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  • 1
    Electronic Resource
    Electronic Resource
    THYMIC MYOGENESIS, T-LYMPHOCYTES AND THE PATHOGENESIS OF MYASTHENIA GRAVIS (1981)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 377 (1981), S. 0 
    Details
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1749-6632.1981.tb33753.x
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  • 2
    Electronic Resource
    Electronic Resource
    Inclusion body myositis. A “slow-virus” infection of skeletal musculature? (1977)
    Springer
    Journal of molecular medicine 55 (1977), S. 1063-1066 
    Details
    ISSN: 1432-1440
    Keywords: Einschlußkörper-Myositis ; Muskelatrophie ; Myopathie ; Nucleocapsid ; Skelettmuskel ; Paramyxoviren ; Inclusion body myositis ; Paramyxoviruses ; Nucleocapside ; Skeletal Musculature
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We report the case of a 56-years old patient with clinical symptoms of an unresolved neuromuscular disease. Thelight microscopic studies of a muscle biopsy from the m. triceps shows the picture of a diffuse muscular atrophy. Byelectron microscopy, myelin-like degeneration zones with tubular-filamentous inclusions can be shown in the cytoplasma of the atrophic muscle cells. These filamentous structures correspond morphologically to the nucleocapside ofparamyxoviruses. These results lead, even without the proof of inflammatory cells, to the diagnosis of an “inclusion body” myositis also taking into account the clinical and electrophysiological findings.
    Notes: Zusammenfassung Es wird über einen 56jährigen Patienten mit den klinischen Symptomen einer unklaren neuromuskulären Erkrankung berichtet. Dielichtmikroskopische Untersuchung einer Muskelbiopsie aus dem m. triceps ergibt das Bild einer diffusen Muskelzellatrophie.Elektronenmikroskopisch werden im Cytoplasma der atropischen Muskelzellen myelinartige Degenerationsherde mit tubulär-filamentösen Einschlüssen nachgewiesen. Diese Filamentstrukturen entsprechen morphologisch dem Nucleocapsid von Paramyxoviren. Dieser Befund führt auch ohne den Nachweis interstitieller Entzündungszellen gemeinsam mit den klinischen und elektrophysiologischen Ergebnissen zur Diagnose einer „inclusion body myositis“.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01489481
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  • 3
    Electronic Resource
    Electronic Resource
    3.1-kb deletion of mitochondrial DNA in a patient with Kearns-Sayre syndrome (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 126-129 
    Details
    ISSN: 1432-0533
    Keywords: Chronic progressive external ophthalmoplegia ; Kearns-Sayre syndrome ; Mitochondrial DNA deletion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Mitochondrial DNA (mtDNA) deletions have been found in the majority of patients with chronic progressive external ophthalmoplegia and Kearns-Sayre syndrome. A large number of different mtDNA deletions have been identified. They generally spare the two origins of replication and are frequently flanked by direct or indirect repeats. We have found a 3.1-kb deletion of mtDNA in a patient with Kearns-Sayre syndrome that has some unusual features. First, it encompasses nucleotides 11259 to 14368, a localization that was not described before. Second, the deletion is not flanked by direct or indirect repeats, supporting the view that homologous recombination and slip-replication do not account for all mtDNA deletions.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294310
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  • 4
    Electronic Resource
    Electronic Resource
    Striated muscle fibres differentiate in monolayer cultures of adult thymus reticulum (1975)
    [s.l.] : Nature Publishing Group
    Nature 256 (1975), S. 493-494 
    Details
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] We have described a tissue culture method that allows growth in vitro of thymus reticulum monolayers derived from adult normal rats or mice7. These cultures at early stages consist of two main cell types, reticular and epithelial. In this communication we report the clonal differentiation of a ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/256493a0
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  • 5
