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  • 1
    Electronic Resource
    Electronic Resource
    CHEMICAL COMPOSITIONS OF BRAIN AND MYELIN IN TWO PATIENTS WITH MULTIPLE SULPHATASE DEFICIENCY (A VARIANT FORM OF METACHROMATIC LEUKODYSTROPHY) (1976)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 27 (1976), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The lipid composition of the brain, including myelin, was studied in detail in two cases with a variant form of metachromatic leukodystrophy (multiple sulphatase deficiency type).In the white matter, the sulphatide concentration was 3–4 times higher than the normal level in both cases. There was a significant accumulation of cholesterol sulphate in the brain, liver and kidney of both cases. The ganglioside pattern in the grey and white matter was abnormal, with a higher proportion of GM3, GM2 and GD3-gangliosides. Non-lipid hexosamine contents were increased 1.5-2 times in brain, 8–10 times in liver and 2–3 times in kidney. Increased amounts of glucocerobroside, ceramide lactoside and ceramide trihexoside were present in grey and white matter of both cases.Recovery of purified myelin from two patients’brains was much less than from control (1–20% in case 1 and 20–30% in case 2). The lipid composition of myelin was almost normal except for a higher proportion of sulphatide, with a decreased amount of cerebroside. The fatty acid compositions of myelin sulphatide and sphingomyelin were almost normal, while non-hydroxy fatty acids of cerebroside contained less long-chain fatty acids, as characterized by a significant increase of C16:0 and C18:0 fatty acids. The myelin polypeptide pattern by SDS-disc gel electrophoresis showed a relative decrease of basic protein and of proteolipid protein.A possible mechanism of myelin loss in MSD is discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1976.tb00310.x
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  • 2
    Electronic Resource
    Electronic Resource
    An Empirical Study of Jungian Typology (1978)
    Oxford, UK : Blackwell Publishing
    The @journal of analytical psychology 23 (1978), S. 0 
    Details
    ISSN: 1468-5922
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Psychology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1465-5922.1978.00226.x
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  • 3
    Electronic Resource
    Electronic Resource
    Transfer factor: A potential agent for immunotherapy of cancer (1977)
    Springer
    World journal of surgery 1 (1977), S. 617-621 
    Details
    ISSN: 1432-2323
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Résumé Le facteur de transfert (TF) est un extrait de leucocytes humains. Il a été démontré qu'il transfère à l'homme anergique une capacité de réaction cutanée spécifique et qu'il augmente la réponse des tests d'activité lymphocytaire in vitro. Le TF a été utilisé, à titre expérimental, pour l'immunothérapie anticancéreuse. Dans plusieurs cas, l'administration de TF a été suivie de régression de tumeurs, de stabilisation temporaire, et l'on a également observé qu'il était possible, sous TF, de réduire les autres besoins thérapeutiques. Le TF est donc peut-Être valable en tant que thérapeutique adjuvante de certains cancers. Cependant, la plupart des travaux publiés sont des études non contrÔlées, et il n'existe aucun accord quant aux critères de définition du donneur de TF. Il sera impossible de tirer des conclusions quant à la valeur des traitements par TF dans le cancer ou d'autres maladies, aussi longtemps que des études contrÔlées n'auront pas été réalisées et que les caractères des donneurs de TF ne seront pas définis.
    Notes: Abstract Transfer factor (TF) is an extract from human leukocytes which has been shown to transfer specific skin test reactivity to previously nonreactive human recipients, and to produce measurable increases in in Vitro tests of lymphocyte activity. The results of the experimental use of TF as an immunotherapeutic agent for cancer are reviewed. TF therapy has been associated with tumor regression, temporary stabilization or reduced need for other treatment modalities in several reported cancer patients, and may have value as an adjuvant therapy in certain malignancies. However, most of the reports involve uncontrolled studies, and no agreement exists about the definition of TF donors. Controlled studies and the use of well characterized TF donors are essential before any conclusions can be drawn about the value of TF in the therapy of cancer and other diseases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01556190
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  • 4
    Electronic Resource
    Electronic Resource
    Neuromyopathy during chronic amiodarone treatment (1979)
    Springer
    Journal of neurology 220 (1979), S. 231-239 
    Details
    ISSN: 1432-1459
    Keywords: Amiodarone side effects ; Neuropathy ; Myopathy ; Lipidosis, drug related
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es werden die Ergebnisse einer klinischen und elektrophysiologischen Untersuchung sowie die Nerven- und Muskelbiopsiebefunde eines Falles berichtet, der 7 Jahre lang mit dem Antiarrhythmikum Amiodarone behandelt wurde. Klinisch-neurologisch fand sich eine distale sensomotorische Neuropathie mit ausgeprägten distalen Muskelatrophien. Die neurophysiologische Untersuchung ergab normale motorische und sensorische Leitgeschwindigkeiten. Die sensorischen Nervenaktionspotentiale waren polyphasisch und zeigten eine niedrige Amplitude. Im M. tibialis anterior und im M. abductor pollicis brevis fanden sich bei der Nadelmyographie deutliche Denervierungszeichen. Die Untersuchung der N. suralis-Biopsie ergab einen kompletten Verlust der großkalibrigen und eine schwere Reduktion der kleinkalibrigen Markfasern. Auch die Anzahl der unbemarkten Axone war deutlich vermindert. Schwannzellen, Fibroblasten und degenerierte Axone enthielten zahlreiche polymorphe, lipidhaltige Einschlußkörper. Die Muskelbiopsie zeigte neurogene und myopathische Veränderungen. Auch Muskelfasern enthielten Lipidakkumulationen. Die physikalische und chemische Untersuchung des Nerven- und Muskelgewebes ergab eine 40- bis 100fache Vermehrung des Jodgehaltes. Die Befunde deuten auf eine Schädigung von Axon, Schwannzelle und Muskelfasern. Ein Zusammenhang zwischen Lipidspeicherung und Akkumulation des Medikamentes bzw. seiner Metaboliten im Nerven- und Muskelgewebe kann vermutet werden.
