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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 10 (1968), S. 132-142 
    ISSN: 1432-0533
    Keywords: Myoclonus body disease ; Lafora bodies ; Electron microscopy ; Corpora amylacea ; Unverricht-Lundborg disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Résumé Les auteurs analysent l'ultra-structure des corps de Lafora à propos d'une biopsie cérébrale pratiquée dans une maladie d'Unverricht-Lundborg caractéristique. La surcharge observée dans les périkaryons neuronaux et dans les prolongements dendroaxonaux, au niveau des synapses, comporte des structures filamenteuses hélicoïdales de 75 Å d'épaisseur, correspondant à des macro-molécules protéiques complexes, des corpuscules denses de 150 à 300 Å et une matrice finement granuleuse. Les corps de Lafora, dépourvus de membrane propre, correspondent à une sécrétion anormale ou à une transformation d'organites cellulaires. Des relations de continuité existent entre les filaments et les ribosomes ergastoplasmiques. Les auteurs soulignent les relations unissant corps de Lafora et corps amylacés. Ils discutent leur morphologie et la comparent à celle de plusieurs structures pathologiques connues d'aspect fibrillaire.
    Notes: Summary The authors analyse the Lafora bodies' ultra-structure about a cerebral biopsy in a typic case of Unverricht-Lundborg disease. This obvious storage is noticed in the nerve cells perikarya, dendro-axonal processes and synapses; it appears to be compound of helicoïdal, filamentous, 75 Å wide profiles corresponding to complex proteic macro-molecules, 150–300 Å wide densities and slightly granulous matrix. The Lafora bodies without any limiting membran correspond to a pathologic neuro-secretion or to changes of cytoplasmic organites. Some continuity is seen between filaments and ribosomes. The authors emphazise the relations connecting Lafora bodies and corpora amylacea. Their morphology is discussed and compared with that of several pathologic fibrillar structures.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Neuroaxonal Dystrophy ; Infantile Autonomi Nervous System ; Electron Microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The autonomic nervous system is studied by electron microscopy for the first time in two siblings born of North African consanguineous parents and affected by infantile neuroaxonal dystrophy. The changes already reported in dystrophic axons of the central and peripheral nervous system, are seen in the myenteric plexus of rectum mucosa. The authors stress the diffuse involvement of the nervous tissue in this degenerative disorder of still unknown nature.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 34 (1976), S. 65-76 
    ISSN: 1432-0533
    Keywords: Niemann-Pick disease, Crocker's type C ; Ultrastructural study of a case
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The authors report electron microscopic findings in brain, bone marrow and liver biopsies in a case of juvenile Niemann-Pick disease (Crocker's type C). The diagnosis was supported by clinical data increase of blood sphingomyelin and vacuolated histiocytes in bone marrow and liver. Neurons and glial cells were filled with two types of cytosomes: classical multilamellar bodies and unusual pleiomorphic bodies. The latter type probably showed some lipofuscinic component. The relationship between type C and classical Niemann-Pick disease is discussed.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 62 (1983), S. 31-40 
    ISSN: 1432-0533
    Keywords: Human pinealomas ; Ultrastructure ; Specific markers ; Pinealocyte
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An ultrastructural study of four pinealomas was carried out to precise eventual specific markers. Dark and clear cells joined with zonulae adherents, extensive and pleiomorphous processes, a complex vacuolar system, and characteristic organelles (lysosome-like structures, clear and dense-core vesicles, vesicle-crowned rodlets and related structures, microtubular sheaves and centriolar derivatives, membranous whorls, fibrous bodies, microtubules, heterogeneous cytoplasmic inclusions) offered a typical pattern. No correlation could be made between the histological and ultrastructural features. The authors stress the ultrastructural similarities between the human tumor cells and the mammalian pineal cells. Pinealomas appeared as a morphological entity distinct from neuronal and astrocytic tumors.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 39 (1977), S. 261-269 
    ISSN: 1432-0533
    Keywords: Giant axons ; Neuropathy ; Neurofilaments ; Microfilaments ; Sympathetic neurons
