[Polymyositis].
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Biomedical subjects
Publications and source records attributed to K G Henriksson.
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An easy and safe method for muscle biopsy is described. The biopsy instrument is an alligator forceps (Weil-Bladesley's conchotome). With this method an adequate amount of muscle tissue can be obtained for both chemical and morphological studies. The method combines the advantage of the "open-surgical" method - sufficient quantity of material - with the advantages of the needle methods - easy to perform, not incapacitating and only slightly uncomfortable for the patient. Out of 959 consecutive biopsies with this method only 19 were impossible to evaluate because of too much artefact or too little tissue.
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Two patients with dystrophia myotonica and pleomorphic adenoma of the parotid gland are described. The probability that this coincidence could be due to chance is less than 1 to 1,000 (P less than or equal to 0.001). There are several reports in the literature of the association of myotonic dystrophy with neoplasia. The reason for their association is not understood.
Two patients with maysthenic syndrome apparently not associated with carcinoma were studied in detail with repetitive stimulation of motor nerves, single fibre electromyography and histology. One patient showed signs of widespread autonomic dysfunction and the other patient had multifocal CNS symptoms of unclear etiology. Both cases showed a marked improvement with guanidine treatment which could be documented by the electrophysiological investigations. Due to severe adverse effects of the guanidine treatment on kidney, pancreatic and bone marrow functions the drug had to be withdrawn. Edrophonium given intravenously caused a marked and longlasting improvement of the muscle weakness and of the neurophysiological parameters. The mechanism of action is suggested to be different from that seen in myasthenia gravis. Single fibre electromyography showed a marked increase of neuromuscular jitter and blockings which decreased with increasing innervation frequency. The morphological study showed a selective affection of type II (fast twitch) fibres, a finding which is suggested to be secondary to an impaired neuromuscular transmission in the type II motor units.
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Changes of the terminal innervation pattern in polymyositis were studied in nine patients using single fiber electromyography (SFEMG) and histochemical investigations of muscle biopsies. In all patients, and in 16 out of 18 muscles studied, reinnervation was found. The preceding denervation could be caused either by segmental necrosis of the muscle fiber that disconnects one part of the fiber from the end-plate region or by direct intramuscular nerve involvement.
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