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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 74 (1987), S. 9-12 
    ISSN: 1432-0533
    Keywords: Axon ; Polyglucosan body ; Sensory neuron ; Thalamus ; Ageing
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Axonal polyglucosan bodies in myelinated axons in the ventral posterolateral nucleus of the human thalamus (VPL) are described. These axonal inclusions were distributed exclusively in the dorsolateral part of the caudal VPL, and their arrangement may be associated with fibres originating from the gracile nucleus. They were not observed in patients under age of 50, and appeared to increase in number and size with advancing age. It is suggested that axonal polyglucosan bodies are an ageing phenomenon of the secondary sensory fibers.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Paraneoplastic cortical cerebellar degeneration ; Cerebellar vermis ; Patchy distribution ; Purkinje cell dendrite ; Alcoholic cortical cerebellar degeneration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case is described of paraneoplastic cortical cerebellar degeneration in a patient with a small cell carcinoma of the lung. Following therapy, clinical improvement of cerebellar ataxia had been observed. The most severe degeneration was found in the superior aspects of the vermis and in the anterior and simple lobes as well as in the inferior aspects of the hemisphere. In addition to this distribution of degenerative lesions, uneven loss of Purkinje cells was apparent. Such distribution patterns in this case were apparently compatible with those of alcoholic cortical cerebellar degeneration (ACD), although the lesions were less severe than in ACD. Furthermore, dendritic changes in the Purkinje cells including loss of the spiny branchlets, focal swelling of the dendrites, and disappearance of secondary and tertiary branches were remarkable. It is noteworthy that these cells showed various stages of degeneration before cell loss occurred. These data suggest that the degree of vulnerability varies among Purkinje cells, and that this could be related to the uneven loss of these cells. It is proposed that, although this case and cases of ACD have both similarities and differences in their neuropathological aspects, it is apparent that both conditions have some common morphopathogenetic factor.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Cerebellum ; Dentatorubral-pallidoluysian atrophy ; Glutamate transporter ; Machado-Joseph ¶disease ; TUNEL
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We examined the mechanism of cerebellar degeneration in brains obtained at autopsy from six cases of hereditary dentatorubral-pallidoluysian atrophy (DRPLA) and six cases of Machado-Joseph disease (MJD), using terminal deoxynucleotidyltransferase-mediated in situ nick end labeling (TUNEL) and immunohistochemistry for apoptosis-related proteins, neurotrophin receptors and glutamate transporters. In three subjects with DRPLA, who developed dementia and cerebellar ataxia at over 50 years of age, the number of Purkinje cells was mildly reduced, TUNEL-positive cells were observed in the molecular layer of the cerebellar cortex, and immunoreactivities for calbindin D28K and excitatory amino acid transporter-1 (EAAT1) were altered in the molecular layer. In addition, all cases of DRPLA showed a reduction of immunoreactivity for EAAT1 in the dentate nucleus. In MJD, augmentation of Bcl-x expression by the Purkinje cells, and increases in Trk B- and GFAP-immunopositive glial cells in the granular layer were observed in half of the cases, whereas immunoreactivity for EAAT-1 was preserved both in the cerebellar cortex and dentate nucleus. One case of MJD showed TUNEL-positive granular cells in the cerebellar cortex. Age-matched control subjects did not show TUNEL-positive cells or immunohistochemical changes in the cerebellum. There were neither TUNEL-positive cells nor alteration of the in situ expression of apoptosis-related proteins in the dentate nucleus in either variant of hereditary spinocerebellar degeneration, although both exhibited grumose degeneration in the dentate nucleus. These findings indicate that latent degeneration in the cerebellar cortex may occur in DRPLA and MJD, in addition to the dentate change, which is the cardinal feature in the neuropathology of these two diseases. The lesion of Purkinje cells and their processes in the molecular layer associated with altered glutamate transport may be important in DRPLA, while the significance of the abnormalities observed in some MJD cases, which might be related to apoptotic mechanism, remains unclear.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 78 (1989), S. 176-182 
    ISSN: 1432-0533
    Keywords: Holoprosencephaly ; Pathology ; Cerebral cortex ; Neuronal migration ; Glomerulus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Abnormal architecture of the telencephalic cortex was studied in six autopsy specimens of alobar holoprosencephaly by histopathological, electron microscopic and immunohistochemical methods. Several common abnormalities are described and their significance discussed. In all of the specimens, the cortices showed excessive thickness and abnormal lamination with a middle cellular layer and an external sparse layer, indicating disturbance of neuronal migration. Neurons of the external sparse layer were segmented into irregularly arranged groups, suggesting disturbance of tisse organization. In three specimens, the internal pyramidal layer contained numerous abnormal glomerular structures which consisted of fine dendrites and axons.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 78 (1989), S. 183-188 
    ISSN: 1432-0533
    Keywords: Holoprosencephaly ; Pathology ; Glia ; Mesenchyme ; Marginal glioneural heterotopia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Abnormalities of glial and mesenchymal elements were studied in six autopsy specimens of alobar holopresencephaly by histopathological and immunohistochemical methods. Marginal glioneural heterotopia was observed in all of the specimens. It was most prominent in the prosencephalic base around the optic chiasm, and extensive in five specimens. The floor of the third ventricle was intermingled with excessive mesenchymal elements, and in three specimens the ventricular cavity was plugged by heterotopic nodules. In the cerebellum, dysplastic nodules were observed in two specimen. In the telencephalon, the marginal and subventricular layers were thickened. The pathological significance of these findings is discussed with their possible relationship to the pathomechanism of holoprosencephaly.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Senile dementia of Alzheimer's type ; Alzheimer's disease ; Pick's disease ; Laminar neuronal loss ; Limbic system
