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  • 21
    ISSN: 1432-0533
    Keywords: Neuronal ceroid-lipofuscinosis ; Membranous cytoplasmic body ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsy case of adult neuronal ceroid-lipofuscinosis was examined. The clinical picture was charaterized by gait disturbance, bulbar palsy and dementia. Histopathologically, diffuse neuronal loss was found throughout the central nervous system. The remaining neurons, predominantly in the motor nuclei of the spinal cord and brain stem, were swollen with storage material. Observed under the electron microscope the storage material showed various ultrastructures, such as lipofuscin-like bodies, pleomorphic lipid bodies, curvilinear profiles and finger-print profiles, in different regions of the central nervous system. In the ballooned neurons of the spinal anterior horn, many membranous cytoplasmic bodies and curvilinear profiles were intermingled within the same cell and were continuous with each other. Biochemically,N-acetyl neuraminic acid content was significantly increased in the spinal anterior horn. These findings suggest the localized increase of ganglioside in that region.
    Type of Medium: Electronic Resource
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  • 22
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 81 (1991), S. 408-417 
    ISSN: 1432-0533
    Keywords: Werdnig-Hoffmann disease ; Immunocytochemistry ; Ultrastructure ; Cytoskeleton ; Ubiquitin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Neuronal alterations in two cases of Werdnig-Hoffmann disease (WH) were investigated immunocytochemically and ultrastructurally. Ballooned neurons (BNs) were found in anterior horn, Clarke's column, dorsal root ganglion and thalamus. Anti-phosphorylated neurofilament antibodies preferentially stained the peripheral perikarya and proximal neuronal processes of BNs, whereas anti-ubiquitin antibodies preferentially stained the central perikarya of BNs. Ultrastructurally, BNs showed degenerative changes ranging from a diffuse increase of neurofilaments to a centrally accentuated accumulation of mitochondria and vesicular or membranous profiles. Our studies suggest that ubiquitinated degradation products accumulate in the center of the BN's perikaryon and displace aberrantly phosphorylated neurofilaments to the periphery. BNs in WH probably reflect an intrinsic alteration in the metabolism of neurofilaments that is associated with regressive changes in the neuron and eventually neuronal death.
    Type of Medium: Electronic Resource
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  • 23
    ISSN: 1432-0533
    Keywords: Dorsal root ganglion ; Onion bulbs ; Initial segment ; amyotrophic lateral sclerosis ; Friedreich's ataxia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We have demonstrated onion bulb-like structures in human dorsal root ganglia (DRG). These onion bulbs morphologically consist of non-continuous layers of supporting-cell cytoplasms that encase thinly myelinated axons and are immunocytochemically recognized by anti-S-100 protein and anti-glial fibrillary acidic protein antibodies. These structures are present in normal controls and preferentially involve the initial complex of the large, light, neurofilament-rich neurons. The number of onion bulbs and their average number of lamellae reach a peak in the third decade and then decline. In three cases of amyotrophic lateral sclerosis (ALS), the onion bulbs involve non-myelinated axons as well as the thinly myelinated portion of the initial complex and are increased both in frequency and in average number of lamellae. Our studies suggest that these onion bulbs represent a normal biological process or DRG neurons that may be accentuated in ALS.
    Type of Medium: Electronic Resource
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  • 24
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 83 (1992), S. 408-414 
    ISSN: 1432-0533
    Keywords: Eosinophilic granular body ; Astrocytoma ; Ultrastructure ; Immunocytochemistry ; αB-crystallin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Eosinophilic granular bodies (EGBs) are studied immunocytochemically and ultrastructurally in a case of low-grade and a case of high-grade astrocytoma. EGBs are recognized as brightly eosinophilic round bodies of variable size in hematoxylin and eosin-stained sections. Immunocytochemically some EGBs are positive for antibodies raised against αB-crystallin, ubiquitin and glial fibrillary acidic protein with the staining patterns for each being different from one another. Ultrastructurally EGBs consist of membrane-bound round body of various diameter ranging from 50 nm to 20 μm. Small EGBs contain electron-dense homogeneous material with occasional myelin figures, while large EGBs contain small EGB-like structures within electron-dense homogeneous material or loose granular profiles. Our studies demonstrate (1) ultrastructural variety of EGB; (2) and αB-crystallin epitope in EGB; and (3) the presence of EGB in high-grade as well as low-grade astrocytoma.
    Type of Medium: Electronic Resource
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  • 25
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 11 (1968), S. 122-139 
    ISSN: 1432-0533
    Keywords: Juvenile Lipidosis ; Electron Microscopy ; Histochemistry ; Biochemistry ; Granular Lipid Inclusions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Elektronenmikroskopische, histochemische und biochemische Untersuchungen eines Falles von juveniler Lipidose werden mitgeteilt. Die neuronalen Einschlüsse sind zumeist von einer unit membrane umgeben und zeigen fingerabdruckähnliche Muster. Membranovesiculäre Körperchen finden sich ebenfalls in den Nervenzellperikaryen; typische granuläre Lipofuscinkörperchen werden aber nicht gesehen. Die Astrocyten der Rinde enthalten eine ähnliche Art von Einschlüssen, von denen aber die Einschlüsse in den Astrocyten der Marksubstanz völlig verschieden sind. Die Lokalisation der Aktivität von saurer Phosphatase in allen Einschlußtypen läßt an ihre mögliche lysosomale Natur denken. Die biochemische Analyse deckt keine spezifischen Abweichungen auf.
