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Results for “Muscle atrophy”

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PubMed1979

Sulphur and phosphorus content in relation to fibre composition and atrophy of skeletal muscle in patients with Parkinson's disease.

Seventeen patients with Parkinson's disease have been compared with 8 normal individuals by biopsy of either the biceps brachii or quadriceps femoris muscles. All biopsies were investigated by enzyme histochemistry. With 13 patients, as well as all controls, scanning electron microscopy with X-ray microanalysis was employed on cryo-sections adjacent to those prepared for light microscopy. Thus, the elemental composition of single muscle fibres was obtained and could be related to histochemical fibre types. Fibre type analysis on the diseased material, based on differential stainability for alkali- and acid-stable ATPase, showed a normal type I and type IIA fibre frequency. A mild type IIB dominance at the expense of type IIA fibres was regarded as a significant deviation from normal. A slight to moderate muscle atrophy affected type IIB fibres almost exclusively. Normal content of sulphur and phosphorus was detected in type I and type IIA Fibres but a lowered sulphur content was obvious in type IIB fibres, especially in the atrophic ones, which also exhibited an increase in phosphorus content. The shift in fibre composition from IIA to IIB, the type IIB fibre atrophy and the change in sulphur and phosphorus content of type IIB fibres are interpreted as signs of a disuse which preferentially affects fast twitch type IIB motor units. These presumably have the highest threshold for activation under pathological conditions characterized by increased muscular tone and difficulties in the performance of rapid and strong voluntary movements.

Adenosine Triphosphatases↗
PubMed1979

Gyrate atrophy of the choroid and retina with hyperornithinemia: tubular aggregates and type 2 fiber atrophy in muscle.

We studied 21 patients with gyrate atrophy of the choroid and retina and hyperornithinemia. Although the patients were not weak, type 2 muscle fibers were almost universally atrophic and had tubular aggregates. Gyrate atrophy is the first disease in which females are shown to have tubular aggregates; the sexes were affected equally. In gyrate atrophy the number of type 2 fibers decreases with age. The muscle and eye changes are probably related to abnormal creatine synthesis, caused, in gyrate atrophy, by the increased body pool of ornithine; muscle abnormalities may also be present in other tapetoretinal dystrophies.

Adolescent↗
PubMed1977

A progressive congenital myopathy. Initial involvement of the diaphragm with type I muscle fiber atrophy.

An uncommon case of initial respiratory distress during the first months of life as the result of bilateral diaphragmatic weakness is presented. The biopsy and necropsy findings show a progressive congenital myopathy with type I muscle fiber atrophy and predominant involvement of the respiratory muscles. The lesions, observed in the central nervous system are due to the severe hypoxia. The morphological findings are discussed in relation to the etiology and the clinical picture of the disease.

Brain↗
PubMedDate not supplied

Fine structure of muscle in human disuse atrophy: significance of proximal muscle involvement in muscle disorders.

The universal occurrence of weakness of skeletal musculature on disuse, however produced, and the paucity of published reports on the fine structural changes in human disuse atrophy, prompted the present investigation. The quadriceps muscle of a leg immobilized in plaster cast (for fracture) and of the opposite non-immobilized limb was biopsied in four adult males, after periods of immobilization from 50 to 75 days. These 8 muscle specimens were examined for histopathological changes, and muscle fibre diameters were measured by micrometry from paraffin sections. The histograms revealed a larger proportion of small fibres (less than 20 micron) and a smaller proportion of large fibres (greater than 40 micron) in the immobilized limb compared to the opposite. Thus, light microscopy showed only atrophic changes. This was confirmed by electronmicroscopy, where atrophy of few to several muscle fibres was seen in the form of loss of myofibrils, collapse and folding of the basement membrane and prominence of glycogen or muscle nuclei. The atrophic change was more severe in the immobilized limbs, but it was also noticeable in all the non-immobilized limbs. Degenerative changes, especially disorganization and breakdown of myofibrils, and fragmentation of plasma membrane, were also seen in occasional atrophied muscle fibres, again more frequently in the immobilized limb. Lipofuscin was often found accumulated in muscle fibres and occasionally in endothelial cells of intramuscular blood vessels; the latter showed prominent pinocytotic vesicles or thickened basement membrane. It is concluded that both atrophy and degeneration of fibres of proximal muscles can occur as non-specific consequences of disuse of the limb in man, that degeneration is a latter and more severe change, that muscles even of the non-immobilized leg are subjected to disuse atrophy during bed-rest, and that the proximal muscles in man seem to have a natural susceptibility to atrophy and degeneration in any muscular disorders.

