ISSN:
1432-0533
Keywords:
Rimmed vacuole
;
Distal myopathy with rimmed vacuole formation
;
Inclusion body myositis
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract In 11 patients with distal myopathy with rimmed vacuole formation (DMRV), a well-known autosomal recessively inherited disorder, the rimmed vacuole formation appears to be the main pathological change accounting for the progressive muscle fiber degeneration. To gain a better understanding of the pathophysiology of the vacuole formation, we applied Congo red and immunohistochemical stains to muscle biopsies from these patients and the results were compared with those of patients with inclusion body myositis (IBM). The vacuoles in DMRV contained Congophilic amyloid material and deposits immunoreactive for β-amyloid protein, both the NH2 and COOH termini of β-amyloid protein precursor, ubiquitin, and tau protein. These results were similar to those seen in our present cases of IBM as well as in previously reported cases. Therefore, there may be no pathogenetic differences in the formation of rimmed vacuoles in DMRV and IBM. Nevertheless, the degenerative process involved in rimmed vacuole formation in various diseases may share a common pathogenetic mechanism with that in amyloid-plaque formation in Alzheimer's disease brain as has been proposed previously.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00294256
