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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 3 (1958), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Gaucher disease ; Brain ; Immunohistochemistry ; Macrophages ; Astrocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Splenectomy in children with the Norrbottnian type of Gaucher disease is followed by increased blood levels of glucosylceramide and imparied neurological and mental status. High blood levels are associated with an increased accumulation of glucosylceramide in perivascular Gaucher cells in the brain compared to non-splenectomised cases. Surrounding the Gaucher cell infiltrates there is loss of neurons and slight demyelinaton in the brain parenchyma. The brains of four cases with the Norrbottnian type of Gaucher disease were examined by immunohistochemical stains in an attempt to further characterize the perivascular Gaucher cells and to examine the reactions of the vessel walls and brain parenchyma to the accumulation of Gaucher cells. The perivascular storage cells showed granular staining with antibodies to muramidase and α1-antichymotrypsin confirming that they are blood-derived macrophages belonging to the monocyte-macrophage system. The Gaucher cells contained material positive for antisera to plasma proteins strongly suggesting that large molecules (including glucosylceramide) can escape from the blood and be taken up by the macrophages in Gaucher disease. The storage cells were surrounded by a reticulin network stained by antisera to collagen type III, type IV and laminin. The infiltrates were bounded from the brain parenchyma by a membrane strongly positive with antiserum for the basal lamina protein collagen type IV and laminin. The formation of a basal lamina around the Gaucher cell cuffs probably constitutes a protective phenomenon governing the brain parenchyma against the foreign cells. A focal loss of neurons but only minor loss of axons could be demonstrated with the antiserum to neurofilament. The brain parenchyma surrounding the Gaucher cell infiltrates showed marked astrogliosis in the anti-glial fibrillary acidic protein stain. In the two cases previously shown to have higher blood levels of glucosylceramide there were astrocytes positive for plasma proteins indicating passage of plasma proteins into the brain, this was not seen in the non-splenectomised cases. The additive effect of low-grade tissue damage in the vicinity of the Gaucher cell infiltrates is probably enough to explain the increased neurological symptoms and mental retardation following splenectomy in the Norrbottnian type of Gaucher disease.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 75 (1988), S. 481-490 
    ISSN: 1432-0533
    Keywords: Sialuria ; Salla disease ; CNS pathology ; Neurofibrillary tangles
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A neuropathological study was performed on two patients with Salla disease, one male and one female, from different families. They both died at the age of 41 years. Both patients showed increased excretion of free sialic acid in the urine, psychomotor retardation starting in the 1st year of life, ataxia and spasticity. Several family members of both families were affected with the same disease indicating the hereditary character of the disorder. The neuropathological investigation revealed strikingly similar changes in the two cases. Macroscopically the cerebral white matter was severely reduced. Histologically marked loss of axons and myelin sheaths was accompanied by pronounced astrocytic proliferation. The remaining axons frequently showed ovoid swellings surrounded by a myelin sheath. The reduction of the number of myelin sheaths seemed proportional to the numerical reduction of axons. Many cortical nerve cells displayed in relation to age an abnormal amount of lipofuscin. Neurofibrillary tangles were observed in nerve cells of the neo-cortex, nucleus basalis of Meynert and locus ceruleus. Cerebellum showed moderate loss of Purkinje cells. In the spinal cord axonal degeneration was observed in both ascending and descending tracts.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 79 (1989), S. 149-153 
    ISSN: 1432-0533
    Keywords: Lead ; Rat ; Cerebellum ; Particle-induced X-ray emission (micro-PIXE)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The distribution of lead in the cerebellum of suckling Sprague-Dawley rats was examined using a nuclear microprobe for elemental mapping of tissue sections (particle-induced X-ray emission, 3-μm beam of 2.5 MeV protons; micro-PIXE). The rats were injected intraperitoneally with a lead-containing vehicle or vehicle only from ages 1 to 14 days. The calculated doses were 7.8 (low-dose) and 15.6 (high-dose) μg lead/g body weight. The rats were killed at 20 days of age. The vascular system was rinsed quickly with 0.15 M ammonium acetate to obtain determinations of intra-parenchymal lead with minimal influence of lead bound to erythrocytes and plasma proteins. Brains were frozen in propane/propylene in liquid nitrogen. Cryostat sections, 15 μm thick, were air dried on formvar coats that covered a hole, 15 mm in diameter, in a plastic disc, and were used for lead analysis by micro-PIXE. Very low concentrations of lead were found in the brain of controls. Lead levels in homogenates from cerebrum and cerebellum measured by atomic absorption spectrometry (AAS) were: low-dose 1.2–2.2 μg/g wet weight and high-dose 1.4–2.4 μg/g wet weight. The lead levels measured with the micro-PIXE method were in good agreement with the levels found with AAS. Lead was present in the cerebellar white matter in two to three times higher amounts than in the cortical grey (low-dose white matter 11–18 μg/g dry weight, grey matter 2.0–5.5 μg/g dry weight). This was true for both low and high dose exposed rats. Lead concentrations in rats subjected the high-dose lead exposure were approximately 60% higher than those in low-dose exposed rats. Concentrations were lower in the Purkinje cell layer than in other parts of the cortex. These new findings on the distribution of lead in suckling rats are discussed in relation to the pathogenesis of experimental lead encephalopathy.