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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Enzyme histochemistry and acridine orange (AO) fluorescence techniques were used for studying muscle biopsy specimens of progressive muscular dystrophy in 75 cases. Five characteristic pathologic patterns for diagnosis were summarized. The level of serum CPK was be used as a marker for judging necrotic fibers. The result of AO staining showed that the number of regenerating IIc type fibers in DMD increased by 5-20%. This indicates that the numbers of the IIc type fibers are also related to necrotic fibers. The authors consider that the regenerative course is a compensatory repair reaction on necrotic fibers. But clinically, the speed of necrosis development is much higher than that of regeneration. Thus, enhancing the synthesis of proteins and promoting the capacity of regeneration should be considered as a new approach to effective therapy for DMD patients.
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PMID:[Pathohistology of progressive muscular dystrophy and the relationship between necrotic and regenerative fibers]. 227 5

For the purpose of clarifying the cause of white muscle disease (WMD) in calves, tocopherol and selenium levels and blood glutathione peroxidase (GSH-Px) activity were measured on 10 calves with WMD and nine of their dams. The main clinical symptoms of the 10 calves with WMD were motor disturbances including recumbency and stiffness. Serum enzyme activities (GOT, GPT, CPK, LDH) in calves with WMD increased markedly, and this increase was also observed in some of their dams. Serum tocopherol levels of calves with WMD were low, 70% of which showing deficient levels of less than 70 micrograms/100 ml. Serum selenium levels of all the calves were lower than 35 ppb, indicating a deficiency, and were accompanied by low blood GSH-Px activity. alpha-Tocopherol and selenium concentrations in organs were very low. Dams of calves with WMD showed low serum tocopherol levels, 22% of which indicating deficient levels below 150 micrograms/100 ml. Serum selenium levels in dams showed a marked decrease to under 20 ppb, and also low blood GSH-Px activity. Feedstuffs supplied in the farms to affected calves indicated very low alpha-tocopherol contents (below 3 mg/100g DM) and low selenium concentrations below 50 ppb in DM. It was concluded that WMD in calves was attributable to nutritional muscular dystrophy caused by deficiencies in tocopherol and selenium in feedstuffs supplied to their dams.
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PMID:Studies on serum tocopherol, selenium levels and blood glutathione peroxidase activities in calves with white muscle disease. 258 29

In this paper are described seven men with a scapula-back type of x-linked recessive muscular dystrophy in a family. Onset began in boyhood. The scapula-back muscles were affected, but the calf muscles were normal. All of the patients were able to walk. The oldest patient was thirty-seven years old. Muscular dystrophy was confirmed by electro-myography (EMG), pathologic and CPK examinations. Pedigree analysis indicated x-linked recessive inheritance.
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PMID:[A family of scapula-back type of x-linked recessive muscular dystrophy]. 259 Dec 65

Seven cases of benign form of spinal muscular atrophy were studied to evaluate the importance of detecting hand tremors, muscle fasciculation, evertion of foot and ECG tremors to distinguish these cases from muscular dystrophy. Taken in combination, diagnosis of all the seven cases was possible without the need for application of more sophisticated and invasive investigations, e.g., EMG, nerve conduction study, CPK levels and muscle biopsy.
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PMID:Spinal muscular atrophy: some easy clues to diagnosis. 263 Apr 60

Twelve cases of progressive muscular dystrophy were treated with external counterpulsation (ECP). The therapeutic results of ECP were shown as follows: markedly improved, 3 cases: improved 6 cases: slightly improved 2 cases and no effect 1 case after ECP Clinical, improvement in various degrees developed in all the cases. Dystrophy muscle enlarged in 11 of the 12 cases. Gastrocnemius muscles softened and lessened in 3 of 8 cases. CPK markedly decreased in 9 of 10 cases. In electron microscopic examination the ultrastructure of the diseased muscles was found to have obviously improved, the structure and form of most mitochondria returned to normal, and collection of glycogen was rarely seen.
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PMID:[Observation on the therapeutic effect of external counterpulsation in progressive muscular dystrophy]. 279 63

Clinical and biochemical responses were studied after taking the measures to prevent nutrition muscular dystrophy in young cattle in the given ecological conditions. Analyzing the biological material (blood, hair, feed, soil), we found the sufficiently high saturation of heifer organisms with the microelement selenium and on the contrary, vitamin E deficiency. Sensitive indicators of the break-down of muscular tissue were the enzymes aspartate aminotransferase (AST), alanine aminotransferase (ALT), and mainly creatinine kinase (CPK): the activities of these enzymes increased significantly after the heifers had been driven to pasture. The stay of animals in the run to get them used to the physical load before going to the pasture was not found to be a sufficient measure to prevent muscular nutrition myodystrophy if the animals had not been administered vitamin E and selenium supplements. Of the one hundred heifers we examined, seven animals began to show the signs of nutrition muscular dystrophy; none of these animals had been administered vitamin E and selenium supplements.
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PMID:[Clinical and biochemical response in the prevention of nutritional myodystrophy in heifers]. 310 11

