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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 57 (1982), S. 137-142 
    ISSN: 1432-0533
    Keywords: Medulloepithelioma ; Peripheral nerve ; Congenital
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of congenital peripheral medullo-epithelioma arising in the sciatic nerve of a 6-month-old boy is reported. Histologically, areas of primitive neural tumor merged with areas showing differentiation into ependymoma, astrocytoma, oligodendroglioma, and ganglioneuroma. These lines of differentiation were confirmed by electron microscopy and immunohistochemistry. Despite the malignant nature of the tumor, 7 years after amputation of the affected leg, the child is alive without recurrence.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Microglia ; Ricinus communis agglutinin-1 ; Lectin ; Histochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Microglia were demonstrated in paraffinembedded human nervous tissues with an avidinbiotin peroxidase method andRicinus communis agglutinin-1 (RCA-1). Specific staining was observed in cell bodies and processes of microglia. Although endothelial cells and blood cells reacted with RCA-1, they were easily distinguished morphologically from microglia. Astrocytes, oligodendrocytes, and neurons did not react with RCA-1. These results suggest that RCA-1 can be used as a new histochemical marker for microglia in normal human brain.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 47 (1979), S. 189-196 
    ISSN: 1432-0533
    Keywords: Childhood dermatomyositis ; Muscle biopsy ; Electron microscopy ; Tubular arrays
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Muscle biopsies from 12 patients with a typical clinical picture of dermatomyositis have been examined by electron microscopy. Endothelial cells of intramuscular blood vessels, their basal lamina, pericytes, muscle fibers, and satellite cells show degenerative or regenerative alterations. In nine patients, tubular arrays were noted in the cisterns of endoplasmic reticulum of endothelial cells, pericytes, lymphocytes, macrophages and satellite cells. Other types of inclusions were also observed. The pathogenesis of the disease is discussed.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 75 (1988), S. 233-240 
    ISSN: 1432-0533
    Keywords: Osteopetrosis ; Ceroid lipofuscin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Neurological manifestations in infantile osteopetrosis are common and varied, and not always attributable to the skeletal pathology. An unusual association of osteopetrosis with neuronal storage of ceroid lipofuscin is reported in two infant brothers born of nonconsanguinous parents. The first child became symptomatic at age 5 days with weight loss and vomiting. He had poor head control, hypertonia, and persistent fisting, and died at age 2 months. In the second infant, the diagnosis of osteopetrosis was confirmed at age 2 days. His neurological symptoms inlcuded blindness, deafness, and recurrent seizures. The infant died at 7 months of age. In both cases, autopsy confirmed the diffuse bony sclerosis with hepatosplenomegaly and extramedullary hematopoiesis. Neuropathological examination revealed cerebral atrophy with ventricular dilation, neuronal loss, and astrogliosis. The most striking finding was widespread accumultion of neuronal ceroid lipofuscin associated with formation of axonal spheroids. The optic nerves were compressed at the optic foramina and showed loss of myelinated axons and gliosis. Rapid Golgi impregnations of neurons from the calcarine cortex in the second infant were analyzed quantitatively, showing a reduction in the total dendritic length and number of branches. The primary defect in osteopetrosis is thought to be a lysosomal dysfunction involving the monocyte cell line from which osteoclasts are derived. Thus, the association in two brothers of osteopetrosis with accumulation of neuronal ceroid lipofuscin may not be fortuitous. The neuronal storage disorder in this instance probably reflects lysosomal dysfunction.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 60 (1983), S. 271-277 
    ISSN: 1432-0533
    Keywords: Tuberous sclerosis ; Subependymal giant-cell tumor ; Immunohistochemistry ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Tissue from seven patients with tuberous sclerosis and subependymal giant-cell tumors was examined with special stains, immunohistochemistry, and electron microscopy. Immunoreactive glial fibrillary acidic protein (GFAP) was not found in the giant cells of four tumors, but was present in some tumor cells in the other three. Immunoreactive S-100 protein was present in tumor cells of six cases; it was also seen in more tumor cells than was GFAP. Electron microscopy was similar in all cases and showed that the tumor cells had numerous organelles — many dense bodies thought to be primary lysosomes, swollen mitochondria, Golgi complexes, rough and smooth endoplasmic reticulum, free ribosomes, and sparsely distributed intermeadiate filaments. In one case, neurosecretory granules, microvilli, and synapses were observed. In another subject, prominent, thick bundles of glial filaments were seen. These findings suggest that the tumor is made up of unique cells in addition to cells with recognizable neuronal or astrocytic features.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1076
    Keywords: Multiple respiratory chain defects ; Skin fibroblasts ; Pyruvate dehydrogenase complex
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A female child suffering from intrauterine growth retardation was born by caesarean section at 32 weeks. In the immediate newborn period there was a metabolic acidosis but this resolved. Hypotonia, muscular weakness and poor respiratory effort were evident and the child died at 6 days of age. A previous male sibling had died at 3 months of age after similar symptoms with seizures and a dysmyelination disorder. Post-mortem examination of both children showed damage to the basal ganglia. Defects in the activities of the pyruvate dehydrogenase complex, cytochrome oxidase and succinate cytochrome c reductase were found in cultured skin fibroblasts. Similar defects were found in isolated muscle mitochondria but not in isolated liver mitochondria from the patient. Immunoblotting for cytochrome oxidase showed that the multienzyme complex was not assembled in muscle and skin fibroblast mitochondria, but was assembled in liver mitochondria. Similar results were obtained in cultured skin fibroblast mitochondria for complex I of the mitochondrial respiratory chain. This is the first occasion that multiple defects have been demonstrated both in tissue and in culture skin fibroblasts in mitochondrial respiratory chain complexes.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] Modification of synaptic strength in the mammalian central nervous system (CNS) occurs at both pre- and postsynaptic sites,. However, because postsynaptic receptors are likely to be saturated by released transmitter, an increase in the number of active postsynaptic receptors may be a more ...
