Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 8 patients with chronic progressive ocular muscular dystrophy, of whom some showed a weakness of lid closure, and in 3 normal persons (aged 62 to 69 years) biopsies of the orbicularis oculi muscle were investigated with the light- and electron microscopes. We tried to find out if the histopathological investigation alone of the orbicularis oculi muscle can establish the diagnosis of ocular muscular dystrophy or not. Both groups of investigated persons showed myopathic changes in the orbicularis oculi muscle. These changes were explained as followings of catabiosis (so called "secondary myopathic reaction") in the normal persons. The changes of the patients with myopathies, however, were explained as primarily myopathic, especially with regard to the anomalies of structure, and the pathological inclusions of the mitochondria. The findings showed that there occurred similar histopathological phenomena seen by light microscope in both groups of investigated persons; the muscle cell changes of the myopathy patients, however, were more prominent in the examination of the ultrastructure.
Klin Monbl Augenheilkd 1978 Sep
PMID:[Light-and electron microscopic appearances of the orbicularis oculi muscle in patients with chronic progressive ocular muscular dystrophy and in normal persons (author's transl)]. 75 Jul 6

Systolic time intervals (STIs) were measured in 57 patients with progressive muscular dystrophy (PMD) of the Duchenne type, and were correlated with the stages of physical disability. The total electromechanical systole (Q-A2), pre-ejection period (PEP), and left ventricular ejection time (LVET) were corrected for heart rate using the linear regression equations calculated from 91 normal subjects. Each systolic time interval index (STII), i.e. corrected STI, was analyzed. In 15 ambulatory patients (Group P-1), the STIIs did not differ significantly from those in normal controls. In 37 patients who were unable to walk (Group P-2) and 5 patients who were confined to bed (Group P-3), there was a significant increase (p less than 0.001) in the PEP index (PEPI), a decrease (p less than 0.001) in the LVET index (LVETI), and an increase (p less than 0.001) in the PEP/LVET compared with those in controls. The PEPI (p less than 0.001), LVETI (p less than 0.025), and PEP/LVET (p less than 0.001) in Group P-2 differed significantly from those in Group P-1. In Group P-3, the PEPI (p less than 0.005), LVET (p less than 0.001), and PEP/LVET (p less than 0.001) were significantly changed in comparison with those in Group P-2. Remarkable myocardial histopathologic changes suggestive of dystrophy were revealed in autopsies of 3 patients who had been in the severely disabled stage and had shown impaired STIs. It is suggested that cardiac function in patients with moderate to advanced PMD of the Duchenne type is deteriorated in proportion to the disability stages, i.e. the severity of changes in the skeletal muscles.
Jpn Heart J 1977 Sep
PMID:Systolic time intervals in patients with progressive muscular dystrophy of the Duchenne type. 92 10

There has been accumulation of the nutritional muscular dystrophy of the cattle in a certain western district of Finland where the prevalence of multiple sclerosis (MS) is also highest. This animal disease is due to lack of selenium (Se) and vitamin E. The Se content of whole blood was low (52.6 +/- 11.3 ng/ml) in MS patients from this high-risk area compared to the controls (68.8 +/- 11.0). The data for serum failed to confirm this tendency. All Se values appeared to be lower than international values suggested. The values for both vitamin E and copper were within the international normal range.
Acta Neurol Scand 1976 Sep
PMID:Selenium, vitamin E and copper in multiple sclerosis. 96 80

The present studies were conducted to determine whether inherited muscular dystrophy in the 129/ReJ-dy mouse was associated with differences in specific activity, substrate availability, or apparent Km of glutathione peroxidase. The results indicate that glutathione peroxidase is elevated in skeletal muscle of mice with genetic muscular dystrophy when the activity is expressed on a protein basis. This elevation precedes the development of severe paralysis since muscles from the fore legs showed increased enzyme activity as early as the more severely affected hind legs. There was no difference in glutathione peroxidase activity in tissues other than skeletal muscle. GSH concentration was elevated in muscle and normal in other tissues of dystrophic mice, showing that adequate substrate was available to the enzyme. The apparent Km for cumene hydroperoxide was also similar for muscle of normal and dystrophic mice. This report provides further evidence that mice with dystrophia muscularis have a functional glutathione peroxidase system in all tissues including skeletal muscle, and that a defect in this in vivo protective system is apparently not a contributing factor in the pathology of the disease.
Proc Soc Exp Biol Med 1976 Sep
PMID:Glutathione peroxidase activity and glutathione concentration in genetically dystrophic mice. 96 80

We have previously shown that diethylstilbestrol (DES) almost always, and prednisolone (Pr) less frequently, lowered the high serum enzyme activities in Duchenne's muscular dystrophy (DMD). In experimental studies, it was shown that pretreatment of mice with each of these agents lowered enzyme efflux from isolated skeletal muscle incubated in vitro, but efflux was augmented by higher doses of Pr. This suggested that these agents may influence skeletal muscle enzyme efflux in man also, producing the effects noted in DMD. The present studies were undertaken to assess the effect on enzyme efflux from skeletal muscle and heart that these two agents would exert when given in combination. Four groups of mice (14/group) were injected with saline, 250 mug DES, 35 mug Pr, or 250 mug DES plus 35 mug Pr in saline every other day for 22 days. The left gastroecnemius and heart were isolated from animals of each group, and placed in separate tubes containing incubation medium at 25 degrees C. The efflux of creatine phosphokinase (CPK) and lactate dehydrogenase (LDH) which issued from each organ was determined over a 5 hour period. In the doses tested, it was found that: 1) DES selectively reduced enzyme efflux from skeletal muscle, but had no effect on enzyme efflux from heart; 2) a Pr dose which decreased enzyme efflux from the heart, augmented efflux from the gastrocnemius; and 3) DES prevented the enhanced enzyme efflux produced by Pr. These studies indicate that these hormones, in pharmacological doses, influence enzyme efflux from muscle. This suggests, but it is not established, that these hormones also exert a similar physiological role. Finally, this experimental model appears to be useful in assessing the effects of single agents, and agents in combination, on enzyme efflux, and should be of aid in selecting appropriate agents which may be therapeutically useful in Duchenne's muscular dystrophy.
Res Commun Chem Pathol Pharmacol 1976 Sep
PMID:Disparate effects of diethylstillbestrol and prednisolone on enzyme efflux from heart and skeletal muscle. 96 73

