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  • 1
    Electronic Resource
    Electronic Resource
    Myopathology of myotonic dystrophy (1979)
    Springer
    Acta neuropathologica 45 (1979), S. 231-240 
    Details
    ISSN: 1432-0533
    Keywords: Myotonic dystrophy ; Myopathology ; Histometry ; Morphometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A histometric analysis of 16 biopsies taken from patients with myotonic dystrophy predominantly revealed type 1 atrophy, type 1 and 2 hypertrophy and type 1 predominance. These changes do not occur in all biopsies and are not pathognostic. Under the electron microscope otherwise apparently normal muscle fibres often demonstrated a single change: a swelling and proliferation of the sarcoreticulotubular system in the I-band region. Morphometry of randomly selected fibres from 15 biopsies revealed a significant increase in the sarcotubular membrane profile concentration in longitudinally and in transversally oriented sections. In addition, a shifting and disappearance of thin filaments in the I-band was noted to be an early sign. Pathological changes in the plasma membrane such as excessive foldings, expulsion of vesicular material and periodic densities were also seen. The morphometric analysis did not reveal any significant change in the mitochondrial fraction and mean mitochondrial size. All the ultrastructural changes of skeletal muscle decribed in myotonic dystrophy are unspecific. Even the alterations of the plasma membrane and the sarcoreticulotubular system described here may not be a primary correlate to myotonia, but represent secondary alterations or may belong to the dystrophic changes. Spontaneous human and experimental myotonia may exist without such alterations. Some biopsies demonstrated light and electron microscopic findings which may indicate a neurogenic process. However, it appears more probable that both the nerve and the muscle may be independently affected by the pleiotropic action of the responsible gene.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00702675
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  • 2
    Electronic Resource
    Electronic Resource
    Evidence for a chronic axonal atrophy in oculopharyngeal “muscular dystrophy” (1982)
    Springer
    Acta neuropathologica 57 (1982), S. 209-216 
    Details
    ISSN: 1432-0533
    Keywords: Oculopharyngeal muscular dystrophy ; Chronic axonal atrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report on morphometric investigations of peripheral nerves in a woman, who died at the age of 69, presenting the classical symptoms of oculopharyngeal muscular dystrophy (OPMD) and a typical family history with several members (males and females) affected over three generations. Evidence for chronic axonal atrophy was found in peripheral nerves and especially in oculomotor nerves with severe axon loss in endomysial nervetwigs of extra-ocular, laryngeal, and tongue muscles. Whereas limb muscles presented features of neurogenic atrophy, severe changes of “myopathic” type were evident in extrinsic eye muscles, laryngeal constrictor, tongue, and diaphragma. However, we interpreted these changes as neurogenic in origin in view of the severe denervation found in those muscles. Our findings suggest that OPMD is a disease of primary neurogenic origin rather than a primary myopathic disorder.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00685391
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  • 3
    Electronic Resource
    Electronic Resource
    Bioptische Befunde des T-Systems und tubulärer Strukturkomplexe bei neoplastischer Cortisonmyopathie (1970)
    Springer
    Acta neuropathologica 14 (1970), S. 338-344 
    Details
    ISSN: 1432-0533
    Keywords: T-System ; Tubular Network ; Muscle Regeneration ; Cushing-Myopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Ein autoptisch verifiziertes ACTH-aktives Lebercarcinom mit sekundärer Nebennierenrindenhypertrophie und Cushing-Myopathie zeigt in regenerierenden Fasern zahlreiche T-Systeme und tubuläre Strukturkomplexe. Mehrmals kann eine offene Kommunikation der transversalen Tubuli mit dem Extracellulärraum und andererseits auch mit den tubulären Strukturkomplexen nachgewiesen werden. Damit werden entsprechende tierexperimentelle und kulturelle Befunde am humanen Biopsiematerial bestätigt. Die cytoplasmatische Differenzierung der T-Systemstruktur scheint mit der der Myofilamente und des sarkoplasmatischen Reticulums einzusetzen.
