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  • Myopathy  (4)
  • Demyelination  (3)
  • 1
    ISSN: 1432-0533
    Keywords: Amylo-1,4-1,6 transglucosidase ; Branching enzyme ; Myopathy ; Type IV glycogenosis ; Polyglucosan
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Polyglucosan body diseases in adults, contrary to infantile cases (Andersen's disease or type IV glycogenosis or amylopectinosis), are usually not associated with a significant deficiency of the branching enzyme (=amylo-1,4-1,6 transglucosidase). We, therefore, report on a 19-year-old male with complete branching enzyme deficiency presenting with severe myopathy, dilative cardiomyopathy, heart failure, dysmorphic features, and subclinical neuropathy. His 14-year-old brother had similar symptoms and was erroneously classified by a previous muscle biopsy as having central core disease but could later be identified as also having polyglucosan body myopathy. The skeletal muscle, endomyocardiac, and sural nerve biopsies as well as the autopsy revealed extraordinarily severe deposits of polyglucosan bodies not only in striated and smooth muscle fibers, but also in histiocytes, fibroblasts, perineurial cells, axons and astrocytes. Occasional paracrystalline mitochondrial inclusions were also noted. Thus, this patient represents to our knowledge the first juvenile, familial case of polyglucosan body disease with total branching enzyme deficiency and extensive polyglucosan body storage.
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  • 2
    ISSN: 1432-0533
    Keywords: Colchicine ; Myopathy ; Tubulin ; Microtuubules ; Familial mediterranean fever
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Severe colchicine myopathy occurred in a 24-year-old patient treated with colchicine because of familial mediterranean fever complicated by renal amyloidosis. In addition to prominent autophagic vacuoles containing heterogeneous osmiophilic material and pleomorphous bodies, cytoplasmic deposits of finely granular material were detected that have not been noted in previous cases of colchicine myopathy. This granular material was immunoreactive for antibodies to tubulin, α-tubulin, and β-tubulin. These observations substantiate the suggestion that alterations of the microtubular network represent the initial step in the pathogenesis of colchicine myopathy.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Zidovudine ; Myopathy ; Nucleus ; Mitochondria ; AIDS
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Long-term zidovudine (also termed azidothymidine, AZT) treatment of AIDS patients may cause severe myopathy characterized by conspicuous mitochondrial and nuclear changes. The mitochondrial changes are attributed to an inhibitory effect of AZT on the mitochondrial γ-polymerase in a variety of cells. Inhibition of the nuclear α-polymerase is another well-known side effect of AZT, whereas the (nuclear) β-polymerase appears to be rather insensitive. The nuclear changes seen in AIDS patients are usually considered secondary to the human immunodeficiency virus infection. To eliminate the influence of the virus on the nuclei, we studied the effect of AZT on non-infected, organotypic co-cultures of spinal ganglia, spinal cord, and skeletal muscle from fetal rats. We noted significant changes not only in the mitochondria but also in the nuclei of spinal ganglia, spinal cord, and muscle cells, which depended more on the duration of AZT application (1, 3, 5, and 8 days) than on the concentration (0.1, 1, 10, 100 and 1000 μM). The alterations of the mitochondria consisted mainly of swelling, loss of cristae and, finally, disappearance. The nuclei showed nucleolar segregation, marginal condensation of heterochromatin, formation of interchromatin and perichromatin granules, nuclear protrusions and pseudoinclusions and, finally, disintegration. The changes were not as pleomorphic as those seen in biopsy specimens from AIDS patients who had received long-term treatment with AZT. However, this difference can easily be attributed to the short duration of drug application in tissue culture compared to the long-term medication in patients.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Key words Schwann cell inclusions ; Demyelination ; Myelinated nerve fibers ; Morphometry ; Peripheral ; neuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In the cytoplasm of Schwann cells of a sural nerve biopsy from a 21-year-old female patient with chronic neuropathy we noted numerous unique, usually double membrane-bound, osmiophilic, granular or globular inclusions, approximately 30–600 μm in diameter. Some of these membrane-bound vesicular or tubular structures contained less dense or no osmiophilic inclusions. Morphometry revealed a reduction of the myelin area per endoneural area to approximately 13% (normal value: 20– 30%) and of the density of myelinated nerve fibers to 5,412/mm2 (normal value at this age: 6,000–9,000/mm2). Large myelinated nerve fibers were predominantly reduced in number, and no myelinated nerve fibers with diameters larger than 4.5 μm were seen. Numerous, usually small onion bulb formations indicated a predominantly demyelinating type of neuropathy. This is to the best of our knowledge the first case of a chronic demyelinating neuropathy in which this kind of presumably pathognostic deposits in the cytoplasm of Schwann cells was detected.
