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Biomedical subjects

F Jerusalem

Publications and source records attributed to F Jerusalem.

At least 73 records · Page 4Linked to original sources
PubMed1977

[Exercise-induced muscular weakness, myalgia and contractures. II. Casuistic contribution].

Two sisters with exercise-induced myalgias, muscular weakness and contractures are reported. Neurologic and psychiatric findings were normal, nor did electromyography, serum enzymes and ischemic forearm exercise test reveal specific findings. Under the light microscope multiple muscle fibers contained small vacuoles which stained positive with Oil Red O. No further information resulted from histochemical examinations. In the more seriously affected patient morphometric analysis of electronmicrographs showed a tenfold increase of fat content in comparison to normal controls. Alterations in the enzymatic activities could be demonstrated in glycogenolysis, glycolysis and in fat metabolism, but there were no previously described enzyme defects such as phosphorylase deficiency, phosphofructokinase deficiency, phosphohexoisomerase inhibitor, carnitine or carnitine-palmityltransferase deficiency. Myopathy with lactic acidosis, prolonged muscular relaxation due to deficient reabsorption of sarcoplasmic calcium in the tubular system and dyskalemic paralysis can be ruled out on the basis of the clinical signs. This myopathy may be due to an as yet unknown metabolic disturbances.

Adult↗
PubMed1977

[Myasthenia gravis. New results on etiology, diagnosis and therapy].

Myasthenia gravis is discussed with reference to recent immunologic findings, improvements in diagnostic procedure, and therapy. Myasthenic reactions can be produced in animals by injection of receptor proteins. In addition, the injection of thymopoietin causes neuromuscular block. The majority of patients with myasthenia gravis have antibodies against acetylcholine receptors, and there is a markedly reduced number of bungarotoxin binding receptors at the endplates. Further, recent immunologic findings indicate that genetic factors may be involved in the etiology of myasthenia gravis. The recently developed single fiber electromyography is useful in the diagnosis of myasthenia gravis. New statistics demonstrate the value of thymectomy in the treatment of myasthenia gravis. Good results have been reported following treatment with corticosteroids and azathioprine.

Adrenal Cortex Hormones↗
PubMed1976

[Selective muscle fiber type anomalies in neuromusclar disorders. An analysis of 124 consecutive muscle biopsies (author's transl)].

The following parameters were measured and calculated in 124 consecutive muscle biopsies: mean fiber diameter, standard deviation, percentage of type I and Type II fibers, variability coefficient, hypertrophy and atrophy factor. Twenty percent of the histometrically analyzed biopsies showed a type II atrophy and four percent a type I atrophy. Type II atrophy was found particularly in the following disorders: collagen vascular diseases, steroid myopathies, cachexia and as a result of inactivity. Some neurogenic processes also demonstrated a selective type II atrophy. The combination of a grouped type II atrophy with a type I hypertrophy is characteristic of chronic and usually heredodegenerative disorders of the motoneurons. The presence of a selective type II atrophy argues against a genetically determined muscular dystrophy. A mixed atrophy classified here as strong or very strong primarily suggests a neuropathy. A selective type I hypertrophy has been found exclusively in neurogenic processes, and type II hypertrophy predominantly in the cases of chronic heredodegenerative neurogenic and primarily myopathic diseases. An increase of the variability coefficient of both types of muscle fibers is more frequent and pronounced in neurogenic processes than in myopathic syndromes. Type II fibers show a selective increase in the variability coefficient considerably more often than type I fibers. In contrast to other reports we seldom found a fiber type predominance or a pathological type-grouping. Only two out of five biopsies with pathological fiber type-grouping were definitely neurogenic. In special cases the histometric analysis of muscle fiber types improves the diagnostic efficiency of muscle biopsies.

Atrophy↗
PubMed1976

[Myasthenia gravis in old age. A retrospective study of 58 patients].

The course in 58 cases of myasthenia gravis in subjects over 50 years of age is reported. They total about 30% of cases in all age-groups. 36.2% of the patients died after an average period of illness of 3.4 years. 58.6% initially showed only ocular symptoms and in 58.8% of these the myasthenia was likewise located only in the eyes in the further course of the illness. The prognosis of ocular myasthenia is very good if the myasthenic process does not extend to other muscle groups in the first or second year of illness. 41.2% of primary ocular myasthenia turned into other types, in most cases a generalized type, and these had a poor prognosis. 69.2% of these patients died after an average period of illness of 1.5 years due to myasthenia-induced complications. Cases of myasthenia which initially are of the generalized type, in which the average duration is only 2.7 years until death, and to some extent the bulbar types, also have a poor prognosis. Ocular myasthenic cases generally need only small doses of cholinesterase inhibitors. Some patients had no medical treatment because there was no subjective or objective need for it. Several patients were given doses of cholinesterase inhibitors which were too large; excessive doses are to be avoided because of the proven negative influence of cholinesterase inhibitors on the myoneural system. It is recommended that types of myasthenia with a poor prognosis be treated early with corticoids, ACTH or azathioprin, if the cholinesterase inhibitors have proved ineffective or it their effect is unsatisfactory. Thymectomy is advisable only in patients under 60 years of age.

Aged↗
PubMed1976

Hypotheses and recent findings concerning aetiology and pathogenesis of the muscular dystrophies.

The survey reports recent findings and current hypotheses on the aetiology and pathogenesis of the muscular dystrophies. Briefly presented are (1) biochemical anomalies of structure and metabolism, (2) membrane defects, (3) the neural hypothesis, (4) the vascular hypothesis, and (5) the connective tissue hypothesis. At present, research interest is focused primarily on membrane structure and biochemistry, on neural muscle trophism, and on the genetic aspects of abnormalities in molecular biology. Whether the progressive muscular dystrophies are primary disorders of voluntary muscle or whether the primary alteration is located outside of the muscle still remains unknown.

Cell Membrane Permeability↗
PubMed1975

Lipid storage myopathy with normal carnitine levels.

A 28-year-old female, who showed a floppy baby syndrome during early infancy, had a non-progressive proximal muscle weakness with easy fatiguability since childhood. Two muscle specimens biopsied at the age of 28 years revealed myriads of 1-3-mum wide abnormal spaces containing neutral fat in type I and type II fibers. Both biopsies demonstrated a type I fiber preponderance. Electron microscopy demonstrated lipid excess and normal mitochondria by simple inspection. The mitochondrial area and sarcotubular membrane profile concentration in morphometry of longitudinal sections were also normal. Cross-sections, however, revealed a slight decrease of the individual mitochondrial size and of the sarcotubular membrane profile concentration . Serum and muscle carnitine levels and the muscle carnitine palmityltransferase level were all within normal range. Besides carnitine deficiency other biochemical defects can occur in lipid storage myopathy, which represents a syndrome rather than a unique disease entity.

Acetyltransferases↗
PubMed1975

Human muscle fiber fine structure: morphometric data on controls.

Muscle fiber fine structure was quantitatively analyzed in 70 longitudinally and 65 transversely sectioned fibers from 10 control subjects without weakness. The average mitochondrial fraction of the fiber volume is close to 4 percent and the mean size of a mitochondrion is about 0.1 mum2. The sarcotubular surface area per unit fiber volume is close to 1.5 mum2/mum3 in transverse sections and 0.65 times this value in longitudinal sections. Only one-third of all fibers contain lipid droplets in the sectioned plane, and for all fibers the droplets account for approximately 0.12 percent of the fiber volume. Variations with the age and sex of the subjects and with different muscles were analyzed and the feasibility of typing human muscle fibers at the ultrastructural level was evaluated.

Adult↗