Alterations in muscle spindle morphology in advanced stages of murine muscular dystrophy

Muscle spindles in the soleus of 1-year-old dystrophic mice of the C57BL/6J dy2J/dy2J strain were studied by microscopic and morphometric methods, and comparisons were made with those in age-matched normal tissue. Transverse epon sections were cut through various regions of an individual receptor, and subsequent 90 degrees reorientation enabled longitudinal examination of the same spindle. In dystrophy, alterations were detected in the outer capsule and consisted of a significant increase in its overall thickness in equatorial regions. Perineurial proliferation accompanied histiocyte and collagen infiltration. Within the equator, intrafusal fibers and sensory terminals appeared unaffected by dystrophy. Alterations in the intrafusal fibers were restricted to polar zones where the mean diameters of chain and bag fibers were significantly reduced. Polar chain fibers exhibited a greater degree of atrophy in dystrophy with a 40% diminution in size. Ultrastructural changes in intrafusal fiber polar regions were less pronounced compared to the surrounding dystrophic muscle. Mitochondrial alterations in affected intrafusal fibers included intramatrix inclusions and glycogen deposition. Vacuolization of the sarcoplasmic reticulum and subsarcolemmal tubular aggregates were also observed in polar regions of dystrophic chain fibers. Regional variation in spindle involvement in advanced murine dystrophy provides evidence that the equatorial contents of this receptor are sequestered from the deleterious effects of the disease. Capsular thickening in the equator may be an adaptive response, preventing the intrafusal fibers from undergoing the moderate change and atrophy observed at their polar ends.