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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 99 (2000), S. 39-47 
    ISSN: 1432-0533
    Keywords: Key words Sural nerve ; Muscle fiber diameter ; Morphometry ; Electron microscopy ; Myotonic ¶dystrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We compared peripheral nerve fibers and muscle fibers in myotonic dystrophy (MD) using a computer-assisted device for morphometry. In the 17 cases with MD studied, the sural nerves of 14 cases (82%) showed various degrees of reduction of the myelin sheath area (MSA) per endoneurial area. Of these, 8 cases (47%) presented with a mild reduction of the MSA, 5 cases (29.4%) with moderate reduction, and one case (6%) with severe reduction. The number of myelinated nerve fibers was not significantly reduced in MD when compared with control nerves, due to clusters of small regenerated nerve fibers. The mean diameter of the muscle fibers in 6 of the 17 cases was less than 40 μm. Of these 6 severely affected cases, ¶5 revealed a considerable reduction of the MSA. Other cases, which appeared to be normal in respect to the diameter of muscle fibers, showed various degrees of reduction of the MSA. Thus, there is usually, but not always a morphometric correlation of the severity of changes between peripheral nerves and muscle. The severity of the peripheral neuropathy appears to depend largely on the patient’s age, the stage of the disorder, and the time of progression. Electron microscopic examination of sural nerves showed significant, though non-specific pathological changes.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 10 (1968), S. 218-241 
    ISSN: 1432-0533
    Keywords: Myotonic dystrophy ; Sarcoplasmic masses ; Striated annulets (Ringbinden) ; Muscle fiber ; Electron microscopy ; Phase microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Das Biopsiematerial von drei Patienten mit unterschiedlichen klinischen und pathologischen Stadien der myotonischen Dystrophie wurde phasenkontrast-und elektronenmikroskopisch untersucht. Beim 1. und 2. Fall waren “sarkoplasmatische Massen” und “Ringbinden” besonders zahlreich, während sie beim 3. Fall mit klinisch erheblich fortgeschrittener Muskelatrophie nur ausnahmsweise vorkamen. Elektronenmikroskopisch erschienen fast alle Elemente der Muskelfasern, zumindest an einigen Stellen, von dem atrophischen oder dystrophischen Prozeß betroffen: Myofilamente, Z-Streifen, Triaden, Kerne, Mitochondrien und Sarkolemm. Außerdem fanden sich cytoplasmatische Cysten, multilamellierte Körperchen, zahlreiche Lipofuscingranula und verschiedene andere abnorme Strukturen. Besonders kennzeichnend für die Erkrankung waren die “sarkoplasmatischen Massen”, die mit variablen Mengen von fehlorientierten Myofilamenten, aber auch von anderen Komponenten der Muskelzelle ausgefüllt waren. Die Ringbinden wurden von fehlorientierten Myofibrillen gebildet, indem sie meist kreisförmig die im Zentrum normal ausgerichteten Myofibrillen umschlossen. Einzelne Veränderungen, die bisher weder bei der myotonischen Dystrophie noch in irgendeiner anderen Muskelkrankheit nachgewiesen worden sind, konnten in leicht dystrophischen wie auch in hochgradig atrophischen Fasern nachgewiesen und identifiziert werden: Große homogene Lacunen, die sich vom sarkoplasmatischen Reticulum herleiten und eigenartige geometrische Anordnungen der terminalen Cisternen. Der Ursprung bestimmter anderer Strukturen blieb unklar. In unserem Material fanden sich keine segmentalen Nekrosen, so daß sich die Relation des dystrophischen Prozesses zur Zenkerschen Degeneration und zur Atrophie, den nach histologischen Untersuchungen wesentlichsten Befunden bei der Steinertschen Erkrankung, nicht eindeutig bestimmen ließ.
    Notes: Summary Biopsies from three patients with different clinical and pathological stages of myotonic dystrophy were studied by phase and electron microscopy. Large sarcoplasmic masses and spiral annulets were prominent in Cases 1 and 2 while in Case 3, featured clinically by atrophic weakness, they were infrequent. In the latter the residual fibers were either large and dystrophic or small and atrophic. Electron microscopically, nearly all components of the muscle cell, in places, were involved in the dystrophic or atrophic process, such as myofilaments, Z discs, triads, nucleic, mitochondria, and the sarcolemma. In addition, cytoplasmic cysts, multilammellated bodies, and lipofuscin granules were observed. The most characteristic feature of the disease were sarcoplasmic masses, filled with varying amounts of disoriented myofilaments and other sarcoplasmic components. Disoriented myofibrils often encircled the remaining core of normal myofibrils, thus forming the striated annulets (Ringbinden). Several other abnormalities, not previously reported in myotonic dystrophy or any other myopathy, were identified in slightly dystrophic as well as in severaly atrophic fibers. These were large, homogenous lacunes derived from the sarcoplasmic reticulum and peculiar geometric arrangements of terminal cisternae. The origin of some other structures remains obscure. The relation of the dystrophic process to segmental degeneration and atrophy, the principal histologic findings in Steinert's disease, is unsettled since segmental necrosis was not observed in our samples for phase and electron microscopy.
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  • 3
    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.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Cell & tissue research 128 (1972), S. 393-405 
    ISSN: 1432-0878
    Keywords: Peripheral nerve ; Polyamines ; Autoradiography ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Description / Table of Contents: Zusammenfassung Mit Hilfe der Autoradiographie von Semi- und Ultradünnschnitten wurde die Verteilung der Radioaktivität nach Applikation von 3H-Putrescin im intakten und im degenerierenden N. ischiadicus der Ratte sowie in Spinalganglien untersucht. Im intakten und im geschädigten Nerven war die Radioaktivität, die zum weit überwiegenden Anteil als Spermidin und Putrescin vorkam, in allen zellulären Bestandteilen des Nerven, im Cytoplasma, in den Kernen und sehr deutlich auch in den Markscheiden, lokalisiert. Im extrazellulären Raum und über den Kollagenfibrillen war demgegenüber nur eine sehr geringe Radioaktivität festzustellen. Die physiologische Funktion von Spermidin und Putrescin im Myelin und den anderen Zellbestandteilen wird in erster Linie im Zusammenhang mit der in diesen Strukturen ebenfalls lokalisierten RNA diskutiert, da zahlreiche Hinweise für eine Rolle der Polyamine in der RNA- und Proteinsynthese vorliegen.
    Notes: Summary The distribution of radioactivity from 3H-putrescine was studied in intact and degenerated sciatic nerves, and spinal ganglia of rats by means of high resolution autoradiography. During the first three days after the administration of the labeled putrescine, the main proportion of radioactive material in the nerves was represented by spermidine and putrescine. Both, in intact and degenerating nerves, developed silver grains were deposited in all cellular components of the nervous tissue, the myelin sheath being markedly tagged. Perineural tissue was also labeled considerably, however, there was no significant amount of label in the extracellular space and in the collagen fibrils. The possible physiological significance of putrescine and spermidine in myelin and in other cellular components of nerves is discussed.
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