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  • Neuropathy  (4)
  • Demyelination  (3)
  • 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.
    Type of Medium: Electronic Resource
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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.
    Type of Medium: Electronic Resource
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  • 3
    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.
    Type of Medium: Electronic Resource
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  • 4
    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.
    Type of Medium: Electronic Resource
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  • 5
    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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  • 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.
    Type of Medium: Electronic Resource
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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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