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PubMed1989

Similarity of IQs of siblings with Duchenne progressive muscular dystrophy.

The similarity of IQs of siblings with Duchenne progressive muscular dystrophy (DMD) was examined to determine whether the view that downward shift in IQ of patients with DMD is a genetically determined primary manifestation of the disease. The mean IQ difference was smaller and the IQ correlation was higher for siblings with DMD than for siblings without the disease. Moreover, these two indices were closer to those for healthy monozygotic twins reared together. These results suggest that the lowered IQ in patients with DMD has a genetic background.

Adolescent↗

PubMed1985

[Features of tolerance of somatotropic hormone to insulin in progressive muscular dystrophy].

Thirty-two patients with primary forms of progressive muscular dystrophies (PMD) and denervational amyotrophies were examined. The basal blood levels of somatotrophic hormone (STH) were significantly decreased in the majority of PMD forms. The insulin test showed quantitatively different types of reactions of STH in denervational amyotrophies versus primary myodystrophies.

Adolescent↗

PubMedDate not supplied

Gastric emptying time in children with progressive muscular dystrophy.

Gastric emptying was evaluated in 11 male children (mean age 8.2 +/- 3.2 years) with progressive muscular dystrophy to detect gastrointestinal smooth muscle involvement. No patient had gastrointestinal symptoms. Gastric emptying studies were performed by using 500 microCi of technetium 99 m sulfur colloid bound to a scrambled egg, and scintigraphic measurements were taken continuously for 60 to 90 minutes. The gastric emptying studies were compared with those of eight male children (mean age 8.2 +/- 2.8 years) without gastrointestinal or muscular disorders. The mean percentage of retention of gastric isotope was significantly greater in the study group than in the control group. These data suggest that dysfunction of the smooth muscle of the upper gastrointestinal tract is detectable in children with progressive muscular dystrophy, even when gastrointestinal symptoms are absent.

Case-Control Studies↗

PubMed1987

[Study of clinical polymorphism of Becker's progressive muscular dystrophy and associated sexual disorders].

Clinical polymorphism of Becker's progressive muscular dystrophy and related sexual disorders were studied in 13 patients aged 19 to 39 years. Characteristic disturbances included inhibited or delayed pubertal development and manifestations of hyposexuality in mature age. Pubertal retardation was diagnosed in 2 patients who also displayed an earlier development of manifestations of the first symptoms of Becker's progressive myodystrophy associated with hyperestrogenemia and hypoprolactinemia.

Adult↗

PubMed1975

[Treatment of progressive muscular dystrophy].

The paper summarizes experience in treating 306 patients with progressive muscular dystrophy during different phases of the disease. The treatment was made with due consideration for a disturbed protein metabolism, a decreased permeability of the membrane in muscular filaments, a normalization of the metabolic product supply from the blood, improvement of the blood flow and cardio-vascular and respiratory insufficiency. The treatment was carried out in the form of a continuous use in in- and out-patient conditions of a session of anabolic hormones, vascular dilatative drugs, anticholinesterase preparations, amino acids, vitamins, drugs facilitating the cardio-vascular and respiratory activity. Besides, the patients were treated by oxygenobarotherapy, muscular electrostimulation by currents of sound frequencies, remedial gymnastics, massage, hypdrotherapy, etc. The authors propose typical treatment complexes. A follow-up study of patients who received a full course of therapy during three years demonstrated its effectiveness which was expressed by the absence of clinical signs of progressiveness.

Adolescent↗

PubMed1996

Facial structure and functional findings in patients with progressive muscular dystrophy (Duchenne).

The investigation of 15 patients who have a progressive muscular dystrophy of the Duchenne type (PMD) shows the affection of various groups of muscles of the stomatognathic system, studied by means of electromyography and mechanical force measurements. We observed the following deviations in patients with PMD; transversal overdevelopment and sagittal shortening of the dental arch; reduction of overbite and overjet; sagittal underdevelopment of the cranial, maxillary and mandibular base; retrusion of incisors; concave profile; increase in bizygomatic width; and a difference in time between the attack on the musculi orbicularis oris and that on masticatory muscles. In our study, the activity of the jaw muscles diminished 2 years earlier compared with the perioral muscles. This, and also the enlargement of the hypotonic tongue, causes a transversal expansion of skull and dental arches. This expansion was strongly pronounced in the lower jaw; we invariably observed a posterior crossbite. In contrast to the transversal overdevelopment, we observed a sagittal underdevelopment of skeletal and dental parameters, as well as a retrusion of the incisors. The results of surface electromyography showed the affection of the masseter muscle in patients who had PMD. By means of the Fast-Fourier transformation, we observed a displacement of the median frequency as compared with the lower frequency range.

