma and stretching might affect nerve blood flow adversely.
To date, very few studies have investigated the role of muscle dysfunction in the pathogenesis of osteoarthritis (OA). Using largely indirect evidence, this article hypothesizes that motor and sensory dysfunction of muscle may be important factors in the pathogenesis of articular damage and are not simply a consequence of joint damage. A new paradigm is constructed to better describe the complex interrelationship between muscle sensorimotor dysfunction, joint damage, and disability in OA. If the hypothesis is correct, because muscle is a relatively plastic tissue, maintaining well-conditioned muscles may delay or prevent the onset of OA, and rehabilitation exercise therapy that reverses muscle sensorimotor dysfunction may ameliorate the effects of OA.
Polymyositis and dermatomyositis are idiopathic inflammatory myopathies. Respiratory complications are a common feature, but ventilatory insufficiency is rare in these patients. We describe here three patients diagnosed with inflammatory myopathy (polymyositis) with respiratory failure due to muscle weakness who did not respond to immunosuppressive therapy. Mechanical ventilation at home with nasal or tracheal intermittent positive pressure resulted in improved chronic hypoventilation. This treatment improves the quality of life of patients with inflammatory myopathies and can be lifesaving in some cases.
BACKGROUND: It has been suggested that cough effectiveness can be improved by assisted techniques. The effects of manually assisted cough and mechanical insufflation on cough flow physiology are reported in this study. METHODS: The physiological actions and patient self-assessment of manually assisted cough and mechanical insufflation were investigated in 29 subjects (nine normal subjects, eight patients with chronic obstructive pulmonary disease (COPD), four subjects with respiratory muscle weakness (RMW) with scoliosis, and eight subjects with RMW without scoliosis). RESULTS: The peak cough expiratory flow rate and cough expiratory volume were not improved by manually assisted cough and mechanical insufflation alone or in combination in normal subjects. The median increase in peak cough expiratory flow in subjects with RMW without scoliosis with manually assisted cough alone or in combination with mechanical insufflation of 84 l/min (95% confidence interval (CI) 19 to 122) and 144 l/min (95% CI 14 to 195), respectively, reflects improvement in the expulsive phase of coughing by these techniques. Manually assisted cough and mechanical insufflation in combination raised peak expiratory flow rate more than either technique alone in this group. The abnormal chest shape in scoliotic subjects and the fixed inspiratory pressure used made effective manually assisted cough and mechanical insufflation difficult in this group and no improvements were found. In patients with COPD manually assisted cough alone and in combination with mechanical insufflation decreased peak expiratory flow rate by 144 l/min (95% CI 25 to 259) and 135 l/min (95% CI 30 to 312), respectively. CONCLUSIONS: Manually assisted cough and mechanical insufflation should be considered to assist expectoration of secretions in patients with RMW without scoliosis but not in those with scoliosis.
UNLABELLED: We examined the relation of maximum phonation time (MPT) and vital capacity (VC) and reviewed the usefulness of MPT as a respiratory function screening. SUBJECTS: 18 healthy adult subjects (8 men and 10 women), and 32 myopathic patients (24 men and 8 women). METHODS: MPT and VC were measured in sitting position. Six patients were tested with and without air stacking by glossopharyngeal respiration. RESULTS: In healthy subjects, MPT was 29.9 +/- 11.8 seconds in men and 21.7 +/- 7.8 seconds in women. Second trials showed good reproducibility. The healthy group had no correlation between MPT and VC. The patient group showed a significant positive correlation between MPT and VC (r2= 0.25, p=0.003). All patients with MPT less than 15 seconds showed VC less than 1.5 l and %VC less than 50%. Air-stacking by glossopharyngeal respiration significantly increased the MPT. CONCLUSIONS: MPT is a useful screening test for respiratory muscle weakness. The patients are easily aware of the effect of air-stacking by glossopharyngeal respiration.
Explore the source record for details and available documents.
Four cases of patients who described an unpleasant subjective experience of weakness and reduced muscle tone during treatment with clozapine are presented. An exacerbation of muscular dystrophy during clozapine treatment is also described. It is hypothesized that these adverse effects are related to the muscle relaxant properties of clozapine. The differential diagnosis of sedation, fatigue, asthenia, and reduced muscle tone is discussed.
The objective of this study was to evaluate the presence of progressive postpoliomyelitis muscle weakness (PPMW) in affected individuals 20 to 40 years after the initial polio infection. Over a three-year period, the isometric and isokinetic strength of the quadriceps femoris muscle was studied in seven symptomatic patients with previous poliomyelitis (mean = 38.3 years from infection) to determine if quadriceps strength decreased during the three years. Each patient had a quadriceps affected by polio on one side and a clinically nonaffected quadriceps on the contralateral limb. The maximal isometric force and the peak isokinetic force of the affected quadriceps (AQ) and nonaffected quadriceps (NQ) muscles were tested on a computerized isokinetic dynamometer machine at six-month intervals. Isometric force increased significantly, by 29% per year (p less than .02) in the AQ and by 14% per year (p less than .05) in the NQ. Paired analysis to determine the change in strength between the affected and nonaffected muscles for the isometric data showed a mean nonsignificant increase in the AQ of 14% per year (p = .01). The change in peak isokinetic force demonstrated a significant increase in the AQ of 35% per year (p less than .05); whereas, the NQ peak isokinetic force increased 15% per year which was not statistically significant. Paired analysis to determine the change in strength between the affected and nonaffected muscles for the isokinetic data showed a nonsignificant relative increase in the AQ of 20% per year (p less than .06).(ABSTRACT TRUNCATED AT 250 WORDS)
BACKGROUND: A 56-year-old woman was referred to a neurogenetic clinic with a history of stiffness and transient weakness. A previous needle electromyogram had confirmed the presence of myotonia, but a muscle biopsy had revealed no evidence of dystrophy. INVESTIGATIONS: Neurological examination, electrophysiological studies and genetic testing. DIAGNOSIS: Recessive myotonia congenita (Becker's disease). MANAGEMENT: Explanation of the nature of the disease and treatment with mexiletine 200 mg twice daily.
