title: [Significance of rimmed vacuoles in neuromuscular disorders--a comparative immunohistochemical study of inclusion body myositis and distal myopathy with rimmed vacuole formation].
abstract: Of 3,403 muscle biopsies from patients with a variety of neuromuscular disorders, 87 (2.6%) had at least three muscle fibers with rimmed vacuoles in a single low power field (magnificantion x 20). The rimmed vacuole formation may be a crucial pathologic findings to understand the pathogenesis of inclusion body myositis (IBM), distal myopathy with rimmed vacuole formation (DMRV), Marinesco-Sjögren syndrome and oculopharyngodistal myopathy, because the muscle fibers in these biopsies showed no striking necrotic and denervating changes. Since Congo red positive material and ubiquitin- and beta-amyloid protein-positive deposits were found in muscle fibers with rimmed vacuoles in DMRV and various neuromuscular disorders besides IBM, these findings may have no disease-specific significance and may be not helpful in differentiating DMRV from IBM.
authors.affiliation: Department of Pediatrics, Kochi Medical School.
authors.forename: N
authors.initials: N
authors.lastname: Murakami
authors.forename: M
authors.initials: M
authors.lastname: Takemitsu
authors.forename: T
authors.initials: T
authors.lastname: Kurashige
authors.forename: I
authors.initials: I
authors.lastname: Nonaka
mesh_terms: D003937:Diagnosis, Differential; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies; D018485:Muscle Fibers, Skeletal / Q000648:ultrastructure*; D009220:Myositis / Q000473:pathology; D009468:Neuromuscular Diseases / Q000473:pathology*; D014617:Vacuoles / Q000648:ultrastructure