Adolescent↗

PubMed1987

[Features of peripheral circulatory disorders in patients with progressive muscular dystrophies].

A total of 135 patients with primary and secondary progressive muscular dystrophies have been studied rheovasographically. On the basis of the findings obtained the authors have identified the main types and peculiarities of peripheral circulation disorders and described the principal pathogenetic mechanisms of their development. The clinical and physiological correlations are presented. The authors show the importance of detected changes in the peripheral circulation for the medical expert evaluation of working ability.

Adolescent↗

PubMed1993

[Chronologic study of signs of myocardiopathy in progressive muscular dystrophy].

In order to analyze the evolution of cardiomyopathy in progressive muscular dystrophies, thirty-three patients (17 with Duchenne type, 11 with Becker type and 5 with the autosomal recessive type dystrophy) were studied retrospectively. Cardiac and systemic follow-up every 3-6 months was made in 29 patients. The electrocardiogram was the first test that became altered, followed by the echocardiogram and thoracic radiograph and finally heart failure manifestations. There was a direct correlation between age and the appearance of abnormal cardiac tests. Electrocardiographic alterations, in patients who were less than 12.5 years of age, were significantly more frequent in the group with Duchenne dystrophy that in the no-Duchenne group. In regards to the appearance of the echocardiographic and radiographic abnormalities, there were no significant differences between the two groups. However, we have noticed a trend towards a more frequent and earlier presentation of these abnormalities in the Duchenne's muscular dystrophy than in the no-Duchenne group.

Cardiomyopathies↗

PubMed1988

A case of progressive muscular dystrophy with numerous arterioluminal vessels.

A 48-year-old man had histologically demonstrated cardiac involvement associated with progressive muscular dystrophy. On coronary arteriography, numerous vascular communications between the coronary arteries and the left ventricular chamber were found. These vascular communications are considered to be the arterioluminal vessels. This is the first report of a case of progressive muscular dystrophy with numerous arterioluminal vessels.

Coronary Angiography↗

PubMed1989

[Progressive muscular dystrophies within the scope of forensic studies].

Serum myoglobin levels were investigated in patients with different types of progressive muscular dystrophy and controls. The Mb levels were determined by Radioimmuno-assay and found to be significantly elevated in all patients. The application of a specific Mb antibody (rabbit anti-human Mb) makes it possible to recognize marked differences between the Mb bands of patients and controls. All patients with progressive muscular dystrophy had an additional fourth Mb band in contrast to controls with three Mb bands.

Blood Protein Electrophoresis↗

PubMed1989

Hereditary progressive muscular dystrophies: serum myoglobin pattern in patients with different types of muscular dystrophies.

Serum myoglobin (Mb) levels and creatine kinase (CK) activity were investigated in patients with different types of progressive muscular dystrophy and controls. The Mb levels were determined by radioimmunoassay and found to be significantly elevated in all patients under resting conditions. There was no correlation between Mb levels and CK activity. Physical exercise was followed by an increase in Mb levels and CK activity in patients and a minor variation in controls. Isoelectric focusing, electroblotting and application of a specific Mb antibody (rabbit anti-human Mb) make it possible to recognize marked differences between the Mb bands of patients and controls. All patients with progressive muscular dystrophy had an additional fourth Mb band (isoelectric point pH 6.3) in contrast to controls with three Mb bands.

Creatine Kinase↗

PubMed1988

Skeletal muscle pathology in ovine congenital progressive muscular dystrophy. 1. Histopathology and histochemistry.

The histopathological lesions of ovine congenital progressive muscular dystrophy (CPMD) were characterized by myofiber hypertrophy, focal myofibrillar degeneration, formation of peripheral and central sarcoplasmic masses devoid of myofibrils and internal nuclei often in chains. Progressive loss of myofibrils was associated with atrophy of the fiber and eventual collapse of the sarcolemma. The process was polyphasic, consequently in mature lesions there was great variation in fiber diameter. Split fibers were common but ring fibers occurred rarely. Myofiber loss was associated with fatty or fibrous tissue replacement. Only type I (red, slow twitch, oxidative) fibers were affected and there was no histological evidence of effective regeneration. Ovine CPMD has many histopathological features in common with dystrophia myotonica in humans.

Adenosine Triphosphatases↗

PubMed1976

Myocardiopathy in Duchenne progressive muscular dystrophy.

