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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 382 (1979), S. 217-226 
    ISSN: 1432-2307
    Keywords: Axonal swelling ; Spheroid ; Dystrophic axon ; Ultrastructure ; Human gracile nucleus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The morphology of the spheroids in human gracile nuclei was studied by light and electron microscopy. Various spheroids encountered in the present study could be classified into three types based on the internal structure: The first one was chiefly composed of many irregular homogeneous dense bodies, multivesicular bodies and mitochondria. The dense bodies seemed to deposit multicentrically in an axon in the early stage of “balloon” formation and coalesce to form larger ones. The second was characterized by a marked accumulation of closely approximated mitochondria and dense concentric bodies. In the third the most characteristic findings were neurofibrillary accumulation and aggregations of dense bodies. These findings showed some divergence from those of dystrophic axons and the last two mimic those of degenerative or regenerative axons, which suggested that axonal swelling (including dystrophic axon) is not characteristic reaction of specific disease but rather nonspecific one to a variety of noxious stimuli. With light microscope, it was difficult to distinguish balloons with different structures since they were quite diverse and manifold in their shape, size, appearance and stainability. Intra-axonal corpora amylacea were seen in most cases and their incidence appeared to be nonspecific for any diseases.
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 372 (1976), S. 245-251 
    ISSN: 1432-2307
    Keywords: Lipomembranous polycystic Osteodysplasia ; Membranous structure ; Endoplasmic reticulum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of lipomembranous polycystic Osteodysplasia is presented. The clinical features were characterized by multiple cystic changes of the bones and progressive psychomotor retardation. Although the diagnosis of polyostotic fibrous dysplasia of bone was made first because of expanded tumorlike lesions in bones, pathology of bone and bone marrow confirmed the diagnosis of lipomembranous polycystic Osteodysplasia. Light microscopy showed a great number of peculiar undulating membranous structures in the adipose tissue. With the electron microscope the membrane seemed to be composed of an accumulation of endoplasmic reticulum-like tubular profiles. They appear first, in close connection with fat droplets, within the mesenchymal cell.
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  • 3
    ISSN: 1432-2307
    Keywords: Axonal swelling ; Spheroid ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Axonal swellings and spheroids in various human diseases were studied by light and electron microscopy. 4 cases of infantile neuroaxonal dystrophy, 2 of degenerative diseases, 2 brain tumors and 3 of cerebrovascular disease were examined. Ultrastructurally most spheroids in infantile neuroaxonal dystrophy consisted of interconnected tubules, stacked membranotubular profiles, alternating layered membranes and accumulations of neurofilaments. Combinations of these four constituents were seen only in infantile neuroaxonal dystrophy. “Torpedos” (fusiform swelling of the axon of a Purkinje cell) consisted exclusively of neurofilaments. Spheroids in case 6 (mental retardation) and 7 (atypical teratoma) consisted of interwoven skeins of neurofilaments and grouped mitochondria. Spheroids in case 8 (demyelination) and 9 (cerebrovascular disease) consisted of packed complex bodies and mitochondria. Spheroids in cases 10 and 11 (cerebrovascular disease) consisted of degenerating organelles only. The morphological differences between cases 9, 10 and 11 probably depends on the severity and timing of the cerebral injury. Most spheroids show similar histological and histochemical properties, but ultrastructural study may give some clue to the origin of the bodies.
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 415 (1989), S. 467-472 
    ISSN: 1432-2307
    Keywords: Ependymoma ; Clear cells ; Oligodendroglioma ; Haemangioblastoma ; Pathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Four cystic brain tumours, one from the frontal lobe, one in the third ventricle and two in the cerebellum, were studied histologically including immunostaining and electron microscopy. Anatomically, all the tumours were located within or adjacent to the ventricular system. By light microscopy, the tumours had a largely honeycomb pattern and were made up of clear cells. Some of the clear cells showed positive for GFAP. Leu 7, Factor VIII and NSE were negative. Electron microscopy of the areas with clear cells revealed densely packed polyhedral cells with clear cytoplasm. They had well developed intercellular junctions, microvilli and some cilia which confirmed their ependymal derivation. Perivascular pseudorosettes or papillary features were only seen in limited areas, where GFAP was strongly positive in the tumour cell processes. As these tumours mimic oligodendroglioma and cerebellar haemangioblastoma, they are called as “clear cell ependymoma” to obviate such errors in diagnosis.
