Experimental Myopathy After Microarterial Embolization

Duchenne Muscular Dystrophy: Functional Ischemia Reproduces Its Characteristic Lesions

“…2, b and c) surrounded by histologically normal muscle fibers. This pattern was identical to that characteristic of early Duchenne dystrophy (1,2). Sometimes there were larger focal lesions of 20 to 100 fibers or more, in which the interior fibers had preferential loss of mitochondrial oxidative enzymes and nuclei with preservation of myofibrils and myofibrillar adenosine triphosphatase; at the periphery the fibers were being phagocytosed and regeneration was beginning.…”

Possible neurogenic factor in muscular dystrophy: its similarity to denervation atrophy

“…The rate and manner of breakdown of muscle fibres appears more responsible for the release of CPK, than any alleged difference in pathogenesis -such as 'myopathic' or 'neuropathic'. A process such as microinfarction would be more likely to release this enzyme, and the recent suggestion of Hathaway, Engel, and Zellweger (1970), that embolization of intramuscular vessels with resultant infarction closely simulates spontaneous muscular dystrophy, is most thought-provoking. A propos of this, it may be recalled that it is the large hypertrophied fibre which exhibits successively the typical 'dystrophic' changes of rounding and homogenization, central nucleation, and, terminally, either splitting or necrosis.…”

Functional muscle ischemia in Duchenne and Becker muscular dystrophy

“…The idea was that vascular insufficiency at the level of the microcirculation caused selective infarction of only those muscle fibers supplied by the obstructed blood vessels, while nearby fibers supplied by unobstructed vessels were not affected (Bramwell, 1925; Demos and Escoiffier, 1957; Cazzato, 1968). Initial experimental support for this vascular hypothesis came from studies performed more than 40 years ago in which the characteristic focal lesions of DMD muscle were reproduced in the muscles of healthy animals by occlusion of intramuscular arterioles with dextran beads, or by functional ischemia induced by a combination of arterial ligation and vasoconstrictor injection (Hathaway et al, 1970; Mendell et al, 1971, 1972). However, subsequent morphological studies did not reveal any fixed anatomical abnormalities in the skeletal muscle microcirculation of DMD patients, with the exception of replication of the capillary basal lamina (Jerusalem et al, 1974; Musch et al, 1975; Koehler, 1977; Leinonen et al, 1979).…”