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Biomedical subjects

L Z Stern

Publications and source records attributed to L Z Stern.

At least 73 records · Page 4Linked to original sources

Ultrastructural fiber typing in normal and diseased human muscle.

The differential staining of the 3 fiber types for oxidative enzyme activity at the histochemical level provides the basis of their identification at the ultrastructural level. Type I fibers have the largest and most numerous mitochondria, the Type IIA smaller and less numerous mitochondria, and Type IIB have the smallest and least numerous mitochondria as studied in 4 patients without neuromuscular disease. Type I fibers could be distinguished from Type II fibers on the basis of mean Z-line width and IIA fibers could be distinguished from IIB fibers on the basis of mean M-line width. Type I fibers had wide Z-lines (95 nm) and wide M-lines (89 nm), Type IIA had narrow Z-lines (74 nm) and wide M-lines (79 nm) and Type IIB fibers had narrow Z-lines (69 nm) and narrow M-lines (60 nm). This system of fiber typing based on relative Z-line and M-line widths was applied to several abnormal muscle biopsies each of which showed a deficiency in at least 1 of the major histochemical fiber types. In each case, the same deficiency was revealed at the ultrastructural level by measuring the relative Z-line and M-line widths of the remaining fiber types.

Adenosine Triphosphatases↗

Hyperthyroid myopathy. Intracellular electrophysiological measurements in biopsied human intercostal muscle.

Morphological and electrophysiological studies were performed on intercostal muscle biopsies from 2 thyrotoxic patients. The diseased fibers had numerous areas of subsarcolemmal glyogen accumulations and abnormal membranous projections. Both Type I and Type II muscle fibers were atrophied. Diseased fibers were substantially depolarized and when artifically hyperpolarized showed earlier inactivation of the sodium conductance as a function of membrane potential, and a critical depolarization potential more depolarized than in normal fibers. When stimulated at 20 pulses/sec, or faster, the diseased fibers could not generate normal action potentials due to membrane depolarization and the appearance of a marked after-hyperpolarization. Muscle weakness associated with hyperthyroidism is attributed to the reduced membrane excitability.

Adult↗

Intercostal muscle biopsy in human neuromuscular disease. Histochemical and electron microscopic studies.

External intercostal muscle biopsies were examined histochemically and by electron microscopy. The use of this muscle allowed correlation with physiological and pharmacological studies on the same specimens. Changes observed in musclar dystrophy and motor neurone disease resembled those previously described in biopsied limb muscle and underline the particular usefulness of this preparation in the study of human neuromuscular disease.

Adenosine Triphosphatases↗

Myopathy associated with linear scleroderma. A histochemical and electron microscopic study.

Pathologic findings in biopsied rectus femoris muscle underlying an area of linear scleroderma are described. The affected muscle was weak and atrophic. On electromyography it showed motor unit potentials of decreased amplitude and duration. Light microscopic changes were minimal. There was atrophy of some histochemical type I fibers. More prominent changes were found at the ultrastructural level, where many of the capillary basal laminae were thickened and reduplicated. Most striking was the presence of two types of electron-dense, rounded inclusions within the mitochondria, one ranging from 29 to 47nm in diameter and the other from 54 to 131 nm in diameter.

Action Potentials↗

Adult-onset acid maltase deficiency: a postmortem study.

In a postmortem study of a patient with adult-onset acid maltase deficiency (AMD), morphological abnormalities were confined to skeletal muscle and consisted of a vacuolar myopathy. Acid maltase activity, however, was approximately 6% of normal in muscle, liver, and brain, and 3% of normal in heart. Kinetic characteristics, and inhibition by antibodies and Zn++, showed that the residual activity was "authentic" acid maltase. Neutral maltase activity was normal in muscle and liver, but decreased in brain (55% of normal) and heart (19% of normal). Although the relative decrease of acid maltase was similar in different tissues, absolute residual activity was lowest in skeletal muscle: this may explain the selective involvement of this tissue in late-onset AMD.

Adult↗

Electrophysiologic properties of intercostal muscle fibers in human neuromuscular diseases.

Electrophysiologic properties of biopsied normal and diseased intercostal muscle fibers were examined using intracellular microelectrode techniques. The resting potentials of all diseased muscle fibers were found to be depolarized. Those from Duchenne dystrophy patients showed the largest depolarization, followed by those from patients with myotonic muscular dystrophy, myotonia congenita, and motor neuron disease. All of the diseased fibers except those from myotonia congenita patients demonstrated an imparied ability to generate action potentials. In the latter fibers, the higher-than-normal membrane resistance was associated with hyperexcitability. When the membrane was hyperpolarized to the normal range, however, action potential characteristics in all fibers were near normal, except in motor neuron disease. All action potentials were blocked by tetrodotoxin. These findings--i.e., that all fibers were capable of generating action potentials when hyperpolarized, and that all action potentials were blocked by tetrodotoxin--suggest the relative intactness, in the disease studied here of the regenerative sodium conductance mechanism.

Action Potentials↗

Observations on the innervation of human extraocular muscles.

The innervation of human extraocular muscles (EOM) was studied using a comprehensive histological and histochemical approach. In contrast to previous concepts, we have demonstrated that the coarse felderstrucktur muscle fibers are singly innervated by en plaque endings and that the fine and granular fibrillenstruktur fibers are multiply innervated by en grappe endings. Each of the fine and granular fibers is innervated by a single thin axon that travels along the fiber and sends collaterals to the en grappe endings. These findings may provide a new basis for the correlation of morphological and physiological properties of EOM.

Adenosine Triphosphatases↗

[Myositis specific autoantibodies as a new diagnostic criterion for idiopathic inflammatory myopathies].

Myositis specific autoantibodies (MSA) are the most specific diagnostic criteria for idiopathic inflammatory myopathies (IIM). There is no evidence of MSA presence in patients with other neuromuscular or connective tissue diseases. MSA are associated with homogeneous clinical syndromes: antisynthetases with antisynthetase syndrome, anti-SRP with severe, resistant to treatment myositis, anti-Mi-2 with classic, benign dermatomyositis. Therefore it is important to include the myositis specific antibodies into routine diagnostic scheme of IIM.

Autoantibodies↗