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  • 1
    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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  • 2
    ISSN: 1432-0533
    Keywords: Nasu-Hakola's disease ; Membranous lipodystrophy ; Axonal spheroids ; Membranocystic change ; Glio-myelin dissociation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsy case of Nasu-Hakola's disease (membranous lipodystrophy) is reported. A 43-year-old Japanese man, whose parents were not consanguineous, had been suffering from frequent long bone fractures since the age of 10. Neuropsychiatric symptoms, which were characterized by euphoria, disturbance of attention and dementia, appeared at his thirties and generalized and/or localized seizures and apallial syndrome at the later stage. The neuropathology revealed diffuse leukoencephalopathy of the cerebrum. The peculiar aspects in this case were membranocystic changes in the lungs [Yagishita et al. Virchows Arch [A] 408:211–217 (1985)], diffuse degeneration of the cerebral cortex, chiefly in frontal and temporal lobes, and many axonal spheroids throughout the cerebral cortex. The ultrastructure of spheroids in the cerebral cortex demonstrated aggregations of mitochondria, dense bodies and minute concentric bodies and a small amount of neurofilaments.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 78 (1989), S. 131-136 
    ISSN: 1432-0533
    Keywords: Senile plaque ; Alzheimer's disease ; Brain stem ; Distribution ; Morphology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The morphology, incidence and distribution of senile plaques in the brain stem were examined in 15 cases of Alzheimer's disease, using mainly the Methenamine-Bodian method. The plaques were found in all cases and were grouped into three types according to their morphology. They were not randomly scattered in the brain stem, but had a distribution common to all cases. There were numerous plaques in the periaqueductal gray, superior colliculus, fourth-ventricle floor and superior central nucleus. The plaques were also found less abundantly in the reticular formation, substantia nigra, pontine nucleus and inferior olivary nucleus. There was a tendency for certain plaque types to be associated with specific regions. In the familial cases, the plaques tended to occur even in the regions where they were rare in other cases. The capillaries with plaque-like degeneration were observed not infrequently in the brain stem. The distribution of plaques did not always coincide with that of neurofibrillary tangles.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Senile plaque ; Morphology ; Familial Alzheimer's disease ; Methenamine-Bodian method
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The distribution and morphology of senile plaques (SPs) in the cerebral cortices and subcortical nuclei of six cases of familial Alzheimer's disease (AD) were examined using the Methenamine-Bodian method and compared with those of sporadic AD cases. SPs were grouped into three types according to their morphology. SP types were generally constant at each anatomical site in all of the cases. The SPs of familial cases, however, had a greater tendency to fuse together than those of sporadic cases, especially in the cingulate cortex, presubiculum and striatum. This tendency was more evident in cases with severe amyloid angiopathy. Here it appeared that a SP type corresponding to “diffuse plaques” at least in part, might be formed by transformation from another type. In the globus pallidus, all the familial cases had many compact-like plaques which appeared to be derived from “drusige Entartung” of the capillaries. Furthermore, the regional proportion of two types of SPs occuring in this nucleus varied along its anteroposterior axis. These findings may be the histological hallmarks of atypical AD rather than familial AD.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Tardive dyskinesia ; Antipsychotic drugs ; Oral dyskinesia ; Cerebellar dentate nucleus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Four autopsied cases of tardive dyskinesia manifesting oral hyperkinesia revealed markedly inflated neurons in the cerebellar dentate nucleus (DN), which had not been described previously. The inflation of the neurons was proved to be statistically significant (P〈0.01) by morphometric study. The nuclei were usually situated in the central portion of the cytoplasm. This inflated change was different from both central chromatolysis and grumose degeneration of the DN, typically observed in progressive supranuclear palsy and dentatorubropallidolysian atrophy, and seemed to be easy to miss without careful observation, since neuronal loss and gliosis were very mild in the DN. Among a few autopsied cases of tardive dyskinesia reported previously, degeneration of the DN was described in only two. It is believed, however, that the inflated neurons of the DN may not be so rare and may be related to the occurrence of some involuntary hyperkinesia, especially oral hyperkinesia following some neurotoxic disorders and/or neuroleptic medications.
    Type of Medium: Electronic Resource
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