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  • 1
    Electronic Resource
    Electronic Resource
    Neuropathological findings of an autopsy case of adult β-galactosidase and neuraminidase deficiency (1983)
    Springer
    Acta neuropathologica 61 (1983), S. 283-290 
    Details
    ISSN: 1432-0533
    Keywords: β-Galactosidase and neuraminidase deficiency ; Neuronal inclusion bodies ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsy case of a Japanese male with familial β-galactosidase and neuraminidase deficiency is reported. The clinical picture was characterized by adult onset, a gargoyle-like face, cerebellar ataxia, myoclonus, convulsions, retinal degeneration and cortical blindness. Histopathologically, most neurons seemed to have become degenerated in the whole cerebral cortex. Moreover, the calcarine cortex appeared spongy with depopulation of nerve cells. Stuffed neurons or neuronal storage changes were found throughout the brain, especially in the motor nuclei of the spinal cord and brain stem. The inclusions in the stuffed neurons revealed various profiles on the electron microscope. They were composed of membranous lamellar and/or multilamellar structures, often accompanying vacuoles and reminiscent of lipofuscin-like profiles.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00691999
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    A case of adult neuronal ceroid-lipofuscinosis with the appearance of membranous cytoplasmic bodies localized in the spinal anterior horn (1987)
    Springer
    Acta neuropathologica 72 (1987), S. 362-368 
    Details
    ISSN: 1432-0533
    Keywords: Neuronal ceroid-lipofuscinosis ; Membranous cytoplasmic body ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsy case of adult neuronal ceroid-lipofuscinosis was examined. The clinical picture was charaterized by gait disturbance, bulbar palsy and dementia. Histopathologically, diffuse neuronal loss was found throughout the central nervous system. The remaining neurons, predominantly in the motor nuclei of the spinal cord and brain stem, were swollen with storage material. Observed under the electron microscope the storage material showed various ultrastructures, such as lipofuscin-like bodies, pleomorphic lipid bodies, curvilinear profiles and finger-print profiles, in different regions of the central nervous system. In the ballooned neurons of the spinal anterior horn, many membranous cytoplasmic bodies and curvilinear profiles were intermingled within the same cell and were continuous with each other. Biochemically,N-acetyl neuraminic acid content was significantly increased in the spinal anterior horn. These findings suggest the localized increase of ganglioside in that region.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687268
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  • 3
    Electronic Resource
    Electronic Resource
    Pleomorphic intra-neuronal polyglucosan bodies mainly restricted to the pallidium (1983)
    Springer
    Acta neuropathologica 62 (1983), S. 159-163 
    Details
    ISSN: 1432-0533
    Keywords: Pleomorphic polyglucosan body ; Bielschowsky body ; Lafora body ; Corpora amylacea
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A rare case with pleomorphic intra-neuronal polyglucosan bodies is reported. A 45-year-old man died of gastric carcinoma with extensive visceral involvement. The autopsy showed numerous intra-neural pleomorphic PAs-positive deposits reminiscent of Bielschowsky bodies in the lateral segments of the pallidum, substantia nigra, and brain stem on both sides. Their histochemical properties were consistent with polyglucosan. The bodies were ultrastructurally composed of filamentous structures and seen in perikarya, axons, and dendrites. Occasionally, spotty electron-dense material was seen in the center of the bodies. The filaments were selectively stained with thiocarbohydrazide method after Thiéry, indicating their chief component as glucose polymers. The bodies are compared with Bielschowsky bodies, Lafora bodies, corpora amylacea, and related bodies, and their nature and significance are briefly discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00684936
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  • 4
    Electronic Resource
    Electronic Resource
    Glycogen in leukocytes from patients with hepatic encephalopathy (1984)
    Springer
    Acta neuropathologica 62 (1984), S. 284-290 
    Details
    ISSN: 1432-0533
    Keywords: Hepatic encephalopathy ; Leukocyte ; Glycogen ; Glycogenolytic enzymes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Accumulations of glycogen in leukocytes from patients with hepatic encephalopathy and controls were studied electron-microscopically and biochemically. Extensive glycogen accumulation was observed in polymorphonuclear leukocytes from these patients, the content being 1.5 or 3.4 times higher than in the controls. The absorption spectra of glycogen with an iodine reagent exhibited no difference between patients and controls. Phosphorylasee activity was reduced but acid alpha 1,4-glucosidase activity elevated in the leukocytes from the patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687610
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  • 5
    Electronic Resource
    Electronic Resource
    Nasu-Hakola's disease (membranous lipodystrophy) (1987)
    Springer
    Acta neuropathologica 74 (1987), S. 294-299 
    Details
    ISSN: 1432-0533
    Keywords: Nasu-Hakola's disease ; Membranous lipodystrophy ; Axonal spheroids ; Membranocystic change ; Glio-myelin dissociation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsy case of Nasu-Hakola's disease (membranous lipodystrophy) is reported. A 43-year-old Japanese man, whose parents were not consanguineous, had been suffering from frequent long bone fractures since the age of 10. Neuropsychiatric symptoms, which were characterized by euphoria, disturbance of attention and dementia, appeared at his thirties and generalized and/or localized seizures and apallial syndrome at the later stage. The neuropathology revealed diffuse leukoencephalopathy of the cerebrum. The peculiar aspects in this case were membranocystic changes in the lungs [Yagishita et al. Virchows Arch [A] 408:211–217 (1985)], diffuse degeneration of the cerebral cortex, chiefly in frontal and temporal lobes, and many axonal spheroids throughout the cerebral cortex. The ultrastructure of spheroids in the cerebral cortex demonstrated aggregations of mitochondria, dense bodies and minute concentric bodies and a small amount of neurofilaments.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688195
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  • 6
    Electronic Resource
    Electronic Resource
    Clinical and neuropathological studies of myoclonic epilepsy (1965)
    Springer
    Acta neuropathologica 4 (1965), S. 370-379 
    Details
    ISSN: 1432-0533
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Zwei Fälle von familiärer Myoklonus-Epilepsie wurden sowohl klinisch als auch neuropathologisch untersucht. Die Krampfanfälle traten in einem Fall im 12. Lj., im anderen im 18. Lj. auf, während myoklonische Zuckungen mit 14 bzw. 21 Jahren einsetzten. Klinisch verlief der erste Fall mit progredientem psychischem Abbau, der zweite verfügte bis ins Endstadium über eine relativ gute geistige Leistungsfähigkeit. Die neuropathologische Untersuchung deckte in beiden Fällen die Hauptläsionen im Kleinhirn und Hirnstamm mit Ausfällen von Purkinjezellen auf; eine eigenartige Zellerkrankung konnte in verschiedenen Kernen des Hirnstamms und im N. dentatus festgestellt werden. In der Medulla oblongata wurden zahlreiche eosinophile Körperchen gefunden. Die klinischen Unterschiede zwischen dem Lafora-Körperchen-Typ und dem degenerativen Typ von Myoklonusepilepsie, das Wesen der eigenartigen Zellerkrankung und der eosinophilen Körperchen werden diskutiert.
