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  • 1
    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
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  • 2
    ISSN: 1432-0533
    Keywords: Peripheral neuropathy ; Diabetes mellitus ; Arteritis ; Hypoxic axonopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The peripheral nerve was taken from 25 diabetic patients with arteritis just before or after amputation of a leg. The ultrastructural study of myelinated fibers showed several alterations. Loss of fibers varied greatly from case to case. This was accompanied in some cases by hypertrophy of the Schwann cells around demyelinated fibers as has been observed in some ordinary diabetic neuropathies. Unusual abnormalities consisted of particular axonal degeneration and evidence of aberrant remyelination. This axonal injury was peculiar because of the accumulation of organelles which tended to transform some of the fibers into enlarged axons. Such a lesion might correspond to a hypoxic axonopathy.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1459
    Keywords: Axon ; Lymphoma ; Myelin ; Peripheral neuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Recent progress in immunopathological studies of peripheral nerve and lymph node fragments together with 16 personal cases and numerous clinicopathological reports have suggested a new classification of peripheral neuropathies (PN) and lymphomas. These are: (1) PN due to local infiltrations by a T-cell lymphoma; (2) acute polyradiculoneuritis due to active demyelination and associated with infiltrates of a T-cell lymphoma in the epineurium, resembling Marek's disease (which is a T-cell lymphoma); (3) B-cell lymphoma proliferation which may be restricted to or predominate in the peripheral nervous system, with a large clinicopathological heterogeneity ranging from localized forms to ascending polyradiculoneuropathies; (4) angiotropic lymphoma, which is a B-cell lymphoma and may present as an acute mononeuropathy; (5) patients with acquired immunodeficiency syndrome due to lymphomatous infiltrates in the endoneurium, of which 2 cases of PN have been reported; (6) PN associated with organomegaly, endocrinopathy, M-component and skin lesions, certain cases being associated with a plasmocytoma and sometimes Castleman's disease but without any monoclonal gammopathy; (7) classic Guillain-Barré syndrome, prone to develop in patients with extraneural lymphoma but without any lymphomatous infiltrates in the peripheral nervous system; (8) certain cases (4 out of 16 in our series) where there is no clear relationship between PN and lymphoma, and there are mainly features of axonal degeneration. Inflammatory perivascular infiltrates were sometimes present in the epineurium.
    Type of Medium: Electronic Resource
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