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Results for “Muscle Weakness”

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At least 109 records · Page 6Linked to original sources

PubMed2002

Isometric strength measurement for muscle weakness in cancer patients: reproducibility of isometric muscle strength measurements with a hand-held pull-gauge dynamometer in cancer patients.

Our aim was to determine several indexes of reproducibility for strength measurements with a hand-held pull-gauge dynamometer (MFB50K) in cancer patients. Two independent testers performed repeated measurements of maximal isometric elbow and knee strength. The measurements were gathered in a convenience sample of 40 patients (27 men, age 20-72 years) with various types and stages of cancer. These patients were recruited from the medical oncology clinic of a university hospital. The Pearson product moment correlation coefficient, the intraclass correlation coefficient (ICC), the standard error of measurement (SEM) and its associated 95% confidence interval, the smallest detectable difference (SDD) and the limits of agreement were determined as outcome measures for reproducibility. The results for the Pearson product moment correlation and the ICC were 0.90 for elbow and 0.96 for knee extension strength. The results in terms of the SDDs were 29.4 newton (N) for elbow and 54.8 N for knee strength. The limits of agreement for elbow strength were -21.7 N and 38.5 N (mean 8.4 N). For knee strength the limits of agreement were -49.8 N and 59.8 N (mean 5 N). The Pearson product moment correlation and the ICC suggested good reproducibility. However, the more informative indices of reproducibility, i.e. the SEM and the SDD, showed relatively large measurement error between the testers. Therefore, the current use of the MFB50K for the measurement of muscle strength is not supported in cancer patients.

Adult↗

PubMed2000

[Respiratory muscle weakness after prolonged use of hydrocortisone and pancuronium bromide].

A 30-year-old man was admitted because of status asthmaticus. He required 7 days of artificial ventilation and was treated with hydrocortisone 1.2 g.day-1 and bronchodilaters. Pancuronium bromide 0.08 mg.kg-1.hr-1 was given for 64 hours for the ease of artificial ventilation. On day 3, severely elevated airway pressure resulted in left pneumothorax and isoflurane 1% was given for the following 2 days. Respiratory muscle weakness was evident 24 hours after discontinuation of pancuronium infusion on day 5 while full 4 twitches of TOF on the adductor pollicis muscle were seen at the time. The respiratory muscle weakness continued for another 3 days and he was extubated on day 8. Serum creatine kinase concentration rose to 2178 U.l-1 on day 6 and returned to normal on day 11. Hematurea, hyperpyrexia and metabolic acidosis were never seen during the course. Acute corticosteroid myopathy was suspected to be the cause of the prolonged respiratory muscle weakness.

Acute Disease↗

PubMed1990

[Mitochondrial encephalomyopathy (focal cytochrome c oxidase deficiency) with transient episodes of muscle weakness and elevation of serum creatine kinase activity].

A 17-year-old boy who had mitochondrial encephalomyopathy with focal deficiency of cytochrome c oxidase (CCO) activity is described. He experienced 3 episodes of muscle weakness, fatigability, nausea, vomiting and concomitant increase of serum creatine kinase activity, at the age of 13, 15 and 17 years. During interval there was no muscle weakness and the serum creatine kinase activity was within normal range. Increased levels of lactic acid and pyruvic acid were observed in the blood and cerebrospinal fluid. After an aerobic exercise test, lactic acid and pyruvic acid in the blood increased to an abnormally high level, and the arterial blood became acidic (pH 7.297). On EEG, occasional intermittent irregular theta activities were observed in the anterior region, but there were no abnormalities on CT and MRI in the central nervous system. In the biopsied muscle, ragged-red fibers comprised 20% on modified Gomori-trichrome staining and a number of fibers with no CCO activity were scattered throughout. The CCO activity in the mitochondria isolated from the biopsied muscle was reduced to 49.2 nmol/min/mg protein (normal range 144.7-355.8), while other mitochondrial enzyme activities in the electron transport system were normal. From these data, the patient was considered to have a unique form of mitochondrial encephalomyopathy. By the administration of a large amount of coenzyme Q10, episodes of muscle weakness and nausea, and an increase of lactic acid and pyruvic acid in the blood after aerobic exercise test were no longer observed.

Adolescent↗

PubMed2001

Myositis associated graft-versus-host-disease presenting as respiratory muscle weakness.