The AA. have carried out in patients affected by proved myocardial infarct, by other cardiac diseases and by muscular dystrophy the following enzymes determinations: total CPK, total LDH, SGOT, SGPT, HBDH, CPK isoenzymes by column chromatography (Mercer's method) and LDH isoenzymes either by column chromatography (Mercer's method) and by electrophoretic separation. Some results concerning the appearance of the CK-MB isoenzymes during the acute period of the myocardial infarction are described.
Quad Sclavo Diagn 1976 Sep
PMID:[Determination of CPK isoenzymes by column chromatography (author's transl)]. 102 94

Maximal systolic endocardial velocity (SEVM) and maximal diastolic endocardial velocity (DEVM) were determined echocardiographically in patients with muscular dystrophy (MD). The SEVM of the muscular dystrophy patients was 5.5 +/- 0.9 cm/sec and the DEVM was 13 +/- 3 cm/sec. The SEVM in MD was significantly less than that seen in age-matched normals (P less than 0.05), persons with myotonia congenita (P less than 0.02), deconditioned patients (P less than 0.001), or older normal persons (P less than 0.05). The Sevm of the MD patients was not significantly different from persons with spinal muscular atrophy. The DEVM of the muscular dystrophy patients was significantly less (P less than 0.001-0.05) than any other group. No correlation could be found between age, heart rate, type or severity of dystrophy and SEVM or DEVM values. The echocardiogram was more selective in correctly identigying muscular dystrophy patients than the electrocardiogram. The abnormality in DEVM was present despite lack of symptoms, normal cardiovascular examination, normal chest X-ray and normal electrocardiograms in 18 of 22 patients. We believe that the DEVM correlates with myocardial relaxation.
Circulation 1975 Sep
PMID:Echocardiographic evaluation of posterior left ventricular wall motion in muscular dystrophy. 115 43

The role of the neural tube in the pathogenesis of muscular dystrophy was tested directly. Neural tubes from chicken embryos with hereditary muscular dystrophy and from genetically normal embryos were transplanted into normal recipient embryos. Dystrophic neural tissue induced in muscles of normal hosts high thymidine kinase activity characteristic of dystrophic muscle; normal neural tubes did not. We propose an early inductive effect of the neural tube on the presumptive myoblasts that sets their subsequent course of development, either normal or dystrophic.
Science 1975 Sep 26
PMID:Dystrophic spinal cord transplants induce abnormal thymidine kinase activity in normal muscles. 116 64

(1) Narrowness of the early-diastolic and presystolic peaks on the echo curve of the anterior mitral leaflet and an abnormal step formation at the middle or higher level on the descending limb of the presystolic peak were observed in 7 cases with congestive cardiomyopathy or myocardial fibrosis, 1 case with myocardial infarction, and 1 case with cardiomyopathy due to progressive muscular dystrophy. (2) This abnormal pattern is considered to be closely related to the myocardial condition in the above-mentioned diseases. (3) Probable mechanisms for this pattern formation are considered as follows: (i) a sudden reduction of distensibility of the left ventricle after filling of the ventricle over a certain limit near full-filling, and impaired ventricular contractility, (ii) restriction in the mobility of the mitral valve and its chardae due to thier inability to adapt themselves to a developed dilatation of the left ventricle. (4) A similar presystolic step formation on the echo curve of the anterior mitral leaflet was observed in 3 of 25 cases of hypertrophic cardiomyopathy of Goodwin's sense. Differences between the myocardial state in congestive cardiomyopathy or in similar myocardial diseases and that in hypertrophic cardiomyopathy were also discussed.
Jpn Heart J 1975 Sep
PMID:An unusual pattern of the mitral echocardiogram observed in cases of congestive cardiomyopathy and other myocardial diseases. 117 35

This is a report on two autopsy cases of congenital muscular dystrophy associated with micropolygyria. The first case was that of an 11-year-old boy and the other of a 22-year-old male adult. Both cases had similar clinical features, very early onset of disease, diffuse and extensive wasting of skeletal muscles including facial muscles, contracture of joints, hypotonia and mental retardation. In the familial histories of these two cases, the parents of the boy were consanguineous, and a sister of the adult case suffered from muscle weakness and mental retardation. Both of these two cases were clinically diagnosed as congenital cerebromuscular dystrophy (Fukuyama's type). Autopsy revealed marked dystrophy of generalized skeletal muscles and widespread micropolygyria of the brain in both cases. Spinal cords and peripheral nerves were free from any prominent changes. It was concluded that so-called congenital cerebromuscular dystrophy may be caused by myogenic as well as neurogenic abnormalities during fetal period.
Acta Pathol Jpn 1975 Sep
PMID:Congenital muscular dystrophy associated with micropolygyria - report of two cases. 119 29


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