    Notes: Summary An autoptic verified ACTH-activ liver-carcinom with secundary hypertrophy of the suprarenal glands and with Cushing-myopathy showed in the regenerating fibers many T-systems and tubular networks. Often a communication of the transversal tubuli with the extracellular space and also with the tubular networks could be demonstrated. By these human biopsy results the earlier animal experiments and culture findings were confirmed. The cytoplasmatic differentiation of the T-system structures appeared to start with the differentiation of the myofilaments and the sarcoplasmatic reticulum.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00685789
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  • 4
    Electronic Resource
    Electronic Resource
    Inhibition of carnitine palmitoyltransferase in normal human skeletal muscle and in muscle of patients with carnitine palmitoyltransferase deficiency by long- and short-chain acylcarnitine and acyl-coenzyme A (1993)
    Springer
    Journal of molecular medicine 71 (1993), S. 763-769 
    Details
    ISSN: 1432-1440
    Keywords: Carnitine palmitoyltransferase deficiency ; Metabolic myopathy ; Acetyl-coenzyme A ; Acetylcarnitine ; Palmitoyl-coenzyme A ; Palmitoylcarnitine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The inhibition of total carnitine palmitoyltransferase (CPT) by short- and long-chain acylcarnitine and acyl-coenzyme A (acyl-CoA) was studied in muscle homogenates of normal controls and of five new patients with CPT deficiency using the isotope forward assay. Acetylcarnitine inhibited neither normal CPT activity nor the CPT of patients. d,l-Palmitoylcarnitine almost completely inhibited CPT in patients but only 55% of normal activity. In controls the CPT fraction sensitive to inhibition by palmitoylcarnitine appeared to be identical with the fraction sensitive to inhibition by malonyl-CoA and succinyl-CoA, which probably represents CPT II. The abnormal inhibition of CPT by palmitoylcarnitine was more likely due to product inhibition than to a detergent effect. Acetyl-CoA concentrations up to 0.4 mM and palmitoyl-CoA above optimal substrate concentrations up to 0.3 mM both inhibited normal CPT by about 25%, whereas the CPT of patients was significantly more inhibited by both substances than was normal CPT. The inhibition by acetyl-CoA was probably due to the structural relationship with malonyl-CoA and succinyl-CoA. The abnormal inhibition of CPT in patients by palmitoyl-CoA was due either to an abnormal substrate inhibition or to a detergent effect on CPT II similar to that of Triton X-100. The data indicate that in CPT deficiency total CPT activity is normal under optimal assay conditions. CPT II, however, is abnormally inhibited by fatty acid metabolites that accumulate during fasting.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00190315
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  • 5
    Electronic Resource
    Electronic Resource
    Die Steroidmyopathie im Kindesalter (1974)
    Springer
    European journal of pediatrics 118 (1974), S. 177-196 
    Details
    ISSN: 1432-1076
    Keywords: Steroid myopathy ; Glucocorticoid therapy ; Side effects following
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung 13 Kinder im Alter von 21/2–14 Jahren, die während längerer Zeit Prednison in unterschiedlicher Dosierung erhielten, wurden klinisch, biochemisch und elektromyographisch auf das Vorliegen einer Myopathie hin untersucht. 6 von den 13 Patienten zeigten klinisch eindeutig Zeichen eines vorwiegend proximal an den unteren Extremitäten und am Rumpf lokalisierten Muskelbefalls. Bei 9 Kindern war die Ausscheidung des Kreatins im 24 Std-Urin erhöht, und bei 10 Fällen ergab die quantitative Auswertung des Elektromyogramms einen pathologischen Befund. Bei einem Patienten mit klinischen Symptomen wurde eine Muskelbiopsie durchgeführt. Diese zeigte in der elektronenoptischen Untersuchung deutliche, jedoch unspezifische myopathische Veränderungen, vor allem vermehrte Einlagerung von Glykogen in den intermyofibrillären Räumen, dilatierte Vesikel des sarkoplasmatischen Reticulums und Veränderungen an der Basalmembran von Capillaren. Nach einem steroidreien Intervall von 3–5 Monaten hatten sich bei allen 8 nachkontrollierten Kindern die klinischen und elektromyographischen Veränderungen zurückgebildet, und die Ausscheidung des Kreatins war bei 4 von 6 Patienten wieder im Normbereich. Die pathogenetischen Mechanismen bei der Steroidmyopathie und deren klinisch-pharmakologische Bedeutung im Kindesalter werden diskutiert.
