An electron microscopic study of muscle in Werdnig-Hoffmann's disease
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An ultrastructural study of the quadriceps muscle of a 6 1/2 month old infant with Werdnig-Hoffman's disease has been made. The diagnosis was established on the basis of clinical and laboratory findings, the electromyographic examination and the light microscopic post-mortem study of the spinal cord and different muscles of the extremities.
The fine structural changes of denervation observed in the different components (myofibrils, nucleus, sarcolemmal sheath) of the muscle fibers proper are comparable with those previously described by other authors. The most striking feature in this study is the relative high number of cells which fulfil the criteria given by Mauro (1961) and which are designated satellite cells.
Cells lying free in the interstitial spaces or in proximity of capillaries and showing great morphological resemblance to “pericytes” are thought to be precursors of the satellite cells. The findings further suggest that these satellite cells may assume myoblast-like properties and that regeneration or restoration could be brought about by activity of these cells.
No sufficient evidence can be given that the presence of satellite cells and the submicroscopic changes of the contractile component of the muscle fibres in Werdnig-Hoffmann's disease are due to or represent arrest of maturation.
KeywordsPublic Health Spinal Cord Muscle Fiber Structural Change Microscopic Study
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