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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 55 (1981), S. 281-287 
    ISSN: 1432-0533
    Keywords: AB-variant metachromatic leukodystrophy ; Activator protein deficiency ; Ultrastructure of sulfatide inclusions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The histopathological findings in a sural nerve biopsy of a new distinct variant of metachromatic leukodystrophy (MLD) are compared to those of classical MLD. The clinical and histological features are typical of a sulfatide lipidosis, yet in vitro activities of arylsulfatases A and B and cerebroside sulfatase are normal. Intact skin fibroblasts, when cultured in a medium supplemented with labelled sulfatide, show impaired in vivo sulfatide hydrolysis. A deficiency of the requisite activator protein is postulated.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 61 (1983), S. 61-64 
    ISSN: 1432-0533
    Keywords: Experimental allergic neuritis ; Lymphocytes ; Passive transfer ; Intraneural injection
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Passive transfer of experimental allergic neuritis (EAN) lymph node cells (LNC) by intraneural injection did not produce significant demyelination. EAN-LNC stimulated with myelin in vitro produced mild demyelination while those incubated with Concanavalin A had no effect. The lack of demyelination by unstimulated EAN-LNC is in contrast to the marked demyelination produced by intraneural injection of EAN serum. The mononuclear cell infiltration and demyelination of classical EAN seem to require both cellular and humoral immune responses.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Key words: Niemann-Pick type II – Niemann-Pick type C – Peripheral neuropathy – Nerve biopsy ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. The severe infantile form of Niemann-Pick disease type II was diagnosed in a 4-year-old girl and confirmed by demonstrating in cultured skin fibroblasts a deficiency of low-density lipoprotein-stimulated cholesterol ester synthesis of 〈5% of normal. Electrodiagnostic studies revealed changes of a predominantly demyelinating motor and sensory polyneuropathy. Light microscope and ultrastructural examination of a peroneal nerve biopsy showed unique changes. Compacted myelin sheaths were disproportionately thin with marked globular irregularities in single teased nerve fibres and evidence of chronic demyelination. The majority of axons were preserved but axonal spheroids and cytoskeletal abnormalities akin to neuroaxonal dystrophy were noted. Membrane-bound multilobulated lysosomal inclusions of floccular and electron-dense material were present in Schwann cells (SC), endoneurial fibroblasts, macrophages, pericytes and endothelial cells. SC of myelinated fibres were stuffed with whorls of concentric osmiophilic membranous profiles and electron-lucent material. The findings are diagnostic and differ from those of classical Niemann-Pick disease.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 54 (1981), S. 113-119 
    ISSN: 1432-0533
    Keywords: Multisystem atrophy ; Neuronal intranuclear ; Hyaline inclusions ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An 18-year-old girl died following a slowly progressive neurodegenerative disease of nine years duration. At 9 years of age, she developed intellectual deterioration associated with speach difficulty, pseudobulbar palsy and ataxia. The progression included spastic quadriplegia, anarthria, severe dysphagia, ophthalmoplegia, and pes cavus. There was no family history. The brain was uniformly small and the substantia nigra was not pigmented. Neuronal loss and gliosis involving globus pallidus, subthalamic nucleus, thalamic nuclei, brain stem, cerebellum, and spinal cord gave the picture of multisystem atrophy. Intranuclear hyaline inclusions were observed in numerous neurons of the central and peripheral nervous system. These were auto-fluorescent and were made up of intermingled straight filaments (8–9 nm in diameter). Only two previously reported cases showing these same inclusions are known. They are reviewed, compared, and discussed in relation to primary neuronal degenerations.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 49 (1980), S. 169-176 
    ISSN: 1432-0533
    Keywords: Experimental allergic neuritis ; Demvelination ; Humoral immunity ; Myelin ; Schwann cell
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Serum from rabbits with experimental allergic neuritis (FAN) when injected into rat sciatic nerves produced rapidly evolving demyelination followed by remyelination. Myelinating and non-myelinating Schwann cells as well as myelin itself were damaged by 15 min after injection. Myelin degradation was well advanced prior to involvement by macrophages at 12 h. The demyelinating factor was myelin-specific and complement-dependent. The evidence suggests that the FAN antigen may reside in Schwann cell membranes as well as in myelin.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 68 (1985), S. 101-109 
    ISSN: 1432-0533
    Keywords: Experimental allergic neuritis ; Blood-nerve barrier ; Evans blue-albumin ; Horseradish peroxidase ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The integrity of the blood-nerve barrier (BNB) was studied during the development of experimental allergic neuritis (EAN). Lewis rats immunized with bovine nerve or myelin plus complete Freund's adjuvant developed histological lesions of EAN in nerve roots by 10–12 days and in sciatic nerves by 12–14 days. Evans blue-albumin (EBA) and horseradish peroxidase (HRP) were injected i.v. 1 h prior to killing on days 6–18. Perivascular and diffuse endoneurial leakage of the tracers was seen in nerve roots by 10–12 days post immunization (p.i.) and in sciatic nerves by 12–14 days. This coincided with the appearance of endoneurial infiltration with inflammatory cells and endoneurial proteinaceous edema at a time when Schwann cell and myelin changes were still minimal. Therefore, an alteration in BNB permeability occurs early in EAN, coincident with inflammatory cell infiltration. This could be an expression of delayed hypersensitivity, yet it would also facilitate the entry of anti-myelin antibodies into the endoneurium where they could initiate demyelination.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Palo Alto, Calif. : Annual Reviews
