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  • Electron microscopy  (4)
  • Myopathy  (4)
  • 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.
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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.
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  • 4
    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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  • 5
    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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  • 6
    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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  • 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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