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  • 1
    Electronic Resource
    Electronic Resource
    Clarke's column in sporadic amyotrophic lateral sclerosis (1992)
    Springer
    Acta neuropathologica 84 (1992), S. 465-470 
    Details
    ISSN: 1432-0533
    Keywords: Amyotrophic lateral sclerosis ; Clarke's column ; Bunina body ; Spheroid ; neuronal loss
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Histological, ultrastructural and morphometrical observations on Clarke's column were carried out in 18 patients with sporadic amyotrophic lateral sclerosis (ALS) and 15 age-matched control subjects. Of the 18 ALS patients 6 had been on a respirator before death. Bunina bodies were found in the neuronal cytoplasm in 7 of the 12 non-respirator-supported ALS patients and in 3 of the 6 respirator-supported patients. The number of spheroids was significantly higher in the non-respirator-supported patients (P〈0.01) than in the control subjects; however, the number in the respirator-supported patients was about equal to that in the controls. The number of neurons in Clarke's column in the non-respirator-supported ALS patients was not reduced, but in the respirator-supported patients they tended to disappear with time after respiratory support. These findings suggest that Clarke's column neurons are also involved primarily in the disease process in sporadic ALS. However, they may begin to disappear only after the patients require respiratory support.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00304464
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    The intermediolateral nucleus in sporadic amyotrophic lateral sclerosis (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 190-192 
    Details
    ISSN: 1432-0533
    Keywords: Amyotrophic lateral sclerosis ; Intermediolateral nucleus ; Quantitative examination ; Bunina body ; Neuronal loss
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Histological and morphometrical observations of the intermediolateral nucleus (IML) at the levels of the upper and lower thoracic segments (T2 and T9) were carried out in 18 patients with sporadic amyotrophic lateral sclerosis (ALS) and 15 age-matched control subjects. Of the 18 ALS patients 6 had been on a respirator before death. No Bunina bodies were found in the IML neurons in either the ALS patients or the control subjects. Only a small number of spheroids were encountered rarely in the IML in both the patients and controls. The number of neurons in the IML in the non-respirator-supported ALS patients were reduced at T2, but well preserved at T9 compared with the control subjects. In the respirator-supported ALS patients, there was a marked reduction of IML neurons at both T2 and T9. Considering the absence of direct synaptic contacts with anterior horn cells, these neurons, without the formation of Bunina bodies, appeared to be involved primarily in the disease process in sporadic ALS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00334889
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Hereditary dentatorubral-pallidoluysian atrophy: ubiquitinated filamentous inclusions in the cerebellar dentate nucleus neurons (1998)
    Springer
    Acta neuropathologica 95 (1998), S. 479-482 
    Details
    ISSN: 1432-0533
    Keywords: Key words Dentatorubral-pallidoluysian atrophy ; Cerebellar dentate nucleus neuron ; Skein-like inclusion ; Ubiquitin ; Amyotrophic lateral sclerosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We examined the cerebellar dentate nucleus (CDN) in 16 patients with hereditary dentatorubral-pallidoluysian atrophy (DRPLA), one of the neurodegenerative diseases caused by expansion of a CAG repeat encoding a polyglutamine tract in the disease protein. In all patients, some CDN neurons were found to contain ubiquitinated filamentous inclusions in their cytoplasm. On hematoxylin and eosin preparations, these filamentous inclusions were eosinophilic, basophilic or amphophilic, and were often found in areas of pale cytoplasm. Electron microscopy revealed that they consisted of bundles of filaments that were somewhat thicker than neurofilaments. These features of the present inclusions were indistinguishable from those of skein-like inclusions (SLI) previously described in the lower motor neurons in sporadic amyotrophic lateral sclerosis. We conclude that SLI can also occur in the CDN in DRPLA and believe that they reflect a characteristic pathological process in this disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050828
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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