    Notes: Summary Two cases of familial myoclonic epilepsy were studied clinically and neuropathologically. Convulsive seizures began at 12 years in one case and at 18 years in another, while myoclonic twitchings appeared at 14 and 21 years old respectively. The first patient had a progressive clinical course with mental deterioration, whereas the second retained relatively intact mental functions to the final stage. Neuropathological examination revealed that the major lesions in both cases were in the cerebellum and brain stem. There was loss of Purkinje cells and a peculiar cell disease in the various nuclei of the brain stem and in the dentate nuclei. Numerous eosinophilic bodies were found in the medulla oblongata. The clinical differences between theLafora body type and the degenerative type, and the nature of the peculiar cell disease and of the eosinophilic bodies are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00690672
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  • 7
    Electronic Resource
    Electronic Resource
    Postnatal sudanophilic leukodystrophy in two siblings (1985)
    Springer
    Acta neuropathologica 67 (1985), S. 103-113 
    Details
    ISSN: 1432-0533
    Keywords: Sibling ; Early infancy ; Convulsions ; Demyelination ; Normal long-chain fatty acids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Reported here are two siblings with sudanophilic leukodystrophy occurring in early infancy. Soon after birth, high fever, distension of the abdomen and spasticity of the extremities were noted. Neurologic disorders, frequent convulsive seizures, and malnutrition caused both siblings to die at the age of 3 and 6 months, respectively. Neuropathologic examination of the brain revealed diffuse demyelination in the cerebral and cerebellar white matter as well as a disturbance of the myelination, especially in the descending pathway. Numerous sudanophilic lipids and glial fibers proliferated in the demyelinated areas. neurochemically, there was a marked decrease in all lipid components, cholesterol, phospholipids, and glycolipids and an increase in cholesterol ester. Fatty-acid composition of cerebroside and sulfatide, especially that of long-chain acids, was within normal range, unlike the fatty-acid composition found in connatal Pelizaeus-Merzbacher disease. The brain damage in postnatal infant caused by frequent convulsive seizures and the severe demyelination in the cerebral and cerebellar white matter in both patients are discussed. The cases of sudanophilic leukodystrophy and Pelizaeus-Merzbacher disease occurring in children under 5 years of age are reviewed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688130
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  • 8
    Electronic Resource
    Electronic Resource
    Tardive dyskinesia with inflated neurons of the cerebellar dentate nucleus (1987)
    Springer
    Acta neuropathologica 73 (1987), S. 38-42 
    Details
    ISSN: 1432-0533
    Keywords: Tardive dyskinesia ; Antipsychotic drugs ; Oral dyskinesia ; Cerebellar dentate nucleus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Four autopsied cases of tardive dyskinesia manifesting oral hyperkinesia revealed markedly inflated neurons in the cerebellar dentate nucleus (DN), which had not been described previously. The inflation of the neurons was proved to be statistically significant (P〈0.01) by morphometric study. The nuclei were usually situated in the central portion of the cytoplasm. This inflated change was different from both central chromatolysis and grumose degeneration of the DN, typically observed in progressive supranuclear palsy and dentatorubropallidolysian atrophy, and seemed to be easy to miss without careful observation, since neuronal loss and gliosis were very mild in the DN. Among a few autopsied cases of tardive dyskinesia reported previously, degeneration of the DN was described in only two. It is believed, however, that the inflated neurons of the DN may not be so rare and may be related to the occurrence of some involuntary hyperkinesia, especially oral hyperkinesia following some neurotoxic disorders and/or neuroleptic medications.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00695500
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  • 9
    Electronic Resource
    Electronic Resource
    Comparative biochemical studies of carotenoids in nine species of cephalopoda
    Amsterdam : Elsevier
    Comparative Biochemistry and Physiology -- Part B: Biochemistry and 92 (1989), S. 247-250 
    Details
    ISSN: 0305-0491
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0305-0491(89)90273-3
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  • 10
    Electronic Resource
    Electronic Resource
    A nuclear magnetic resonance spectroscopic and conformational study of eight pseudo-trehaloses (d-glucopyranosyl 5a-carba-d- and -l-glucopyranosides)
    Amsterdam : Elsevier
    Carbohydrate Research 209 (1991), S. 51-65 
    Details
    ISSN: 0008-6215
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0008-6215(91)80144-C
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