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  • 1
    Electronic Resource
    Electronic Resource
    A case of adult neuronal ceroid-lipofuscinosis with the appearance of membranous cytoplasmic bodies localized in the spinal anterior horn (1987)
    Springer
    Acta neuropathologica 72 (1987), S. 362-368 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00687268
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  • 2
    Electronic Resource
    Electronic Resource
    Immunocytochemical and ultrastructural studies of Werdnig-Hoffmann disease (1991)
    Springer
    Acta neuropathologica 81 (1991), S. 408-417 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00293462
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  • 3
    Electronic Resource
    Electronic Resource
    Immunocytochemical and ultrastructural studies of eosinophilic granular bodies in astrocytic tumors (1992)
    Springer
    Acta neuropathologica 83 (1992), S. 408-414 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00713533
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  • 4
    Electronic Resource
    Electronic Resource
    Neuronal degeneration in the brain of the brindled mouse (1979)
    Springer
    Acta neuropathologica 45 (1979), S. 17-25 
    Details
    ISSN: 1432-0533
    Keywords: Brindled mutant mouse ; Male hemizygotes ; Neuronal degeneration ; Ultrastructure ; Giant mitochondria ; Copper deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The brindled mouse (Mobr) is a neurological mutant mouse with clinical and biochemical features closely similar to Kinky hair syndrome (KHS) in humans. Neuronal degeneration in the cerebral cortex and thalamic nuclei was the constant neuropathological lesions in the CNS of the male hemizygotes of this mutant (Yajima and Suzuki, 1978). Ultrastructurally, many cortical neurons contained enlarged mitochondria with prominent tubular or vesicular cristae, which were similar to those described in the Purkinje cells in the human KHS (Ghatak et al., 1972) and in the rat brain with copper deficiency (Prohaska and Wells, 1975). Such mitochondria were observed not only in the degenerating neurons but even in the otherwise normal-appearing cortical neurons, suggesting that the mitochondrial damage possibly related to the deficient activities of the copper containing enzymes (cytochrome oxidase, etc.) preceded the neuronal degeneration. Many mitochondria in the severely degenerated neurons contained numerous electron dense spicules of possible calcium. Although rare, similar morphological alteration of neuronal mitochondria was also noted in the female heterozygotes, indicating the presence of possible subclinical defect in copper transport in the heterozygotes as well.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00691800
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  • 5
    Electronic Resource
    Electronic Resource
    Ultrastructural evidence of mitotic ependymal cells in 6-aminonicotinamide-treated suckling mice (1986)
    Springer
    Acta neuropathologica 70 (1986), S. 71-74 
    Details
    ISSN: 1432-0533
    Keywords: 6-Aminonicotinamide ; Ependymal cell ; Mitosis ; Suckling mice ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Mitotic ependymal cells were encountered in 10-day-old mice treated with 6-aminonicotinamide, an antagonist of niacin. These occurred along the medial surface of the lateral ventricle and the ventral portion of the aqueduct. Electron microscopy revealed that both mitotic ependymal cells had eccentrically placed chromosomes without a nuclear membrane and well-formed gap junctions in contact with adjacent ependymal cells. Microtubules from a centriole radiated to the chromosomes. These data show that cell division occurs in morphologically matured ependymal cells in the postnatal brain under pathological conditions. We believe this to be the first ultrastructural demonstration of this phenomenon.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00689516
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  • 6
    Electronic Resource
    Electronic Resource
    Immunocytochemical and ultrastructural studies of upper motor neurons in amyotrophic lateral sclerosis (1992)
    Springer
    Acta neuropathologica 83 (1992), S. 518-524 
    Details
    ISSN: 1432-0533
    Keywords: Amyotrophic lateral sclerosis ; Upper motor neurons ; Ultrastructure ; Neurofilament ; Ubiquitin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The pathological alterations in upper motor neurons were investigated in 27 cases of adult-onset sporadic amyotrophic lateral sclerosis (ALS). No signficant cytoskeletal alterations were found in the Betz cells of any of the cases except one, although cytoskeletal pathology was consistently present in lower motor neurons. The one case had severe circumscribed atrophy of the precentral gyrus and, microscopically, had argentophilic intracytoplasmic inclusions in Betz cells and other pyramidal neurons in the primary motor area as eell as in the lower motor neurons. Immunocytochemically these inclusions contained the epitope of phosphorylated neurofilament and ubiquitin and ultrastructurally consisted of granule-associated filaments with neurofilaments. This is the first demonstration of alterations of cytoskeleton and ubiquitination in the giant cells of Betz, an established subset of upper motor neurons in ALS. Thus, although uncommon, cytoskeletal changes can be found in upper motor neurons in some ALS cases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00310029
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  • 7
    Electronic Resource
    Electronic Resource
    A histological study on experimental autoimmune myocarditis with special reference to initiation of the disease and cardiac dendritic cells (1995)
    Springer
    Virchows Archiv 426 (1995), S. 493-500 
    Details
    ISSN: 1432-2307
    Keywords: Autoimmune myocarditis ; Cardiac myosin ; Dendritic cell ; Macrophage ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The precise mechanism of myosin-induced autoimmune myocarditis is unknown. The purpose of the present study was to define the immunohistological and ultrastructural characteristics of the infiltrating cells, especially in the initial phase of the myocarditis. It was demonstrated that OX6-positive dendritic cells first infiltrated the cardiocytes on day 13 after immunization. After day 17, OX6-positive cells, which possessed elongated irregular-shaped processes on the cell surface but contained few phago-lysosomes in the cytoplasm, were located at the margin of an inflammatory field and inserted their processes into the sarcoplasm of cardiocytes. The central portion of the inflammatory field was occupied by ED1-positive inflammatory macrophages, which were rich in phagosomes and which were in contact with degenerating cardiocytes. No evidence was obtained which suggested that lymphocytes directly injured the cardiocytes. These results demonstrated ultrastructural evidence that the type of infiltrating cell that first injures cardiocytes is the cardiac dendritic cell. Inflammatory macrophages thereafter serve as scavengers of degenerating cardiocytes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00193173
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  • 8
    Electronic Resource
    Electronic Resource
    Ultrastructural changes in the lung in Niemann-Pick type C mouse (1995)
    Springer
    Virchows Archiv 427 (1995), S. 77-83 
    Details
    ISSN: 1432-2307
    Keywords: Niemann-Pick disease ; Mouse ; Lung ; Electron microscope ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The biochemical and morphological aspects of BALB/c mice with many features of the Niemann-Pick disease type C in man (NP-C mouse) have been studied extensively. However, the pulmonary pathology has not been studied extensively and we describe here some unique ultrastructural features of the lung in the NP-C mouse. Ultrastructurally, macrophages in younger mice contained osmiophilic dense granules and annulolamellar structures, but larger multilamellar concentric structures increased in the macrophages of older mice. In contrast, endothelial cells and type I pneumocytes showed membrane-bound bodies with dense granules and vesicular or vesiculogranular structures as well as amorphous materials. Type II pneumocytes were unremarkable throughout. Our study suggests that endothelial cells and type I pneumocytes are the major site of metabolic derangement resulting in pronounced morphological changes with granular and round membranous structures in the lungs of NP-C mouse. Alveolar macrophages with multilamellar concentric structures may be a result of disturbed disposal of surfactant material from type II pneumocytes rather than that from storage material of type I pneumocyte.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00203741
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