A five year old boy who had received logopedic treatment for more than two years was seen as an outpatient because of speech retardation. He presented with myopathic face, incomplete closure of both lids and severe weakness of facial muscles, bilateral winging of scapulae and hyperlordosis. Extraocular and pharyngeal muscles were not affected. Motor and sensory nerve fibre conductions and electromyography were within normal limits. CPK was moderately elevated (320 U/I). Muscle biopsy of right deltoid muscle revealed unspecific myopathic changes. The patients brother aged 7 also presented with facial weakness, elevated CPK and neurogenic changes in EMG of deltoid muscle. Both parents were clinically and electrophysiologically unremarkable. Although problems to speak distinctly are usually not the first manifestation, we found in this family facio-scapulo-humeral muscular dystrophy.
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PMID:[Articulation disorder as the initial manifestation of facioscapulohumeral muscular dystrophy in childhood]. 370 73

Coenzyme Q(10) (CoQ(10)) exists in human tissue, and is indispensable to mitochondrial enzymes of respiration. CoQ was administered to children with preclinical muscular dystrophy, CoQ enzymology was emphasized, and serum creatine phosphokinase, CPK, (ATP:creatine N-phosphotransferase, EC 2.7.3.2) was repeatedly monitored.A 40-week treatment of an infant, 1-2 years of age, reduced serum CPK (P < 0.001; total CPK assays, 76). A 40-week treatment of a boy, 3-5 years of age, reduced serum CPK (P < 0.01); treatment through 80 weeks reduced CPK (P < 0.001; total CPK assays, 118). This response of preclinical dystrophy to CoQ implies a deficiency of CoQ in skeletal muscle that was actually found previously by assay of the activity of the succinate dehydrogenase:coenzyme Q(10) reductase of the rectus abdominis. The relationships among a CoQ deficiency in muscle, serum CPK, and use of CPK in muscle are uncertain; however, restoration of CoQ enzyme activity in muscle by oral administration of CoQ could lead to increased use of CPK in muscle to form phosphocreatine from creatine and ATP, with a corresponding decrease in serum levels of CPK. The great excess of CPK in serum comes from deteriorating muscle in which CPK is below normal.
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PMID:Effect of coenzyme Q on serum levels of creatine phosphokinase in preclinical muscular dystrophy. 452 74

Two autopsy cases of congenital muscular dystrophy of Fukuyama type (F-CMD) were described. The first case was diagnosed clinically and pathologically as its typical case. Neither his family history nor the history of his prenatal period were contributory. He had suffered from muscle weakness and atrophy since his birth. Serum CPK was markedly elevated. EMG and muscle biopsy proved dystrophic changes of the skeletal muscles. In addition, he manifested mental retardation and attacks of convulsion. EEG failed to elicit remarkable changes, but PEG represented ventricular dilatation. He died of respiratory insufficiency at age 12. His postmortem examination showed variegated anomalies in the nervous system. Extensive micropolygyria was present in the cerebrum and cerebellum accompanied by adhesions between the bilateral cerebral hemispheres. Assymmetry of the longitudinal fibers was pointed out in the pontine base. Anterior horn cells were atrophic and moderately depopulated. On the other hand, the second patient was an atypical F-CMD case in symptoms, signs and pathology. His grand-mothers on both father's and mother's sides wee first cousins. His three siblings showed no similar disorders. His mother developed slight gestational toxicosis in the sixth and seventh months of pregnancy. His muscle weakness, contracture of the bilateral hip-joints and clubfoot had been observed since his birth. Physical and neurological examinations at age 6 showed deformity of the skull, myopathic face, macroglossia, high-arched palate, pigeon chest, scoliosis of the thoracic spine. In addition, generalized muscular atrophy, hypotonia and areflexia were recognized. Pseudohypertrophy of the muscles was absent. Sensation was intact to all modalities. Serum CPK and LDH were moderately increased.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[2 autopsy cases of congenital muscular dystrophy of Fukuyama type--a typical and an atypical cases]. 652 23

The genetic clinical, biological and histological study in five families with a muscular dystrophy are reported. The disease appears to be progressive, affecting both sexes, beginning often in infancy, severe at least in one of the siblings and variable from one case to the other. Hypertrophy of the calves, and weakness of the limb girdle as well as the trunk is present. CPK are much increased. Muscle biopsy shows active degeneration and regeneration. The genetic transmission is suggested to be autosomal recessive. Peculiar attention is drawn to the high incidence (75 per cent) of consanguinity of these cases.
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PMID:[Autosomal recessive severe, proximal myopathy in children, common in Tunisia]. 661 42


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