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of giant axonal neuropathy in a 8 years old child is reported by light and electron microscopy. Clinically, this case is strikingly similar to the rare previous reports and characterized by a distal neuropathy, CNS symptoms and tightly curled hair. Giant axons were found in the sural nerve but had been absent at the onset of the illness. An increase in the number of neurofilaments was found in the axons and neurons of the myenteric plexus. The number of microfilaments was also increased in various types of cells namely Schwann and endothelial cells and fibroblasts: This suggests that the metabolic disorder, probably inborn and genetic, does not only affect the nervous system.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 55 (1981), S. 77-80 
    ISSN: 1432-0533
    Keywords: Esthesioneuroepithelioma ; Olfactory neuroblastoma ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A rare case of esthesioneuroepithelioma infiltrating the right frontal lobe is reported in a 41-year-old man. This tumor composed of compact lobules with tubular arrangements, ultrastructurally showed two different cell types, both devoid of neurosecretory granules and reminiscent of neurons and sustentacular cells of the olfactory epithelium. These features are distinguished from those generally observed in olfactory neuroblastomas.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 56 (1982), S. 151-156 
    ISSN: 1432-0533
    Keywords: Central neuronal tumor ; Third ventricle tumor ; Synapses ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The authors report two cases of a rare tumor in adults which were inserted on the fornix and caused a frontal syndrome. By light microscopy, the tumors, highly calcified, were composed of small clear cells forming dense areas in a patchy fibrillary stroma. Electron microscopy revealed a striking neuronal differentiation with numerous synapses. These tumors, for which the term neurocytomas was proposed, were compared with the other CNS neuronal tumors described in the literature.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 18 (1971), S. 327-341 
    ISSN: 1432-0533
    Keywords: Infantile Neuroaxonal Dystrophy ; Electron Microscopy ; Striated Muscle ; Membranous-Tubular Structures ; Filamentous Structures
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary This paper deals with the first ultrastructural study of muscle fiber in a child affected by infantile neuroaxonal dystrophy or Seitelberger's disease. In a first step, diagnosis was performed by light and electron microscopy in biopsy and autopsy findings in central and peripheral nervous system. Muscle fiber and axonal changes are very similar. The ultrastructure findings in muscle fiber are as follows: 1. neural atrophy, 2. overproduction of membrano-tubular structures related to sarcoplasmic reticulum hyperplasia, 3. filamentous aggregates by presumed overproduction of myofilaments, 4. overproduction of abnormal mitochondria. These changes, already described in various muscular diseases, are not specific; they seem related to an abnormal muscle fiber reaction in close association to dystrophic axonal endings.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0533
    Keywords: Leucodystrophy Metachromatic ; Electron Microscopy ; Prismatic Deposits ; Brain ; Peripheral Nerve ; Kidney ; Rectum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Clinical history, light and electron microscopy findings are reported in four cases of infantile metachromatic leucodystrophy. By electron microscopy, the authors describe the various types of lesions and the different lipid deposits noticed in the central and peripheral nervous system, in the kidney and rectum. They stress the prismatic lipid deposits observed not only in the cerebral white matter and in the kidney but in the peripheral nerves as well.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 28 (1974), S. 353-359 
    ISSN: 1432-0533
    Keywords: Familial Céroïd-Lipofuscinosis ; Batten Disease ; Late Infantile Amaurotic Idiocy ; Consanguinity ; Curvilmear, Finger-Print Inclusions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The authors report histochemical and electron mciroscopic data of brain biopsies in two cases of Battern's disease. The siblings affected, a male and a female, are born from consanguineous North African parents. The diagnosis of Ceroïd-Lipofuscinosis is supported by neuro-glial, endothelial and perithelial autofluorescent cell storage. By electron microscopy the abnormal cytosomes show both curvilinear and finger-print profiles; their lysosomal nature is supported by their obvious acid phosphatase activity. These lipopigment cytoplasmic inclusions are compared with those described in senile brains and in other cases of Batten's disease.
    Type of Medium: Electronic Resource
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