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  • 11
    ISSN: 1432-0533
    Keywords: Acute porphyria ; Porphyric neuropathy ; Axonal degeneration ; Uroporhyrinogen I synthetase ; Ferrochelatase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsy case of a 37-year-old woman with acute porphyria is reported. The patient began to complain of severe menstrual pains, and later developed serious peripheral neuropathy and various autonomic nervous symptoms. The autopsy revealed a marked loss and degeneration of axons and myelin sheaths in the peripheral nervous system (PNS), and prominent central chromatolysis of the spinal anterior horn cells. The predominant process of the peripheral neuropathy appeared to be axonal degeneration. Biochemical analysis showed a marked increase of delta-aminolevulinic acid (ALA), porphobilinogen, uroporphyrin, and coproporphyrin in the urine, and an increase of coproporphyrin and protoporphyrin in the stools and blood. In the analysis of the enzymatic activities of the liver and bone narrow, the activity of ALA synthetase (ALA-S) was markedly increased, and the activities of both uroporphyrinogen I synthetase (URO-S) and ferrochelatase were decreased. It was characteristic in this case that the enzymatic abnormalities found in both acute intermittent porphyria (AIP) and variegate porphyria (VP) coexisted. Biochemical analysis of the sciatic nerve showed an increase of ALA-S activity and a decrease of both URO-S and ALA dehydrase activities. This was the first report that indicated the presence of abnormal activities of the heme biosynthetic enzymes in the peripheral nerves of porphyric patients. The possibility was discussed that these enzymatic abnormalities of the heme biosynthesis in the peripheral nerve itself might be strongly related to the pathogenesis of the porphyric neuropathy.
    Type of Medium: Electronic Resource
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  • 12
    ISSN: 1432-0533
    Keywords: Sudanophilic leukodystrophy ; van Bogaert and Nyssen's disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two autopsy cases of siblings with the adult pigment (Peiffer) type of sudanophilic leukodystrophy (SLD), which demonstrated the full-blown stage (case 1) and early stage (case 2) of demyelination, were examined. Numerous brown pigments deposited in demyelinated cerebral areas were characterized histochemically and ultrastructurally as lipofuscin and ceroid. Under the electron microscope formation of blebs due to myelin splitting associated with deposition of multilamellar myeloid bodies within them was a prominent feature in the demyelinated cerebral areas of case 2 as compared with case 1. However, various features of myelin degradation such as thinning, partial or complete circumferential myelin loss, and deposition of electron-dense material on the interperiodic lines were found in both cases. Blebs occurred in all layers of myelin, and axons were compressed by these blebs or the hydropically swollen inner lips of oligodendroglias. Oligodendroglias were relatively well preserved in the demyelinated and nondemyelinated areas in case 2, although the cytoplasm was hydropic. Many spheroids were present in demyelinated areas and were irregularly distributed in both cases. The peripheral nerves in case 1 presented essentially the same changes as those in the brain, although those in case 2 were not affected. Morphometrically, the results showed that hypomyelination was not the mechanism for this pigment type of SLD. One possible cause may be an accelerated ageing of the metabolic process of myelin turnover.
    Type of Medium: Electronic Resource
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  • 13
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 60 (1983), S. 145-148 
    ISSN: 1432-0533
    Keywords: Intracytoplasmic acidophilic granules ; Distribution ; Lewy bodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Using four autopsied brains, we studied the anatomic locations of the intracytoplasmic acidophilic granule (IAG)-containing neurons. These neurons occurred in the hypothalamus, zona incerta, insular cortex, and the other 23 nuclei. However, IAGs were not observed in neurons of the Ammon's horn, thalamus, dentale nucleus, or in Purkinje cells of the cerebellum. The distribution of IAG-containing neurons does not exactly correspond to that of the neuromelanin- or monoamine-forming neurons. There is, however, a striking parallelism between the IAG-containing neurons map and the Lewy bodies map. It is suggested by the superimposition of both maps that IAG-containing neurons may have a certain metabolic relation to the formation of Lewy bodies.
    Type of Medium: Electronic Resource
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  • 14
    ISSN: 1432-0533
    Keywords: Intracytoplasmic acidophilic granules ; Melanin-bearing neurons ; Round electron-dense body ; Mitochondrial inclusion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Using the brains of 30 patients with mental and neurologic disorders, we studied the intracytoplasmic acidophilic granules in neurons of the substantia nigra and locus ceruleus by light and electron microscopy. The granules were present in all 30 brains, including those with no recognizable pathologic change, there was no correlation between their appearance and the age, sex, disease of, or the medication received by, the patients. In four electron-microscopically examined brains, we noted many small, round electron-dense bodies in the perikarya and neuronal processes of the substantia nigra and locus ceruleus. The bodies were packed tightly within a double membrane; in shape, size, and distribution in the neuronal cytoplasm, they corresponded to acidophilic granules. Some mitochondrial matrices contained one or more similar, but smaller inclusion bodies; larger bodies pushed aside the mitochondrial cristae. We conclude that the acidophilic granules represent highly developed forms of mitochondrial inclusions.
    Type of Medium: Electronic Resource
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  • 15
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 78 (1989), S. 96-100 
    ISSN: 1432-0533
    Keywords: Citrullinemia ; Pseudoulegyric-type hepatocerebral disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsy case of adult-type citrullinemia in a 42-year-old male is reported. The patient neuropathologically presented mixed cerebral changes consisting of the pseudoulegyric and ischemic types of hepatocerebral disease. In common with previously reported cases of the pseudoulegyric type, the nature and localization of the cerebral changes in this case were characteristic, in that neuronal loss occurred most severely and symmetrically in the mediobasal part of the frontal and occipital lobes, gyrus cinguli, claustrum, insula and temporal lobe, and that the watershed area of the cerebral cortex, basal ganglia and Purkinje cells were only slightly affected. The importance of hypercitrullinemia was stressed in the pathogenesis of the cerebral changes evident in adulttype citrullinemia.
