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[Value of electromyography in the child. Apropos of 1,624 examinations performed over a 3 year period].
EMG and nerve conduction studies have limitations and require particular consideration in children. The indications and the main results are considered from the study of 1624 EMGs in 1385 children under 15 years old seen over a period of 3 years. Classification of cases was based on clinical criteria. The diagnostic yield of EMG is emphasized in the evaluation of 122 children with hypotonia and weakness (all under 3 years old); abnormalities were demonstrated in 48% leading to the diagnosis of spinal muscular atrophy in 16%, of congenital myopathies in 11%, and of peripheral neuropathies in 8%. In 103 children with hypotonia associated with mental deficiency and/or seizures, evidence of a peripheral neuropathy was shown in 20%. EMG abnormalities allowing the diagnosis of hereditary motor and sensory neuropathies or myopathies were demonstrated in a wide range of clinical conditions. EMG appears to be useful for the early detection of hereditary myopathies or neuropathies in asymptomatic children. It may have also a prognostic value as in adults, especially in nerve traumas.
[Differential diagnosis of "primary" and "secondary" myopathies by electromyography].
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Pyruvic and lactic acid metabolism in muscular dystrophy, neuropathies and other neuromuscular disorders.
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Enzymes of the glycogen cycle and glycolysis in various human neuromuscular disorders.
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5'-Nucleotidase activity of normal and dystrophic human muscle.
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Developmental and maturational aspects of inherited avian myopathies.
Three inherited abnormalities of muscle growth of poultry are an inherited muscular dystrophy of the chicken, deep pectoral myopathy of turkeys and broilers, and focal myopathy of turkeys. The major features of each are described and compared. Cellular and molecular bases of dystrophy of the chicken and treatments to alleviate the disorder are discussed. The pathologic progression and anatomical basis for deep pectoral myopathy are presented. Evidence is given that focal myopathy of the turkey is a growth-dependent disorder. The implications of the idea that such disorders are partly consequences of selection are discussed in the context of the future needs of a poultry industry emphasizing processing of poultry meat.
[The value of additional diagnosis in the differentiation between neuropathies and myopathies].
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Clinical neurology.
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Rigid spine syndrome: a muscle syndrome in search of a name.
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[Histological observations on muscle biopsy specimens].
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Neuromuscular disorders: clinical aspects.
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[Electrodiagnosis of caudal cranial nerve disorders in infants and small children].
The endoscopic application of electrodes for electromyography of laryngeal and pharyngeal muscles is routinely performed in adults and very helpful in electrodiagnosis of voice and speech disturbances. Dysfunctions of the lower cranial nerves in infancy and childhood are congenital diseases in most of the cases. Up to now the most helpful diagnostic tool has been direct laryngoscopy which, however, could not classify the type of peripheral or central neural or even muscular disease. It is only by electrophysiological methods that the laryngologist can answer these questions. Electromyography and neuromyography as well as reflex myography of the lower cranial nerves have been performed in ten children since 1979, enabling diagnosis and classification of congenital and acquired lesions in the muscles of tongue, soft palate, pharynx and larynx. In particular accurate diagnosis of laryngeal malacia with intact peripheral innervation and voluntary activity during electromyography is always possible. In addition, the method is helpful in the planning of therapy which consists in either conservative or surgical treatment.
On monitoring structural changes of anisotropic tissues by means of statistical analysis of ultrasonic images.
An investigation into statistical properties of ultrasonic image texture from three-dimensional clusters of anisotropic scatterers is carried out. The structural properties of the clusters are modeled after those of soft biological tissues, such as skeletal muscle tissues, both in their healthy condition and at the early stage of degenerative diseases. The average axial autocorrelation function of the intensity of the image texture is used to characterize and monitor changes of the geometrical properties of the tissue components. A distinct local increase of the autocorrelation is observed within a range of small time shifts, and it is explained in terms of the structure of the time-domain backscattered signal from each individual scatterer. It is shown that such an increase is sensitive to structural variations of the cluster similar to those occurring at the early stage of several muscular diseases.
Loss of selenoprotein N function causes disruption of muscle architecture in the zebrafish embryo.
Mutations in the gene coding for selenoprotein N (SelN), a selenium containing protein of unknown function, cause different forms of congenital muscular dystrophy in humans. These muscular diseases are characterized by early onset of hypotonia which predominantly affect in axial muscles. We used zebrafish as a model system to understand the function of SelN in muscle formation during embryogenesis. Zebrafish SelN is highly homologous to its human counterpart and amino acids corresponding to the mutated positions in human muscle diseases are conserved in the zebrafish protein. The sepn1 gene is highly expressed in the somites and notochord during early development. Inhibition of the sepn1 gene by injection of antisense morpholinos does not alter the fate of the muscular tissue, but causes muscle architecture disorganization and greatly reduced motility. Ultrastructural analysis of the myotomes reveals defects in muscle sarcomeric organization and in myofibers attachment, as well as altered myoseptum integrity. These studies demonstrate the important role of SelN for muscle organization during early development. Moreover, alteration of myofibrils architecture and tendon-like structure in embryo deficient for SelN function provide new insights into the pathological mechanism of SelN-related myopathy.
Creatine phosphokinase isozymes in muscles. Human fetus and patients.
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Myosin light chains in normal and pathological human skeletal muscles.
Thirty-nine human skeletal muscle biopsies from 24 individuals were classified as normal, neuropathic, or myopathic muscle according to classical clinical observations and histopathological properties of the muscles. The content in myosin light chains (LC) of each muscle sample was analyzed by means of a new technique of polyacrylamide gel electrophoresis that gives an improved discrimination, involving isoelectrofocusing of the muscle homogenate for the first dimension and successive migration in a urea-containing gel for the second dimension. Four different LC patterns have been observed in the normal muscles; these four patterns and three different ones have been observed in the pathological muscles. No apparent correlation exists between the myosin LC content and the histochemical fiber typing. It is concluded that the myosin LC are apparently not a useful marker to detect the normality or the pathology of human muscle.