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Seven cases of senile dementia of Alzheimer type (SDAT) with unusual clinico-pathological findings are reported. The patients showed neuronal loss in laminar pattern, with gliosis exclusively confined to the CA1 of the hippocampus, the area of the hippocampal gyrus (entorhinal cortex) and medial occipitotemporal cortex. This change was more pronounced in the oral region. The subcortical white matter showed more pronounced fibrillary gliosis than loss of myelin. Both Alzheimer's neurofibrillary tangles and senile plaques were less marked than those usually seen in SDAT. The mental disturbance started after the age of 65 in all patients. The main clinical feature was marked character change in addition to disturbance of cognitive function. Cranial computed tomography showed marked dilatation of the oral portion of the inferior horn of the lateral ventricle in the early stage. It was apparent that although the cases in this group could be incorporated within in the spectrum of SDAT, they could also be considered to represent a variant of SDAT. This group could contribute to an understanding of the clinico-pathological spectrum of SDAT as well as indicating ways of managing such patients.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-0533
    Keywords: Key words de Lange syndrome ; Septo-optic dysplasia ; Cerebellar hypoplasia ; Commissural defects
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Little is known about the neuropathology of Cornelia de Lange syndrome. We report a unique type of cerebral malformation combined with Cornelia de Lange syndrome in a 5-year-old female child. At autopsy, the optic systems, hypothalamic nuclei, corpus callosum and cerebellar vermis were hypoplastic, and the septum pellucidum, fornix and anterior commissure were rudimentary. The brain had malformative features of septo-optic dysplasia combined with commissural dysplasia and cerebellar vermian hypoplasia. This case suggests an interrelationship between Cornelia de Lange syndrome and midline development of the brain.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: Key words Cortical dysplasia ; Dwarfism ; Leprechaunism ; Megalencephaly
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report concerns an autopsy case of megalencephaly exhibiting a unique combination of physical and brain malformations. A 4-year-old boy had a peculiar face, a severe reduction of subcutaneous adipose tissue, severe growth failure and frequent hypoglycemic episodes. These clinical features were compatible with leprechaunism; however, the absence of hyperinsulinemia and insulin resistance prevented the diagnosis of leprechaunism. The autopsy disclosed complex cardiac and brain malformations. Although hydrocephalus coexisted, the brain weight after complete removal of the cerebrospinal fluid was 2260 g, which was greater than the average brain weight of normal Japanese children of the same age. The neuronal density in the cerebral cortex was decreased, while the surface area of the cerebral cortex and white matter were greater than those in an age-matched control. There was cortical dysplasia in the frontal and parietal lobes. Endocrine tests and immunohistochemical analysis of the brain did not demonstrate any abnormalities in the hypothalamus-pituitary system. Megalencephaly can be associated with dwarfism, and this case is important for consideration of the interrelationship between neuronal proliferation and physical growth.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0533
    Keywords: Key words Calcium-binding protein ; Enkephalin ; Hypoxic encephalopathy ; Immunohistochemistry ; Marbled state
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have immunohistochemically analyzed the marbled state in 8 cases of perinatal hypoxic ischemic encephalopathy and 4 cases of infantile hypoxic encephalopathy, using antibodies against calbindin-D28k (CaBD), glial fibrillary acidic protein (GFAP), methionine-enkephalin (MEnk), myelin basic protein (MBP), neurofilament (NF), parvalbumin (PV), substance-P (SuP) and synaptophysin (SP). The marbled state was found in the thalamus in 11 cases, whose age at death was over 10 years. Four cases demonstrated the marbled state in the cerebral cortex, in addition to the striatum and/or the thalamus. The abnormally myelinated fibers in the marbled state were stained with both Klüver-Barrera and Holzer stainings; however, they were partly immunopositive for MBP and completely immunonegative for GFAP, CaBD, MEnk, PV, SuP and SP, although some of the neurons and/or fibers showed immunoreactivities for those calcium-binding proteins and/or neurotransmitters. The axons were visualized in the abnormally myelinated fibers by Bodian staining and/or anti-NF immunostainings in the cerebral cortex and striatum but not in the thalamus. GFAP-positive astrocytes did not show any continuity with the abnormally myelinated fibers. These histological features were seen in the cerebral cortex, striatum and thalamus. Difference of the etiology did not affect the histological features with the exception of anti-PV staining, in which PV-immunopositive neurons were observed only in aged subjects with infantile hypoxic encephalopathy, and seemed to be more severely affected by hypoxic stress during the perinatal period than the early infantile period. These data suggest that the site of lesion or the length of survival period after brain injury might influence the formation of the marbled state rather than the etiology. And the direct relationship between the abnormally myelinated fiber and astrocytic process was not verified.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0533
    Keywords: Demyelinating disease ; Infundibulo-hypophysitis ; Hepatosplenomegaly ; Hemophagocytic lymphohistiocytosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A man aged 31 with remittent fever, leukopenia, polyuria, and splenomegaly in addition to chronic and recurrent episodes of neurologic abnormalities was examined pathologically. A chronic type of perivenous encephalomyelitis was found in the CNS, and chronic inflammatory lesions were noticed in the infundibulo-hypophyseal system and also in the peripheral nerves. Moreover, hemophagocytic lymphohistiocytosis predominated in the reticuloendothelial system. This seemed to be the first adult case of demyelinating disease with hemophagocytic lymphohistiocytosis.
    Type of Medium: Electronic Resource
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