    Notes: Abstract 13 children aged 21/2 to 14 years, who were treated for several months with different dosages of prednisone, were examined for steroid myopathy. Clinical symptoms of a proximal myopathy were present in 6 patients; hypercreatinuria was found in 9, and pathological EMG findings in 10 children, respectively. A muscle biopsy was performed on one clinically affected patient. Moderate and nonspecific electron microscopic alterations, including glycogen storage in the intermyofibrillar spaces, enlargement of vesicula in the sarcoplasmic reticulum, and alterations of capillary basal membranes were present. 8 children were re-examined after a steroid-free interval of 4–5 months. The clinical symptoms and the EMG alterations had normalized in all, and the hypercreatinuria had decreased in 4 out of 6 children. The pathogenetic mechanisms in steroid myopathy and its clinical and pharmacological significance in childhood are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00464609
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  • 6
    Electronic Resource
    Electronic Resource
    Neuropathy and Monoclonal IgM M-Protein with Antibody Activity Against Gangliosides (1988)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 540 (1988), 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.1749-6632.1988.tb27106.x
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  • 7
    Electronic Resource
    Electronic Resource
    Virus-ähnliche Einschlüsse bei chronischen neuro-muskulären Prozessen (1972)
    Springer
    European archives of psychiatry and clinical neuroscience 215 (1972), S. 148-166 
    Details
    ISSN: 1433-8491
    Keywords: Virus-Like Structures ; Neuromuscular Syndrome ; Myopathy ; Myositis ; Virus-ähnliche Strukturen ; Neuromuskuläres Syndrom ; Myopathie ; Myositis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 2 chronisch-progredienten neuromuskulären Erkrankungen, die nach elektromyographischen und bioptisch-histologischen Kriterien neben neurogenen auch myopathische und myositische Komponenten zeigen, wurden elektronenmikroskopisch virusähnliche Strukturen in Kernen und im Sarkoplasma der Muskelfasern gefunden. Sie weisen eine gewisse Ähnlichkeit auf mit denjenigen, die man bei Ortho- und Paramyxovirus-Infektionen findet. Analoge Beobachtungen anderer Autoren wurden bisher bei 4 Polymyositiden und einer chronischen Myopathie gemacht. Die serologischen Reaktionen mit verschiedenen Virusantigenen waren negativ oder ergaben nur niedrige Titer. Die Inokulation von Muskelhomogenat auf Zellkulturen ergab keinen cytopathischen Effekt, und elektronenmikroskopisch konnte in den Zellkulturen kein Virus nachgewiesen werden. Die vor 3 Monaten mit Muskelhomogenat inokulierten Mäuse zeigen bisher keine Krankheitssymptome. Das morphologische Verlaufsspektrum setzt ein mit filamentös tubulären Strukturen in den Kernen zunächst unauffälliger Muskelfasern. Mit ihrer zunehmenden Vermehrung löst sich die Kernstruktur auf, und es entleeren sich die virusverdächtigen Partikel ins Sarkoplasma, wo sie bei weiterer Vermehrung mit einer starken myofibrillären Destruktion und der Bildung zahlreicher Lamellenkörper verbunden sind. Aufgrund morphologischer Kriterien ist anzunehmen, daß die virusähnlichen Strukturen eine pathogenetische Bedeutung haben und es sich nicht um eine belanglose Kontamination handelt.
    Notes: Summary Two cases with a slowly progressive neuromuscular disorder of 9–10 years duration, involving distal and proximal as well as girdle and axial muscles, are described. The muscular atrophy was pronounced in the shanks and finger muscles, also swallowing was disturbed. The tendon reflexes were diminished. By electromyography as well as by muscle biopsy we found a neurogenic pattern and besides that pronounced myopathic and also myositic alterations. By electronmicroscopy there were found accumulations of filamentous tubules in the nuclei of the muscle fibers and in later stages, after the destruction of the nuclei, also in the sarcoplasma where they formed irregularly arranged groups. Also a great number of x-bodies and myeline bodies could be observed. Like Chou (1968) and some other authors, we presume an infection due to a virus. From the morphological viewpoint there seems to be a real pathogenic effect causing the myopathic and myositic alterations; a contamination is not likely. In contrast to the previously published cases of polymyositis and one chronic myopathy our two cases stem from a basic neurogenic alteration. The serological tests and inoculation of homogenized muscle in cell cultures (only case 2) gave no positive results. The animal inoculation tests are not yet completed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00342531
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  • 8
    Electronic Resource
    Electronic Resource
    Zur diagnostischen Spezifität von nemaline-Strukturen (1971)
    Springer
    Journal of neurology 200 (1971), S. 148-157 
    Details
    ISSN: 1432-1459
    Keywords: Nemaline-Structures ; Progressive Muscular Dystrophy ; Dermatomyositis ; Neuromyopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Nemaline-Strukturen bei einer progressiven Muskeldystrophie, einer Dermatomyositis und einer Neuromyopathie werden beschrieben. Es bestätigt sich die auf Grund von Einzelbeobachtungen geäußerte Auffassung, daß nemaline-Strukturen ein morphologisch unspezifisches Symptom darstellen. Nachdem sich auch das klinische Bild der nemaline-Myopathie mit zunehmenden Fall-beobachtungen bezüglich des Verlaufes, der Symptomatik und der Erstmanifestation als recht heterogen erwiesen hat, ist der Begriff nemaline-Myopathie als Krankheitseinheit unzureichen. Gegebenenfalls ist von gutartigen kongenitalen Myopathien mit nemaline-Strukturen zu sprechen.