    Electronic Resource
    Electronic Resource
    3.1-kb deletion of mitochondrial DNA in a patient with Kearns-Sayre syndrome (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 126-129 
    Details
    ISSN: 1432-0533
    Keywords: Key words Chronic progressive external ; ophthalmoplegia ; Kearns-Sayre syndrome ; Mitochondrial DNA deletion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Mitochondrial DNA (mtDNA) deletions have been found in the majority of patients with chronic progressive external ophthalmoplegia and Kearns-Sayre syndrome. A large number of different mtDNA deletions have been identified. They generally spare the two origins of replication and are frequently flanked by direct or indirect repeats. We have found a 3.1-kb deletion of mtDNA in a patient with Kearns-Sayre syndrome that has some unusual features. First, it encompasses nucleotides 11259 to 14368, a localization that was not described before. Second, the deletion is not flanked by direct or indirect repeats, supporting the view that homologous recombination and slip-replication do not account for all mtDNA deletions.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294310
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  • 6
    Electronic Resource
    Electronic Resource
    Beitrag zur Differenzierung von Nerven- und Gliazellen mit Hilfe der Silberimprägnation (1967)
    Springer
    Acta neuropathologica 9 (1967), S. 185-197 
    Details
    ISSN: 1432-0533
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Brain tissue was investigated with the electron microscope. The tissue was stained with silver or lead hydroxide. The capillaries of the neuropil have base membranes which consist of a layer only, the lamina densa. The neurofibriles of the axons can be stained readily by silver. In the nerve cells microtubules can be traced. They differ from the neurofibrils. Surrounded by endoplasmic reticulum, reticular inclusions can be found. Because of the impregnation with silver one can easily differentiate between nerve cells, astrocytes and oligodendrocytes.
    Notes: Zusammenfassung Das Gewebe der Großhirnrinde wurde elektronenmikroskopisch nach Silber-imprägnation (Marx u.Mölbert, 1965) und nach Bleihydroxydkontrastierung untersucht. Im Bereich des Neuropils konnten die Basalmembranen der Capillaren lediglich aus der Lamina densa bestehend beobachtet werden. Die Neurofibrillen der Axone waren stark versilberbar. In den Nervenzellen konnten erstmals Mikrotubuli, die sich von den Neurofibrillen durch ihre verschiedene Kontrastierbarkeit unterscheiden gefunden werden. Ferner konnten “reticular inclusions” im Bereich des endoplasmatischen Reticulums dargestellt werden. Die Unterscheidbarkeit von Nervenzellen einerseits und Astrocyten und Oligodendrocyten andererseits mit Hilfe der Silberimprägnation wird aufgezeigt.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00691443
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  • 7
    Electronic Resource
    Electronic Resource
    Ergebnisse klinischer, biochemischer, lichtmikroskopischer und ultrastruktureller Untersuchungen einer kindlichen Muskelglykogenose bei zwei Brüdern und deren schwester (1973)
    Springer
    European journal of pediatrics 116 (1973), S. 23-41 
    Details
    ISSN: 1432-1076
    Keywords: Infantile muscular glycogenosis type II ; “Floppy infant” ; Acid maltase (α-1,4-glucosidase) deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über eine “abortive muskuläre Form” der Typ II-Glykogenose (Pompe) bei drei Geschwistern (zwei Buben und deren Schwester) berichtet. Die klinischen und routinehistologischen Befunde entsprechen dem Bild einer primär degenerativen Myopathie. Erst spezielle lichtmikroskopische (PAS-Reaktion), elektronenmikroskopische und biochemische Untersuchungen führten zur Sicherung der Diagnose. Im elektronenmikroskopischen Bild weisen die betroffenen Muskelzellen aller drei Geschwister große, autophage Vacuolen auf, die neben aggregierten Glykogengranula häufig myelinartige Abbauprodukte cytoplasmatischer Membranen enthalten. Daneben werden unspezifische Veränderungen an den übrigen Zellorganellen und den Myofibrillen beobachtet. Die biochemische Untersuchung einer Muskelbiopsie ergibt den Nachweis eines Mangels an α-1,4-Glucosidase.
