Amyloidosis causing a progressive myopathy
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Updated: Oct 30, 2026

A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS
Published on: October 6, 2015
N Nadkarni1, M Freimer, J R Mendell
1Department of Neurology, College of Medicine, Ohio State University, Columbus 43210, USA.
Abstract:
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.
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