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  • Electron microscopy  (4)
  • Neuropathy  (4)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 78 (1989), S. 649-661 
    ISSN: 1432-0533
    Keywords: Pyloric stenosis ; Myenteric plexus ; Smooth muscle ; Neuropathy ; Myopathic changes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The myenteric plexus and intramuscular nerve bundles in the circular muscle layer of the pylorus from 37 children with infantile hypertrophic pyloric stenosis (IHPS) obtained at pyloromyotomy were studied by light and electron microscopy and compared to six control cases without clinical evidence of IHPS. In certain IHPS cases degenerative alterations of the axons predominated. The axonal changes consisted of (1) severely increased variability of diameters with evidence of degeneration and regeneration of some axons, (2) accumulation of electron-dense bodies, lysosomes and pleomorphic membranous cytoplasmic bodies, (3) increase in the number of maloriented neurofilaments, and (4) aggregation of glycogen granules. Degenerative changes or immaturity of perikarya of neurons and glial cells in the myenteric plexus were not a significant feature. While axonal changes predominated in some IHPS cases there were severe changes of smooth muscle cells in others suggesting that a primarily neurogenic type of IHPS can be distinguished from a predominantly myogenic type. Although the etiology of the axonal changes in IHPS is not clear, it is suggested that they play an important role in the pathogenesis of pyloric stenosis and hypertrophy.
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 80 (1990), S. 295-306 
    ISSN: 1432-0533
    Keywords: Pyloric stenosis ; Smooth muscle ; Myenteric plexus ; Visceral myopathy ; Neuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Smooth muscle cell biopsies obtained at pyloromyotomy from 37 children with infantile hypertrophic pyloric stenosis (IHPS) were studied by light and electron microscopy and compared with 6 autopsy control cases without any clinical evidence of this disorder. In cases with IHPS an apparently irregular increase in the number of smooth muscle cells by mitosis was accompanied by an increase of the endoplasmic reticulum, proliferation of mitochondria and regressive changes, such as shrinkage, swelling, necrosis and apoptosis of smooth muscle cells. Other alterations, seen in some but not all cases consisted of large numbers of unusual dense granules some of which were clearly associated with actin filaments and, therefore, regarded as derivatives of the normally occurring dense bodies. Furthermore, intermyofibrillar and subsarcolemmal glycogen accumulations, various nuclear abnormalities and pleomorphic membranous cytoplasmic or nuclear bodies occurred. While smooth muscle cell abnormalities predominated in some cases of IHPS, in others there were more severe axonal changes in the myenteric plexus. It is suggested, therefore, that a primarily myogenic type of IHPS can be distinguished from a predominantly neurogenic type.
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 79 (1989), S. 190-199 
    ISSN: 1432-0533
    Keywords: Amyloidosis ; Neuropathy ; Immuno-electron microscopy ; Immunoglobulin light chains ; Perpheral nerve
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Sural nerve specimens from ten patients with amyloidosis (hereditary, associated with lymphoproliferative disorders, or of unknown origin) and peripheral neuropathy were investigated by immunohistochemistry at the light and electron microscopic level. Peroxidase-antiperoxidase and immunogold techniques were applied to glutaraldehyde-fixed, osmicated and epoxy-embedded tissue. In five cases, four of which associated with lymphoproliferative disorders, amyloid deposits strongly and exclusively reacted with antibodies to kappa or lambda light chains, respectively. By electron microscopy, bundles of immunogold-labelled amyloid fibrils could be identified in coated and uncoated single membrane-bound vesicles of endoneurial macrophages. Schwann cells did not contain intracellular amyloid but their processes were entangled in amyloid fibrils and their basement membranes were sometimes fused with the fibrillar masses. It is concluded that immunoglobulin light chains in AL (amyloid of immunoglobulin light chain origin) amyloidosis precipitate, forming amyloid fibrils, in the presence of, and presumably with the assistence of, endoneurial cells. Inefficiency of phagocytosis appears to be one of the major causes for the deleterious effects of amyloid.
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  • 4
    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.
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  • 5
    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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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 65 (1985), S. 285-292 
    ISSN: 1432-0533
    Keywords: Thalidomide ; Neuropathy ; Conduction velocity ; Myelin sheath
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Morphological studies of experimental thalidomide neuropathy have thus far failed to show any significant structural changes. The present investigation was performed on sural nerves of female New Zealand white rabbits showing a reduction of sensory conduction velocity after oral treatment with thalidomide (100 mg/kg b.wt. per day) for a period of 33 weeks. Rabbits of the same strain and equal sex, weight, and number served as controls. Very few nerve fibers were undergoing Wallerian degeneration in both groups, experimental animals and controls. Morphometry, however, revealed a statistically significant reduction of the mean myelin thickness of sural nerve fibers in the thalidomide group of rabbits as compared to controls. The mean myelin thickness of the largest nerve fibers was also significantly smaller than in the control group. On the other hand, axonal diameters were not significantly altered. The association between the decrease of the sensory conduction velocity, the reduction of the myelin sheath thickness, and the chronic thalidomide application is discussed.
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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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  • 8
    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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