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Mucopolysaccharidosis ; Sanfilippo's syndrome ; Ceroid ; Lipofuscin ; Autofluorescence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Characteristics of intraneuronal lipopigment in two siblings with Sanfilippo's syndrome are reported. A lipopigment emission spectrum probably reflects its composition and the (uncorrected) autofluorescence emission spectra results are compared with spectra from non-diseased tissue and from previously reported childhood-onset neuronal ceroidlipofuscinoses (ceroidoses), adult-onset ceroidosis (Kufs' disease) and animal ceroidoses. Values derived from the emission spectra from Sanfilippo's syndrome could be distinguished from those obtained from equivalent regions of non-diseased brains and were within the range of abnormal values previously reported from accumulations of pigment in various types of neuronal ceroidosis. Some abnormal lipopigment in Sanfilippo tissue was indistinguishable from some lipopigment in childhood-onset ceroidosis and in Kufs' disease. These results indicate that the intraneuronal lipopigment which accumulates to an abnormal extent in Sanfilippo's syndrome should not be termed “lipofuscin”, which is a normal cerebral constituent, but “ceroid” to denote lipopigment with abnormal characteristics.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 14 (1970), S. 293-303 
    ISSN: 1432-0533
    Keywords: Chronic Sclerosing Panencephalitis ; Myelin Involvement ; Lipid Composition ; Fatty Acid Composition ; Sphingolipids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In einem subakuten und zwei chronischen Fällen von sklerosierender Panencephalitis wurden der Lipidgehalt und das Fettsäuremuster des Gehirns untersucht. Bei den chronischen Fällen war der Gangliosidgehalt in der Hirnrinde leicht bis mäßig herabgesetzt. Bei allen Fällen fand sich im Mark ein frühzeitig auftretender und starker Verlust an Sphingolipiden, insbesondere Galaktolipiden. Die Fettsäurezusammensetzung der Phosphoglyceride war nur leicht gegenüber der Norm verändert. Die Cholesterinester enthielten sehr niedrige Konzentration an Polyensäuren und unterschieden sich damit grundsätzlich von jenen im normalen menschlichen Gehirn. Das Mark der beiden chronischen Fälle zeigte ein fast völliges Verschwinden von Cerebrosiden und Sulfatiden mit langkettigen Fettsäuren sowie eine relative Zunahme solcher mit mittel- bis langkettigen Fettsäuren und C23 h.
    Notes: Summary In one subacute and two chronic cases of sclerosing panencephalitis (SSPE), the lipid composition and the fatty acid pattern of cerebrum were studied. The ganglioside concentration in the cerebral cortex was slightly to moderately decreased in the chronic cases. In all the cases there was an early and extensive loss of sphingolipids, especially of galactolipids, in the white matter. The fatty acid composition of the phosphoglycerides was only slightly changed from the normal. The cholesteryl esters contained very low concentrations of the polyenoic acids and thus differ completely from those of normal human brain. In cerebral white matter of the two chronic cases there was an almost complete disappearance of cerebrosides and sulfatides with long-chain fatty acids and a marked relative increase of those with medium-short acids and C23 h.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 14 (1970), S. 284-292 
    ISSN: 1432-0533
    Keywords: Chronic Sclerosing Panencephalitis ; Myelin Involvement ; Lipid-Histochemistry ; Pathology of Oligodendroglia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Histologische und lipid-histochemische Untersuchungen wurden an zwei chronischen und einem subakuten Fall von sklerosierender Panencephalitis mit besonderer Berücksichtigung der Markschädigung durchgeführt. In den chronischen Fällen fanden sich fast völliger Markscheidenverlust, Reduktion der Oligodendroglia sowie zahlreiche Makrophagen. Histochemisch wurde in der Marksubstanz ein fast völliger Verlust von Cerebrosiden, Sphingomyelinen und Cholesterin nachgewiesen. Die Makrophagen enthielten vorwiegend Cholesterinester. Die Markscheiden des subakuten Falles erschienen größtenteils erhalten, doch fanden sich in der Oligodendroglia zahlreiche Kerneinschlüsse. Die Befunde werden mit den im Teil II mitgeteilten Ergebnissen der biochemischen Analyse verglichen. Es wird vermutet, daß die Schädigung der Oligodendroglia auch deren Fähigkeit, den Stoffwechsel der Markscheidenlipide aufrechtzuerhalten, beeinträchtigen dürfte. Der neurochemische Nachweis eines sehr niedrigen Gehaltes an Sphingolipiden, vor allem an Galaktolipiden, sowie deutlicher Veränderungen ihres Fettsäuregehaltes, selbst im subakuten Fall mit histologisch und histochemisch fast intakten Markscheiden, weisen auf eine frühe und tiefgreifende Störung des Stoffwechsels der Markscheidenlipide bei der sklerosierenden Panencephalitis hin.