    Notes: Summary Electron microscopic, histochemical and biochemical studies of a case of juvenile lipidosis have been presented. The neuronal inclusions were mostly unit membranebound with fingerprint-like patterns. Membrano-vesicular bodies were also found in the neuronal perikarya, but “typical” granular lipofuscin bodies were not seen. Cortical astrocytes contained a similar type of inclusion but the inclusions in the astrocytes in the white matter were quite different. The localization of acid phosphatase activity in all types of inclusions suggests their possible lysosomal nature. Biochemical analysis failed to pin-point any specific abnormalities.
    Type of Medium: Electronic Resource
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  • 26
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 47 (1979), S. 33-37 
    ISSN: 1432-0533
    Keywords: Pexid (perhexiline maleate) ; Abnormal inclusions ; Retina ; Rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Three morphologically distinct kinds of cytoplasmic inclusion (lamellar, reticular and crystalloid) developed in the retinal cells of suckling rats treated with Pexid (300 mg/kg/day). Lamellar inclusions were most abundant and they were seen in all types of retinal cells. This variety of inclusion was especially numerous in the ganglion cells. Reticular inclusions were encountered less commonly than the lamellar type and their distribution did not show any particular cellular predilection. Crystalloid inclusions were observed only in the pigment epithelial cells. The mechanism of formation of the three types of inclusion is not known, nor is the reason why certain types of inclusion occur most commonly in a particular kind of cell. One can speculate, however, that the dissimilarity of form may reflect differences in the metabolism and physicochemical properties of the various retinal cells.
    Type of Medium: Electronic Resource
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  • 27
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 56 (1982), S. 75-77 
    ISSN: 1432-0533
    Keywords: Bielschowsky bodies ; Lafora body ; Status marmoratus ; Polyglucosan ; Anoxic encephalopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Intraneuronal inclusions, consisting of polyglucosan and having histochemical and ultrastructural features identical to Lafora body of familial myoclonic epilepsy (Unverrict-Lafora disease), have been found restricted to the lateral pallidum in five patients. Two of these patients were also found to have status marmoratus of the basal ganglia. These lateral pallidal inclusions have been named after Bielschowsky, their original discoverer. We report two additional patients with status marmoratus and Bielschowsky bodies and suggest that these two conditions are frequent concomitant phenomena arising independently from a common cause.
    Type of Medium: Electronic Resource
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  • 28
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 58 (1982), S. 269-274 
    ISSN: 1432-0533
    Keywords: Quaking mouse ; Spinal root ; Periaxonal space ; Paranodal region
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Quaking is a neurologic mutant mouse with hypomyelination of CNS and PNS. In this mutant mouse of over 6 months of age, extensive vacuolation was found in the nerve fibers of the spinal roots, mostly in the ventral root. Normal axoplasmic constituents, such as mitochondria, neurotubules, and neurofilaments were, in general, well preserved. Many of these vacuoles appeared to be intra-axonal and only a few showed direct continuity with dilated periaxonal space. However, moderately electron-dense fluffy materials were often found in both the vacuoles and in the dilated periaxonal space, and rare mononuclear cells were found within the vacuoles, suggesting that these vacuoles were likely to be dilated periaxonal spaces. The vacuoles tended to be found more often in the myelinated nerve fibers than non-myelinated fibers. The changes in the periaxonal spaces observed in the old quaking mice were closely similar to those found in the myelinated cultures maintained on low calcium medium (Blank et al. 1974). Since calcium is highly concentrated in the node-paranodal regions and may be involved in the adhesion of Schwann cell loops to the axolemma (Ellisman et al. 1979), disturbed calcium and possibly other ionic concentrations due to structural abnormalities of node and paranodal regions in quaking mouse (Suzuki and Zagoren 1977) are speculated to be responsible for such morphological changes of spinal root in this mutant mouse.
    Type of Medium: Electronic Resource
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  • 29
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 59 (1983), S. 316-318 
    ISSN: 1432-0533
    Keywords: Intracytoplasmic inclusions ; Thalamus ; Young mice ; Neurofilament
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Neuronal intracytoplasmic inclusions were found in the ventrolateral and posterior nuclei of the thalamus of 3-month-old thiamine-deficient mice and quaking mice. An electron-microscopic study revealed that these inclusions were composed of condensation of parallel rectilinear 10-nm filaments with interconnecting cross-bridges as a hallmark of neurofilaments. They were morphologically indistinguishable from those described in aged mice. This observation indicates that neuronal inclusions in the thalamus, which have been constantly reported in aging mice, can be seen even in young mice in the presence of cellular metabolic disturbances.
    Type of Medium: Electronic Resource
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  • 30
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 62 (1984), S. 298-308 
    ISSN: 1432-0533
    Keywords: Globoid cell leukodystrophy ; Twitcher mouse ; Demyelination ; Spinal cord
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Chronologic events of demyelination were investigated in the spinal cord of the twicher mouse, an authentic murine model of human globoid cell leukodystrophy (GLD) from 5 to 45 days postnatal. There was very little evidence of myelin degeneration before day 25 although clustered or scattered globoid cells were already noted in the dorsal columns and intramedullary portion of the ventral roots. Globoid cells contained typical cytoplasmic inclusions and in those which were found adjacent to degenerating myelin and naked axons, myelin debris were conspicuous in their cytoplasm. Vesiculation of myelin and a feature of globoid cells stripping myelin lamellae were noted in the area of demyelination. Myelin and oligodendroglial degeneration became pronounced throughout the spinal white matter after day 40 but globoid cells tended to be more concentrated in the dorsal columns. Our observations suggest that the emergence of globoid cells in GLD is in response to the changes in biochemical environment (i.e., excessive presence of galactosylceramide in the tissue?), and these cells appear to have a role as phagocytic cells in removing myelin lamellae.
    Type of Medium: Electronic Resource
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