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Results for “PROGRESSIVE MUSCULAR DYSTROPHY”

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At least 127 records · Page 7Linked to original sources
PubMed1989

Observation of the curative effect of external counter-pulsation therapy on progressive muscular dystrophy.

External counter-pulsation therapy was applied to 12 cases of progressive muscular dystrophy with effects of varying degressive markably effective in 3 cases, effective in 6, improved in 2 and no effect in one. In 11 of 12 cases the atrophic muscles became normal, in 3 of 8 cases the gastrocnemius softened and in 9 of 10 cases creatine phosphokinase greatly reduced. Electron microscopy found that the ultramicrostructure of the diseased muscles greatly improved, the structure and morphology of most mitochondria became normal, and glycogenesis disappeared.

Adolescent↗
PubMed1995

[Spinal anesthesia for patients with progressive muscular dystrophy].

Spinal anesthesia was applied on 8 occasions to 7 patients with progressive muscular dystrophy (PMD) undergoing orthopedic lower limb surgery. No postoperative complication occurred in all patients. During the operations, however, two patients were subjected to high spinal anesthesia, which caused ventilatory suppression in one patient and bronchial asthma attack in another. Both respiratory complications were easily managed by ventilatory assistance with endotracheal intubation or by administration of bronchodilator, respectively. High spinal anesthesia should be avoided in applying spinal anesthesia to patients with PMD.

Adolescent↗
PubMed1988

Pathogenesis of progressive muscular dystrophy: studies on free radical metabolism in an animal model.

Evidence to suggest the presence of abnormal metabolism of oxygen free radicals in progressive muscular dystrophy is presented using an animal model. In the superficial pectoral muscles of dystrophic chickens, enzyme activities regulating the metabolism of oxygen free radicals, i.e., catalase, superoxide dismutases and glutathione peroxidase, were significantly elevated within 1 week of hatching. Activities of related enzymes, i.e., glutathione reductase, glucose-6-phosphate dehydrogenase, 6-phosphogluconate dehydrogenase were also elevated. In contrast, the specific activity of phosphofructokinase, the rate-limiting enzyme of the glycolytic pathway, was normal during the first 4-week period. These results suggest that there is an increased turnover of oxygen free radicals in the dystrophic muscle. This concept appears important in a further investigation of the pathogenesis and treatment of progressive muscular dystrophies.

Animals↗
PubMed1979

Systolic time intervals in progressive muscular dystrophy.

Indirect systolic time intervals corrected for heart rate were measured at rest, during, and immediately after the isometric handgrip exercise in 70 patients with progressive muscular dystrophy, and these were compared with the values of normal subjects. Those with dystrophy included 47 patients with Duchenne type, 19 with limb girdle type and 4 with facioscapulohumeral type, and each type was subdivided into 2 groups by the severity of the skeletal muscle involvement: 1) mild group, included patients who were still able to walk, 2) severe group, included those who were restricted to wheel chair or confined to bed. Over a half of the patients of the severe Duchenne type group had a longer pre-ejection period (PEP), shorter left ventricular ejection time (LVET), and larger PEP/LVET ratio at rest than the normal group. Increased PEP/LVET ratio during and after isometric handgrip exercise was observed in the severe group of Duchenne type. The patients with limb girdle type, and facioscapulohumeral type showed no significant difference in values of the systolic time intervals at rest and during handgrip exercise compared with the normal subjects. The measurements of resting and exercise systolic time intervals may be useful for clinical recognition of latent left ventricular functional impairement in subjects with progressive muscular dystrophy.

Adolescent↗
PubMed1992

Upper gastrointestinal tract motility in children with progressive muscular dystrophy.