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  • 5
    ISSN: 1432-2307
    Keywords: Axon ; Astrocyte ; Synaptophysin ; Glial fibrillary acidic protein ; Foamy spheroid body
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Foamy spheroid bodies (FSBs) are described, as newly identified pathological structures occurring in human brain. FSBs favoured the substantia nigra pars reticulata (SNPR) and/or globus pallidus (GP) in degenerative conditions especially postencephalitic parkinsonism, progressive supranuclear palsy, pallido-nigro-luysial atrophy and multiple system atrophy. No FSBs were observed anywhere in the presence of substantia nigra pars compacta (SNPC) degeneration, such as occurs in idiopathic Parkinson's disease, or luysio-pallidal system degeneration, such as found in dentato-rubro-pallidoluysial atrophy or Joseph's disease. FSBs were also occasionally identified in the substantia nigra (SN) and/or GP of aged persons. In addition to SN and GP lesions, FSBs were seen in diffuse axonal lesions of long fibre tracts (the corpus callosum, the superior cerebellar peduncle) after non-missile head injuries, and in peri-infarct lesions. Under the light microscope, FSBs appear as slightly eosinophilic, foamy and nearly round objects with vague outlines, measuring approximately 10–50 μm in diameter. Some FSBs contain coarse, eosinophilic clusters at their periphery. FSB stained black when stained by the Gallyas silver method. Some FSBs were immunohistochemically positive for synaptophysin and 68 kDa neurofilament. Glial fibrillary acidic proteinpositive fibres were observed alongside and/or inside some FSBs. Electron microscopically, FSBs were found to consist of collections of neuritic debris containing a variety of dense bodies and a small number of both mitochondria and neurofilaments. Some such collections were surrounded by astrocytic processes. These findings strongly suggest that FSBs are collections of small axonal debris destined for removal by astrocytes in due course. A variety of factors (degeneration of the SNPR and/or the GP, injury, infarction, ageing) seemed to be responsible for the histogenesis of FSBs.
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 31 (1975), S. 191-200 
    ISSN: 1432-0533
    Keywords: Infantile Neuroaxonal Dystrophy ; Spheroid ; Endoplasmic Reticulum and Microtubule ; Synaptic Vesicle ; Crystalloid Inclusion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The findings in a case of infantile neuroaxonal dystrophy are presented. Light microscopy shows wide distribution of spheroids, cerebellar atrophy and striatal degeneration. Electron microscopy shows numerous spheroids consisting of tubulo-vesiculo-membranous profiles. The dystrophic changes appear first in the axon terminals, extend proximally and in the proximal axons seem to be modified by axostasis and/or transneuronal degeneration in addition to the dystrophic changes. The participation of the endoplasmic reticulum and microtubules in spheroid formation and their relation to the malformation of the synaptic vesicles have been discussed. Diverse mitochondrial abnormalities and Hirano like body are also described.
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  • 7
    ISSN: 1432-0533
    Keywords: Key words Paired helical filament ; Polyglucosan ; body ; Alzheimer’s disease ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The coexistence of polyglucosan bodies (PBs) and paired helical filaments (PHFs) in the same neuron is reported in an autopsy case of Alzheimer’s disease. The patient was a 56-year-old Japanese male with a typical clinical course and pathological findings of Alzheimer’s disease. Electron microscopically, numerous neurofibrillary tangles, mainly composed of PHFs, were observed in the neuronal cytoplasm, axons and dendrites. Some of them coexisted with other filamentous structures, which comprised randomly oriented branching filaments with a diameter of 5–10 nm. These structures were compatible with PBs. Glial tangles could not be found. Coexistence of these two structures was thought to occur in neurites.