    Annual Review of Ecology, Evolution, and Systematics 14 (1983), S. 1-33 
    ISSN: 0066-4162
    Source: Annual Reviews Electronic Back Volume Collection 1932-2001ff
    Topics: Biology
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Freshwater biology 14 (1984), S. 0 
    ISSN: 1365-2427
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Biology
    Notes: SUMMARY. 1. Spine development in a clone of unspined Keratella cochlearis was promoted by a water soluble factor released by the copepods Tropocyclops prasinus and Mesocyclops edax and by the predatory rotifer Asplanchna priodonta. Between 9 and 55% of K. cochlearis populations cultured in predator-conditioned media responded to the inducing chemical.2. The K. cochlearis form possessing a posterior spine of medium length was much less susceptible to predation by small Tropocylops and Asplanchna than the form lacking the posterior spine. These predators consistently selected the unspined form over the spined form when offered equal densities of each. However, both spined and unspined forms were equally susceptible to predation by large Mesocyclops.3. The phenotypic variation of offspring produced from induced unspined Keratella females encompassed much of the variation reported for the taxon in North America. These morphotypes are similar to the variation of forms reported for the K. cochlearis tecta series known from Europe. We suggest that the North American morphotypes be identified in studies of this species because the presence of the posterior spine can greatly affect predator selectivity.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Freshwater biology 48 (2003), S. 0 
    ISSN: 1365-2427
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Biology
    Notes: 1. A clonal culture of the peritrich Epistylis pygmaeum was used for all observations and experiments. Motile cells preferentially attached to the eggs of three species of Brachionus but also attached to the body of adult B. angularis. Zooids on the transitory egg substratum developed only short stalks, while those on the body often developed long stalks and branched colonies. Selection for the eggs positions the ciliate near the cloaca, and thus high concentrations of fine particulate material excreted by the host. Settlement on eggs occurred equally well in the light and dark, and on moving and stationary eggs.2. Motile Epistylis cells attached to a wide variety of rotifer and crustacean zooplankton, but exhibited some pronounced selectivity. They readily settled on the eggs of other rotifers (Epiphanes, Polyarthra), on the carapace of several cladocerans (Ceriodaphnia, Daphnia, Diaphanosoma), and on the egg sacs of a copepod (Tropocyclops). They settled less readily on the bodies of the rotifers Asplanchna and Synchaeta, and rarely or never settled on the rotifer Keratella, the cladocerans Bosmina and Scapholeberis, and the body of the copepod.3. Epistylis populations initiated with a single zooid on Brachionus increased exponentially and often contained several hundred attached zooids and motile cells after 3 days at 20 °C. Observations of a culture initiated from a single telotroch provided new information about peritrich life cycles: (1) motile cells reproduced themselves at a rapid rate (λ = 4.26 day−1); (2) telotrochs produced or transformed into swimming zooids and vice versa. Functions of the two types of motile cells remain to be clarified. Telotrochs likely are specialised for finding and attaching to hosts. Swimming zooids can feed and reproduce, producing both their own cell type and telotrochs. Together, they should enhance dispersal and population growth, especially when hosts are rare.4. Life-table experiments with two species of Brachionus showed that colonisation by Epistylis had no effect on adult survival but significantly decreased fecundity, by 29% in both cases. Zooids attached to eggs could be a weight burden, increase drag, and possibly inhibit egg development. Those on the body of B. angularis also could interfere with coronal cilia, inhibiting feeding and further slowing locomotion. The ability of E. pygmaeum to select and then interfere with its hosts indicates that this epibiont has the potential to influence the species structure of zooplankton communities.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0533
    Keywords: Niemann-Pick type II ; Niemann-Pick type C ; Peripheral neuropathy ; Nerve biopsy ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The severe infantile form of Niemann-Pick disease type II was diagnosed in a 4-year-old girl and confirmed by demonstrating in cultured skin fibroblasts a deficiency of low-density lipoprotein-stimulated cholesterol ester synthesis of 〈 5% of normal. Electrodiagnostic studies revealed changes of a predominantly demyelinating motor and sensory polyneuropathy. Light microscope and ultrastructural examination of a peroneal nerve biopsy showed unique changes. Compacted myelin sheaths were disproportionately thin with marked globular irregularities in single teased nerve fibres and evidence of chronic demyelination. The majority of axons were preserved but axonal spheroids and cytoskeletal abnormalities akin to neuroaxonal dystrophy were noted. Membrane-bound multilobulated lysosomal inclusions of floccular and electron-dense material were present in Schwann cells (SC), endoneurial fibroblasts, macrophages, pericytes and endothelial cells. SC of myelinated fibres were stuffed with whorls of concentric osmiophilic membranous profiles and electron-lucent material. The findings are diagnostic and differ from those of classical Niemann-Pick disease.
    Type of Medium: Electronic Resource
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