Adult↗
PubMed1978

Structure and function of the motor unit. Effects of immobilization, monoparesis or paraplegia.

Single motor units have been studied in the medial gastrocnemius muscle in cat following acute or chronic unilateral hind limb immobilization or antero-lateral spinal cord section. Immobilization produces muscle atrophy by 5 weeks and in the chronic phase (4-6 months) is characterized functionally by reduced twitch and tetanus tensions and structurally by fiber atrophy without type grouping or preponderance. Antero-lateral cord section with minimal motor dysfunction does not produce muscle atrophy or change in tension but in the chronic animals there is a slight decrease in twitch-tetanus ratios. Changes in the functional properties of motor units reflect changes in structure and both may result from secondary effects (i.e., type and amount of muscle activity, muscle length, pain, etc.) of a neuromuscular disease on muscle.

Animals↗
PubMed1978

Muscle fiber atrophy after cast immobilization in the rat.

The purpose of this study was to evaluate the effect of 6 weeks of bilateral cast immobilization on the adult Wistar rat plantarflexors. Knee and ankle joints were fixed in the neutral position, under sodium pentobarbital anesthesia. Controls (n = 7) and cast-immobilized (n = 7) rats were sacrificed at 6 weeks after the onset of the experimental period. The muscles were evaluated for muscle weight, protein content, and fiber histochemistry. There was equal weight loss in the gastrocnemius, soleus, and plantaris muscles, and greater loss of myofibrillar protein than of sarcoplasmic protein concentrations. Fiber atrophy of types I and II was equal in the soleus, and that of type II was greater than the type I atrophy in both regions of the plantaris. All soleus and 2 plantaris muscles demonstrated fiber damage. The results of this experiment suggest that the degree of fiber atrophy is specific to the fiber type and to its location in different muscles or muscle regions.

Animals↗
PubMed2026

Clinical and genetic analysis of a family with 16p11.2 microduplication syndrome and variable multisystem manifestations.

16p11.2 microduplication syndrome (OMIM #614671) is a pathogenic recurrent copy-number gain at the 16p11.2 locus and is associated with variable expressivity across neurodevelopmental, growth, and medical phenotypes. Gastrointestinal symptoms have been reported in carrier cohorts, but detailed documentation of gastrointestinal motility and neuromuscular findings remains limited. We performed clinical and genetic analyses in a multigenerational family in which the proband (III1) presented with limb muscle pain, exercise intolerance, and chronic gastrointestinal symptoms. Next-generation sequencing (NGS), low-pass whole-genome sequencing (lpWGS)-based CNV analysis, Sanger sequencing, and qPCR validation identified a 0.8 Mb microduplication at 16p11.2 (BP4-BP5), involving 44 genes including TBX6, inherited from the mother (II2). The proband's clinical manifestations included developmental delay, pointed chin, low body mass index, gastrointestinal dysfunction (chronic abdominal pain, diarrhea, esophageal motility disorder, and rectal prolapse), forward-leaning gait, mild scoliosis, and limb muscle atrophy with inflammatory muscle involvement. Four family members (II2, III1, III2, and III4) carried the microduplication, but their available clinical features varied in severity and system involvement. The proband's twin brother (III2) had left ear deafness and epilepsy, individual II2 had blindness from cone-rod dystrophy, and III4 showed more pronounced scoliosis. This family provides a detailed clinical and genetic description of 16p11.2 microduplication carriers with prominent gastrointestinal motility and neuromuscular manifestations, thereby enriching the clinical characterization of this recurrent CNV and supporting substantial intrafamilial phenotypic heterogeneity.