Explore the source record for details and available documents.
A 16-year-old woman was admitted to our hospital because of abdominal pain, paresis of extremities, and muscle weakness. Bartter's syndrome was suspected because of the features of the hypokalemia, hyperaldosteronism, hyperreninemia, increased concentration of plasma angiotensin I & II, the defect in distal fractional reabsorption of chloride and normotension. The concentrations of the plasma angiotensin II and aldosterone, however, were decreased to normal levels after admission probably due to a decrease in the salt intake because of the regular hospital diet. Furthermore, the hyperplasia of the juxtaglomerular apparatus was not found. Therefore, at first we thought we were dealing with another disease, that is pseudo-Bartter's syndrome, which is caused by different pathogenesis. Gill et al. reported that the defect in distal fractional reabsorption of chloride was a characteristic feature in the diagnosis of the Bartter's syndrome. Thus, we tried to explain the clinical symptoms and diagnosis of this case as the Bartter's syndrome according to the theory of Gill et al. If the defect of chloride reabsorption was the only pathogenesis of the Bartter's syndrome, other symptoms seen in our case could be thought of as secondary (or new) changes. Therefore, this case could be differentiated from the pseudo-Bartter's syndrome or the "true" Bartter's syndrome because of the clinical symptoms and the defect in chloride reabsorption.
Tau, one of the major neuronal microtubule-associated proteins (MAPs), is important for neuronal cell morphogenesis and axonal maintenance. Tau is also known to be a component of the paired helical filaments (PHFs) in Alzheimer's disease patients. Recently, mutations in the tau gene were found in a hereditary neurodegenerative disease called frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) which exhibits various neurological and neuropathological characteristics including PHF-like intracellular tau deposit formation. Currently, the phenotype of the disease is thought to be due to: (1) the toxicity of mutant tau molecules and and/or; (2) the loss of function of normal tau molecules in patients' brains. To test the latter hypothesis, we performed behavioral and neurological tests on tau-deficient mice. Tau-deficient mice showed muscle weakness in the wire-hanging test, hyperactivity in a novel environment, and impairment in the contextual fear conditioning. They also had a tendency to fall more easily in the rod-walking test. These phenotypes parallel some signs and symptoms of FTDP-17 patients. Our results show that the loss of tau protein may itself lead to some of the neurological characteristics observed in FTDP-17 patients.
Resting energy expenditure (REE) was assessed and its relationship to nutritional status, pulmonary function and respiratory muscle function was studied in 25 patients with pulmonary emphysema. The mean value of REE was 1413 +/- 251 Cal and the ratio of REE/REEpred was 1.398 +/- 0.23, suggesting the existence of a hypermetabolic state in these patients. REE/REEpred ratio was inversely correlated with plasma amino acid BCAA/AAA ratio and body muscle mass; and PImax. REE was inversely correlated with FEV1.0%. REE in the patient subgroup with severe hyperinflation (%RV > or = 200) was significantly higher than that in the subgroup with moderate hyperinflation. Malnourished patients showed significantly more severe hyperinflation than well-nourished patients. These findings suggest that augmented REE contributes to malnutrition in patients with emphysema, and that the increase in REE was related to the increase in mechanical work load on the basis of airway obstruction, respiratory muscle weakness and hyperinflation.
BACKGROUND: It is well established that patients with longstanding weakness of the respiratory muscles have a reduction in lung distensibility. Although this occurs in most patients without any radiographic changes suggesting parenchymal lung disease, it has been attributed to the development of microatelectasis. METHODS: A high resolution computed tomographic (CT) scanner was used in eight patients with traumatic tetraplegia and six patients with generalised neuromuscular disorders to look for areas of atelectasis. With the patient in the supine posture scans of 1 mm thickness were obtained at total lung capacity at intervals of 1 cm from the apex to the base of the lung. RESULTS: Vital capacity, total lung capacity, and inspiratory muscle strength were reduced to a mean of 59.5%, 73.9%, and 51.1% of predicted values, respectively. Static expiratory lung compliance was decreased in 12 of the 14 patients and averaged 69.1% of the predicted value. The CT scans revealed only small areas of atelectasis in one tetraplegic patient and in one patient with a generalised neuromuscular disorder; no parenchymal abnormality was seen in the other 12 patients. CONCLUSIONS: In many patients with chronic weakness of the respiratory muscles the reduced lung distensibility does not appear to be caused by microatelectasis. It might be related to alterations in elasticity of the lung tissue.
We report 27 children, aged 14 months to 9 years, who had megalencephaly, hypotonia, proximal muscle weakness, speech and motor delay, and increased intracellular lipid (myoliposis) in needle muscle biopsy specimens. The patients had many features of the Ruvalcaba-Myhre-Smith syndrome, and in 17 families we confirmed the autosomal dominant inheritance pattern previously suggested. Muscle carnitine content was low in all 11 patients and all 4 affected relatives tested. All 27 probands were treated with oral L-carnitine; a clinical response was noted in 17. We speculate that myoliposis may be found in other disorders with megalencephaly and muscle symptoms. In such cases, muscle carnitine deficiency should be considered. The reason for the reduced muscle carnitine content is not known.
Explore the source record for details and available documents.