    Notes: Summary Nemaline-structures seen in muscular dystrophy, dermatomyositis, and atypical neuromuscular disease with myxoviruslike inclusions are described. These observations confirm the interpretation that nemaline structures are an unspecific reaction arising in a heterogenic group of myopathies. Therefore, the appearance of nemaline structures doses not justify the assumption af a particular myopathic disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00316522
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  • 9
    Electronic Resource
    Electronic Resource
    Ataxia teleangiectatica; elektronenmikroskopische Biopsiebefunde des Nervus suralis von 2 Fällen (1972)
    Springer
    Journal of neurology 202 (1972), S. 128-138 
    Details
    ISSN: 1432-1459
    Keywords: Ataxia teleangiectasia ; Peripheral Nerve ; Sural Nerve Biopsy ; Lipid Metabolism ; Louis Bar-Syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Nach zahlreichen klinischen, elektromyographischen, bioptisch-lichtmikroskopischen und autoptischen Erfahrungen ist auch das periphere Nervensystem häufig am Krankheitsprozeß der Ataxia teleangiectatica (AT) beteiligt. Elektronenmikroskopisch zeigen Suralisbiopsien eines Brüderpaares mit AT eine Speicherung diverser Lipidartikel in Schwannschen Zellen bemarkter und unbemarkter Nervenzellen sowie leichte axonale Degenerationen. Diese Befunde bestätigen weitgehend die Ergebnisse, die Gardner u. Goodman (1969) bei der bisher einzigen derartigen Biopsie beschrieben. Gestützt auf die dargestellten morphologischen Beobachtungen wird eine enzymatische Störung des Lipidmetabolismus oder eine lysosomale Fermentanomalie als pathogenetischer Faktor der AT diskutiert.
    Notes: Summary Neumerous clinical, electromyographic and morphological findings are indicating that the peripheral nerve is often involved in ataxia teleangiectasia (AT). Electron micrographs of sural nerves of two brothers with AT reveal various lipid-containing particles within Schwann cells of myelinated fibers and, in addition, a slight axonal degeneration. These results confirm the findings of Gardner and Goodman (1969) in the only biopsy to be reported to date. The morphological alterations demonstrated raise the question whether an enzymatic error of lipid metabolism or a lysosomal enzyme anomaly represent a pathogenetic factor in AT.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00316162
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  • 10
    Electronic Resource
    Electronic Resource
    Autoradiographische Untersuchungen über die Regeneration am denervierten Rattenmuskel (1968)
    Springer
    Journal of neurology 194 (1968), S. 128-141 
    Details
    ISSN: 1432-1459
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Die Regeneration am denervierten und innervierten Rattenmuskel wird autoradiographisch und lichtmikroskopisch untersucht. Durch einen Vergleich der3H-Thymidin-Indices der Muskelzellkerne und der Myoblasten sowie des Auftretens und Wachstums von Muskelschläuchen und jungen Muskelfasern im innervierten und denervierten Muskel wird nachgewiesen, daß die Regeneration in der Zeitspanne von 12 Std bis 21 Tage nach der Muskelläsion durch eine Denervation nicht nachteilig beeinflußt wird. Außerdem stützen die Befunde die heute gültige Annahme, daß sich aus den Kernen geschädigter Muskelfasern nach einer cytoplasmatischen Dedifferenzierung und DNS-Synthese Myoblasten entwickeln. Die Bildung und das Wachstum von Muskelschläuchen vollzieht sich nicht durch mitotische oder amitotische Kernteilungen, sondern durch Fusion der Myoblasten.
    Notes: Summary The regeneration of innervated and denervated rat muscle was studied by autoradiography. The thymidine-3H index of nuclei and myoblasts of muscle cells as well as the appearance of sarcoplasmic tubules and new muscle cells were compared both in the innervated and denervated muscle. The results showed that denervation does not affect the regeneration during the time interval studied, i.e. from 12 hours 21 days after the muscle was damaged. Moreover the results support the view that nuclei of damaged muscle cells turn into myoblasts consequent to cytoplasmic dedifferentiation and DNA synthesis; sarcoplasmic tubules arise from myoblasts upon fusion. There is no mitotic or amitotic division of nuclei of the tubules.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00244221
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