Amyloidosis causing a progressive myopathy.
A 62-year-old woman developed profound weakness secondary to a progressive myopathy associated with primary systemic amyloidosis. The characteristic apple-green birefringent amyloid deposits were demonstrated surrounding individual muscle fibers in Congo red stained sections. Electron microscopy demonstrated amyloid filaments in close apposition to muscle fibers exhibiting excessive corrugations of the sarcolemmal membrane. The pathological features of progressive amyloid myopathy associated with primary systemic amyloidosis are distinct from the intracellular amyloid deposits characteristic of sporadic inclusion body myositis and inherited inclusion body myopathy.
Nadkarni
N
N
Department of Neurology, College of Medicine, Ohio State University, Columbus 43210, USA.
Freimer
M
M
Mendell
J R
JR
D000686:Amyloidosis / Q000473:pathology*; D005260:Female; D006801:Humans; D008475:Median Nerve / Q000502:physiology; D008854:Microscopy, Electron; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000473:pathology; D009135:Muscular Diseases / Q000473:pathology*; D009431:Neural Conduction / Q000502:physiology; D014459:Ulnar Nerve / Q000502:physiology