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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 55 (1981), S. 39-46 
    ISSN: 1432-0533
    Keywords: Polyneuropathy ; Intermediate filaments ; Connatal diseases ; Schwann cells ; Demyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of connatal polyneuropathy is described in a boy who died of pneumonia at the age of 2 years, and from whom sural nerve biopsies had been taken when he was 4 and 16 months old. Clinically, his disease was characterized by motor weakness and muscular flaccidity in the presence of normal intellectual development. The evolution of the connatal peripheral nerve lesion could be followed from the age of 4 months to death: The first biopsy evidenced the most serious pathologic changes. The findings were reminiscent of those encountered in a fetal nerve at 18 weeks of gestation. Furthermore, it showed numerous filamentous inclusions in Schwann cells. The second biopsy showed a sparsely myelinated nerve with bands of basement membrane apparently unrelated to cells arranged around the nerve's fibers. A few Schwann cells containing filamentous inclusions were still present. At autopsy, the findings were identical to those of the second biopsy. The possibility that this patient was transitionally exposed to a neurotoxic agent during pregnancy and that the biopsy findings represent a lesion that is still florid in the first and in a residual state in the second biopsy is considered.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 55 (1981), S. 157-162 
    ISSN: 1432-0533
    Keywords: Epileptic seizures ; Bicuculline ; Protein biosynthesis ; Autoradiography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The incorporation ofl-[3,5-3H]tyrosine into cerebral proteins was investigated during the initial phase (30 min) of bicuculline-induced status epilepticus. Autoradiographs of different parts of the cerebral hemispheres, brain stem, and cerebellum were prepared. Marked local reduction of amino acid incorporation was evident in bilaterally symmetrical areas of the cerebral cortex, hippocampus, thalamic nucleic, and the region of the medial geniculate body. No apparent difference of local [3H]tyrosine incorporation was observed in the lower brain stem nuclei and in the cerebellum of control and convulsed animals. The territories showing a decrease of protein synthesis during epileptic seizures coincide largely with the regions of maximal local glucose metabolism and cerebral blood flow. The present investigation demonstrates that autoradiography of regional protein biosynthesis is a suitable method for the visualization of neuronal populations at risk in the very early stages of seizure activity.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Neurofibromatosis 2 ; Bilateral acoustic neurofibromatosis ; Ghal hamartomas ; Immunohistochemistry ; S-100 protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Bilateral acoustic neurofibromatosis (neurofibromatosis 2, NF2) accounts for less than 10% of all cases of neurofibromatosis and manifests itself with bilateral acoustic schwannomas, multiple schwannomas of spinal nerve roots, meningiomas, glial tumors and hamartomatous CNS lesions. We have observed dysplastic foci of immature neuroectodermal cells in the cerebral cortex and basal ganglia of six patients afflicted with neurofibromatosis 2, ranging from occasional clusters of immature, dysplastic cells to numerous, confluent lesions. These cells, although often polymorphic and multinuclear did not show mitotic acitivity or a tendency for neoplastic transformation. To determine the histogenesis of these foci, extensive immunocytochemical reactions were carried out with antibodies to a variety of glial, neuronal and nonneural cell lineages. With the exception of S-100 protein, no immunoreactivity was detectable. S-100 was consistently expressed in these foci, irrespective of their size, location, and degree of polymorphism. On the basis of cytological appearance, distribution and immunoreactivity we tentatively designate these foci as glial micro-hamartomas. Although we did not systematically analyze the CNS of patients with von Recklinghausen neurofibromatosis (neurofibromatosis 1, NF1), the present study strongly suggests that these micro-hamartomas constitute a morphological hallmark of bilateral acoustic neurofibromatosis (NF2).