    Type of Medium: Electronic Resource
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  • 16
    ISSN: 1432-0533
    Keywords: Mitochondrial disease ; Myoclonus epilepsy and ragged red fibers (MERRF) syndrome ; Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome ; Polarographic studies ; Immunoblot studies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Mitochondrial respiratory chain function was investigated with polarographic and enzymatic studies, and correlated with immunoblot studies using a battery of probes against respiratory chain holocomplexes in a series of patients with myoclonus epilepsy and ragged red fibers (MERRF) syndrome. State III respiration rates in intact skeletal muscle mitochondria were normal in two cases, suggested site I deficiency in one case and a midrespiratory defect in another. Immunological studies of complex I showed reduced levels of several subunits with the apparent absence of two bands (which at 45 and 42 kDa, coincide with the predicted electrophoretic mobility of the ND5 gene product) in one case. Complex I, III and IV composition was normal in the other three cases indicating no major disruption of complex assembly. A differing severity of skeletal muscle respiratory chain impairment in a group of unrelated patients with severe cerebral clinical involvement is best explained by uneven tissue distribution between brain and muscle of a heteroplasmic mtDNA mutation. The relationship between MERRF and mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) encephalopathies is reappraised by extension of this hypothesis.
    Type of Medium: Electronic Resource
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  • 17
    ISSN: 1432-1459
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 18
    ISSN: 1432-1114
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Notes: Abstract A new experimental technique is developed by combining a flash photolysis method with a laser-photodiode system to measure the velocity of the flow of thin liquid films. The technique is applied to the measurement of the velocity of the liquid-fuel film within the intake pipe of an internal combustion engine operated under firing condition. The results show that the velocity of the fuel film is of the order of 1/100 of the mean air velocity inside the intake pipe.
    Type of Medium: Electronic Resource
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  • 19
    Electronic Resource
    Electronic Resource
    Springer
    Experimental brain research 121 (1998), S. 92-98 
    ISSN: 1432-1106
    Keywords: Key words Saccade ; Latency ; Fixation ; Smooth pursuit ; Gap ; Human
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  To examine the effects of smooth-pursuit eye movements on the initiation of saccades, their latency was measured when subjects initially fixated or pursued a target. In half of the block of trials, the fixation or pursuit target was extinguished 200 ms before the saccade target was illuminated (gap trials). Reduction of the mean saccade latency in the gap trials (the “gap effect”) was evident even when the subjects were pursuing a moving target, consistent with previous observations. The effect of pursuit direction on saccade latency was also examined. Saccades in the same direction as the preceding pursuit (forward saccades) had shorter latencies than those in the opposite direction (backward saccades). This asymmetry was observed in both the gap and nongap trials. Although the forward-backward asymmetry was much smaller than the “gap effect”, it was statistically significant in six of eight cases. These results suggest that the preparation of saccades is affected by smooth-pursuit eye movements.
    Type of Medium: Electronic Resource
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  • 20
    ISSN: 1432-1106
    Keywords: Visual association cortex ; Prelunate gyrus ; Area 19 ; Colour ; Texture ; Visual neurones ; Awake monkey
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We have analysed, in the awake monkey (Macaca sylvana) the functional properties of 489 neurones in the prelunate visual area (PVA, largely corresponding to V4). PVA has a coarse retinotopic organization with the lower quadrant of the visual field represented along the prelunate gyrus. The visual periphery is located medio-dorsally, the central visual field laterally near (and within?) the inferior occipital sulcus and the upper quadrant latero-ventrally. The vertical meridian runs caudally within the lunate sulcus, the horizontal meridian crosses the prelunate gyrus and continues into the superior temporal sulcus. Receptive field diameters of neurones vary between 1° and 10° with increase towards the visual periphery, but are strictly confined to the contralateral visual field. 28% of the neurones showed spectral sensitivity. About half of these cells had strong spectral opponency, the other half showed only weak opponency with broader spectral response curves. 11 cells (2%) showed striking centre/surround interactions with inhibition, disinhibition or occlusion of the two mechanisms, and different spectral response ranges of the centre and the surround, respectively. 43% of the prelunate cells were responsive to various spatial features without spectral sensitivity. We distinguished on- and off-center cells (2%), direction and movement sensitive cells (10%) and cells sensitive to gratings of parallel lines within a limited range of orientations (about 10%). A special group were cells which responded strongly to stimuli which contained many contrasts (textures without specific orientations and without regular spatial arrangements) (9%). Many of these cells were specifically responsive to variations of the internal structure of such stimuli. 3% of the cells were strongly activated in connection with behaviour: 11 neurones discharged strongly when the monkey looked attentively at a human face or when he responded with facial expressions to a threatening expression of a person. Photographs of faces were not effective. Some neurones (1%) were activated in connection with eye movement. These neurones were found in the lateral part of the prelunate gyrus. Neurones with spectral or non-spectral properties were clustered within small, irregularly shaped patches of 1–4 mm diameter. It is concluded that the prelunate visual cortex, which we consider as part of area 19, is not just a “colour area”, but represents various features of the visual environment (including colour, luminance, movement, texture and behavioral significance), and relates them — through its subcortical and cortical outputs — to behaviour. The various visual cortical areas may be seen as a cooperative of several connections between visual input and behaviour output rather than as links in a hierarchical chain of perceptual and cognitive representations.
    Type of Medium: Electronic Resource
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