    Notes: Summary Clinical, electrophysiological, and the nerve and muscle biopsy findings from a case treated with amiodarone are reported. Marked distal motor and sensory impairment and distal muscular atrophy were observed clinically. The electrophysiological examination revealed normal motor and sensory conduction velocities in the median nerve; the sensory action potentials were polyphasic and reduced in amplitude. Electromyography revealed denervation potentials and severe loss of motor units in the M. extensor digitorum brevis and in the M. tibialis anterior. The light and electronmicroscopical study of a N. suralis biopsy displayed total loss of large myelinated fibers and an almost total reduction of small myelinated fibers. The number of unmyelinated axons was markedly reduced. Fibrocytes and degenerative axons polymorphous inclusion bodies were present in Schwann cells. The muscle biopsy revealed both neurogenic and myopathic changes. Lipid storage was also present in the muscle fibers. Physical and chemical analysis of the nerve and muscle biopsy revealed the content of iodine to be more than 40 times increased. The findings indicate damage of axons, schwann cells and muscle fibers. It is suggested that the lipid storage in nerve and muscle tissue might be related to the accumulation of the drug or its metabolites.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00314147
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  • 5
    Electronic Resource
    Electronic Resource
    Dynamic aspects of peripheral nerve changes in progressive neural muscular atrophy (1976)
    Springer
    Journal of neurology 211 (1976), S. 111-124 
    Details
    ISSN: 1432-1459
    Keywords: Peripheral nerve ; Peroneal muscular atrophy ; Nerve biopsy ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 2 Patienten mit progressiver neuraler Muskelatrophie wurden Nervenbiopsien jeweils in einem frühen und in einem fortgeschrittenerem Stadium der Erkrankung entnommen und verglichen. In beiden Fällen zeigten bereits die frühen Biopsien ein völliges Fehlen der großkalibrigen, dickbemarkten Axone. Ebenfalls als frühe Veränderung wurde eine Erweiterung des endoneuralen Interstitiums festgestellt. Eine geringe Anzahl der vorhandenen bemarkten und unbemarkten Axone in allen Biopsien wies degenerative Veränderungen auf. Die für die progressive neurale Muskelatrophie typische Zwiebelschalenbildung der Schwannschen Zellen — möglicherweise eine Reaktion auf wiederholte De-und Remyelinisierungsvorgänge um dystrophische Axone — trat erst in den späteren Biopsien deutlicher hervor. Hinsichtlich der formalen Genese der hypertrophischen Neuropathie bei neuraler Muskelatrophie sind nach diesen Beobachtungen axonale Dystrophie und interstitielle Veränderungen des Endoneuriums als primäre Entmarkung und Zwiebelschalenbildung als sekundäre Phänomene zu betrachten. Die Möglichkeit einer kausalen Beziehung zwischen axonaler Dystrophie und interstitiellen Veränderungen wird an Hand der vorliegenden Befunde und Literatur diskutiert.
    Notes: Summary Serial nerve biopsies were performed at an early, and at an advanced stage of the disease in 2 patients with progressive neural muscular atrophy. The early biopsy showed a complete loss of the large diameter and thickly myelinated fibres, as well as an expansion of the endoneurial interstitium in both cases. Myelinated and unmyelinated fibres exhibited axonal degeneration in all biopsies occasionally. “Onion bulb” formation, a typical feature of peripheral neuropathy in neural muscular atrophy, was found to be prominent only in the latter biopsies. As regards the formal pathogenesis of hypertrophic neuropathy in neural muscular atrophy, axonal dystrophy and interstitial changes of the endoneurium were regarded as primary phenomena, demyelination and “onion bulb” formation as secondary. A possible causal relation between axonal dystrophy and interstitial changes, observed in these cases, is discussed in the light of the present literature.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00313355
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  • 6
    Electronic Resource
    Electronic Resource
    Morphological observations in the nervous system of prenatal mucopolysaccharidosis II (M. Hunter) (1979)
    Springer
    Acta neuropathologica 48 (1979), S. 139-143 
    Details
    ISSN: 1432-0533
    Keywords: Mucopolysaccharidosis II ; Prenatal diagnosis ; Electron microscopy ; Lysosomal storage
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Light and electron microscopic findings in the nervous system of a 23-week-old fetus are reported, in which MPS II was diagnosed prenatally. The degrees of myelination and neuronal differentiation were similar as in a normal fetus of the same age. A storage of mucopolysaccharides in typical vacuolar inclusion bodies was present throughout the peripheral and central nervous system, mainly in cells of mesenchymal origin. “Zebra” bodies and granulo-membranous bodies, which are thought to represent secondary ganglioside accumulation were only found in the well developed neurons of the spinal cord and spinal ganglia, but not in the poorly developed neurons of the cerebellar and cerebral cortex. Mucopolysaccharide storage in endothelial cells of cerebral blood vessels precedes the appearance of lipid storage in cerebral neurons.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00691155
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