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  • 8
    ISSN: 1432-0533
    Keywords: Key words Homogeneous dense body ; Alzheimer’s disease ; Ultrastructure ; Axonal dystrophy ; Eosinophilic body
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The light microscopical, immunohistochemical and ultrastructural aspects of eosinophilic bodies in the cerebral cortex from patients with Alzheimer’s disease (AD) are described, based on a study of 16 cases of AD, 5 elderly non-demented controls and, as disease controls, 5 cases of Pick’s disease, 9 with progressive supranuclear palsy, 5 with Creutzfeldt-Jakob disease and 1 with Binswanger’s disease. At the light microscopy level, the bodies were clearly separated from the surrounding tissues and were mostly round or elliptic with a diameter of 5–30 μm and a central, intensely eosinophilic core. Ultrastructurally, they consisted of a central homogeneous electron-dense body (HDB), and filamentous structures (resembling either neurofilaments or paired helical filaments) or other small organelles in the periphery. Immunohistochemically, some of these bodies exhibited ring-shaped rims which were positive with antibodies against paired helical filaments, tau-2, phosphorylated neurofilaments and ubiquitin. The bodies were widely distributed throughout the cerebral cortex, but were not observed in the white matter. These bodies were thought to be compatible with one type of axonal dystrophy in the gracile nucleus (termed ‘old’ spheroid by Jellinger), and are here referred to as the HDB-type spheroid based on their ultrastructure. In this study HDB-type spheroids were found in high incidence in the AD cases, but only two HDB-type spheroids were seen in one case of Pick’s disease, and none in any of the other cases of neurodegenerative diseases or in the elderly non-demented controls. It seems plausible that the incidence of HDB-type spheroids in the cerebral cortex might be related to a pathological process and not to a physiological ageing phenomenon, and might be characteristic of, but not unique to, AD.
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  • 9
    ISSN: 1432-0533
    Keywords: Key words Progressive supranuclear palsy ; Astrocyte ; Tuft-shaped astrocyte ; Neurofibrillary tangle ; Diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Progressive supranuclear palsy (PSP) is known to have tau-positive cytoskeletal abnormalities in astrocytes and oligodendroglia as well as neurons. Astrocytic tau-positive structures (tuft-shaped astrocytes; Tu-SA) were studied to elucidate their proper significance in the neuropathological diagnosis of PSP. The distribution and incidence of Tu-SA were examined in 26 cases of PSP. The disease specificity of Tu-SA was demonstrated by comparison with diseases accompanied by neurofibrillary tangles (NFTs) and those with or without cytoskeletal abnormalities other than NFTs. In PSP, Tu-SA appeared prominently in the precentral and premotor cortex (areas 4 and 6) of the superior and middle frontal gyri, but were quite scare in the temporal lobe and limbic area. In the subcortical nuclei, they appeared preferentially in the putamen and were also scattered in other degenerating regions. In the cerebrum the Tu-SA and NFTs were distributed in quite different regions. The assessment of the incidence of Tu-SA in area 6 revealed that only 5 of 26 PSP cases lacked Tu-SA in the examined fields. In contrast, in the control diseases, Tu-SA were found only rarely in cases of corticobasal degeneration in the cerebral cortex among other frequent tau-positive structures. One case of Pick’s disease showed occasional Tu-SA but only in the hippocampal region and not in the frontal lobe or putamen. In summary, although the absence of Tu-SA does not necessarily exclude the possibility of PSP, Tu-SA in the frontal lobe and putamen is highly suggestive for PSP. Thus, detection of Tu-SA and the ranking of the characteristic distribution of NFTs contribute to the neuropathological diagnosis of PSP.
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  • 10
    ISSN: 1432-0533
    Keywords: Gerstmann-Sträussler syndrome ; Variant ; Amyloid plaques ; Alzheimer's neurofibrillary tangles ; Prion protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary This report presents a variant of Gerstmann-Sträussler syndrome (GSS). A 53-year-old female had developed slowly progressive dementia and atactic gait since the age of 45. No myoclonic jerks and periodic synchronous discharges were observed throughout the illness. The neuropathological study revealed that many amyloid plaques and widespread Alzheimer's neurofibrillary tangles (NFTs) appeared in the cerebral cortex. Characteristically, the plaques reacted with anti-prion protein and none of them reacted with anti-β protein, and they were made of many components, including amyloid cores, macrophages laden with lipid granules and/or degenerated neurites. Neuropil threads were seen mainly in amyloid plaques. Moreover, plaques appeared which were confluent and laminar in arrangement in the fifth and sixth cortical layers and had a close relationship to the neuronal loss. There was no spongiform change in the cerebral cortex or cerebellum. The cerebellum was almost intact except for a few amyloid plaques. Ultrastructurally, some of the plaques simulated kuru plaques and others had many degenerated neurites possessing paired helical filaments and other accumulated organelles. GSS has been proposed to include cases with progressive ataxia, dementia and massive multifocal plaques in the brain with or without cerebral spongiform changes. The case presented here is a very peculiar case of GSS. Recently, similar cases have been reported in some large families, diagnosed as familial Alzheimer's disease. These cases may be a telencephalic form with numerous NFTs of GSS.
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