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At least 235 records · Page 13Linked to original sources
PubMed1983

Breathing patterns during curare-induced muscle weakness.

This study examines the pattern of breathing used by normal subjects to compensate for an acute decrease in muscle strength. A continuous infusion of curare was used to reduce peak inspiratory pressure in six normal subjects from normal control levels to -45 cm H2O (moderate weakness) and to -70 cm H2O (mild weakness). Before administration of curare, inspiratory pressure exceeded -120 cm H2O. A canopy-computer-spirometer system was used for noninvasive spirometry and measurements of gas exchange. Partial curarization to a mild level of muscle weakness did not produce significant changes in the respiratory functions studied. With a moderate level of muscle weakness, there were significant increases in tidal volume from 166 to 186 ml/m2 and in inspiratory time from 1.51 to 1.71 sec (P less than 0.05). Minute ventilation and inspiratory flow did not change. However, when given 3% CO2, both normal and partially curarized subjects increased minute ventilation, from 2.3 to 5.7 L/min/m2 and from 2.5 to 6.7 L/min/m2, respectively. The increases in both conditions were secondary to increases in tidal volume. There was also a small increase in respiratory frequency from 15.4 to 18 breaths/min, P less than 0.01 in the partially curarized group given 3% CO2. Because minute ventilation was preserved while vital capacity decreased, it is proposed that respiration is maintained in the presence of muscle weakness associated with curare by diaphragmatic function which remains relatively unaffected by curarization.

Adult↗
PubMed1987

Adult onset supranuclear ophthalmoplegia, cerebellar ataxia, and neurogenic proximal muscle weakness in a brother and sister: another hexosaminidase A deficiency syndrome.

An Ashkenazi Jewish brother and sister developed progressive ataxia and proximal neurogenic muscle weakness, associated with supranuclear ophthalmoplegia, in the fourth decade of life. Hexosaminidase A activity, assayed using both synthetic and natural substrates, was severely reduced in the patients' plasma, leukocytes, and skin fibroblasts. Enzyme activity in their parents was in a similar range to that seen in heterozygotes for Tay-Sachs disease. The increasing evidence for marked clinical and molecular heterogeneity in the GM2 gangliosidoses warrants their consideration in the diagnosis of multisystem degenerative neurological disorders, even if onset of symptoms is in adult life.

Age Factors↗
PubMed1990

A noninvasive procedure to detect muscle weakness in the mdx mouse.

The forward pulling tension exerted by individual mice was measured nearly isometrically in a simple apparatus designed to determine whole body tension (WBT). WBT determinations on control (C57Bl10/SnJ) and experimental (C57Bl10-mdx) mice indicate a muscle weakness which lasts throughout the lifespan of mdx mice. Direct muscle stimulation experiments in vivo also showed significant decreases in peak twitch and tetanic tensions in adult mdx muscle with no obvious alterations in twitch time course or in twitch: tetanus ratios. We suggest that the noninvasive WBT procedure may be used to partially assess various therapies on this murine model of Duchenne muscular dystrophy.

Animals↗
PubMed1999

Case of the month: February 1999--54 year old man with severe muscle weakness.

A 54 year old man developed rhabdomyolysis one year after beginning treatment with a combination of lovastatin (an HMGCoA reductase inhibitor) and niacin. Muscle biopsy showed a severe necrotizing myopathy affecting both fibre types. Recovery occured gradually with cessation of medication. The spectrum of cholesterol lowering agent myopathy may include delayed cases of unusual severity.

Biopsy↗
PubMed2006

Fukutin gene mutations cause dilated cardiomyopathy with minimal muscle weakness.

OBJECTIVE: The fukutin gene (FKTN) is the causative gene for Fukuyama-type congenital muscular dystrophy, characterized by rather homogeneous clinical features of severe muscle wasting and hypotonia from early infancy with mental retardation. In contrast with the severe dystrophic involvement of skeletal muscle, cardiac insufficiency is quite rare. Fukuyama-type congenital muscular dystrophy is one of the disorders associated with glycosylation defects of alpha-dystroglycan, an indispensable molecule for intra-extra cell membrane linkage. METHODS: Protein and functional analyses of alpha-dystroglycan and mutation screening of FKTN and other associated genes were performed. RESULTS: Surprisingly, we identified six patients in four families showing dilated cardiomyopathy with no or minimal limb girdle muscle involvement and normal intelligence, associated with a compound heterozygous FKTN mutation. One patient died by rapid progressive dilated cardiomyopathy at 12 years old, and the other patient received cardiac implantation at 18 years old. Skeletal muscles from the patients showed minimal dystrophic features but have altered glycosylation of alpha-dystroglycan and reduced laminin binding ability. One cardiac muscle that underwent biopsy showed altered glycosylation of alpha-dystroglycan similar to that observed in a Fukuyama-type congenital muscular dystrophy patient. INTERPRETATION: FKTN mutations could cause much wider spectrum of clinical features than previously perceived, including familial dilated cardiomyopathy and mildest limb girdle muscular dystrophy.

Adult↗
PubMedDate not supplied

Muscle weakness caused by an iodine-deficient diet: investigation of a nutritional myopathy.

