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PubMed1979

[Further observations by electron microscope of striated muscle in progressive Duchenne muscular dystrophy].

Gastrocnemius muscle fragments of children affected by clinically diagnosed progressive muscular dystrophy of Duchenne have been studied. At the light microscope, in the semi-thin sections, the more evident changes are represented by a wide diameter range of the fibers and fatty infiltration. Some fibers show numerous nuclei in their central part, a sarcoplasmic degeneration of vacuolar type and an irregular and tortuous course of the myofibrils. Moreover, the ultrastructural findings have shown characteristic changes in myofilaments and Z bands represented by: streaming of the Z bands, collection of the triads and concentric laminated bodies. These observations have pointed out a certain gradualness of the alterations, starting from focal changes of Z band to the complete disarrangement of myofilaments.

Child↗
PubMed1989

[Effect of sinestrol on the course of the myodystrophic process in progressive Duchenne muscular dystrophy].

Therapeutic effect of sinestrol was investigated in 15 patients with progressive muscular dystrophy of Duchenne (PMDD) aged 7 to 10 years, at stage II of the disease. The drug was given orally 1 mg twice a day for 3 weeks. Control group consisted of 14 patients with PMDD aged 7 to 9 years. By the end of the course a several relief of motor constraint was noted in 10 patients with functional tests improved, tendon reflexes increased. The results of clinico-electromyographic investigation performed 6 months after the sinestrol withdrawal evidenced progressive course of the disease, though its rate was significantly lower in sinestrol-treated group. The treatment did not produce considerable changes in the baseline hormonal profile (gonadotropins, prolactin, sexual steroids).

Administration, Oral↗
PubMed1993

Effects of electrical stimulation on muscles of children with Duchenne and Becker muscular dystrophy.

Twelve children with progressive muscular dystrophy (10 Duchenne and 2 Becker type) were included in a low-frequency electrical stimulation (LFES) program of the right tibialis anterior (TA) muscle for three months. Muscle strength was estimated by measuring torques in the ankle during short attempts of maximal voluntary isometric contraction (MVIC) in the direction of dorsal flexion of the foot. Muscle fatigue was assessed by the decrease of force during sustained (1-minute) voluntary contraction. The measurements were carried out before the beginning of the stimulation program and immediately after its conclusion. At the end of the stimulation program there were higher torques in 10 out of 12 children in the stimulated leg. The increase in torques in the stimulated leg was statistically significant (p < 0.01). Regarding the fatigue of the stimulated muscle there was no change after the conclusion of stimulation.

Child↗
PubMed1990

[Computerized tomography findings in malignant progressive Duchenne muscular dystrophy].

A correct diagnosis of progressive muscle dystrophy type Duchenne is important for consultation of the family of patients. The muscles of neck, shoulder, back, pelvis and legs of 23 patients were examined by computed tomography. The density of selected muscles were measured and compared with that of normal probands. Typical changes in the density-pattern were found. It is show, that the measurement of the density-pattern of muscles in pelvis and legs is sufficient for a sure diagnosis of progressive muscle dystrophy Duchenne.

Adolescent↗
PubMed2000

Elevated plasma levels of transforming growth factor beta1 in patients with muscular dystrophy.

The progression of muscular weakness of patients suffering from muscular dystrophies directly correlates with the progressive loss of myofibers, accompanied by fibrosis. Since transforming growth factor beta1 (TGF-beta1) promotes tissue fibrosis, we measured the plasma TGF-beta1 level in patients with various muscular dystrophies in order to determine whether the level is elevated in patients with muscular dystrophy and if the level reflects the severity of tissue fibrosis. The plasma TGF-beta1 level was significantly elevated in patients with Duchenne muscular dystrophy and congenital muscular dystrophy (CMD), but not in those with Becker muscular dystrophy. Growth factors related to muscle fiber regeneration and fibrosis might be a key factor in the progression of muscular dystrophy and could be a target for therapeutic studies.

Adolescent↗
PubMed1996

Exercise and clenbuterol as strategies to decrease the progression of muscular dystrophy in mdx mice.