Gastric emptying was evaluated in 15 children (mean age, 8.0 years) with progressive muscular dystrophy to detect early gastrointestinal smooth muscle involvement; 10 of the children also underwent esophageal manometry. Clinical evidence of skeletal muscle dysfunction was minimal in 14 of the 15 patients; 10 of them had no gastrointestinal symptoms. Gastric emptying studies were performed by using 500 muCi of technetium 99m-sulfur colloid bound to a scrambled egg, and scintigraphic measurements were made continuously for 60 to 90 minutes. Gastric emptying studies and manometric tracings were compared with those from 11 children (mean age, 8.4 years) without gastrointestinal or muscular disorders. Mean (+/- SD) percentage retention of gastric isotope was significantly greater in patients with muscular dystrophy than in control subjects. No differences were found between the two groups in distal esophageal motility or in upper and lower esophageal sphincter pressures or relaxation. Contraction amplitudes in the upper portion of the esophagus, however, were significantly lower in patients with myopathy than in control subjects. These data suggest that dysfunction of smooth muscle of the upper gastrointestinal tract is detectable in children with muscular dystrophy early in the course of the disease, even when gastrointestinal symptoms are absent and skeletal muscle symptoms are minimal.

Adolescent↗
PubMed1976

General anaesthesia for a patient with progressive muscular dystrophy.

The case is reported of a four-year-old boy with progressive muscular dystrophy who underwent anal fistulectomy under neuroleptanaesthesia with Thalamonal. Recovery was uneventful. Anaesthetic problems associated with the disease include sudden death due to myocardial involvement and high postoperative mortality due to pulmonary infection. Mental retardation and ECG abnormalities may be present and acute postoperative gastric dilatation has been reported. Narcotics or minor tranquilizers and hyoscine are recommended for premedication. Light anaesthesia should be maintained throughout surgery and minimal doses of non-depolarizing muscle relaxants may be used if necessary. Suxamethonium chloride should be avoided because muscle damage and cardiac arrest might occur.

Benperidol↗
PubMed1998

Nine-year follow-up study of heart rate variability in patients with Duchenne-type progressive muscular dystrophy.

OBJECTIVES: The purpose of this study was to investigate the progression of autonomic dysfunction in patients with Duchenne-type progressive muscular dystrophy (DMD) over time by using heart rate variability. BACKGROUND: Although previous studies suggest the presence of autonomic dysfunction in patients with DMD, the precise cause is not known. On the other hand, it is well known that analysis of heart rate variability provides a useful, noninvasive means of quantifying autonomic activity. High frequency power is determined predominantly by the parasympathetic nervous system, whereas low frequency power is determined by both the parasympathetic and sympathetic nervous systems. METHODS AND RESULTS: Frequency and time domain analyses of heart rate variability during ambulatory electrocardiographic monitoring were performed in 17 patients with DMD over a 9-year period. At the time of entry, the mean patient age was 11 years and the mean Swinyard-Deaver stage was 4. In the first year, high frequency power was significantly lower and the ratio of low frequency to high frequency was significantly higher in patients with DMD than in the normal control subjects. These differences become significantly greater as the disease progressed. At the time of entry, low and high frequency powers increased at night in both groups. However, over time, high and low frequency powers at night tended to decrease. All of the time domain parameters were significantly lower in the patients with DMD at all time points compared with the normal control subjects. CONCLUSIONS: We concluded that DMD patients have either a decrease in parasympathetic activity, an increase in sympathetic activity, or both as their disease progresses.

Adolescent↗
PubMed1986

Progressive muscular dystrophy with congenital adrenal hypoplasia: an unusual autopsy case.

A 3 1/2-year-old child with progressive muscular dystrophy (PMD) and congenital adrenal hypoplasia (CAH) is described. Symptoms and signs of adrenocortical insufficiency appeared shortly after birth. Despite corticosteroid therapy, the muscular weakness and elevated CK level continued. A diagnosis of Duchenne muscular dystrophy was made on the basis of clinical signs and characteristic muscle biopsy. The affection of his older brother suggests an X-linked recessive inheritance. The autopsy revealed a very rare combination of cytomegalic type CAH and PMD. This combination suggests that a small deletion of X-chromosome might be responsible for the two disorders.

Adrenal Glands↗
PubMed1978

An ultrastructural basis for electrocardiographic alterations associated with Duchenne's progressive muscular dystrophy.

