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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 155 (1968), S. 0 
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 155 (1969), S. 0 
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Notes: Histochemical and electron microscopic studies on the nature of the azurophilic hypergranulations of leukocytes in Batten's disease are reported. The exact nature of the phenomenon could not be elucidated, but it appears that the abnormal granulation is due to the prevalence of an intermediate granule in the polymorphonuclear cells. In electron microscopic preparations, the leukocyte granules from patients with Batten's disease are bounded by a unilaminar membrane, in contrast to the trilaminar membrane of the granules in controls. The possibility that this finding may represent a molecular lesion is discussed.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    [s.l.] : Nature Publishing Group
    Nature 221 (1969), S. 974-974 
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] A biopsy of a brain from a clinically, serologically and pathologically documented child with SSPE was minced, trypsinized and cultured according to standard procedures4. Dispersed cells were washed three times with Hanks balanced salt solution and then resuspended in Earle's minimum essential ...
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    [s.l.] : Nature Publishing Group
    Nature 183 (1959), S. 1337-1338 
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] Since protein in the denatured state has more ionized tyrosine hydroxyl and cysteine sulphydryl groups available than in the native state, iodination, by lowering the pK of the protein, will further increase its affinity for basic dyes4. Based on these considerations, the following technique has ...
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 177 (1958), S. 451-457 
    ISSN: 1432-1459
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Zusammenfassung Es wird über Untersuchungen zur Verhütung der experimentellen allergischen Encephalomyelitis auf immunologischem Wege berichtet Die Analyse der an Mäusen und Meerschweinchen erzielten Ergebnisse deutet darauf hin, daß mit der gewählten Technik — Schutz durch infantile Injektionen von homologem Hirnbrei in Adjuvant — wahrscheinlich keine immunologische Toleranz erzielt wurde. Vielmehr deuten klinische und pathologische Befunde darauf hin, daß die gefundene Schutzwirkung auf einem endokrinologischen Phänomen, nämlich einer Aktivierung der Nebennierenfunktionen, beruht.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 212 (1976), S. 107-115 
    ISSN: 1432-1459
    Keywords: Radiation injury ; Radiation necrosis delayed ; Radiation myelopathy ; Radiation therapy ; Radiation idiosyncrasy ; Myelopathy, radiation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Die Einführung des Konzeptes der „Nominalen Standarddosis“ und dessen Präzisierung durch Zeit-, Dosis- und Fraktionierungsfaktoren ermöglicht scheinbar eine eindeutige Festlegung von Toleranzdosen für gesundes Gewebe in der Strahlentherapie. In dieser Studie wird gezeigt, daß die allgemein akzeptierten Schwellendosen keine absolute Gültigkeit haben, sondern bestenfalls als Richtlinien gelten können. Die Strahlentoleranz ist nämlich nicht nur durch die Effektivdosis bestimmt, sondern auch durch eine Reihe von individuellen Faktoren. Diese Faktoren müssen vom Therapeuten in den Bestrahlungsplan miteinkalkuliert werden. Von besonderer Wichtigkeit sind die relativen Dimensionen des bestrahlten Gewebsvolumens, etwaige krankhafte Veränderungen im bestrahlten Gewebe und die Entwicklung pathologischer Vorgänge nach Beendigung der Bestrahlung. Selbst wenn diesen und anderen modifizierenden Faktoren Rechnung getragen, die Toleranzdosis entsprechend eingestellt und nicht überschritten wird, lassen sich Strahlenschäden des Nervensystems nicht immer vermeiden. Aus diesem Grunde ist der Strahlentherapeut verpflichtet, nach Möglichkeiten zu suchen, die die Belastung des normalen Nervengewebes so niedrig wie möglich halten. Moderne Beschleuniger machen dieses Ziel erreichbar.
    Notes: Summary The introduction of the concept of Nominal Standard Dose and of Time, Dose Fractionation Factors ostensibly permits definition of tolerance doses for normal tissues in unequivocal terms. However, even with these refinements, tolerance doses remain, at best, guidelines, because radiobiologic effectiveness is governed not only by the effective dose, but also by individual factors, which will modify the response. Attention must be accorded to these biologic parameters, in order to prevent injury to healthy tissues. Of particular significance are the relative size or volume of the irradiated tissue, the possible presence of co-existing pathology in the exposed organ and the development of disease after the termination of the treatment. Even if these factors are properly respected, the risk of radiation injury cannot entirely be eliminated. The radiotherapist is therefore obligated to use an approach which minimizes the exposure of the healthy nervous tissue, a goal which has become attainable with the advent of modern accelerators as radiation sources.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 5 (1965), S. 26-39 
    ISSN: 1432-0533
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Die Krankengeschichten von zwei Brüdern, die wahrscheinlich an “Dégénérescence systématisée optico-cochléo-dentelée” (Nyssen-van Bogaert) litten, wurden mitgeteilt und die am Probanden, dem älteren Bruder, erhobenen Sektionsbefunde beschrieben. Zusammen mit den beiden neuen Beobachtungen sind nunmehr neun Fälle dieser Krankheit bekannt, die sowohl klinisch als auch pathologisch-anatomisch miteinander verglichen werden. Als gemeinsamer Befund liegt den fünf autoptisch gesicherten Beobachtungen eine Degeneration der Opticus-, Cochlearis-, Dentatum-und medialen Schleifensysteme zugrunde. Dagegen bleiben die basalen Ganglien und das Zwischenhirn vom Prozeß weitgehend verschont. Eine erhebliche Nervenzellichtung der Großhirnrinde scheint vor allem bei den im Säuglingsalter beginnenden Fällen vorzukommen. Die in unserem Fall auffallend schwere Kleinhirnrindenschädigung war auf Grund ihres Verteilungsmusters als Krampfschaden zu deuten.
