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LATE ONSET MYOPATHY WITH ROD‐LIKE PARTICLES
Author(s) -
Kamieniecka Zofia
Publication year - 1973
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1973.tb01328.x
Subject(s) - muscle biopsy , biceps , myopathy , biopsy , medicine , pathology , anatomy , trichrome stain , nemaline myopathy , immunohistochemistry
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.