    Notes: Summary A histological and lipid-histochemical study, with emphasis on myelin involvement, has been made of two chronic and one subacute case of sclerosing panencephalitis. An almost total loss of myelin sheaths was found in the brains of the chronic cases, accompanied by a reduction in the number of oligodendroglial cells and the occurrence of numerous compound granular corpuscles. Histochemically a practically complete disappearance of cerebrosides, sphingomyelins, and cholesterol was demonstrated in the white matter. The compound granular corpuscles mainly contained cholesteryl esters. The myelin sheaths of the subacute case appeared for the most part well preserved but numerous intranuclear inclusion bodies were observed in the oligodendroglia. The findings are discussed against the results of the biochemical analysis, presented in Part II. It might be supposed that the oligodendroglial lesions would also affect the capacity of the oligodendrocytes to maintain the metabolism of myelin lipids. In fact, the neurochemical observations of a very low content of sphingolipids, especially of galactolipids, and a marked change of their fatty acid composition, even in the subacute case with histologically and histochemically almost intact myelin sheaths, suggest an early and profound involvement in the metabolism of the myelin lipids in sclerosing panencephalitis.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: Herpes simplex encephalitis ; Histochemistry ; Capillaries
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An enzyme histochemical study of experimental herpes simplex encephalitis of the mouse has revealed a decrease in the number of capillaries displaying alkaline phosphatase activity. Glial cells showed increased Inosine 5 diphosphatase and ATPase activity. These enzyme histochemical changes were distributed throughout the nervous parenchyma while the lesions, seen by light microscopy, are localized to well defined areas. Mice inoculated with a pure culture of irradiated HSV failed to show the above mentioned modifications.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0533
    Keywords: Polyneuropathy ; Hereditary disease ; Chiloren ; Sural nerve biopsy ; Electron microscopy ; Morphometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Ten autosomal recessive/sporadic cases of hereditary motor and sensory neuropathy type I (HMSN I), nine of which originated from the northern part of Sweden, were included in the study. Parents were free from neurologic symptoms. Motor and sensory conduction velocity was normal when recorded, i.e., in 19 and 17 parents, respectively. Sural nerve biopsies from the ten cases revealed a varying degree of onion bulb formation. In eight of the cases the onion bulbs consisted of abundant basement membranes, whereas the Schwann cells were few and sometimes lacking. There were in some cases considerable differences between separate fascicles as to the loss of myelinated nerve fibers. In the six biopsies in which teasing was performed signs of present and previous demyelination were noticed. Numerous internodal segments were abnormally thin with reference to their length. In many such segments there were marked local thickenings of the nerve fiber. In cross sections the probable counterparts to these thickenings were nerve fibers with unduly thick myelin sheaths and complex folding of the myelin. Ultrastructural axonal changes were seen in the majority of the cases. The pathogenetic and diagnostic implications of the present findings are discussed.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 54 (1981), S. 287-292 
    ISSN: 1432-0533
    Keywords: Mucopolysaccharidoses III ; Sanfilippo's syndrome ; Histochemistry ; Electron microscopy ; Lipofuscin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A histochemical and ultrastructural study was made on the brain of a 23-year-old man with Sanfilippo's syndrome. In accordance with previous reports the cortical nerve cells contained a PAS-positive lipid storage substance. This showed intense autofluorescence in UV-light and was positive with various stains for lipofuscin. The storage material appeared ultrastructurally as inclusion bodies composed of short lamellated membranes, granular material, and vacuoles. In addition, concentrically and transversely lamellated membranous cytoplasmic bodies were observed in the nerve cells. It is concluded that the PAS-positive lipid storage material in the neurons was composed partly of lipofuscin in addition to other lipids presumably glycosphingolipids.
    Type of Medium: Electronic Resource
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