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 43 (1978), S. 105-109 
    ISSN: 1432-0533
    Keywords: Brain neoplasms ; 3,3-Dimethyl-1-phenyltriazene ; DNA alkylation ; DNA repair ; Transplacental carcinogenesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The role of DNA alkylation by the neurooncogenic agent 3,3-dimethyl-1-phenyltriazene (DMPT) was investigated perinatally and in adult rats. Following a single subcutaneous injection of14C-DMPT (100 mg/kg) on the 21st day of gestation, the concentration of methylated purines was similar in both fetal liver and brain whereas during postnatal growth this treatment resulted in an increasingly preferential methylation of liver DNA. In 30-day-old and adult rats the concentration of 7-methylguanine in liver was about 8 times higher in brain DNA, suggesting that during prenatal development both liver and brain DNA are transplacentally methylated by a proximate carcinogen produced by maternal organs. Multiple doses of14C-DMPT (50 mg/kg) to adult rats led to a preferential accumulation of O6-methylguanine in cerebral DNA. This supports the hypothesis that the deficient repair excision capacity of the central nervous system is a significant factor in the organ-specific carcinogenicity of DMPT and related carcinogens.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Acquired immune deficiency syndrome (AIDS) ; Leukoencephalopathy ; Cytomegalovirus ; Papovavirus ; HTLV-III/LAV
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two adult patients with acquired immune deficiency syndrome (AIDS) presented with psychoorganic symptoms produced by an extensive cerebral and cerebellar leukoencephalopathy. Diffuse loss of myelin and axons with reactive astrocytosis and distinctive multinucleated giant cells were prominent in the deep white matter, but less so in the subcortical white matter and in compact myelinated pathways. Bilateral involvement of the centrum semiovale produced distal Wallerian degeneration of the descending pyramidal tracts, which in one patient correlated with progressive paraparesis and bladder dysfunction. Although there were morphological indications of cytomegalovirus infection and immunohistochemical evidence of papovavirus antigens, the neuropathology did not resemble that usually associated with infection by these opportunistic agents. The possibility is entertained that the progressive diffuse leukoencephalopathy (PDL) in these patients was directly related to infection with human T-cell lymphotropic virus (HTLV-III/LAV), the etiologic agent of AIDS.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Germ cell tumor ; Syncytiotrophoblastic giant cells ; Germinoma ; Embryonal carcinoma ; Precocious puberty ; Ki-67
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 9-year-old male patient developed a germ cell tumor in the right basal ganglia which secretedβ-human chorionic gonadotropin (β-HCG) and caused precocious puberty. Histology and immunohistochemical staining for placental alkaline phosphatase (PLAP), α-fetoprotein (α-FP), andβ-HCG showed a mixed population of neoplastic germinocytes, embryonal carcinoma, and syncytiotrophoblastic giant cells (STGC). Immunohistochemical double-staining for α-FP andβ-HCG revealed that these two markers were produced by different subsets of cells. Expression of the proliferation marker Ki-67 showed a growth fraction of 53% for the neoplastic germinocytes and embryonal carcinoma cells, but only 21% for the STGC.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-0533
    Keywords: Medullomyoblastoma ; Neuronal differentiation ; Hamartoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary This report describes an unusual medullo-myoblastoma which developed in the cerebellar vermis of a 6-year-old girl. Histological investigation showed a highly cellular and predominantly undifferentiated tumor. Myogenic differentiation was prominent in clusters of large tumor cells with eosinophilic cytoplasm and immunoreactivity for desmin and myoglobin. Electron microscopy revealed the presence of immature Z-bands. Immunohistochemically, numerous cells showed incipient expression of myoblastic marker antigens, supporting the view that medulloblastomas and related primitive neuroectodermal tumors possess the potential for non-neural differentiation. In addition, there was evidence of advanced neuronal differentiation, with expression of neuron-specific enolase, synaptophysin, retinal S-antigen, and the formation of ganglioid tumor cells. Occassional neoplastic cells expressed glial fibrillary acidic protein without morphologically detectable astrocytic differentiation. Associated with the neoplasm was brain tissue containing clusters of neuronal cells and focal accumulations of immature oligodendroglia-like cells which expressed neuronal marker antigens. This unusual component resembled a hamartomatous lesion and would support the hypothesis that the cerebellar medullomyoblastoma originated from a teratomatous or malformative lesion. Alternatively, this component may constitute the end stage of advanced neuronal differentiation of a primitive neuroectodermal tumor.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 70 (1986), S. 91-102 