The effects of exercise and the combination of exercise and clenbuterol on progression of muscular dystrophy were studied in mdx mice. At 3 wk of age, mdx mice were randomly assigned to sedentary (MS), exercise (ME), or combined exercise and clenbuterol (MEC) groups. Clenbuterol was given in the drinking water (1.0-1.5 mg . kg body weight-1 . day-1), and exercise consisted of spontaneous running activity on exercise wheels. At 3 mo or 1 yr of age, ventilatory function, contractile properties, and morphological characteristics of the soleus (Sol) and diaphragm (Dia) muscles were measured. The mdx mice receiving clenbuterol ran less than the mice without clenbuterol. The combination of clenbuterol and exercise was associated with an increase in Sol muscle weight and a muscle weight-to-body weight ratio of 30-35% compared with the sedentary group and approximately 20% compared to exercise alone. Myosin and total protein concentrations of the Sol and Dia increased in the MEC group at 1 yr of age only. Normalized active tension was increased in the Dia at 1 yr of age in both the ME and MEC groups by approximately 30%. Absolute tetanic tension of the Sol was increased at both 3 mo and 1 yr of age in the MEC compared with the MS group. At 1 yr of age, there was an additional 23% increase compared with the ME group. Fatigability increased in the MEC group by approximately 25% in the Sol and Dia muscles at both ages compared with the MS and ME groups. Results indicate that exercise and exercise plus clenbuterol decrease the progression of muscular dystrophy. However, different mechanisms may be involved because the combination of clenbuterol and exercise resulted in increased fatigability and the development of deformities, whereas exercise alone did not. Therefore, clenbuterol may not be suitable for use in patients with muscular dystrophy.

Animals↗
PubMed1978

[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)].

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.

Adult↗
PubMed1992

Long-term electrical stimulation of muscles in children with Duchenne and Becker muscular dystrophy.

Nine children suffering from progressive muscular dystrophy (7 Duchenne and 2 Becker) were included in a program of low-frequency electrical stimulation (LFES) of the right tibialis anterior (TA) muscle. Muscle strength and muscle fatigue were estimated by measuring torques in the ankle during attempts of maximal voluntary contraction (MVC) in the direction of dorsal flexion of the foot and during electrically evoked contractions (EEC). No important increase in the strength of the stimulated muscles was noticed in 4 boys whose muscles were stimulated for 3 months. The muscles of 5 boys who were subjected to electrical stimulation for 9 months showed an improvement; 6 measurements made during the stimulation program revealed that changes of torques in the ankle of the right stimulated extremity were significantly different (P less than 0.001) from the changes of torques in the ankle of the left nonstimulated extremity.

Child↗
PubMed1989

Downward shift in IQ in persons with Duchenne muscular dystrophy compared to those with spinal muscular atrophy.

Patients with Duchenne progressive muscular dystrophy (DMD) and those with spinal progressive muscular atrophy (SPMA) were compared on the basis of memory and IQ. The DMD group was inferior to the SPMA group in memory tests and Full Scale IQ. The DMD group showed poorer scores, despite the fact that functional disabilities and social environments were similar for the two groups. Results thus support the current view that lower IQ is one of the primary manifestations of DMD.

Adolescent↗
PubMed2005

Fascioscapulohumeral muscular dystrophy: a progressive degenerative disease that responds to diltiazem.

The authors believe that with fascioscapulohumeral muscular dystrophy (FSHD), like Duchenne muscular dystrophy, there is Ca2+ dysregulation in the muscle cells. The dysregulated Ca2+ can cause cell death in various ways. One mechanism may be Ca2+ triggering abnormal levels of tumor necrosis factor (TNF-alpha). Another mechanism may involve excessive Ca2+ levels within the mitochondria which would cause this organelle's membrane to collapse ultimately inducing apoptosis and/or necrosis. With this in mind, it has been reported that in FSHD there is over expression of adenine nucleotide translocator-1 (ANT-1). This Ca2+ dependent protein, which is a component of the mitochondrial permeability transition pore, could be an important culprit in mitochondrial membrane collapse. Therefore, dysregulated Ca2+ as well as TNF-alpha, in addition to over-expression of ANT-1, may result in cell disruption ultimately causing the characteristic dystrophic muscle wasting. The present investigators have noted that some individuals with FSHD benefit from a regimen of diltiazem, a Ca2+ channel blocker. Initial results using diltiazem may represent the first beneficial treatment for a form of muscular dystrophy. Even if there is only a slowing of progression, this would be a positive first step. A combination of several different Ca2+ regulating agents and TNF-alpha inhibitors may be necessary to truly alter and/or reverse the deleterious effects of this form of muscular dystrophy.

Adenine Nucleotide Translocator 1↗
PubMed1977

An autoradiographic study of muscular dystrophy, motor neuron disease and Charcot-Marie-Tooth disease.

The autoradiographic findings using tritiated leucine are described in muscle biopsy material from five patients with progressive muscular dystrophy (P.M.D.), three with motor neuron disease (M.N.D.) and four with Charcot-Marie-Tooth disease (C.M.T.). In progressive muscular dystrophy there is a marked increase in uptake of leucine into cytoplasmic proteins and precursors, and reduced incorporation into structural protein. In Charcot-Marie Tooth disease muscle there is a significantly increased uptake into cytoplasmic elements and a normal uptake into structural protein. In motor neuron disease the uptake into cytoplasmic elements appears normal but is reduced into structural proteins. The abnormal uptake in C.M.T. could be explained as a product of regenerative efforts associated with reinnervation. However, the abnormal uptake may represent the primary effects of gene action in the muscle, as seems probable in progressive muscular dystrophy.

Charcot-Marie-Tooth Disease↗