Electrocardiographic abnormalities were identified in 63 (84%) of 75 patients with Duchenne's progressive muscular dystrophy. A tall R wave over V1 with an abnormal R/S ratio was seen in 64% of the patients, a deep and narrow Q wave greater than 4 mm over leads I, V5, and V6 in 44%, sinus tachycardia in 32% and right axis deviation in 16%. Other ECG abnormalities included an abnormal PV1 index in 14% of patients and a short P-R interval in 6%. Ultrastructural characteristics of the heart were determined for two patients with characteristic electrocardiographic abnormalities. Common to both hearts was a total loss of thick as well as thin myofilaments, which gave a "moth-eaten" appearance to the myofiber. This feature, combined with preservation of the transverse tubular system, formed the most characteristic ultrastructural finding and was seen most consistently in the posterobasal area of the left ventricle. Alterations of Z-band material; accumulation of mitochondria, occasionally containing electron-dense bodies and showing loss or discontinuity of cristae; dilatation of sarcoplasmic reticulum with striking ectasia of cisternae; depletion of glycogen particles; a paucity of lipoid or lipochrome granules; and the absence of virus-like particles were other consistent ultrastructural features. Comparison of skeletal and cardiac muscle disclosed identical subcellular changes. These observations support the contention that the distinctive ECG pattern associated with Duchenne's dystrophy results from multifocal degenerative changes involving myocardium, predominantly the posterobasal region of the left ventricle and the posterior papillary muscle.

Adolescent↗
PubMed1988

Pulmonary hypertension in progressive muscular dystrophy of the Duchenne type.

Right heart catheterization was performed in 8 patients with progressive muscular dystrophy of the Duchenne type (DMD) at the advanced stage. A mean pulmonary arterial pressure in excess of 20 mmHg was observed in all cases. Five of them showed severe pulmonary hypertension with a mean pressure above 40 mmHg. Since pulmonary hypertension was relieved by correction of hypoxemia, this represented a precapillary pulmonary hypertension caused by constriction of the pulmonary artery. Furthermore, elevation of the mean right atrial pressure above 5 mmHg was observed in 6 of the 8 cases, indicating the possible presence of right ventricular failure. Despite the presence of left ventricular dysfunction as assessed by echocardiogram, no manifestations of left ventricular failure, such as dyspnea and pulmonary rales, were noted in any of the patients. In conclusion, it can be said that even in the terminal stage of DMD, the left ventricular function may, in fact, still remain not markedly involved, and that respiratory failure, as well as right ventricular failure caused by precapillary pulmonary hypertension, will tend to occur frequently and may play a determinant role in prognosis of the advanced DMD patient.

Adolescent↗
PubMed1982

Effects of therapeutic exercise on masticatory function in patients with progressive muscular dystrophy.

The slope of the curve relating integrated electromyographic activity of masseter muscle to biting force, the latency of the jaw-jerk reflex, and masticatory performance wee estimated in patients with Duchenne type of progressive muscular dystrophy before and during therapeutic exercise of the somatogenc system. The slope and latency were slightly decreased, and masticatory performance was increased during exercise. These results suggest that therapeutic exercise of the stomatognathic system is effective in improving masticatory function in patients with progressive muscular dystrophy.

Adolescent↗
PubMed1994

Cardiac involvement of progressive muscular dystrophy (Becker type, Limb-girdle type and Fukuyama type) evaluated by radionuclide method.

Tl-201 SPECT and Tc-99m-Human serum albumin (HSA) multigated radionuclide ventriculography were performed on 11 patients with progressive muscular dystrophy (Becker type 2, Fukuyama type 2, Limb-girdle type 7) to evaluate myocardial involvement. Hypoperfusion was detected in 8 patients on Tl-201 SPECT. Decreases in both systolic function (left ventricular ejection fraction; LVEF) and diastolic function (peak filling rate; PFR) were also seen in these patients. A high incidence of myocardial involvement of these kinds of progressive muscular dystrophy was suggested.

Adolescent↗