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  • 1
    Electronic Resource
    Electronic Resource
    Progression of myopathology in Kearns-Sayre syndrome: a morphological follow-up study (1993)
    Springer
    Acta neuropathologica 85 (1993), S. 679-681 
    Details
    ISSN: 1432-0533
    Keywords: Myopathy ; Kearns-Sayre-syndrome ; Cytochrome c oxidase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report on the progression of myopathology by comparing two biopsies from a patient with a Kearns-Sayre-Syndrome. The first biopsy was taken in 1979 and showed 10% ragged-red fibers. Myopathic changes were slight including internal nuclei and fiber splitting in 10% of the fibers. Electron microscopy revealed typical mitochondrial abnormalities with regard to number and shape. In 1989 a second biopsy was performed for an extended analysis of mitochondrial DNA. This time less than 5% of all fibers were ragged-red. Severe myopathic changes could be detected which so far has rarely been reported in mitochondrial cytopathy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00334681
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Glucocorticoid-sensitive hereditary inclusion body myositis (1996)
    Springer
    Journal of neurology 243 (1996), S. 126-130 
    Details
    ISSN: 1432-1459
    Keywords: Inclusion body myositis ; Heredity ; Immunosuppressive therapy ; Morphology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a hereditary muscle disorder with features of inclusion body myositis (IBM) in two adult sisters with slowly progressive asymmetrical muscle weakness. The findings of light microscopic and ultrastructural investigations of muscle biopsy specimens were consistent with a diagnosis of IBM. Both patients improved and stabilized on immunosuppressive treatment with corticosteroids and azathioprine. This differentiates our patients from other sporadic and familial cases of IBM. Clinical and histological features are described and compared with those of other previously reported families with IBM.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02444002
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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