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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
    Acta neuropathologica 79 (1989), S. 310-316 
    ISSN: 1432-0533
    Keywords: Childhood dermatomyositis ; Cylindric confronting cisternae (CCC) ; Tubuloreticular structures (TRS) ; Circulating lymphocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Tubuloreticular structure (TRS) and cylindric confronting cisternae (CCC) have been observed in circulating lymphocytes and in the muscle of six children with dermatomyositis. The presence of TRS was seen in all cases investigated, the number of CCC increased in various cells with the severity of the disease. Extensive formation of TRS and CCC in childhood dermatomyositis probably reflects local or systemic α-interferon production and suggests that some viral factor is responsible for the disease.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 81 (1991), S. 572-577 
    ISSN: 1432-0533
    Keywords: Human ontogeny ; Cell death
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Naturally occurring muscle cell death in normal human fetal muscle was examined to determine the timing and structural differences with respect to muscle maturity. Two types of degenerative changes in developing muscle were found: cytoplasmic and nuclear. Degeneration of the primary and mature myotubes between 10 and 16 weeks of gestation entailed cytoplasmic condensation and disruption, swelling of mitochondria and dilatation of sarcoplasmic reticulum. In contrast, the formation of immature muscle fibres was associated with disintegration of satellite myofibres characterized by nuclear degenerative changes. These findings indicate that naturally occurring muscle cell death appears as a two-successive-stage phenomenon of cell necrosis. Initially, at the myotube stage, a number of muscle cells are eliminated. In the later stage a single cell is removed from the cluster which seemingly is responsible for final shape and size of the muscle fibre.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 64 (1984), S. 161-166 
    ISSN: 1432-0533
    Keywords: Leaking vessels ; Arthus-like reaction ; Myopathy ; Ultrastructural study
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Specific blood-tissue barrier alterations were observed in a 2-year-old boy with a myopathic lesion in his muscle. Close by, degenerative changes were present in muscle fibers, three types of vascular abnormalities, i.e. increased vascular permeability, neutrophil aggregation, and damage of endothelium with thrombi formation, suggest that a process of vasculitis took place in the muscle of this patient. On the basis of current information it is not entirely clear whether this Arthus-like reaction observed in the primarily affected muscle represents a widespread vascular bed defect or whether it is the consequence of an additional secondary symptom possibly dependent upon muscle necrosis.
    Type of Medium: Electronic Resource
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  • 5
    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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