16p11.2 microduplication syndrome↗
PubMed1979

Caloric and exercise requirements of space flight: biostereometric results from skylab.

The biostereometric study of the Skylab astronauts used stereophotogrammetry to make accurate three-dimensional measurement of body form, from which regional and total body volumes were derived. Volume changes in the thighs and calves, over the course of the flight, showed a high correlation with inflight exercise on the bicycle ergometer, and suggested that an exercise level of 80-100 W-min/d/kg lean body mass would be necessary to prevent inflight muscle atrophy. The bicycle ergometer is thus a relatively inefficient means of preventing leg muscle atrophy. Inflight caloric intake showed a high correlation with the change in volume of the buttocks, the abdomen, and the body as a whole, and suggested that a caloric intake of 47-51 kcal/d/kg lean body mass would be necessary to prevent a change in body fat. Only one of the astronauts exceeded this range and gained body fat; the group as a whole showed a mean fat loss of 1.2 kg.

Adult↗
PubMed1978

Lipid storage myopathy in von Gierke's disease: a case report.

An 18-year-old girl with von Gierke's disease associated with a lipid storage myopathy is reported. The diagnosis of von Gierke's disease was made from decreased activity in glucose-6-phosphatase in the jejunal biopsy specimen. Neurologically she showed generalized hypotonia of the muscles, atrophy of bilateral proximal muscles of the lower extremities, weakness in neck flexors, deltoid and lumbar girdle muscles, and a positive Gower's sign. Muscle biopsy from flexor femoris muscle revealed fatty deposition in type 1 fibers and atrophy of type 2 fibers and the diagnosis of an accompanying lipid storage myopathy was made. This case also had a ventricular septal defect confirmed by right cardiac catheterization.

Adolescent↗
PubMed1978

[Changes in cholinesterase activity of gastrocnemius and soleus muscles of the rat after crush of the sciatic nerve, and effects of a vitamin B complex on those changes (author's transl)].

In an analysis of the gastrocnemius muscle, the microsomal fraction showed the highest cholinesterase (ChE) activity. The ChE activity of all fractions decreased to a greater extent after strong nerve crushing than after weak crushing. This change in the activity in the microsomal fraction was the most marked change observed. Although in the analysis of the soleus muscle the ChE activity was measured only in the homogenate and in the microsomal fraction, the results were the same as those obtained with the gastrocnemius. A preparation of vitamins B1, B2, and B12 (B complex) had little effect on the ChE activity in the gastrocnemius muscle. In the soleus muscle on the lesion side, the B complex increased the ChE activity to some extent after nerve crushing, but such was not significant. However, the B complex signifiicantly increased this activity in the soleus on the intact side. In the soleus muscle, strong nerve crushing induced more marked muscle atrophy than weak crushing. On the other hand, no significant difference was found in the gastrocnemius. Effects of the B complex on muscle atrophy were found in the soleus, but not in the gastrocnemius. These results suggest that the ChE activity in the microsomal fraction containing sarcoplasmic reticulum reflects the nervous disorders clearly, and that the B complex increases the ChE activity and muscle weight in the soleus, but not in the gastrocnemius muscle.

Animals↗
PubMed2026

miR-191 affects skeletal muscle differentiation by regulating Wwp1 in mouse myoblasts.

