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Results for “PROGRESSIVE MUSCULAR DYSTROPHY”

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At least 73 records · Page 4Linked to original sources
PubMed1987

[Function of the heart conduction system in patients with progressive muscular dystrophy].

Intracardiac electrophysiologic studies were carried out in 9 patients with progressive muscular dystrophy. Diagnostic stimulation of the heart demonstrated no sinus mode dysfunction. The intra-atrial conduction time increased to 72 +/- 7.1 ms (p less than 0.05), and the interatrial conduction time, to 97 +/- 12.5 ms (p greater than 0.05) in response to shortened testing stimulus delay in 3 patients. All 9 patients showed great variation in the duration of effective refractory atrial and atrioventricular periods (97.0 +/- 12.5 ms). The H-V interval increased from 43.9 +/- 4.3 to 73.9 +/- 7.6 ms (p less than 0.01) in response to shortened delay in 7 of 9 patients. Atrial pre-excitation resulted in a block of the right and left limbs of the His bundle in 5 of 7 patients. Third-degree atrioventricular block was recorded by continuous ECG monitoring in 7 patient. It is suggested that an apparent or latent intraventricular conductivity disorder may be present in most cases of progressive muscular dystrophy.

Adolescent↗
PubMed2005

[Social support and acceptance of the disease by parents of children who have progressive muscular dystrophy].

The purpose of this study was to investigate the relationship in the parents among social support, psychological stress response, and acceptance of the fact that their children had progressive muscular dystrophy. Parents of progressive muscular dystrophy patients, 326 in total, completed a questionnaire that included scales of social support, acceptance of muscular dystrophy, positive thought on child's muscular dystrophy, and psychological stress response. Results of analysis of variance (ANOVA) indicated that features of the social support received by the parents differed at different stages of the disease, but levels of acceptance of the disease and psychological stress response were not different. Results of analysis of covariance structure suggested that social support increased parent's positive cognition about the disease, the cognition in turn increased their acceptance of the disease, and the acceptance reduced psychological stress responses. Finally, social support systems that were available to the parents of children with muscular dystrophy were discussed.

Adaptation, Psychological↗
PubMed1985

[Blood lipid disorders in Becker-Kiner progressive muscular dystrophy].

The article is devoted to one of the rare forms of hereditary neuromuscular diseases--Becker-Kiner progressive muscular dystrophy. Blood lipids were determined in nine patients aged 16 to 45 years with Becker-Kiner progressive muscular dystrophy. Profound disorders of the blood lipid metabolism were established which were expressed in hyperbeta-lipoproteinemia, hypertriglyceridemia, and hypercholesterinemia. The examination of these parameters is useful for objective assessment of the given form of progressive muscular dystrophy.

Adolescent↗
PubMed1985

Anaesthesia and progressive muscular dystrophy.

The presentation and features of Duchenne's progressive muscular dystrophy (Duchenne's PMD) are described and the increased risks associated with anaesthesia are considered. Hazards associated with induction of anaesthesia and immediate postoperative recovery have been stressed in recent case reports, and these are summarized. Features of a hyperpyrexia-like response including cardiac arrest, increased serum creatine phosphokinase concentration, myoglobinuria and metabolic acidosis following suxamethonium or halothane, or both, have been described in patients with Duchenne's PMD. Subsequent in vitro muscle tests have suggested that it is possible that a malignant hyperpyrexia response to general anaesthesia may occur. Six children known to have Duchenne's PMD who developed delayed respiratory insufficiency following anaesthesia and required controlled pulmonary ventilation are reported. In five of the children, cardiac arrest occurred despite apparently adequate respiratory support. Suxamethonium was common to the anaesthetic received by all six patients. In one of these patients subsequent anaesthetics, without suxamethonium, were uneventful and delayed muscle weakness did not occur.

Adolescent↗
PubMed1980

Primary progressive muscular dystrophy in the dog.

A case of primary progressive muscular dystrophy in a labrador dog is described. The differential diagnosis with respect to certain muscular and nervous disorders is discussed. The possibility of the hereditary nature of the disease is indicated.

Animals↗
PubMed1989

[Plasma alpha/beta lipoprotein coefficient in the diagnosis of progressive muscular dystrophy].

Ten patients with Erb-Roth's progressive muscular dystrophy and 10 ones with Charcot-Marie neural amyotrophy have been examined. The blood plasma lipoproteins have been studied by disk electrophoresis in polyacrylamide gel with Reanal reagents. The studies have revealed different patterns of dyslipidemia and demonstrated the significance of alpha/beta lipoprotein coefficient in the diagnosis of such conditions.

Charcot-Marie-Tooth Disease↗
PubMed1980

[Allopurinol in progressive muscular dystrophy (author's transl)].

