Elsevier

Human Pathology

Volume 29, Issue 10, October 1998, Pages 1128-1133
Human Pathology

Original contribution
Rimmed vacuoles with β-amyloid and ubiquitinated filamentous deposits in the muscles of patients with long-standing denervation (postpoliomyelitis muscular atrophy): similarities with inclusion body myositis

https://doi.org/10.1016/S0046-8177(98)90425-7Get rights and content

Abstract

In the chronically denervated muscles of patients with prior paralytic poliomyelitis, there are secondary myopathic features, including endomysial inflammation and rare vacuolated fibers. To assess the frequency and characteristics of the vacuoles and their similarities with those seen in inclusion body myositis (IBM), we examined 58 muscle biopsy specimens from patients with prior paralytic poliomyelitis for (1) the presence of rimmed vacuoles; (2) acid-phosphatase reactivity; (3) Congo-red-positive amyloid deposits; (4) electron microscopy, searching for tubulofilaments; and (5) immunoelectron microscopy, using antibodies against β-amyloid and ubiquitin. We found vacuolated muscle fibers in 18 of 58 (31%) biopsies, with a mean frequency of 2.06 ± 0.42 fibers per specimen. The vacuoles contained acid phosphatase-positive material in 6 of the 18 (33.30%) specimens and stained positive for Congo red in five (27.80%). By immunoelectron microscopy, the vacuoles contained 5.17 ± 0.13 nm fibrils and 14.9 ± 0.31 nm filaments that immunoreacted with antibodies to β-amyloid and ubiquitin in a pattern identical to the one seen in IBM. We conclude that vacuolated muscle fibers containing filamentous inclusions positive for amyloid and ubiquitin are not unique to IBM and the other vacuolar myopathies but can also occur in a chronic neurogenic condition, such as postpoliomyelitis. The chronicity of the underlying disease, rather than the cause, may lead to vacuolar formation, amyloid deposition, and accumulation of ubiquitinated filaments.

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      Citation Excerpt :

      These proteins may accumulate in the cytoplasm of muscle fibers due to malfunctioning of the proteasome machinery, direct toxicity of monomers or oligomers of aberrant proteins and generation of cell stress, as described by Askanas et al. [1–3,7]. Many of the noted accumulations however do not appear specific for IBM because they are also found in other vacuolar myopathies especially myofibrillar, hereditary IBM or even in chronic neurogenic conditions such as the postpolio syndrome [89–96]. Autophagic processing, which is relevant to degradation of intracellular proteins, may also play a role since the vacuoles have autophagic properties [97] involved in processing of APP/β-amyloid.

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