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  • 1
    ISSN: 1432-0533
    Keywords: Key words Neuronal intranuclear inclusion ; Neurodegenerative diseases ; Polyglutamine ; Ubiquitin ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Neuronal intranuclear hyaline inclusion disease (NIHID) is a group of neurodegenerative disorders characterized by the presence of intranuclear inclusions in neurons (NIs). We report here clinicopathological findings of a 25-year-old female patient who died after 13 years of a clinical course characterized by progressive gait disturbance and movement disorders. Histological examination revealed widespread NIs with neuronal loss in restricted regions; neuronal loss was severe in the subthalamic nucleus, internal pallidum, substantia nigra, Edinger-Westphal nucleus and Purkinje cell layer. Quantification of the NIs combined with a graded evaluation of neuronal loss revealed an overall tendency for more severe neuronal loss to be accompanied by a lower frequency of NIs. A morphological similarity to the nuclear inclusions recently identified in several CAG repeat diseases prompted us to examine the immunolocalization of ubiquitin and expanded polyglutamine stretches, which demonstrated the presence of ubiquitin at the periphery of most NIs. An expanded polyglutamine stretch was seen in the center of limited number of NIs. These findings indicate that abnormal fragments such as expanded polyglutamine regions are incorporated into the inclusion, aggregated in its center, and thereby metabolized by a ubiquitin-dependent proteolytic pathway. Although it remains to be elucidated how the formation of NIs is related to neuronal degeneration, our findings suggest that NIs are formed in the process of sequestering or degrading abnormal protein fragments and formation of NIs may not be immediately toxic to neurons.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Key words Alzheimer’s disease ; Corticobasal ¶degeneration ; Paired helical filaments ; Thiazin red ; Tau
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Thiazin red (TR), a fluorochrome that has an affinity to fibrillary structures such as neurofibrillary tangles (NFTs) or senile plaques, was utilized to investigate assembly of tau protein into fibrils in tau-immunopositive neocortical neurons of corticobasal degeneration (CBD) and of Alzheimer’s disease (AD). Double fluorescence with anti-paired helical filament monoclonal antibody (AT8) and TR was followed by either the Gallyas or Bodian silver impregnation method, which enabled a comparison of the staining features by three different methods on the same neuron. NFTs of AD were uniformly stained by TR and Gallyas method. Most of tau-immunopositive neurons of CBD were similarly stained by Gallyas method but barely or only weakly by TR or Bodian method, suggesting that tau in neocortical neurons of CBD is less liable to form fibrillary structures than in those of AD, easily distinguishable by TR staining. Clarifying the process of tau assembly using this fluorochrome will give a clue to understanding mechanisms of tau deposition, which may be different in various neurological disorders.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 81 (1990), S. 155-161 
    ISSN: 1432-0533
    Keywords: Pick's disease ; Substantia nigra ; Pigmented neurons ; Morphometry ; Parkinsonism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Loss of neurons from the substantia nigra (SN), which is often encountered in Pick's disease, was quantitatively analyzed in 13 cases of Pick's disease and 19 age-matched controls. On sections from the upper and lower portions of the SN, the pigmeted zone (zona compacta) and the non-pigmented zone (zona reticulata) were delineated, and these zones were partitioned into quarters: medial, mid-medial, mid-lateral and lateral. Neuronal loss was fairly severe and more evident in the upper section of the SN (−40%), especially in the midmedial and lateral quarters. In the lower section (neuronal loss:−28%), the medial quarter was most severely affected. Non-pigmented neurons were preserved. Fibrillary gliosis was denser in the zona reticulata, where neuronal loss was minimal. These findings revealed a selective vulnerability of nigral neurons according to their topography and pigmentation and suggests the primary involvement of some neuronal groups (especially the pigmented neurons) of the SN in Pick's disease.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 83 (1992), S. 271-276 
    ISSN: 1432-0533
    Keywords: Alzheimer's disease ; Substantia nigra ; Pigmented neurons ; Neurofibrillary tangles ; Morphometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Loss of neurons from the substantia nigra (SN), which is sometimes observed in Alzheimer's disease (AD), was quantitatively analyzed in 10 cases of presenile AD and 19 age-matched controls. On sections from the upper and lower portions of the SN, the pigmented zone (zona compacta) and the nonpigmented zone (zona reticulata) were delineated, and these zones were partitioned into quarters: medial, mid-medial, mid-lateral and lateral. This approach clarified topographical preference of neuronal depletion in the SN of AD; namely (1) pigmented neurons were more severely affected than non-pigmented neurons, (2) neuronal depletion was more marked in the lower SN (−38%, P〈0.001), where the pigmented neurons in the medial quarter were most severely affected (−51%, P〈0.001), (3) in the upper SN (neuronal loss: −21%, P 〈0.01), the pigmented neurons in the mid-medial quarter were most severely affected (−43%, P〈0.01). These findings suggest that some groups of nigral neurons are primarily involved in presenile AD. Gallyas staining after bleaching of melanin pigments uncovered a large number of neurofibrillary tangles (NFTs) mainly in the pigmented zone, especially in the medial quarter. A large number of NFTs, scarse senile plaques, and substantial depletion of neurons form an unique combination of Alzheimer pathology in the SN not well recognized so far.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Key words Corticobasal degeneration ; Tau ; Gallyas ; Autopsy ; Cytoskeletal protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An autopsy case of clinically diagnosed “corticobasal degeneration (CBD)” was investigated. In addition to status spongiosus and neuronal achormasia around the central sulcus, cortical pyramidal neurons and thread-like structures were densely stained by Gallyas stain and tau immunohistochemistry, but apparent fibrillary structures like Alzheimer's disease neurofibrillary tangle were absent. Bodian, methenamine-Bodian, Congo red, thioflavin S, or Bielshowsky stains failed to visualize these structures. They were not stained by immunohistochemical stain with anti-ubiquitin antibody. The widespread cytoskeletal pathology, which is distinct from that in Alzheimer's disease or progressive supranuclear palsy, is suggestive of CBD.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Journal of Photochemistry and Photobiology A: Chemistry 66 (1992), S. 379-385 
    ISSN: 1010-6030
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Journal of Photochemistry and Photobiology A: Chemistry 67 (1992), S. 101-107 
    ISSN: 1010-6030
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 236 (1989), S. 186-186 
    ISSN: 1432-1459
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 241 (1993), S. 92-95 
    ISSN: 1432-1459
    Keywords: Action myoclonus ; Visually guided movement ; Sialidosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In order to determine what kind of voluntary movement induces action myoclonus, we gave two siblings with sialidosis two kinds of tasks. When the patients were asked to move their index fingers following a smoothly moving target, action myoclonus became prominent. In contrast, when they were asked to perform the same movements with their eyes closed, they could move their index fingers very smoothly. This shows that action myoclonus was induced by visually guided movement, but not by self-paced movement. Our observations might reflect a disorder of the cerebellum, which controls visually guided movement.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1920
    Keywords: Radiation myelopathy ; MRI ; Gadolinium ; DTPA enhancement
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We described two cases of chronic progressive radiation myelopathy (CPRM), in which magnetic resonance imaging (MRI) was of great value for the diagnosis. Gadolinium-DTPA (Gd-DTPA) enhancement delineated precise lesions responsible for Brown-Séquard syndrome caused by CPRM in both cases. This safe, sensitive procedure will be a requisite study in patients who are suspected of CPRM.
    Type of Medium: Electronic Resource
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