Myositis associated with graft-versus-host-disease (GVHD) typically presents with proximal muscle weakness, myalgias, and a raised creatinine phosphokinase (CPK) level. We report a case of a 51 year old man who developed respiratory muscle weakness five years after an allogeneic bone marrow transplant for multiple myeloma. His symptoms included tachypnoea, abdominal paradox, and orthopnoea. Pulmonary function tests revealed diminished vital capacity and maximal inspiratory and expiratory pressures. Serum CPK levels were raised and a peripheral muscle biopsy specimen was consistent with GVHD. He improved with immunosuppressive therapy.

Bone Marrow Transplantation↗

PubMed1999

A novel 7.4 kb mitochondrial deletion in a patient with congenital progressive external ophthalmoplegia, muscle weakness and mental retardation.

We present a patient with external ophthalmoplegia, bilateral ptosis, progressive muscle weakness with "ragged-red fibres" and mental retardation. Mitochondrial DNA analysis by Southern blot revealed heteroplasmy in muscle for a 7.4 kb deletion. In white blood cells, the deletion was only detectable by PCR. There was no evidence for duplications, nor for multiple deletions in the proband or siblings. PCR analysis did not reveal the presence of a mitochondrial DNA defect in the parents and siblings. Thus, there is no experimental support for a maternally inherited mitochondrial DNA deletion. We consider this a sporadic case with a de novo deletion. Diabetes and complaints of fatigue, also seen in this family, are probably coincidental. Mental retardation has been reported occasionally in patients with mitochondrial deletions, but is not common.

Adult↗

PubMed1981

The effects of intermittent positive pressure breathing on patients with respiratory muscle weakness.

Marked reductions in pulmonary compliance have been noted in patients with respiratory muscle weakness, which greatly contribute to the losses of lung volume. In an attempt to reverse this abnormality and to decrease the work of breathing, we evaluated the effects of a 15-min period of positive pressure, mechanical hyperinflation of the lungs in 10 patients with generalized neuromuscular disorders and long-standing involvement of the respiratory muscles. The vital capacity was 46, and the static expiratory lung compliance was 59% of control values. The recoil pressure of the lung at full inflation was 19 cm H2O. Using positive pressure breathing devices, we inflated the lungs either with inflation pressures ranging between 20 and 40 cm H2O or with volume that were at least twofold larger than the patients' natural inspiratory capacity. Lung volumes, including functional residual capacity, and static pulmonary compliance were not modified by these procedures. Maintaining 10 cm H2O of positive end-expiratory pressure did not alter the results. These findings suggest that (1) alveolar collapse, rather than a simple decrease in the compliance of the surface film of the lung, is the major cause of the low pulmonary compliance in patients with chronic respiratory muscle weakness; (2) these patients do not acutely benefit from intermittent positive breathing treatment.

Adolescent↗

PubMed2006

Hereditary hypotonia, muscle weakness, failure to thrive, and cognitive delay in a large moslem kindred.

Five infants of a Moslem-Arab extended family were evaluated for common and characteristic clinical findings of failure to thrive, extreme muscle weakness, severe motor delay, and moderate to severe cognitive and verbal delay. All children were below the third percentile in weight and height, and three of them had head circumference below the third percentile. Neurologic examination revealed severe hypotonia, muscle weakness, and absent deep tendon reflexes. Two children died at 2 years of age, and none of the children acquired full head control and the motor milestones of rolling and sitting. Laboratory evaluation including muscle biopsies, genetic studies, and metabolic evaluation was nondiagnostic.

Biopsy↗

PubMed1999

Dystonin-deficient mice exhibit an intrinsic muscle weakness and an instability of skeletal muscle cytoarchitecture.