In a retrospective study of hospital and autopsy records of 19 male subjects with the Duchenne type of progressive muscular dystrophy the incidence of cardiac involvement of the heart almost invariably develop heart failure; an early sign may be persistent tachycardia and, possibly, electrocardiographic changes, in case of which institution of digitalis treatment should be considered. Cardiac and pulmonary complications were equally frequent causes of death (42%) but, death from cardiac complication occurred only patients with Duchenne progressive muscular dystrophy very often develop cardiac complications, and when relating the available information on treatment to the autopsy findings it should be stressed that early and intensive therapy of the cardiac symptoms is of the greatest importance to the patient.

Adolescent↗

PubMed1984

The pyrophosphate heart scintigram in children with progressive muscular dystrophy.

A pyrophosphate heart scintigram was obtained in 16 boys with progressive muscular dystrophy Duchenne. All of them showed pathological ECG findings and high plasma levels of CK, AST, ALT and LD. In 4 patients the scintigram was distinctly positive and in further 3 it reached borderline values. The remaining 9 boys had normal scintigraphic findings. Those with a positive heart scintigram had very high plasma levels of the enzymes under study which was suggestive of current progression of the disease. There was, however, no relation between heart scintigraphy and the affliction of the skeletal muscles expressed by means of an index.

Adolescent↗

PubMed1987

[Analysis of motor control and kinesthetic perception in patients with progressive muscular dystrophy].

The purpose of this study was to examine motor control and kinesthetic perception of upper extremity in patients with Duchenne progressive muscular dystrophy. Nine normal subjects and nine subjects with muscular dystrophy performed a pursuit tracking task with step wave target by means of isometric contraction, and simultaneously estimated magnitude of muscular tension during tracking behavior. The results were as follows: (1) the muscular electrical activity measured from the EMGs was directly proportional to the muscular tension for both the normal and the muscular dystrophy groups, (2) the speed of step response for the muscular dystrophy group was slower than that for the normal group, and (3) the exponent of power function for the muscular dystrophy group tended to be smaller than that for the normal group. These results were discussed in terms of the stage of disability in progressive muscular dystrophy.

Child↗

PubMedDate not supplied

Clinico-morphological correlative aspects in progressive muscular dystrophy.

The author's 15-year experience in the investigation of progressive muscular dystrophy (PMD) is presented. Clinical and paraclinical (biochemical, electromyographical and morphohistochemical) parameters were estimated and correlated in patients with facioscapulohumeral (FSH), Duchenne (D) and limb-girdle (LG) types of muscular dystrophy. The value of each kind of investigation was also estimated in the establishment of the anatomoclinical diagnosis of a degenerative muscular affection.

Adolescent↗

PubMed2002

[Association between dystrophin and neuronal nitric oxide synthase in muscles of progressive muscular dystrophy].

OBJECTIVE: To study the association between dystrophin and neuronal nitric oxide synthase in muscles of progressive muscular dystrophy patients and the role of deficiency of nNOS in pathogenesis of muscular dystrophy. METHODS: NADPH diaphorase enzyme histochemistry and anti-nNOS, anti-dystrophin, and anti-alpha, beta, gamma, delta-sarcoglycan antibody immunohistochemistry were used to analyze the muscle specimens from progressive muscular dystrophiy patients. RESULTS: Both nNOS and dystrophin were absent in the sarcolemma region of Duchenne muscular dystrophy (DMD) patients. Dystrophin was reduced, and nNOS was absent or reduced in the sarcolemma region of Becker muscular dystrophy (BMD) patients. Both nNOS and dystrophin were expressed normally in the sarcolemma region of limb girdle muscular dystrophy (LGMD) patients. CONCLUSION: Deficiency of nNOS is associated with deficiency of dystrophin in the sarcolemma. Dystrophin may have a novel role in localizing nNOS to sarcolemma and regulating the expression of nNOS. Aberrant regulation of nNOS may contribute to degeneration of muscle fibers in DMD.

Adolescent↗

PubMed1988

[Pseudohypertrophic forms of progressive muscular dystrophy with the onset at puberty and a malignant course of the myodystrophic process].

The article describes two familial cases of pseudohypertrophic progressive muscular dystrophy with an onset in the pubertal age and a malignant course of the myodystrophic process. The cases presented are the first ever reported in the world literature. The questions of inter- and intrafamilial polymorphism of recessive X-linked forms of progressive muscular dystrophies are discussed.

Adolescent↗