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  • 1
    ISSN: 1432-0533
    Keywords: Progressive supranuclear palsy ; Neurofibrillary tangles ; Pick body ; Immunocytochemistry ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Degeneration of heterogeneous systems in the central nervous system, with widespread distribution of argyrophilic neuronal fibrillary inclusions, was found in a patient with presenile dementia. Atrophy was circumscribed in the frontal and temporal lobes. Neuronal loss was severe in the basal ganglia, subthalamic nucleus, and substantia nigra. Immunocytochemical study using anti-phosphorylated tau and anti-ubiquitin antibodies in conjunction with ultrastructural observations revealed two types of inclusions: neurofibrillary tangles (NFTs) of progressive supranuclear palsy (PSP) in the Edinger-Westphal nucleus, locus coeruleus, cerebellar dentate nucleus, inferior olivary nucleus, and posterior horn of the spinal cord; and Pick bodies (PBs) in the atrophied cerebral cortex and red nucleus. PSP-type NFTs and PBs have been demonstrated in a single case for the first time. Despite their pathognomonic significance in certain disorders, we suggest that these inclusions may reflect a form of cytoskeletal disorganization, which is not entirely restricted to a single disease entity.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 79 (1990), S. 629-633 
    ISSN: 1432-0533
    Keywords: Pick's disease ; Locus coeruleus ; Cell counts ; Statistical analysis ; Pick body
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Brains affected by the fronto-temporal type of Pick's disease were classified into two subgroups according to whether Pick bodies (PBs) were detectable in cerebral cortex (PB-positive group, six cases) or not (PB-negative group, eight cases), and examined neuropathologically. Controls included seven patients with non-degenerative diseases. The neuronal population in the locus coeruleus (LC) was estimated quantitatively in preparations from the middle part of the LC. The data were analyzed statistically by the Mann-Whitney U-test. Histological and ultrastructural studies were also carried out. The following results were obtained: (1) there were no appreciable differences between the PB-positive and PB-negative groups with regard to age at onset, age at death, duration of illness, clinical stage at death, and brain weight; (2) the mean nerve cell counts in the LC were 43.7±5.2 in the controls, 28.8±11.7 in the PB-positive group, and 42.9±7.6 in the PB-negative group. The nerve cell count in the PB-positive group was significantly lower (P〈0.05) than those in the controls and the PB-negative group; and (3) in each of the PB-positive cases, PBs were disclosed in the LC, in medium-sized melanin-laden neurons and small neurons. PBs were globular or lobulated, and their fine structure was identical to that of typical PBs in the cerebral cortex. In conclusion, PB formation may play an important role in neuronal decrease in the LC of PB-positive cases, whereas the LC may not be affected in PB-negative cases. In this respect, Pick's disease with PB formation appears distinct from that without PB formation.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Multiple system atrophy ; Ubiquitin ; Neuronal cytoplasmic inclusions ; Thickened neurites ; Granule-associated filaments
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Neuronal alterations in five cases of multiple system atrophy (MSA) were investigated histologically, immunocytochemically and ultrastructurally. Argentophilic neuronal cytoplasmic inclusions (NCIs) were observed in all cases. They were distributed, in order of decreasing frequency, in the pontine nucleus, striatum, subiculum, amygdala, hippocampus, dentate fascia, substantia nigra and inferior olivary nucleus. Anti-ubiquitin antibodies visualized many thickened neurites in the degenerating gray matter as well as NCIs. Some NCIs were also recognized by anti-phosphorylated neurofilament antibodies. Ultrastructurally, NCIs consisted of a meshwork of granule-associated filaments, the diameter ranging from 18 to 28 nm, that were mixed with neurofilaments. The granule-associated filaments were also present in the axoplasm of myelinated fibers. Our studies demonstrate widespread distribution of NCIs in the central nervous system of MSA. The same pathological process that forms the granule-associated filaments in axons may also be responsible for the formation of ubiquitin-positive thickened neurites. These axonal alterations, as well as neuronal perikaryal changes, may play an important role in the impaired neuronal function in MSA.
    Type of Medium: Electronic Resource
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