Dystonia musculorum (dt) was originally described as a hereditary sensory neurodegeneration syndrome of the mouse. The gene defective in dt encodes a cytoskeletal linker protein, dystonin, that is essential for maintaining neuronal cytoskeletal integrity. In addition to the nervous system, dystonin is expressed in a variety of other tissues, including muscle. We now show that dystonin cross-links actin and desmin filaments and that its levels are increased during myogenesis, coinciding with the progressive reorganization of the intermediate filament network. A disorganization of cytoarchitecture in skeletal muscle from dt/dt mice was observed in ultrastructural studies. Myoblasts from dt/dt mice fused to form myotubes in culture; however, terminally differentiated myotubes contained incompletely assembled myofibrils. Another feature observed in dt/dt myotubes in culture and in skeletal muscle in situ was an accumulation and abnormal distribution of mitochondria. The diaphragm muscle from dt/dt mice was weak in isometric contractility measurements in vitro and was susceptible to contraction-induced sarcolemmal damage. Altogether, our data indicate that dystonin is a cross-linker of actin and desmin filaments in muscle and that it is essential for establishing and maintaining proper cytoarchitecture in mature muscle.

Actins↗

PubMed2005

Gene dosage-dependent transmitter release changes at neuromuscular synapses of CACNA1A R192Q knockin mice are non-progressive and do not lead to morphological changes or muscle weakness.

Ca(v)2.1 channels mediate neurotransmitter release at the neuromuscular junction (NMJ) and at many central synapses. Mutations in the encoding gene, CACNA1A, are thus likely to affect neurotransmitter release. Previously, we generated mice carrying the R192Q mutation, associated with human familial hemiplegic migraine type-1, and showed first evidence of enhanced presynaptic Ca(2+) influx [Neuron 41 (2004) 701]. Here, we characterize transmitter release in detail at mouse R192Q NMJs, including possible gene-dosage dependency, progression of changes with age, and associated morphological damage and muscle weakness. We found, at low Ca(2+), decreased paired-pulse facilitation of evoked acetylcholine release, elevated release probability, and increased size of the readily releasable transmitter vesicle pool. Spontaneous release was increased over a broad range of Ca(2+) concentrations (0.2-5mM). Upon high-rate nerve stimulation we observed some extra rundown of transmitter release. However, no clinical evidence of transmission block or muscle weakness was found, assessed with electromyography, grip-strength testing and muscle contraction experiments. We studied both adult ( approximately 3-6 months-old) and aged ( approximately 21-26 months-old) R192Q knockin mice to assess effects of chronic elevation of presynaptic Ca(2+) influx, but found no additional or progressive alterations. No changes in NMJ size or relevant ultrastructural parameters were found, at either age. Our characterizations strengthen the hypothesis of increased Ca(2+) flux through R192Q-mutated presynaptic Ca(v)2.1 channels and show that the resulting altered neurotransmitter release is not associated with morphological changes at the NMJ or muscle weakness, not even in the longer term.

Acetylcholine↗

PubMed2005

Non-collagenic etiologies of muscle weakness with joint deformities: about two paradigmatic case reports.

Muscle weakness associated to marked joint deformities is not an uncommon clinical situation in daily neuromuscular clinics. These abnormalities encompass a large variety of conditions including non-primary muscle disorders. Besides well-defined and rather readily recognisable hereditary syndromes such as Bethlem myopathy or Ullrich congenital muscular dystrophy, some unusual etiologies should also be considered. We report here two paradigmatic cases in which we found mutations in two novel genes corresponding to two newly described entities (progressive pseudorheumatoid dysplasia, PPD, and infantile systemic hyalinosis, ISH) both conditions in which the clinical picture can mimick primary muscle disease.

CCN Intercellular Signaling Proteins↗

PubMed2005

Muscle weakness and imbalance in older dizzy patients.

BACKGROUND AND AIMS: Dizziness is common in older adults, and may be associated with limitations in physical performance. The purpose of this study was to investigate if leg muscle weakness and imbalance exist in older dizzy patients. METHODS: Twenty-three older dizzy patients and an age-matched group of 16 healthy non-dizzy older adults participated in the study. Isometric strength of leg muscles, center of pressure (COP) during quiet standing, and maximal distance of reaching forward in standing were tested. All patients answered the Dizziness Handicap Inventory (DHI). RESULTS: Most patients reported that they were handicapped by dizziness (DHI mean 31.8, SD 22.1). Compared with the healthy group, they showed significantly weaker strength in hip extension, knee extension and ankle dorsiflexion (all p < 0.000). Imbalance, indicated by greater COP trajectory (p = 0.008) and anteroposterior range of displacement (p = 0.001) during quiet standing, and a smaller forward reach distance (p < 0.000), was also found in patients. CONCLUSIONS: Leg muscle weakness and imbalance were more common in older dizzy patients than in healthy subjects. Clinical management should include assessment and therapy for these problems.

