A 62-year-old woman developed profound weakness secondary to a progres
sive myopathy associated with primary systemic amyloidosis. The charac
teristic apple-green birefringent amyloid deposits were demonstrated s
urrounding individual muscle fibers in Congo red stained sections. Ele
ctron microscopy demonstrated amyloid filaments in close apposition to
muscle fibers exhibiting excessive corrugations of the sarcolemmal me
mbrane. The pathological features of progressive amyloid myopathy asso
ciated with primary systemic amyloidosis are distinct from the intrace
llular amyloid deposits characteristic of sporadic inclusion body myos
itis and inherited inclusion body myopathy. (C) 1995 John Wiley and So
ns, Inc.