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  • Electronic Resource  (3)
  • Central nervous system  (2)
  • Electron microscopy  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 80 (1990), S. 541-546 
    ISSN: 1432-0533
    Keywords: Mitochondria ; Central nervous system ; Skeletal muscle ; Pathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The neuropathological changes found at autopsy in a case of Kearns-Sayre syndrome are described. We have previously analyzed the respiratory chain function in isolated muscle mitochondria and also described a large deletion of muscle mitochondrial DNA (mtDNA) in this case. The neuropathological examination revealed prominent neuronal degeneration and gliosis of the basal ganglia and there were bilateral areas of softening and total loss of nerve cells in the lenticular nuclei. The pallidum and caudate nucleus disclosed accumulation of iron-containing pigment. The white matter in the cerebrum, brain stem and cerebellum showed widespread and focally accentuated spongy change due to splitting of myclin lamellae. It is suggested that deficiency of respiratory chain enzymes due to the mtDNA deletion is of pathogenetic importance in the development of the described changes.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 54 (1981), S. 121-127 
    ISSN: 1432-0533
    Keywords: Rat ; Protein deprivation ; Nerve fibre degeneration ; Central nervous system ; Peripheral nervous system
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Knowledge from previous reports that kwashiorkor in man may lead to nerve fibre degeneration prompted this study on rats. The rats were subjected to severe protein deprivation from 6 weeks of age. Protein deprivation was achieved by feeding the rats ad lib with a diet containing only 1.5% protein. Control rats received an iso-caloric diet with 14% protein. The vitamin content in both diets was well above normal requirements. In relation to body weight the protein-deprived rats did not consume less food than the control rats. Protein deprivation resulted in stunted body growth, markedly reduced values of serum albumin, and changes in the fur accompanied by areas of alopecia. Furthermore, the protein-deprived rats showed degeneration of nerve fibres in the medial parts of the posterior columns of the cervical but not the sacral part of the spinal cord and nerve fibre degeneration in the distal but not the proximal parts of the longitudinal tail nerves. Teased nerve fibre preparations of the tail nerves revealed changes consistent with the Wallerian type of degeneration. It is concluded that severe protein deprivation in young rats may lead to a “dying-back” type of neuropathy in the central and peripheral nervous systems.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 54 (1981), S. 287-292 
    ISSN: 1432-0533
    Keywords: Mucopolysaccharidoses III ; Sanfilippo's syndrome ; Histochemistry ; Electron microscopy ; Lipofuscin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A histochemical and ultrastructural study was made on the brain of a 23-year-old man with Sanfilippo's syndrome. In accordance with previous reports the cortical nerve cells contained a PAS-positive lipid storage substance. This showed intense autofluorescence in UV-light and was positive with various stains for lipofuscin. The storage material appeared ultrastructurally as inclusion bodies composed of short lamellated membranes, granular material, and vacuoles. In addition, concentrically and transversely lamellated membranous cytoplasmic bodies were observed in the nerve cells. It is concluded that the PAS-positive lipid storage material in the neurons was composed partly of lipofuscin in addition to other lipids presumably glycosphingolipids.
    Type of Medium: Electronic Resource
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