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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 12 (1969), S. 329-338 
    ISSN: 1432-0533
    Keywords: Hypokalemic Periodic Paralysis ; Electron Microscopy ; Myopathy ; Sarcoplasmic Reticulum ; Glycogen, Muscular
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Licht- und elektronenoptische Untersuchungen wurden an Muskelbiopsien von 4 Fällen hypokaliämischer periodischer Paralyse durchgeführt. Die frühesten Veränderungen betreffen nach Meinung der Verfasser das sarkoplasmatische Reticulum: Vacuolisierung des Längssystems und vermutlich Multiplikation des Quersystems. Es wird vermutet, daß die Vacuolenbildung die Folge einer Fusion der Vesikeln des Quersystems darstellt. Destruktion von Myofibrillen und Vergrößerung der, interfibrillären Räume treten erst in späteren Phasen des Krankheitsprozesses auf. Ausdehnung und Intensität dieser Veränderungen bedingen das typische histologische Bild myogener Läsionen bei Fällen m klinisch fixierten Symptomen der Myopathie.
    Notes: Summary Light and electron-microscopic studies were performed on the muscles taken from 4 cases with hypokalemic periodic paralysis. The authors conclude that the earliest changes concern the sarcoplasmic reticulum: vacuolisation of the longitudinal system and probably, multiplication of the transverse system. It is suggested, that vacuoles are formed as a result of fusion of the vesicles of the transverse system. Destruction of myofibrilles and enlargement of the interfibrillar spaces appear at a later period of the disease process. Extensive and intensive changes of that kind present a typical appearance of myogenic changes in histological study observed in the cases with clinically fixed symptomes of myopathy.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 226 (1981), S. 205-212 
    ISSN: 1432-1459
    Keywords: Ceroid lipofuscinosis ; Cytoplasmin inclusions ; Autosomal dominant
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The study of the ultrastructure of the sural nerve and peripheral blood lymphocytes of a boy with late-infantile neuronal ceroid-lipofuscinosis revealed the presence of ‘curvilinear bodies’ and ‘fingerprint profiles’. The elder sister of the patient died at the age of 7 years after progressive mental and motor deterioration. The same kind of cytoplasmic inclusions was found in the lymphocytes of the father of these children, who had had epilepsy since the age of 32. Clinical data and the results of the ultrastructural study suggest that in the same family two different forms of ceroid-lipofuscinosis appear and that the disease is inherited as an autosomal dominant trait. This family seems to suggest the nosological unity of clinically different forms of ceroid-lipofuscinosis.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 220 (1979), S. 169-184 
    ISSN: 1432-1459
    Keywords: Guillain-Barré syndrome ; Polyradiculoneuropathy ; Acute idiopathic polyneuropathy ; Conduction velocity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wurden 50 Fälle von Guillain-Barré-Strohl-Syndrom sowohl klinisch wie elektrophysiologisch untersucht, 20 davon in der akuten Phase und 30 viele Jahre später im Rahmen einer katamnestischen Untersuchung. In 6 Fällen wurde eine Suralisbiopsie ausgeführt. Es wurde keine offensichtliche Korrelation zwischen klinischer Symptomatologie und der Verlangsamung der motorischen oder sensiblen Erregungsleitung im peripheren Nerven nachgewiesen. Die Erregungsleitung wurde nach Beginn der klinischen Besserung verlangsamt. Die elektrophysiologischen Normabweichungen betrafen sowohl die sensiblen wie auch die motorischen Fasern, trotz dem häufigen Fehlen von klinisch faßbaren sensiblen Ausfällen. Die sogenannten langen Nerven waren früher und in ausgeprägterem Maße befallen als die kurzen. Sowohl die Erregungsleitung wie die distale Latenz waren in gleicher Weise betroffen. Leichte elektrophysiologische Normabweichungen konnten sogar viele Jahre nach der akuten Phase und bei völliger Symptomfreiheit der Patienten noch nachgewiesen werden. Gewisse Beziehungen bestehen zwischen den Veränderungen der Erregungsleitungsgeschwindigkeit und den histologischen Befunden.
    Notes: Summary Fifty cases of the Guillain-Barré-Strohl syndrome were investigated clinically and electrophysiologically—20 in the acute phase, and 30, as a matter of followup, many years after. The sural nerve was biopsied in six cases. There was no evident correlation between clinical symptoms and slowing of motor and sensory conduction. Nerve conduction velocity became slower after the beginning of clinical improvement. The electrophysiological abnormalities concerned both sensory and motor fibers despite the frequent absence of clinical sensory manifestations. The so-called long nerves were involved earlier and more markedly than the so-called short nerves. Conduction velocity and distal latency were equally affected. A slight electrophysiological defect was noticeable even many years after the acute phase of the syndrome, in completely symptoms free patients. Some correlation existed between conduction velocity changes and histological findings.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of epidemiology 7 (1991), S. 501-504 
    ISSN: 1573-7284
    Keywords: Creutzfeldt-JaKob disease ; Young people ; Poland
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three neuropathologically confirmed cases of Creutzfeldt-Jakob disease in young people (19, 23, and 27 years of age) are described. None had received pituitary hormone therapy. At the onset of illness all patients were suspected of having SSPE or other viral encephalitis, because of the similarity of clinical symptomatology and the shift towards older age of SSPE onsets observed in Poland in recent years.
    Type of Medium: Electronic Resource
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