摘要:

AbstractA 41‐year‐old man had ichthyosis, ectropion, steatorrhea, and slowly progressive proximal limb weakness. Biopsies showed abnormal lipid accumulation in muscle, liver, skin, leukocytes, and gastric mucosa. Lipid storage was particularly marked in cultures of skin and muscle, and it increased in subsequent cell generations. By electron microscopy, the lipid globules showed no limiting membranes. The stored lipid was identified by thin‐layer chromatography as triglyceride; there was no excess of cholesterol or cholesteryl esters. Muscle carnitine concentration and activities of carnitine palmityltransferase and acid lipase were normal;14CO2production from labeled palmitate in leukocytes was not impaired. The excessive accumulation of triglyceride in different tissues and in the progeny of cells in tissue culture suggests a genetic error of lipid metab

ISSN:0148-639X    

DOI:10.1002/mus.880020102

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractForty patients with polymyositis or dermatomyositis underwent detailed electromyographic evaluation. The paraspinal muscles of all patients were examined, as were several extremity muscles. The distribution of fibrillation potentials (FPs) in different muscles is discussed. FPs were most frequent in paraspinal muscles. We conclude that, for any patient suspected of having a myopathy, electromyographic examination should include the paraspinal muscles.

ISSN:0148-639X    

DOI:10.1002/mus.880020103

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractHypoxanthine‐guanine phosphoribosyltransferase (HGPRT) activity was measured in red cells and in skeletal muscles of normal and Duchenne subjects. [8‐14C] hypoxanthine was used as substrate, and 5‐phospho‐α‐D‐ribose 1‐diphosphate (PRPP) was used as the ribose‐5‐phosphate donor. The [8‐14C] inosine monophosphate (IMP) formed was separated by high‐voltage electrophoresis, and radioactivity was measured by liquid scintillation counting. HGPRT activity in Duchenne and normal red‐cell hemolysates was similar, but such activity was significantly higher in Duchenne than in normal muscle homogenates. Red cells of both normal and Duchenne subjects had significantly higher enzyme activity than did skeletal muscles. It is suggested that increased HGPRT activity may be involved in enhancing protein synthesis by increasing intracellular levels o

ISSN:0148-639X    

DOI:10.1002/mus.880020104

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractPerhexiline maleate therapy may produce a polyneuropathy as well as other side effects in man. The Schwann‐cell origin of the neuropathy is demonstrated by morphologic studies. Ultrastructural studies of muscle and nerve biopsies showed the presence of intracytoplasmic inclusions, primarily in Schwann cells, muscle fibers, and endothelial cells. These inclusions were polymorphic and varied from one cell type to another. They were frequently associated with calcium deposits, and their structure suggested a lysosomal origin. It was possible experimentally to reproduce similar inclusions in mice. Furthermore, numerous tubular aggregates were observed in intoxicated mouse muscle fiber

ISSN:0148-639X    

DOI:10.1002/mus.880020105

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractMyosin in striated muscle exists in at least two immunologically distinct forms. Human autoantibodies specific for either form show selective muscle fiber reactivity. One group of antibodies reacts with type 2b (white) muscle fibers, the other with type 2a (red) and type 1 (intermediate) fibers. All of these antibodies react with the A band of glycerinated myofibrils. Both groups of antibodies react with cardiac muscle. Muscle‐fiber‐specific antibodies are detected in approximately 1% of sera submitted for diagnostic screening. Sera from eight patients were studied further. The two patients with antibodies against type 2b (white) muscle myosin had rheumatoid arthritis. The six patients with antibodies against type 2a (red) and type 1 (intermediate) myosin had clinical features which included various autoimmune and infectious disord

ISSN:0148-639X    

DOI:10.1002/mus.880020106

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractPalmaris longus (PML) muscles of rhesus monkeys were transplanted, with or without anastomosis of the median nerve, to the nerve stump of the autograft. Because PML autografts revascularize spontaneously, vascular anastomoses were not performed. Muscle fibers regenerated in all autografts with neuroanastomosis, but in only three of eight autografts without neuroanastomosis. Five autografts without neuroanastomosis were replaced by noncontractile connective tissue. Growth and differentiation of muscle fibers into three fiber types and development of capillarity were analyzed histochemically, and succinate oxidase activity of whole‐muscle homogenates was determined. None of these measures reached values for control PML muscles within 100 days of transplantation. In comparison to control muscles, autografts had slower times to peak tension and less absolute tension, but similar tension per square centimeter of muscle fiber cross‐sectional area. Monkey PML autografts with neuroanastomosis were similar in structure and function to cat extensor digitorum longus autografts that had not had neuroanastomo

ISSN:0148-639X    

DOI:10.1002/mus.880020107

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractA double‐blind, controlled study was conducted to assess the effects of allopurinol on Duchenne muscular dystrophy. Six patients—three treated with allopurinol and three given a placebo—were followed up for one year and were evaluated by functional and manual muscle testing as well as by determination of serum creatine kinase (CK) levels. Patients in both the allopurinol‐treated group and the placebo group deteriorated at varying rates without evidence of therapeutic benefit. To exclude the possibility of error attributable to small sample size, all six patients were treated with allopurinol for an additional eight‐week period. Muscle strength continued to deteriorate during this phase of

ISSN:0148-639X    

DOI:10.1002/mus.880020108

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractLine 413 early‐onset, genetically homozygous dystrophic chickens were given twice‐daily intraperitoneal injections of the antiserotoninergic drug cinanserin, alone or in combination with methysergide. Other trials consisted of penicillamine treatment in combination with either methysergide or cyproheptadine. Chemotherapy significantly prolonged the righting ability of treated dystrophic chickens, as measured by a periodic standardized fliptest procedure. Plasma creatine kinase activities were not affected by any of the various drug treatments. However, the blood serotonin levels of the dystrophic chickens (grand mean 1.47 μg serotonin/ml blood) were found to be significantly higher (p<0.001) than those in the corresponding normal chickens (0.99 μg/ml). This finding may partially account for the antiserotoninergic drug enhancement in righting ability that was demonstrated in the drug‐treated dystrophic c

ISSN:0148-639X    

DOI:10.1002/mus.880020109

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractWe have introduced the termF chronodispersionto denote the scatter or dispersion of the relative latencies of statistically significant numbers of consecutively recorded F waves. Five patients with chronic renal failure and five control subjects were studied. The two groups were matched for age, sex, proximal and distal motor conduction velocity, and latency of fast F waves plotted against distance traveled. We found that the extent of F chronodispersion of the deep peroneal nerve did not exceed 7.5 msec in control subjects, while it was longer than this value in all uremic patients studied. Measurements of this type may prove to be a useful diagnostic tool for cases in which traditional electrophysiologic measurements are normal.

ISSN:0148-639X    

DOI:10.1002/mus.880020110

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY

摘要:

AbstractA 75‐year‐old French‐Canadian woman with familial oculopharyngeal dystrophy demonstrated histopathologic alterations similar to those of idiopathic polymyositis. A second biopsy obtained 15 months later was more consistent with previously reported cases. It is suggested that certain patients with oculopharyngeal dystrophy may pass through an initial phase of secondary muscle inflammation similar to that seen in some other heritable myopa

ISSN:0148-639X    

DOI:10.1002/mus.880020111

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1979

数据来源: WILEY