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  • 1
    Digitale Medien
    Digitale Medien
    Springer
    Acta neuropathologica 53 (1981), S. 169-172 
    ISSN: 1432-0533
    Schlagwort(e): Pallido-nigro-luysial atrophy ; Combined system degeneration ; Progressive supranuclear palsy ; Corpora amylacea
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary A case of pallido-nigro-luysial atrophy, characterized by symptoms typical of progressive supranuclear palsy, presented (1) severe symmetrical abiotrophic degeneration of the globus pallidus, substantia nigra, and subthalamic nucleus and (2) widespread accumulations of corpora amylacea in the CNS. The former is thought to be consistent with pallidonigro-luysial atrophy and we regard it as a combined system degeneration of both the pallido-nigral and the pallido-luysial systems. The significance of the latter is quite obscure. As far as we know, only three cases of pallido-nigro-luysial atrophy have been reported. However, there is no reported case of this disorder with such widespread and massive accumulations of corpora amylacea as in our case.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    ISSN: 1432-0533
    Schlagwort(e): Key words Dentatorubropallidoluysian atrophy ; Dementia ; Nucleus basalis of Meynert ; Neuropathology
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract This report concerns a clinicopathological study including a quantitative pathological study on the nucleus basalis of Meynert (nbM) of seven Japanese autopsy cases (four male, three female) of dentatorubropallidoluysian atrophy (DRPLA) with special reference to the clinicopathological correlation of dementia in DRPLA. In each case the pattern of the inheritance was consistent with that of an autosomal dominant trait. The neurological examination revealed that all seven individuals had cerebellar signs. Six patients had epilepsy and choreoathetoid involuntary movement; myoclonus was evident in five patients. Dementia was noted in all seven patients. Degeneration of the globus pallidus (particularly the lateral segment) and of the dentate nucleus was the principal pathological feature. Brain weights at autopsy ranged from 1020 to 1400 g (average 1241 g: male 1320 g, female 1135 g). The quantitative evaluation revealed no significant loss of neurons in the nbM as compared with a control group. There was no clinicopathological correlation between dementia and involvement of the nbM. We suggest that the dementia of DRPLA is due not to the involvement of the nbM, but to – as yet – unidentified pathology elsewhere.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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