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  • 1
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Morbus Parkinson ; Motorisches System ; Limbisches System ; Lewy-Körper ; Lewy-Neuriten ; Zytoskelett ; α-Synuklein ; Key words Parkinson's disease ; Motor system ; Limbic system ; Lewy bodies ; Lewy neurites ; Cytoskeleton ; α-Synuclein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Morbus Parkinson is a progressive degenerative disease of the human central, peripheral, and enteric nervous systems. In the course of the disease, not only the substantia nigra, but also extranigral components of the motor system, as well as numerous limbic system and autonomic centers undergo serious damage. Accordingly, Parkinson's disease is a multisystem disorder. Only specific types of projection neurons fall victim to it. The first manifestation of the pathological process which causes the disease are abnormalities of the neuronal cytoskeleton. Intracytoplasmic inclusions evolving in the form of Lewy bodies in perikarya and Lewy neurites in the neuronal processes result in premature cell death of the affected neurons. On the basis of this selective neuronal vulnerability, a categorization emerges of the pathological changes within the nervous system and corresponding functional impairments.
    Notes: Zusammenfassung Der Morbus Parkinson ist eine stetig voranschreitende degenerative Erkrankung des zentralen, peripheren und enterischen Nervensystems des Menschen. Im Verlauf der Erkrankung erleiden neben der Substantia nigra eine Reihe von extranigralen Komponenten des motorischen Systems sowie zahlreiche Zentren des limbischen Systems und der autonomen Regulation schwerwiegende Zerstörungen. Der Morbus Parkinson ist daher eine Multisystemerkrankung. Nur bestimmte Arten von Projektionsneuronen entwickeln die für die Erkrankung charakteristischen Veränderungen des neuronalen Zytoskeletts. Das Ergebnis dieser Veränderungen sind intraneuronale Einschlusskörper in Form von Lewy-Körpern in den Zellleibern und Lewy-Neuriten in den Zellfortsätzen, die zum vorzeitigen Absterben der betroffenen Nervenzellen führen. Aufgrund der selektiven neuronalen Vulnerabilität ergibt sich eine charakteristische Verteilung der Veränderungen innerhalb des Nervensystems mit entsprechenden Einbußen der Funktionsfähigkeit zahlreicher Systeme.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Key words Parkinson’s disease ; α-Synuclein ; Limbic ¶system ; Motor system ; Reticular formation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Pathological changes which consistently develop in the lower brain stem of patients suffering from Parkinson’s disease are described against the background of the internal organization and interconnections of the involved nuclei, i.e., the gigantocellular reticular nucleus, bulbar raphe nuclei, and coeruleus-subcoeruleus area. Immunoreactions against the presynaptic protein α-synuclein reveal not only the voluminous forms of Lewy bodies and Lewy neurites but also the otherwise inconspicuous dot- or thread-like types. These lesions develop solely in specific neuronal types. Lipofuscin- or neuromelanin-laden projection cells which at the same time generate a long, unmyelinated or sparsely myelinated axon are particularly susceptible to developing the changes. The bulbar nuclei under consideration receive strong input from supramedullary sources, above all from higher order centers of the limbic system such as the central amygdalar nucleus, periaqueductal gray, and parabrachial nuclei. In turn, they generate descending projections to premotor and motor neurons of the somatomotor system. The disease-related deterioration of both the supramedullary limbic centers and the bulbar brain stem nuclei reduces the limbic influence and markedly impairs the control of premotor and motor neurons. This functional deficit most probably contributes to the overall dysfunction of the motor system typically evolving in the course of Parkinson’s disease.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Huntington's disease ; Human brain ; Thalamus ; Nuclei centromedianus-parafascicularis ; Neurone number
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The centromedian-parafascicular complex represents a nodal point in the neuronal loop comprising striatum – globulus pallidus – thalamus – striatum. Striatal neurone degeneration is a hallmark in Huntington's disease and we were interested in estimating total neurone and glial number in this thalamic nuclear complex. Serial 500-μm-thick gallocyanin-stained frontal sections of the left hemisphere from six cases of Huntington's disease patients (three females, three males) and six age- and sex-matched controls were investigated applying Cavalieri's principle and the optical disector. Mean neurone number in the controls was 646,952 ± 129,668 cells versus 291,763 ± 60,122 in Huntington's disease patients (Mann-Whitney U-test, P 〈 0.001). Total glial cell number (astrocytes, oligodendrocytes, microglia, and unclassifiable glial profiles) was higher in controls with 9,544,191 ± 3,028,944 versus 6,961,989 ± 2,241,543 in Huntington's disease patients (Mann-Whitney U-test, P 〈 0.021). Considerable increase of fibrous astroglia within the centromedian-parafascicular complex could be observed after Gallyas' impregnation. Most probably this cell type enhanced the numerical ratio between glial number and neurone number (glial index: Huntington's disease patients = 24.4 ± 8.1; controls = 15.0 ± 5.2; Mann-Whitney U-test, P 〈 0.013). The neurone number in the centromedian-parafascicular complex correlated negatively, although statistically not significantly, with the striatal neurone number. This lack of correlation between an 80% neuronal loss in the striatum and a 55% neurone loss in the centromedian-parafascicular complex points to viable neuronal circuits connecting the centromedian-parafascicular complex with cortical and subcortical regions that are less affected in Huntington's disease.
    Type of Medium: Electronic Resource
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