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  • Intracytoplasmic inclusion bodies  (1)
  • Rimmed vacuolar distal myopathy  (1)
  • 1
    ISSN: 1432-0533
    Keywords: Myotonic dystrophy ; Intracytoplasmic inclusion bodies ; Marinesco bodies ; Thalamus ; Substantia nigra
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Intracytoplasmic inclusion bodies of the thalamus and the substantia nigra, and Marinesco bodies have been studied in four patients with myotonic dystrophy (MyD), eight patients with other neurological diseases (control A), and eight patients without neurological diseases (control B). The percentages of the affected cells were calculated by dividing the number of neurons including intracytoplasmic inclusion bodies of the thalamus and the substantia nigra, and Marinesco bodies, by the total cell count in these respective regions. Statistical analyses were performed with regard to the frequency of these bodies by using Student'st test. There was a significantly higher incidence of intracytoplasmic inclusion bodies of the thalamus (13.2% versus 0.7%,P〈0.001) and the substantia nigra (20.4% versus 2.7%,P〈0.001), and Marinesco bodies (37.4% versus 4.1%,P〈0.001) in patients with MyD than in controls A and B. From our observations, it is suggested that the presence with a high frequency, in combination, of these bodies is not an incidental finding but may have an intimate and important relationship with the pathogenesis of MyD, and may be a conspicuous and diagnostically important feature of MyD.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 234 (1987), S. 137-145 
    ISSN: 1432-1459
    Keywords: Rimmed vacuolar distal myopathy ; Autophagic vacuole ; Golgi's apparatus ; Lysosome ; Tubulomembranous structure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An ultrastructural study of biopsied muscles was performed in seven patients with rimmed vacuolar distal myopathy, which was characterized by prominent rimmed vacuoles in the muscle fibers. The earliest changes noted were focal proliferation of the Golgi's apparatus and mitochondrial degeneration with myofibrillar loss. A proliferation of the T-system appeared later. Secondary lysosomes (autophagosomes) could be noted much later and gradually increased in number. Autophagosomes tended to coalesce and became larger autophagic vacuoles, which were surrounded in part by relatively preserved myofibrils and partly by a single membrane. Gently curved laminated structures (tubulomembranous structures) were seen in the degenerating muscle fibers and also in relatively intact fibers, satellite cells, and interstitial cells in all cases. They were closely associated with lipofuscin-like material. These findings suggest that an abnormality of the lysosomal system might be essential in the pathogenesis of rimmed vacuolar distal myopathy.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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