Amyloid myopathy: A rare cause of quadreparesis with bulbar palsy

Abstract

Amyloidosis is a systemic disease that results in the deposition of amyloid fibrils in a variety of tissues causing their progressive dysfunction. Systemic amyloidosis involves Kidney, Heart, Skin, Peripheral nerve and rarely muscle. Amyloid myopathy (AM) is a rare manifestation of primary systemic amyloidosis (AL). Amyloid myopathy frequently occurs in the setting of systemic amyloidosis and less commonly in isolation (isolated amyloid myopathy). It can present with proximal muscle weakness and an increased creatinine kinase level. Muscle involvement is associated with amyloid deposits around small blood vessels and muscle fibers. We present a case of rapidly progressive muscle weakness with dysphagia. The diagnosis was confirmed by demonstration of amyloid protein by Thioflavin immune-florescence staining on abdominal fat pad biopsy.