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  • 1
    Digitale Medien
    Digitale Medien
    Miniplaques and shapeless cerebral amyloid deposits in a case of Gerstmann-Sträussler-Scheinker's syndrome (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 532-535 
    Details
    ISSN: 1432-0533
    Schlagwort(e): Amyloid ; Prion protein ; Plaques ; Transmissible spongiform encephalopathy
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Multicentric plaques of typical pathomorphology have been described in two members of the well-documented German family “Sch” with Gerstmann-Sträussler-Scheinker's syndrome (GSS). The case of a third affected member with a clinical course of 10 years is now reported. In addition to multicentric plaques, small granules of prion protein (PrP) with stellate ultrastructure (miniplaques) were found in the tractus perforans and alveus of the hippocampal area. At the ultrastructural level, shapeless amyloid deposits of irregularly arranged bundles of amyloid fibrils were also observed in the same region. All amyloid precipitates apparent at the light microscopic level immunostained for PrP. These observations widen the known spectrum of amyloid plaque morphology in GSS. The multicentric plaque should still, however, be considered the diagnostic hallmark of this disease.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00228593
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  • 2
    Digitale Medien
    Digitale Medien
    Subakute spongiforme Encephalopathie mit multiformer Plaquebildung (1980)
    Springer
    Acta neuropathologica 49 (1980), S. 205-212 
    Details
    ISSN: 1432-0533
    Schlagwort(e): Spinocerebellar atrophy ; Dementia ; Plaques ; Slow virus ; Spongiform encephalopathies
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Report of two unrelated cases of a rare familiar disease of degenerative nature, from a clinical point of view belonging to the spinocerebellar atrophies, combined with dementia. According to the pedigrees, the disease can be followed up to 3–4 generations. Microscopic study reveals glioneuronal dystrophy with spongiform changes together with kuru plaques and atypical plaque-like formations, hitherto only described in this disease. Nosological aspects of these findings are discussed.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00707108
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 3
    Digitale Medien
    Digitale Medien
    Gerstmann-Sträussler-Scheinker's disease (1980)
    Springer
    Acta neuropathologica 52 (1980), S. 203-211 
    Details
    ISSN: 1432-0533
    Schlagwort(e): Dementia ; Plaques ; Kuru ; Spongiform encephalopathies
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Light and electron microscopic observations are reported on a brain biopsy of a man of 59 with a rare familial disease of the CNS and a 5-year clinical course. Electron micrographs of the frontal biopsy reveal plaque-like deposits composed of amyloid cores, often multicentric in the cortex and subcortical white matter. They are localized between enlarged astrocytic processes. In the neuropil they are sometimes associated with abnormal neuritic processes, in the white matter with processes of fibrous astroglia and basement membranes. There are no signs of primary neuritic or synaptic involvement in the plaque formation which is more obviously associated with altered astrocytic processes. Moreover, degenerative alterations in the cortical vessels and slight astroglial spongiform changes as well as oligodendroglial proliferation can be found. Plaques are considered to be mainly of the kuru type; the relationship with transmissible spongiform encephalopathies is discussed.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00705808
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