    Notes: Summary Clinical observations of two brothers and pathoanatomical alterations on the older brother were found to be very similar to those in cases reported in the literature as “dégénérescence systématisée optico-cochléo-dentelée”. Including these two observations, a total of 9 patients believed to have been affected by this condition are now known. Clinical and pathological features are critically compared and their implications discussed. While some definite differences, possibly determined by the age of onset of the disease, exist, the common denominator is seen in a selective, systematized degeneration of the optic, the cochlear, the dentate and the medial lemniscal systems, a process which correlates well with the evolution of the clinical picture. The basal ganglia and most of the diencephalon remain unaffected. Degeneration of the pallium was noted in some instances, the degree of which relates well with the motorsensory retrogression. Cerebellocortical damage was severe only in our observation and is considered to be ictogenic.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 3 (1963), S. 144-149 
    ISSN: 1432-0533
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Elektronenmikroskopische Studien am Gehirngewebe von vier Patienten mit juveniler amaurotischer Idiotie (Batten-Spielmeyer-Vogtsche Krankheit) ergaben, daß die akkumulierten Lipoide in der Form von cytoplasmischen Organellen vorhanden sind. Diese Lipoidkörper sind pleomorph, von überaus wechselnder Größe, und zeigen zwei Typen von verschiedener interner Strukturierung. Einmal handelt es sich um ein dichtes, granuläres Stroma; in anderen Lipoidkörpern finden sich runde, ovale und tubuläre Membranen, die dem Ganzen ein multilokuläres Aussehen verleihen. Auf Grund gewisser morphologischer Ähnlichkeiten wird die Entstehung der Lipoidkörperchen aus degenerierten Mitochondrien erwogen. Daß ihre Feinstruktur nicht altersbedingt ist, geht daraus hervor, daß die Lipoidkörperchen von einer 9 Jahre alten Patientin mit denen von einer 23 Monate alten Patientin identisch waren. Die Lipoidkörper der juvenilen amaurotischen Idiotie sind grundverschieden von denen der infantilen amaurotischen Idiotie, den sogenannten „membranous cytoplasmic bodies” (Terry andKorey).
    Notes: Summary Electronmicroscopic examination of brain tissue from patients with juvenile amaurotic idiocy (Batten-Spielmeyer-Vogt disease) revealed a highly characteristic fine architecture of the lipid bodies accumulated in the perikarya. These pleomorphic bodies present two different types of internal structure, either a densely granular stroma, or round, oval and tubular membranes giving the cross section a multilocular appearance. There is some suggestion that the bodies might derive from degenerating mitochondria. In all four patients, regardless of age, the lipid bodies were similar in structure but they were distinctly different from the membranous cytoplasmic bodies of infantile amaurotic idiocy (Tay-Sachs disease) as described byTerry andKorey.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 4 (1964), S. 28-45 
    ISSN: 1432-0533
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 30 (1974), S. 155-173 
    ISSN: 1432-0533
    Keywords: GM1-Gangliosidosis ; Glycoproteins ; β-Galactosidase ; Lysosomal Disease ; Electron Microscopy ; Genetics of GM1-Gangliosidosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Post-mortem studies on a 6-year old boy with GM1-gangliosidosis, Type II revealed no evidence of accumulation of residual bodies nor of gangliosides or glycoproteins in liver and spleen. In brain tissue the ganglioside GM1 accounted for 70% of the ganglioside fraction and ganglioside-NANA was increased 3.6 fold over controls. In addition, the brain tissue contained large amounts of glycoprotein, glycoprotein derived galactose being increased 2.5 times. The neuronal accumulation of tertiary lysosomes exhibited a characteristic distributional pattern: in general the large neuronal perikarya were more consistently involved with the exception of the motor cells of the cranial nerve nuclei, III, IV, and VI. In addition to characteristic MCB's, the nerve cells contained residual bodies with a granulo-floccular matrix, presumed to represent glycoproteins. The distribution of the mutant gene was studied among 30 blood relatives of the proband at risk and 6 carriers could be ascertained on the basis of a reduced leukocytic β-galactosidase activity. The partly purified enzyme from the patient's liver revealed 20% activity as compared to that of normal controls. All three fractions obtained by DEAE cellulose column chromatography exhibited markedly reduced activity at pH 3.6, but nearly normal activity at pH 6.6. The reduced activity corresponded to the B component of the enzyme as shown by electrophoretic separation. It is pointed out that this case cannot be diagnosed as “generalized gangliosidosis” for the process of ganglioside accumulation was restricted to nervous tissue.
    Type of Medium: Electronic Resource
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