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Relationship of spontaneous fibrillation potentials to muscle fibre segmentation in human muscular dystrophy

Abstract

DENERVATED skeletal muscle fibres have long been known to show spontaneous fibrillation potentials, this being one of the features resulting from the removal of the trophic influence of the motor nerve1. Electromyography (EMG) has shown spontaneous fibrillations in human myopathies such as myositis or muscular dystrophy2–6, but their true incidence and mechanism is still obscure. We propose that myopathic fibrillations result from segmental necrosis of muscle fibres so that a distal fibre segment is separated from the part carrying the motor endplate. We show here, first, that after experimental myotomy in the baboon biceps muscle, the nerve-free segments develop fibrillation after a consistent delay and second, that by comparing different clinical types of human myopathies a correlation can be found between the incidence of focal necrosis and spontaneous fibrillation potentials. Our findings are consistent with our previous report of collateral innervation of newly formed nuscle fibres in Duchenne muscular dystrophy7.

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DESMEDT, J., BORENSTEIN, S. Relationship of spontaneous fibrillation potentials to muscle fibre segmentation in human muscular dystrophy. Nature 258, 531–534 (1975). https://doi.org/10.1038/258531a0

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