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  • 1
    Electronic Resource
    Electronic Resource
    Septooptische Dysplasie (de-Morsier-Syndrom) (1998)
    Springer
    Der Nervenarzt 69 (1998), S. 352-356 
    Details
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Septooptische Dysplasie ; De-Morsier Syndrom ; Optikushypoplasie ; Septum-pellucidum-Agenesie ; Key words Septo-optic dysplasia ; De Morsier syndrome ; Optic nerve hypoplasia ; Agenesis of the septum pellucidum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Septo-optic dysplasia (or de Morsier syndrome) is a congenital disorder characterised by anomalies in cerebral midline structures, optic nerve hypoplasia, and hormonal deficiencies. Diagnosis should be made early, due to the possibility of treating the hormonal disturbances. We describe here a case with decreased visual acuity, one-sided hemianopia, nystagmus und agenesis of the septum pellucidum and discuss the heterogeneous appearance of this syndrome. There are two theories regarding its pathogenesis. The first postulates simultaneous damage to both cerebral structures and optic nerve development around the 6th week of gestation, while the other favours secondary degeneration of optic nerve fibres due to a cerebral lesion.
    Notes: Zusammenfassung Die septooptische Dysplasie (oder de-Morsier-Syndrom) ist ein kongenitales Fehlbildungssyndrom zerebraler Mittellinienstrukturen mit Agenesie des Septum pellucidum, Hypoplasie der Nervi optici und Störungen des hypothalamisch-hypophysären Systems. Ein frühzeitiges Erkennen des Syndroms und rechtzeitige Hormonsubstitution beeinflussen entscheidend den Verlauf der Erkrankung. Anhand der Krankengeschichte einer Patientin mit Visusminderung, einseitiger Hemianopsie, Nystagmus sowie Agenesie des Septum pellucidum beschreiben wir das klinische Spektrum dieses sehr heterogenen Syndroms. Die Pathogenese ist nicht geklärt. Zum einen wird eine Schädigung von Optikusneuronen und Hirnstrukturen in der 6. Entwicklungswoche vermutet, zum anderen eine spätere Läsion mit sekundärer Degeneration retinaler Ganglienzellen diskutiert.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001150050282
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  • 2
    Electronic Resource
    Electronic Resource
    Vascular permeability changes in tumours of the peripheral nervous system (1992)
    Springer
    Virchows Archiv 420 (1992), S. 59-64 
    Details
    ISSN: 1432-2307
    Keywords: Peripheral nervous system tumours ; Vascular permeability ; Serum proteins ; Immunohistochemistry ; Neurofibromatosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Vascular permeability changes were examined in 34 tumours of the peripheral nervous system by immunohistochemical demonstration of serum proteins as endogenous tracers. The blood-tumour barrier was impaired in the reticular (Antoni type B) portions of neurinomas (Schwannomas) and in cutaneous neurofibromas but was similar to the normal blood-nerve barrier in fibrillary (Antoni type A) neurinomas, in most neurofibromas, in ganglioneuromas and in anaplastic tumours. These differences in permeability are discussed in relation to aspects of pathological tumour vascularization, the histogenesis of microcystic changes, and systemic therapeutic approaches.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01605985
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  • 3
    Electronic Resource
    Electronic Resource
    Immunochemistry of ethylnitrosourea-induced rat neurinomas, the RN6 neurinoma cell line and their transplantation tumors (1991)
    Springer
    Acta neuropathologica 82 (1991), S. 78-85 
    Details
    ISSN: 1432-0533
    Keywords: Ethylnitrosourea-induced neurinomas ; RN6 neurinoma cell line ; Transplantation tumors ; Immunohistochemistry ; Intermediate filaments
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The expression of glial fibrillary acidic protein (GFAP), vimentin, S-100 protein (S-100), HNK-1, myelin basic protein (MBP) and fibronectin was investigated immunohistochemically in 51 ethylnitrosourea (ENU)-induced neurinomas of the rat. Additionally, 90 transplantation tumors derived from ENU-induced neurinomas and the RN6 rat neurinoma cell clone were studied. Vimentin immunoreactivity was shown in 50/51 primary neurinomas and 60/90 transplantation tumors. In contrast, GFAP was expressed in only 23/51 primary tumors and in 5/90 transplantation tumors. In the RN6 neurinoma clone, vimentin and GFAP could be demonstrated both in vivo and in vitro GFAP expression varied depending on the tumor localization, i.e., tumors of distal portions of peripheral nerves were more frequently GFAP positive than tumors of the spinal roots or of cranial nerves. The same tendency was observed for S-100. In the series of transplantation tumors S-100 and GFAP immunoreactivity decreased with increasing numbers of transplantation passages. Only individual cells in 5 primary tumors were HNK-1 positive and no MBP-immunorcactive cells were observed. Our results demonstrate that the expression of differentiation antigens in ENU-induced experimental neurinomas parallels the results reported for human neurinomas.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00310927
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  • 4
    Electronic Resource
    Electronic Resource
    Immunohistochemistry of primitive neuroectodermal tumors in infants with special emphasis on cytokeratin expression (1991)
    Springer
    Acta neuropathologica 82 (1991), S. 494-501 
    Details
    ISSN: 1432-0533
    Keywords: Primitive neuroectodermal tumor ; Cytokeratin immunohistochemistry ; Double labelling ; Electron microscopy ; Immunoblotting
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Eleven primitive neuroectodermal tumor (PNET) biopsies from infants under the age of 3 years were studied for the presence of various differentiation markers for neuroectodermal stem cells. Special emphasis was placed on the expression of cytokeratin proteins. The tumor cells expressed different cytokeratin proteins (CK8, CK13, CK18, CK19, KL1, AE1/AE3, MNF16) in 3 of 11 cases. These cases were furthermore characterized by a strong expression of glial fibrillary acidic protein, S-100 protein and vimentin. Vimentin and cytokeratin proteins were co-expressed; cross-reactivity between these two intermediate filaments could be excluded by immunoblotting. It is noteworthy that the three positive tumors were all from infants in their 1st year. We assume that PNETs in early infancy are characterized by a particularly wide range of differentiation patterns. The presence of cytokeratin proteins in these cases seems to be associated with the expression of vimentin and must be regarded as an indicator of an early developmental stage of the tumor cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00293384
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