LATE ONSET MYOPATHY WITH ROD-LIKE PARTICLES

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Abstract
A 74-year-old man gave a 17-year history of progressive, proximal weakness in the extremities. He had electrophysiological signs of myo-pathy. Biopsy from two proximal muscles in the leg 7 years after the onset of the disease showed inflammatory infiltrates. Ten years later a biopsy from the brachial biceps muscle showed numerous rod-like structures in atrophic fibres on Gomori's trichrome stain. Histochemical study of the brachial biceps muscle showed increased cross-sectional area occupied by fibres rich in mitochondrial enzymes (type C). One third of the fibres of type C were hypertrophic and half of the fibres of type A or B were atrophic. Nemaline myopathy was originally described as a congenital disease (Shy et al. 1963, Engel et al. 1964, Spiro & Kennedy 1965). Since then five patients have been reported in whom the myopathy had its onset late in life (Engel 1966, Engel & Resnick 1966, Hopkins et al. 1966, Hefferman et al. 1968). The present report deals with a sixth patient with late onset who was studied histochemically.

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