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  • 1
    Electronic Resource
    Electronic Resource
    Cathepsin B, D and K expression in adamantinomatous craniopharyngiomas relates to their levels of differentiation as determined by the patterns of retinoic acid receptor expression (2003)
    Oxford, UK : Blackwell Science Ltd
    Histopathology 43 (2003), S. 0 
    Details
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Aims:  To investigate the potential predictive value of cathepsins B, D and K in a series of 51 adamantinomatous craniopharyngiomas. While almost always benign, craniopharyngiomas exhibit a high propensity to recur postsurgically and biological markers are therefore needed to predict their recurrence. We have previously demonstrated the potential predictive value of retinoic acid receptors (RARs) (Lefranc et al., J. Neurosurg. 2003; 98; 145–153).Methods and results:  Computer-assisted microscopy was used to determine quantitatively the immunohistochemical levels of expression of the α, β and γ RAR subtypes and cathepsins B, D and K. The levels of expression of cathepsin D and of cathepsin B correlated significantly with the levels of expression of RARβ. The levels of expression of cathepsin K correlated significantly with the levels of expression of RARγ.Conclusions:  Recurrent adamantinomatous craniopharyngiomas are characterized by low levels of RARβ and high levels of RARγ. The tendency to recurrence seems, at least partly, to relate to the fact that (i) craniopharyngiomas with low levels of RARβ express low levels of cathepsin D, and (ii) craniopharyngiomas with high levels of RARγ express high levels of cathepsin K.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2559.2003.01751.x
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  • 2
    Electronic Resource
    Electronic Resource
    Developmental aspects of type II lissencephaly (1994)
    Springer
    Acta neuropathologica 89 (1994), S. 72-84 
    Details
    ISSN: 1432-0533
    Keywords: Key words     Type II lissencephaly ; Polymicrogyria ; Development ; Meninges ; Neurocristopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report neuropathological studies of five cases of type II lissencephaly from three fetuses and two infants. This comparative study allowed us to determine the developmental course of the cerebral lesions. Two distinct developmental events seem to generate this type of brain malformation: firstly, an early disturbance in cortex formation, which results both from a disorder of radial migration and a pial barrier disruption; secondly, a late perturbation of cerebral surface organization, resulting in fusion of the cerebral surface. All these features can be related to a primitive meningeal pathology, and more generally, to a neurocristopathy. Accordingly to our observations, this brain malformation appears during both migrational and post-migrational stages and may be considered more like a polymicrogyria than a lissencephaly.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294262
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    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Uncompacted myelin lamellae in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome (1994)
    Springer
    Acta neuropathologica 87 (1994), S. 302-307 
    Details
    ISSN: 1432-0533
    Keywords: Castleman disease ; Monoclonal gammopathy ; Myelin ; Peripheral neuropathy ; POEMS syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Mechanisms of peripheral neuropathies in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome are poorly understood. A peripheral nerve biopsy was performed in 22 patients. Of these 9 had histological features of Castleman's disease on lymph node biopsies, and 19 had a monoclonal lambda light chain in their serum. Certain nerve fragments were paraffin embedded, others were frozen and studied by direct immuno-fluorescence, and others were fixed for ultrastructural examination. Paraffin-embedded fragments did not show any amyloid deposits, and at direct immunofluorescence there was no immunoglobulin fixation. At ultrastructural examination, features of uncompacted myelin lamellae (UML) were present in 19 patients, and their frequency varied from 1% to 16% of myelinated fibres. Up to now UML have been reported only in 7 patients with POEMS syndrome in the literature. UML have also been noticed in a few cases of inflammatory demyelinating polyradiculoneuritis and inherited tendency to pressure palsy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296746
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    In vivo diagnosis of Kufs’ disease by extracerebral biopsies (1998)
    Springer
    Acta neuropathologica 96 (1998), S. 102-108 
    Details
    ISSN: 1432-0533
    Keywords: Key words Adult ceroid lipofuscinosis ; Kufs’ disease ; Electron microscopy ; Extracerebral biopsies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In almost all of the earlier reported cases of Kufs’ disease, the adult form of ceroid lipofuscinosis, the diagnosis was ascertained by cerebral tissue examination, while peripheral biopsy examination revealed an apparent poor diffusion of specific lipofuscinic deposits, the finger print profiles (FPs). We report the ultrastructural data from skin, muscle and rectal biopsy specimens from two siblings, both still living, who present clinical features of Kufs’ disease. We observed the presence of FPs in locations that differ from the previous classic reports. Our results emphasize the value of extracerebral biopsies for the diagnosis of Kufs’ disease in vivo, and suggest some physiopathological assumptions based on vascular wall involvement.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050866
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    Paper (German National Licenses)
    Fulltext
  • 5
    Electronic Resource
    Electronic Resource
    Uncompacted myelin lamellae in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome (1994)
    Springer
    Acta neuropathologica 87 (1994), S. 302-307 
    Details
    ISSN: 1432-0533
    Keywords: Key words: Castleman disease – Monoclonal gammopathy – Myelin – Peripheral neuropathy – POEMS syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Mechanisms of peripheral neuropathies in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome are poorly understood. A peripheral nerve biopsy was performed in 22 patients. Of these 9 had histological features of Castleman's disease on lymph node biopsies, and 19 had a monoclonal lambda light chain in their serum. Certain nerve fragments were paraffin embedded, others were frozen and studied by direct immunofluorescence, and others were fixed for ultrastructural examination. Paraffin-embedded fragments did not show any amyloid deposits, and at direct immunofluorescence there was no immunoglobulin fixation. At ultrastructural examination, features of uncompacted myelin lamellae (UML) were present in 19 patients, and their frequency varied from 1% to 16% of myelinated fibres. Up to now UML have been reported only in 7 patients with POEMS syndrome in the literature. UML have also been noticed in a few cases of inflammatory demyelinating polyradiculoneuritis and inherited tendency to pressure palsy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296746
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 6
    Electronic Resource
    Electronic Resource
    Developmental aspects of type II lissencephaly (1995)
    Springer
    Acta neuropathologica 89 (1995), S. 72-84 
    Details
    ISSN: 1432-0533
    Keywords: Type II lissencephaly ; Polymicrogyria ; Development ; Meninges ; Neurocristopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report neuropathological studies of five cases of type II lissencephaly from three fetuses and two infants. This comparative study allowed us to determine the developmental course of the cerebral lesions. Two distinct developmental events seem to generate this type of brain malformation: firstly, an early disturbance in cortex formation, which results both from a disorder of radial migration and a pial barrier disruption; secondly, a late perturbation of cerebral surface organization, resulting in fusion of the cerebral surface. All these features can be related to a primitive meningeal pathology, and more generally, to a neurocristopathy. Accordingly to our observations, this brain malformation appears during both migrational and post-migrational stages and may be considered more like a polymicrogyria than a lissencephaly.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294262
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
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