Authors have treated with allopurinol ten children with progressive muscular dystrophy, whose ages were between 15 months to eleven years. An initial and monthly clinical evaluation of muscular strength, Gowers's sign and pseudohypertrophy was performed. Monthly determinations of CPK, LDH, aldolase, GOT and serum urate; every two months electromyography was also made. In three patients a complete recovery of strength, Gowers's sign was found and at the same time pseudohypertrophy diminished; the age average was three years and four months. In four patients a partial recovery was found; the age average was nine years and four months. In three patients there was no answer to treatment, they were contracted and had deformities, being age average ten years. No variations on monthly controls of CPK, LDH, aldolase and GOT were observed. On these facts their opinion is that allopurinol is an effective drug in the treatment of progressive muscular dystrophy and that its' effectivity is better when treatment is initiated early and, of course, before patients present deformities and contractures.

Allopurinol↗
PubMed1980

[Features of the course and treatment of progressive muscular dystrophy associated with chronic suppurative diseases].

A comparison of the clinical course of progressing muscular dystrophy with laboratory and electrophysiological findings has shown that combination of the dystrophy with chronic tonsillitis, purulent otitis, etc. aggravate the course of the underlying disease. The existing methods of therapy are ineffective: this has been established in 14 out of 33 observations. On these grounds the authors suggest that the principal methods of treating progressing muscular dystrophy should be supplemented with the use of antibiotics, desensitizers, detoxicants, salicylates, and means enhancing the body resistance. If the conservative therapy is of no effect tonsillectomy should be performed.

Adolescent↗
PubMed1980

Central nervous system involvement in progressive muscular dystrophy.

Several abnormalities in the central nervous system were shown in patients with progressive muscular dystrophy using computerised tomography (CT) scans, electroencephalograms, psychometry, and ophthalmological methods. In congenital muscular dystrophy, the most characteristic finding in the CT scan was a low density area in the white matter, seen in 14 (56%) out of 25 cases. In Duchenne dystrophy, slight cerebral atrophy was observed in 20 (67%) out of 30 cases. It was interesting that in the case of Duchenne dystrophy the older the patient, the more severe were the CT findings. In congenital muscular dystrophy half the patients with a low density area showed a spike or a spike-and-wave complex in the electroencephalogram, and optic atrophy was evident in several cases. It is concluded that progressive muscular dystrophy is not only a myogenic disorder but also one which affects the central nervous system.

Adolescent↗
PubMed2002

[Propofol anesthesia for a patient with progressive muscular dystrophy].

We gave propofol anesthesia to a patient with limb-girdle type of progressive muscular dystrophy. A 42 year-old male was to have skin graft for third degree burn. His respiratory function test showed %VC of 73.6% and %FEV1.0 of 107.6%. Arterial blood gas data were within normal ranges. He was anesthetized with propofol, fentanyl, vecuronium and nitrous oxide. During position change, Wenckebach type of second degree AV block occurred. AV block returned to sinus rhythm easily by injection of ephedrine hydrochloride and atropine sulfate, and reduction of propofol infusion rate. There were no perioperative respiratory complications and no clinical manifestations of malignant hyperthermia. Propofol anesthesia is suitable for limb-girdle type of progressive muscular dystrophy, because of very little possibility of triggering malignant hyperthermia, rapid awaking, minimal residual effects of the respiratory system, and easiness in controlling anesthetic depth.

Adult↗
PubMed1991

Progressive muscular dystrophy with particular reference to muscle regeneration.

In various progressive muscular dystrophies (PMD), there were a number of fibers with histological and histochemical characteristics of regenerating fibers. With a morphometric analysis in diseased muscles, an identification of type 2C fiber on ATPase stain was the most simple and reliable method for fiber type evaluation. The type 2C fiber comprised 16.5% in Duchenne and 27.5% in Fukuyama type congenital MD, suggesting an active regenerating process taking place in both diseases. Regenerative capacity of muscle fibers after experimental damage to dystrophic chicken and mdx mouse muscles was similar to those seen in nondystrophic control muscles. From the above results, we speculate that a certain environmental factor such as interstitial fibrosis seems to play a major role in delaying regeneration in dystrophic muscles.

Animals↗
PubMed1991

[General anesthesia with sevoflurane and vecuronium for patients with dystrophia myotonica and progressive muscular dystrophy].

A 9 year old male previously diagnosed as progressive muscular dystrophy whose serum CPK5430IU.l-1 was very high received general anesthesia. Before anesthesia, dantrolene sodium 2 mg.kg-1 was given. Anesthesia was induced with thiamylal 100 mg and vecuronium bromide 3 mg. Anesthesia was maintained with sevoflurane (0.5%) in nitrous oxide (66%) and oxygen (33%). The course of anesthesia was uneventful. The operative time was 80 minutes. At the end of the operation, the patient recovered smoothly from anesthesia. A 46 year old female with dystrophia myotonia also received general anesthesia. The patient was diagnosed as having this disease 26 years previously. Preoperatively, the patient was suspected to have cardiac damage. Anesthesia was induced with thiamylal 100 mg, fentanyl 100 micrograms, midazolam 5 mg and vecuronium bromide 4 mg, and maintained with sevoflurane (1.0%) in nitrous oxide (66%) and oxygen (33%). Anesthesia was uneventful, but at the end of the operation, the patient could not breath fully by herself. She was placed on a ventilator and observed carefully. The endotracheal tube was removed 150 minutes after the induction of anesthesia. In these two cases, sevoflurane and vecuronium bromide were used safely.

Anesthesia, Inhalation↗