Aged↗

PubMed1996

Recurrent hypokalemic muscle weakness as an initial manifestation of Wilson's disease.

A 24-year-old man had several episodes of hypokalemic muscle weakness of undetermined etiology since the age of 13 years. Wilson's disease (WD) was not diagnosed until the age of 18 when wing-beating tremor in the left upper limb was noted. Renal function study revealed incomplete proximal renal tubular acidosis. The hypokalemic muscle weakness and wing-beating tremor had subsided after long-term penicillamine therapy. The present case indicates that recurrent hypokalemic paralysis due to renal tubular acidosis is a rare initial presentation of WD and may respond to penicillamine therapy.

Acidosis, Renal Tubular↗

PubMed1998

Severe muscle weakness secondary to paraneoplastic hypophosphatemia in neuroblastoma.

We report a 28-year-old woman who presented with severe proximal muscle weakness secondary to paraneoplastic hypophosphatemia and associated with recurrent neuroblastoma. The biochemical findings included hyperphosphaturia, a reduced serum level of 1,25-dihydroxyvitamin-D3, elevated alkaline phosphatase and normocalcemia which are pathognomic for paraneoplastic hypophosphatemia. Following systemic chemotherapy and supplementation of 1,25-dihydroxyvitamin-D3 a complete remission of the neuroblastoma was achieved and all features of the paraneoplastic hypophosphatemia gradually disappeared. In the differential diagnosis of muscle weakness, hypophosphatemia should be included. Paraneoplastic hypophosphatemia associated with metastatic neuroblastoma has not been reported previously. Diagnosis, mechanism and therapy of paraneoplastic hypophosphatemia are shortly reviewed.

Adult↗

PubMed2004

Inspiratory muscle weakness in diastolic dysfunction.

OBJECTIVES: To test the hypothesis that patients with well-documented diastolic dysfunction (DD) in the setting of normal systolic function will have inspiratory muscle weakness when compared to normal control subjects, and will experience dyspnea and tachypnea during exercise. BACKGROUND: Respiratory muscle weakness has been described in patients with (systolic) congestive heart failure; however, whether or not patients with DD may present with the findings of congestive heart failure is not known. METHODS: We selected for study 14 patients with DD previously referred for cardiopulmonary evaluation whose diagnosis had been confirmed by data obtained at cardiac catheterization. Seven control subjects matched for age, sex, and weight were recruited from the hospital community. Subjects performed both basic pulmonary function tests and tests of muscle strength: handgrip strength (Hgr), and maximal subatmospheric static inspiratory muscle pressure (Pimax). Subjects then performed a graded exercise test on a bicycle ergometer. Minute ventilation, oxygen consumption, carbon dioxide production, and heart rate were monitored continuously. Echocardiography was performed three times: before exercise, at a selected submaximal exercise level (20% of a predicted maximal workload), and at maximal exercise. Subjects rated their degree of dyspnea using the Borg scale at the same three time intervals. RESULTS: Pimax was - 102 +/- 17 cm H(2)O in control subjects, and - 77 +/- 19 cm H(2)O in patients with DD (p = 0.013) [mean +/- SD]. Hgr was similar between the groups. At the selected submaximal exercise level, patients with DD rated dyspnea to be 2.6 +/- 2.2 Borg scale units (control subjects, 0.5 +/- 0.8 Borg scale units). Hey plots described a rapid, shallow breathing pattern in patients with DD during exercise. Patients with DD and control subjects achieved similar maximal work loads. CONCLUSION: Patients with DD have diminished Pimax, adopt a rapid, shallow breathing pattern during exercise, and experience dyspnea at low work loads when compared to matched control subjects.

Aged↗

PubMed2006

A boy with muscle weakness, hypercarbia, and the mitochondrial DNA A3243G mutation.