Newborn rats raised on a commercially available iodine-deficient diet developed severe muscle weakness, affecting predominantly their proximal hind limbs, that electrophysiologically and morphologically was determined to be myopathic in type. Follow-up dietary studies, utilizing different combinations of vitamins, minerals, casein and elemental iodine, demonstrated that the myopathy was the result of a deficiency of multiple dietary constituents, particularly casein, and was not due to a deficiency of iodine alone. These findings were compared with those observed in earlier investigations of a variety of nutritional myopathies. In the laboratory study of animals raised on experimental diets, it becomes important to consider the possible contributions of multiple dietary deficiencies in the evaluation of any abnormalities found.

Animals↗
PubMed1989

[Cachexia, pain and muscle weakness in a 69-year-old woman].

The case is reported of a 69-year-old woman who for the last 2 years of her life had severe muscle weakness (with elevated muscular enzymes), thyroid abnormalities (autoantibodies and increase in TSH) and a slowly growing mass in the anterior mediastinum. She died in cardiogenic shock. The diagnosis of "nodular polymyositis" (painful nodules palpable in the muscles) with heart involvement was strongly suspected. The thyroid abnormalities were considered to be "biological thyroiditis" i.e. a "disease" frequent in normal females (5-10%) and, after differential diagnosis of anterior mediastinal masses, the diagnosis of thymoma was adopted in view of its frequent association with autoimmune diseases. Moreover, in rare cases of thymoma associated with polymyositis, histology has disclosed "giant cell polymyositis". Autopsy revealed giant cell polymyositis, a thymoma and lymphocytic infiltration of the thyroid.

Aged↗
PubMed1991

Adult onset lipid storage in gastric mucosa and skeletal muscle fibers associated with gastric pain, progressive muscle weakness and partial deficiency of cytochrome C oxidase.

We report on the first case, a 21-year-old man, with partial deficiency of cytochrome c oxidase, lipid storage myopathy and concomitant lipid storage in the gastric mucosa affecting chief, parietal, and argentaffine cells as well as interstitial macrophages. The clinical symptoms consisted of increasing muscle weakness, cramps of the legs, and severe gastric pain that was resistant to treatment. Muscle biopsy specimens showed severe lipid storage in muscle fibers. Enzyme histochemistry revealed partial deficiency of cytochrome c oxidase (COX) with scattered non-reactive fibers among a majority of COX-positive fibers whereas biochemical analysis of muscle homogenates resulted in no corresponding defect of mitochondrial enzymes. Gastric biopsy specimens showed similarly to muscle fibers an extensive accumulation of lipid droplets in the chief cells, HCl producing parietal cells, macrophages, neutrophilic and eosinophilic leucocytes, and to a lesser degree also in argentaffine cells and unmyelinated axons of the gastric mucosa. The lipid droplets were associated with an insignificant increase in the number and size of mitochondria although paracristalline mitochondrial inclusions were neither noted in muscle fibers nor in cells of the gastric mucosa. These findings resemble those in multisystem triglyceride storage disease although the clinical signs were not reminiscent of this disease, and indicate that among the clinically heterogeneous group of cytochrome c oxidase deficiencies lipid storage may not be confined to muscle, but can affect the gastric mucosa as well.

Adult↗
PubMed1988

Genetic deficiency of short-chain acyl-coenzyme A dehydrogenase in cultured fibroblasts from a patient with muscle carnitine deficiency and severe skeletal muscle weakness.

Genetic deficiency of short-chain acyl-coenzyme A (CoA) dehydrogenase activity was demonstrated in cultured fibroblasts from a 2-yr-old female whose early postnatal life was complicated by poor feeding, emesis, and failure to thrive. She demonstrated progressive skeletal muscle weakness and developmental delay. Her plasma total carnitine level (35 nmol/ml) was low-normal, but was esterified to an abnormal degree (55% vs. control of less than 10%). Her skeletal muscle total carnitine level was low (7.6 nmol/mg protein vs. control of 14 +/- 2 nmol/mg protein) and was 75% esterified. Mild lipid deposition was noted in type I muscle fibers. Fibroblasts from this patient had 50% of control levels of acyl-CoA dehydrogenase activity towards butyryl-CoA as substrate at a concentration of 50 muM in a fluorometric assay based on the reduction of electron transfer flavoprotein. All of this residual activity was inhibited by an antibody against medium-chain acyl-CoA dehydrogenase. These data demonstrated that medium-chain acyl-CoA dehydrogenase accounted for 50% of the activity towards the short-chain substrate, butyryl-CoA, under these conditions, but that antibody against that enzyme could be used to unmask the specific and virtually complete deficiency of short-chain acyl-CoA dehydrogenase in this patient. Fibroblasts from her parents had intermediate levels of activity towards butyryl-CoA, consistent with the autosomal recessive inheritance of this metabolic defect.

Acyl-CoA Dehydrogenase↗
PubMedDate not supplied

Carnitine deficiency with hyperbilirubinemia, generalized skeletal muscle weakness and reactive hypoglycemia in a patient on long-term total parenteral nutrition: treatment with intravenous L-carnitine.

Low levels of plasma carnitine and reduced urinary carnitine excretion with persistently elevated plasma bilirubin levels, reactive hypoglycemia and generalized skeletal muscle weakness are described in a patient requiring long-term total parenteral nutrition (TPN). Intravenous administration of L-carnitine at 400 mg/day for 7 days and subsequently a maintenance dose of 60 mg/day corrected the plasma carnitine deficiency and reactive hypoglycemia and was associated with a return to normal plasma bilirubin levels and a restoration of skeletal muscle strength.

Adult↗