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  • 5
    ISSN: 1432-0533
    Keywords: Zidovudine ; Myopathy ; Nucleus ; Mitochondria ; AIDS
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Zidovudine (formerly azidothymidine) is a potent inhibitor of the human immunodeficiency virus (HIV) reverse transcriptase and represents the first approved drug showing clinical efficacy in HIV-associated diseases. However, considerable toxicity causing macrocytic anemia, neutropenia, and myopathy has been reported, with severe mitochondrial alterations as a special feature of this myopathy. The mitochondrial changes are consistent with the fact that zidovudine acts as an inhibitor of the mitochondrial gamma-polymerase. Electron microscopically, we could confirm the presence of severely altered mitochondria in a 32-year-old male, who developed a necrotizing myopathy after daily administration of 1,000 mg zidovudine over a period of 15 months. In addition, there were even more severe nuclear changes that, for the most part, have not been documented electron microscopically in HIV-related myopathy either with or without zidovudine treatment, especially in non-necrotic and non-regenerating fibers. Since various in vitro studies have shown interference of zidovudine with nuclear DNA metabolism even in human cell lines, we assume that the nuclear changes that we observed are at least in part related to zidovudine treatment.
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 14 (1970), S. 261-283 
    ISSN: 1432-0533
    Keywords: Allergic Neuritis ; Electron Microscopy ; Mononuclear Cells ; Demyelination ; Remyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Kaninchen mit einer experimentell-allergischen Neuritis (EAN) wurden 8 Tage bis 4 Monate nach der Injektion einer Emulsion aus heterologem Nervengewebe mit Freundschem Adjuvans phasenkontrast- und elektronenmikroskopisch untersucht. Im akuten Stadium der Demyelinisation ist eine Beteiligung von mononucleären Infiltratzellen am Vorgang der Entmarkung peripherer Nervenfasern in der gleichen Weise nachweisbar, wie es Lampert (1969) bei der EAN von Ratten dargestellt hat. Da die Markscheidenschäden jedoch nicht immer in unmittelbarem Kontakt mit den Infiltratzellen auftreten, sind humorale Faktoren, die möglicherweise von den Infiltratzellen ausgeschieden werden, als Ursache der Markscheidenschäden nicht mit Sicherheit auszuschließen. Der weitere Abbau der geschädigten Markscheiden findet dann sowohl in den zu Makrophagen transformierten Infiltratzellen als auch in den proliferierenden Schwann-Zellen statt. Ausnahmsweise dominieren unter den Infiltratzellen auch bei der einfachen experimentell-allergischen Neuritis neutrophile Leukocyteninfiltrate; sie kommen in Zusammenhang mit anderen Zeichen einer schweren Störung der Gefäßnervenschranke wie Erythrodiapedesen und Fibrinexsudaten im fortgeschrittenen Stadium der Gewebsschädigung vor. Auf Axonläsionen und die im Ausheilungsstadium der EAN vorkommenden Zwiebelschalenformationen sowie die wiederholt beobachteten Gruppen regenerierter, von einer gemeinsamen Basalmembran gebündelter Nervenfasern wird kurz hingewiesen.
    Notes: Summary Rabbits with experimental allergic neuritis (EAN), induced by intradermally injected emulsified heterologous antigen together with Freund's adjuvant, were investigated by phase and electron microscopy 8 days to 4 months after the injection. Early lesions of the myelin sheaths in EAN can be demonstrated to occur in close contact with infiltrated mononuclear cells as has been reported by Lampert (1969) in rats. Yet since myelin lesions are not always restricted to areas of immediate contact with infiltrated mononuclear cells, it cannot be excluded that humoral factors, possibly excreted by the infiltrated cells, may initiate the myelin lesions. Further breakdown of myelin sheaths takes place in proliferating Schwann cells as well as in infiltrated mononuclear cells. Occasionally, neutrophilic leucocytes predominate among the cellular infiltrates. They occur together with erythrodiapedesis, and fibrinous exsudates in areas of severance of the blood-nerve barrier. Axonal lesions, and during remyelination, “onion bulb” formation were also seen as a sequence of the demyelinating lesions. Also, bundles of small regenerated nerve fibers enclosed by a single basement membrane were repeatedly observed in areas with remyelinated nerve fibers.
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  • 7
    ISSN: 1432-1459
    Keywords: Ethylene oxide ; Peripheral neuropathy ; Morphometry ; Electron microscopy ; Demyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case is reported of ethylene oxide polyneuropathy after 5 months of exposure. There was symmetrical distal weakness of both lower extremities and transitory reduced nerve conduction velocities with increased latencies. Sural nerve biopsy revealed nerve fibre degeneration of the Wallerian type, associated with reduction of axonal cross-sectional areas and some degree of nerve fibre regeneration that could be confirmed morphometrically. In addition, there was conspicuous paranodal vesicular disintegration of individual myelin lamellae. Unusual cisternae with introverted hemidesmosomes were noted in endoneurial fibroblasts.
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