Skeletal muscle atrophy is a key complication of various diseases, such as chronic obstructive pulmonary disease (COPD) and cancer. The mechanisms by which these diseases affect skeletal muscle metabolism need to be deeply explored. By analyzing the miRNA expression profiles in the plasma of patients with COPD, we found that miR-191 expression was significantly altered and it may influence skeletal muscle metabolism by regulating ubiquitination and the mTOR pathway. Using a mouse model of skeletal muscle injury induced by cardiotoxin, we found that miR-191 and Wwp1 showed a dynamic negative correlation in injury repair. Transfection with miR-191 mimics significantly inhibited the expression of myogenic regulatory factor Myog and differentiation markers Myh1/7/8, while downregulating key genes in the mTOR pathway. Molecular mechanism studies showed that miR-191 could directly act on the 3' untranslated region of the Wwp1 gene to inhibit its expression. This study reveals the important role of the miR-191/Wwp1 axis in skeletal muscle differentiation and provides a novel theoretical basis for research on muscle atrophy induced by COPD, cancer cachexia, and other diseases.

Animals↗
PubMed1976

[Amyotrophic lateral sclerosis with myoclonic bodies (Lafora bodies) (author's transl)].

In the present investigation 2 sporadic cases of amyotrophic lateral sclerosis with the occurrence of myoclonic bodies (Lafora bodies) in the CNS are reported. The patients died after about 14 respectively 8 months lasting disease at the age of 41 and 43, respectively under the clinical signs of an ALS. Morphology, staining qualities, and the distribution of the myoclonic bodies in our cases correspond to the adult form of the myoclonic body disease (type Lundborg). In the literature we found further 6 cases of neurogenic muscle atrophy with the occurrence of myoclonic bodies in the CNS so that one might suppose a syntropy of these two affections. The possible pathophysiological relations of neurogenic muscle atrophy to the occurrence of myoclonic body disease in adults are briefly discussed.

Adult↗
PubMed1979

Clioquinol and 2,5-hexanedione induce different types of distal axonopathy in the dog.

The central distal axonopathy induced in dogs by the administration of high doses of clioquinol is contrasted with the central-peripheral distal axonopathy precipitated by intoxication with 2,5-hexanedione. Mature, pure-bred Beagle dogs received a daily oral dose of 400 mg/kg of clioquinol for up to 7 months, or 1 ml per animal (approximately corresponding to 110 mg/kg) of 2,5-hexanedione for up to 5 months. Intoxicated and control animal were killed and perfused at monthly intervals, so that the spatial-temporal development of the lesion could be followed and correlated with clinical symptoms. During the treatment, dogs intoxicated with 2,5-hexanedione developed symptoms of peripheral neuropathy consisting of flaccid weakness, muscle atrophy, hind-limb foot-drop and areflexia. By contrast, the dogs surviving clioquinol intoxication exhibited a stiff-legged gait, hyperreflexia but no muscle atrophy. Light and electron microscope examination of central and peripheral nervous tissue from dogs intoxicated with 2,5-hexanedione revealed giant axonal swelling and distal axonal degeneration. By contrast, dogs receiving clioquinol showed a distal axonal degeneration confined to the optic tract and the long spinal cord tracts, without any visible involvement of peripheral nerves.

Animals↗
PubMed1975

Lipid disturbances associated with spiral muscular atrophy. Clinical, electromyographic, histochemical, and lipid studies.

Twelve patients with juvenile- and adult-onset spinal muscular atrophy have been studied. Eleven of the twelve patients had either type II, type IV, or borderline abnormal phenotypes, suggesting a possible relationship between serum lipid abnormalities and neuronal degeneration in the spinal muscular atrophies. Muscle enzyme histochemical studies provided valuable diagnostic information. Extensor toe signs and talipes cavus were common clinical observations.

Adenosine Triphosphatases↗
PubMed1979

Haemophilic neuromyopathy.

Twenty haemophilic patients were examined neurologically and found to have varying degrees of muscle atrophy. The pathogenesis of the atrophy was investigated by electromyography, serum muscle enzyme levels, and muscle biopsies. In most cases the EMG showed such abnormalities as reduced numbers of functioning motor units and "myopathic" motor unit potentials. Serum enzyme levels were frequently raised, and type 2 fibre atrophy was found in the three patients who underwent muscle biopsy. These findings suggest the presence of neuromyopathic involvement in haemophilic patients.

Adolescent↗