[Primitive megalencephaly with growth retardation and deficiency in growth hormone (author's transl)].
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Biomedical subjects
Publications and source records attributed to M Nathanson.
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Scattered reports, both clinical and experimental, have been accumulating in the past 20 years indicating that true seizures may, indeed, originate from the brain stem and its immediate connections. Four cases are reported that give further strong presumptive evidence that this is so. All the seizures were confined to axial structures (face, tongue, palate, pharynx, diaphragm, and abdomen), and in one case each seizure had an identical "Jacksonian march." The phenomena were documented by 16-mm motion pictures, brain stem signs, and electroencephalography. The EEG consisted of periods of burst activity followed by relative interictal electrocerebral silence.
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A 41-year-old woman, who was receiving methysergide maleate for the treatment of severe headaches, had occlusion of the left brachial artery, confirmed by arteriography. The occlusion was believed to result from the use of methysergide and the drug was therefore withdrawn. Within 4 days the distal pulses were normal, as confirmed by arteriography, and she was asymptomatic.
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A method allowing for direct observation of intern and resident physicians while interviewing and examining patients has been in use on our medical wards for the last five years. A large number of errors in the performance of the medical examination by young physicians were noted and a classification of these errors into those of technique, omission, detection, interpretation, and recording was made. An approach to detection and correction of each of these kinds of errors is presented, as well as a discussion of possible reasons for the occurrence of these errors in physician performance.
The ultrastructural appearance of the endoplasmic reticulum of the hepatocytes was found to be normal in a 5-year-old girl with alpha-1-antitrypsin deficiency with the genotype Pi--. The liver ultrastructure of this variant is therefore different from that of alpha-1-antitrypsin deficiency with the genotype PiZZ in which aggregates of an abnormal, unsecreted alpha-1-antitrypsin accumulate in the endoplasmic reticulum of the hepatocytes. The normal appearance of the endoplasmic reticulum in alpha-1-antitrypsin deficiency with the genotype Pi-- is compatible with the hypothesis, in this variant, synthesis of alpha-1-antitrypsin is completely, or nearly completely, absent; an alternative hypothesis would be that an abnormal alpha-1-antitrypsin is produced by the liver and secreted into the plasma, but disappears rapidly from the plasma.
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