    ISSN: 1432-0533
    Keywords: Iridium-192 ; Interstitial radiation ; Brachytherapy ; Radionecrosis ; Delayed-radiation damage
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary To investigate the effects of a permanent interstitial source of γ-irradiation on normal brain tissue, single iridium-192 (Ir-192) wires (1.05 mCi) were stereotactically implanted into the left centrum semiovale of adult dogs (survival times, 25, 46, 74, 230 and 362 days). Within 25 days, a coagulation necrosis developed in the immediate vicinity of the radioactive source. In later stages, the necrosis increased in size and became progressively mineralized. Staining for extravascular immunoreactive serum proteins revealed the presence of a chronic perifocal vasogenic edema, which extended into the white matter of the entire ipsilateral hemisphere. This edema persisted through all stages and showed a significant decrease only in the animal with a 1-year survival. A reactive gliosis with formation of a dense network of glial fibrillary acidic protein-positive astrocytes developed around the central necrosis in the adjacent white matter and, at later stages, in the contralateral hemisphere. Demyelination was restricted to the ipsilateral centrum semiovale without affecting the internal capsule or the contralateral hemisphere. It was present as early as 25 days and showed no tendency to increase at later stages. Analysis of the sequential morphological changes following Ir-192 implantation suggests that the central coagulation necrosis represents a direct radiation effect, the sharp focal delineation of which can be explained by the physical characteristics of the radiation source, i.e., rapid falloff of the dose at short distances. Due to the continuous emission of radiation energy, there is a perifocal zone with overlapping of progressive radiation damage and tissue organization. This focus becomes the source of a chronic vasogenic edema, which in turn is most likely to be responsible for the partial demyelination of the adjacent centrum semiovale. The widespread reactive gliosis observed at all stages may also, in part, be a consequence of chronic vasogenic edema, but its distribution suggests that direct radiation effects may also be involved.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 72 (1986), S. 23-28 
    ISSN: 1432-0533
    Keywords: Hypoglycemia ; Cerebellum ; Selective vulnerability ; Neural grafts ; Protein synthesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Prolonged insulin-induced hypoglycemia causes widespread loss of neurons and permanent brain damage with irreversible coma. Although the deprivation of carbohydrate stores affects all brain regions, the breakdown of energy metabolism and cessation of protein synthesis occur predominantly in the cerebral cortex, caudoputamen and hippocampus. The cerebellum, brain stem and hypothalamus are largely resistant. Following transplantation of the cerebellar anlage of rat fetuses (day 15 of gestation) into the caudoputamen of adult rats, the grafts were allowed to differentiate for a period of 8 weeks. The host animals were then subjected to 30 min of severe hypoglycemia with isoelectric EEG (‘coma’). In contrast to the surrounding vulnerable brain structures, protein synthesis was fully preserved within the cerebellar transplant. Grafting of fetal forebrain cortex to the same location did not result in escape from hypoglycemic cell injury. This indicates that resistance to hypoglycemia is part of the programmed differentiation of the cerebellum and develops irrespective of its location and functional integration within the nervous system.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0533
    Keywords: HIV encephalitits ; HIV myelitis ; Human immunodeficiency virus ; Polymerase chain reaction ; In situ hybridization
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The presence and distribution of human immunodeficiency virus (HIV) were examined in the CNS of two children with severe HIV encephalitis and myelitis. Using polymerase chain reaction-mediated DNA amplification and subsequent Southern analysis, proviral HIV gag sequences were identified in brain tissue of both patients. In situ hybridization using antisense oligonucleotide probes revealed abundant HIV gag and env/nef RNAs selectively in areas with histopathological evidence for HIV-induced tissue damage. The spinal cord of one patient exhibited a striking subpial accumulation of HIV RNAs strongly suggestive of a liquorigenic spread of the infection. HIV RNAs were typically associated with cells of the monocyte/macrophage lineage, as shown by a combined immunohistochemical and in situ hybridization procedure. The present study supports the view that the pattern and distribution of HIV-induced brain lesions is largely determined by the extent of focal HIV replication within the CNS.
    Type of Medium: Electronic Resource
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