    Notes: Summary This report describes a mild muscular form of type II glycogenosis (Pompe) in 3 children (two boys and their sister). The clinical picture and results of routine histological tests were in keeping with the picture of a primary degenerative myopathy. It was not possible to confirm the diagnosis until special preparations were examined by light microscopy (PAS stain) and by electromicroscopical and biochemical techniques. Electron-microscopical examination of muscle cells from the affected areashowed large, autophagic vacuoles in all three sibs, which contained aggregated glycogen granules and myelin-like degradation products of cytoplasmic membranes. Nonspecific changes of the other cell organelles and the myofibrils were also observed. Biochemical examination of a muscle biopsy revealed an α-1,4-glucosidase deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00438826
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  • 8
    Electronic Resource
    Electronic Resource
    Foamy myocardial transformation in a child with a disturbed respiratory chain (1987)
    Springer
    European journal of pediatrics 146 (1987), S. 582-586 
    Details
    ISSN: 1432-1076
    Keywords: Foamy myocardial transformation ; Lactic acidosis ; Respiratory chain defect
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A female infant presented with signs of general muscle hypotonia and increasing hypertrophy of the heart muscle. There was a constant lactic acidosis worsened after glucose intake. Serum lactate levels were increased up to 6.7 mmol/l. Serum alanine levels reached maximum concentrations of 2800 μmol/l. The patient died after sudden cardiac arrest at 22 months of age. Histology of the heart showed the pattern of foamy myocardial transformation with accumulation of mitochondria and an increased glycogen content. Biochemical analysis of the skeletal muscle and the heart demonstrated signs of a disturbed respiratory chain. The content of cytochromeaa3 protein was decreased in skeletal muscle and heart mitochondria. It is suggested that respiratory chain defects may be the cause of foamy myocardial transformation of the heart.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02467358
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  • 9
    Electronic Resource
    Electronic Resource
    A double-blind placebo controlled trial of diltiazem in duchenne dystrophy (1988)
    Springer
    Journal of molecular medicine 66 (1988), S. 565-570 
    Details
    ISSN: 1432-1440
    Keywords: Diltiazem ; Duchenne muscular dystrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The role of calcium accumulation in the pathogenesis of Duchenne muscular dystrophy (DMD) has already been discussed. Several trials with different calcium-blocking drugs have revealed no clinical benefit. In addition, the present study includes histological investigations and computer tomography to verify therapeutic effects. In a randomized placebo-controlled double-blind study, 13 DMD patients aged from 3–10 years (mean, 7 years) were treated with 5 mg/kg diltiazem daily for 1 year. Compared with before therapy, the number of calcium-positive muscular fibres was remarkably reduced in the treated DMD patients, but not in the placebo group. The evaluation of all other biochemical and clinical parameters revealed no significant effects of the diltiazem therapy. The muscularX-ray density measured by computer tomography decreased under treatment. After the evaluation of the double-blind study, the code was broken. Therapy, however, was continued in the treated group and started in the placebo group. After 3 years of diltiazem therapy the clinical status of all 26 patients of the study and 20 additional DMD patients who were treated with diltiazem was compared with 46 untreated DMD patients of the same age and stage in our department. No obvious clinical benefit of diltiazem therapy could be observed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01720830
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  • 10
    Electronic Resource
    Electronic Resource
    Passagere Hemiparese und Raynaud-Symptomatik bei eosinophiler Fasziitis (1997)
    Springer
    Der Internist 38 (1997), S. 688-691 
    Details
    ISSN: 1432-1289
    Keywords: Schlüsselwörter Eosinophile Fasziitis ; Raynaud-Symptomatik ; Hemiparese
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Zusammenfassung Es wird über einen 56jährigen Patienten berichtet, der im Dezember 1994 wegen Ödemen der Extremitäten mit anschließender Ausbildung einer verdickten, indurierten und erythematösen Haut, Myalgien, einem starken Gewichtsverlust und Krankheitsgefühl stationär aufgenommen wurde. Im Verlauf der Behandlung kam es zu einer vorübergehenden Hemiparese und Raynaud-Symptomatik, die am ehesten auf eine begleitende Vaskulitis zurückzuführen waren.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001080050081
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