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 90 (1995), S. 532-538 
    ISSN: 1432-0533
    Keywords: Key words Familial erythrophagocytic ; lymphohistiocytosis ; Vascular occlusion ; Brain pathology ; Cytokines
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Neuropathological findings in two siblings with familial erythrophagocytic lymphohistiocytosis (FEL) are reported. Case 1 showed the typical neuropathological findings of FEL with lymphohistiocytic infiltration of the leptomeninges and perivascular spaces. A characteristic erythrophagocytosis was detected in inguinal lymph nodes, lung and bone marrow. Case 2 revealed calcification and necrotic lesions in the brain. In the necrotic areas, parenchymal calcification, vascular medial calcification, and occlusion of many vessels due to subendothelial fibrosis were detected. The areas of necrosis correlated with the distribution of occluded vessels. These changes were most prominent in putamen, internal capsule, thalamus and dentate nucleus. Hypercytokinemia is suspected to be the underlying mechanism for the clinical and laboratory findings in patients with FEL, although the relationship to the vascular pathology is unclear.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 90 (1995), S. 532-538 
    ISSN: 1432-0533
    Keywords: Familial erythrophagocytic ; lymphohistiocytosis ; Vascular occlusion ; Brain pathology ; Cytokines
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Neuropathological findings in two siblings with familial erythrophagocytic lymphohistiocytosis (FEL) are reported. Case 1 showed the typical neuropathological findings of FEL with lymphohistiocytic infiltration of the leptomeninges and perivascular spaces. A characteristic erythrophagocytosis was detected in inguinal lymph nodes, lung and bone marrow. Case 2 revealed calcification and necrotic lesions in the brain. In the necrotic areas, parenchymal calcification, vascular medial calcification, and occlusion of many vessels due to subendothelial fibrosis were detected. The areas of necrosis correlated with the distribution of occluded vessels. These changes were most prominent in putamen, internal capsule, thalamus and dentate nucleus. Hypercytokinemia is suspected to be the underlying mechanism for the clinical and laboratory findings in patients with FEL, although the relationship to the vascular pathology is unclear.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 92 (1996), S. 90-97 
    ISSN: 1432-0533
    Keywords: Key words Hydrocephalus ; Ependyma ; Vimentin ; Glial fibrillary acidic protein ; Intermediate filament
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In order to elucidate the immunohistochemical features of hydrocephalic ependyma, immunohistochemical examination was undertaken in 11 normal, post-mortem brains (age range, 11 weeks’ postconception to 6 months after birth) and 12 hydrocephalic brains (three cases each of congenital aqueductal stenosis, Dandy-Walker malformation, Arnold-Chiari type II malformation and posthemorrhagic hydrocephalus) by using antisera to nestin, vimentin and glial fibrillary acidic protein (GFAP). In normal brains, nestin was predominantly expressed in neuroepithelial cells and radial glial fibers during the period of neuronal migration. Vimentin immunoreactivity was principally detected in immature ependymal cells and their basal fibers after the period of neuronal migration, then partly replaced by GFAP reactivity during late gestation. In hydrocephalus, the areas of ependymal disruption were covered with nestin- or vimentin-positive cells. Nestin and vimentin were also expressed in immature ependymal cells or their basal processes in anatomical regions such as the roof or floor plate of the fourth ventricle or the cerebral aqueduct, and the ventral part of the third ventricle. These results suggest that the overexpression of nestin and vimentin in hydrocephalus follows two patterns: a reactive pattern of proliferating immature glial cells associated with ependymal cell loss and an abnormal developmental pattern of immunopositivity associated with anatomical regions in the midline mesencephalon.
    Type of Medium: Electronic Resource
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