The point mutation in the mitochondrial genome tRNA(Leu_ (A3243G) is associated with the syndrome of mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS). We report a boy presenting with respiratory compromise and hypercarbia owing to severe muscle weakness. Historically, he demonstrated idiopathic growth hormone deficiency, retarded bone age, and exercise avoidance. Owing to severe respiratory compromise out of proportion to expected recovery, a metabolic work-up was performed. Muscle biopsy demonstrated abnormal mitochondria structure and heteroplasmic A3243G mutation. Idiopathic growth hormone deficiency and retarded bone age have not been previously reported in MELAS, and these findings delayed testing for mitochondrial disease. This case demonstrates that isolated muscle weakness in the context of other organ system abnormalities should make the investigator consider MELAS. (J Child Neurol 2006;21:77-79).

Biopsy↗

PubMed2002

Proximal muscle weakness--an unusual presentation of celiac disease.

The identification of the etiology of metabolic bone disease that leads to myopathy is important as this type of muscle weakness often responds fully to treatment. We present the case history of an 11-year-old girl with celiac disease who had rickets masquerading as muscle weakness and bone pains.

Biopsy↗

PubMedDate not supplied

[Respiratory system elastance and resistance measured by proportional assist ventilation in patients with respiratory muscle weakness].

OBJECTIVE: Non-invasive ventilatory therapy has prolonged survival of myopathy patients with hypoventilation. Efficacy of non-invasive ventilation depends on both elastance and resistance of the respiratory system. Although these parameters are important in the prescription of respiratory management, conventional respiratory function test does not show the appropriate answer in patients with severe respiratory muscle weakness. In muscular dystrophy, muscle tends to be shortened due to its fibrosis, when muscle becomes atrophic and weak; fibrosis of respiratory muscle tissues presumably causes high thoracic elastance. We evaluated the total respiratory system elastance and resistance during proportional assist ventilation (PAV) in myopathy patients. METHODS: In PAV with 100% assist, using BiPAP Vision ventilator, airway pressure exceeds 20 cmH2O or tidal volume exceeds 1.5 liter (run-away phenomenon) when the volume assist or the flow assist is higher than the individual elastance or the resistance, respectively. Twenty myopathy patients with ventilatory failure and 7 healthy controls were evaluated, including 7 patients with Duchenne muscular dystrophy (DMD), 2 patients with congenital myopathy (CM), 1 patient with limb-girdle muscular dystrophy (LG), 6 patients with myotonic dystrophy (MyD) and 4 patients with acid maltase deficiency (AMD). Seventeen patients used a nasal mask and 3 patients had a tracheostomy tube. Fifteen patients used a pressure-preset ventilator, and 3 patients used a volume-preset ventilator. RESULTS: In all patients with DMD, CM and LG, respiratory system elastance was higher than 20 (cmH2O/L) and than in all patients with AMD and MyD except 1 MyD patient. Follow-up measurement after half a or one year showed increase of respiratory system elastance in 2 DMD patients and 1 CM patient, but almost no change in 3 AMD patients. The elastance measured during PAV was consistent with the clinical impression of muscle shortening. One exceptional MyD patient showed extremely high elastance (more than 58 cmH2O/L), which reflected the fixed thoracic spine and increase of abdominal visceral fat. Resistance was normal in all patients except a LG patient with pulmonary aspergillosis and a history of pulmonary tuberculosis who showed 14 (cmH2O/L/s). In a CM patient who developed emphysema, resistance increased from 5 to 12 (cmH2O/L/s) in a year, although forced expiratory volume 1.0% (FEV1.0/FVC) remained normal. Respiratory system resistance measurement was useful to detect a lung disease, because obstructive disorder is underestimated with FEV1.0/FVC when vital capacity is low. CONCLUSION: The respiratory system elastance and resistance measured during PAV are useful parameters in evaluation of mechanical features of the lung, thorax and airway. It is recommended to keep both parameters normal in patients who may require ventilatory assist due to progression of respiratory muscle weakness.

Adult↗

PubMed2001

Muscle weakness in a Japanese family of Arg1239His mutation hypokalemic periodic paralysis.

Familial hypokalemic periodic paralysis is an autosomal dominant disorder. Recently, three predominant mutations were found in the muscle dihydropyridine-sensitive calcium channel alpha 1-subunit gene. We present a Japanese family that displays one of these, the Arg1239His mutation. All the affected individuals of this family displayed this mutation. Two cases had a history of recurrent episodes of muscle weakness and difficulty in running before their first paralytic attack. It is suggested that there are no racial differences with this mutation, and that mild muscle weakness occurs not only after but also preceding the first attack.

Adult↗