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  • 1
    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.
    Type of Medium: Electronic Resource
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  • 2
    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.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 29 (1974), S. 115-126 
    ISSN: 1432-0533
    Keywords: Infantile Neuroaxonal Dystrophy ; Spheroid ; Striatal Degeneration ; Cerebellar Atrophy ; Endoplasmic Reticulum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two cases of infantile neuroaxonal dystrophy in female siblings are reported. The clinical features, with a fatal outcome within 7 years and 3 years respectively, were characterized by progressive psycho-motor retardation. The first manifestation of the disease appeared at the age of one and half years and at the age of 4 months, respectively. Light microscopy shows spheroids with a selective distribution in medulla, posterior horn of spinal cord and cerebral cortex, diffuse cerebellar cortical atrophy with sclerosis, sudanophilic fatty deposits and status dysmyelinisatus in the striatum. Electron microscopy shows tubulomembranous structures composed of usually compactly arranged smooth membranes, aggregations of mitochondria in the spheroids, filamentous inclusions simulating Hirano bodies in the spheroid and axon. Spheroids were present in nerve cell perikarya, axons, dendrites, and presynaptic terminals.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 378 (1978), S. 181-197 
    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.
    Type of Medium: Electronic Resource
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  • 5
    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.
    Type of Medium: Electronic Resource
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  • 6
    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.
    Type of Medium: Electronic Resource
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  • 7
    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.
    Type of Medium: Electronic Resource
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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.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0533
    Keywords: Key words Dentate nucleus ; Dentatorubro-pallidoluysian atrophy ; Progressive supranuclear palsy ; Purkinje cell ; Torpedo
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cerebellar torpedoes can be induced by direct damage to Purkinje cell axons. This raises the possibility that the loss of dentate nucleus neurons (DNNs) may also cause torpedo formation through synaptic detachment between DNNs and Purkinje cell axon terminals. To investigate this possibility, we conducted a morphometric study with an image analyzer in progressive supranuclear palsy (PSP) and dentatorubro-pallidoluysian atrophy (DRPLA). Using horizontal and sagittal sections of the cerebellar hemispheres containing the greatest proportion of the dentate nucleus, we determined the line densities of torpedoes, Purkinje cells, and DNNs. In PSP and DRPLA, the densities of DNNs were significantly lower, and the densities of torpedoes much greater than in controls, while those of Purkinje cells were normal in both diseases. In addition, the torpedo densities in PSP and DRPLA showed a strong negative correlation with the DNN densities. Thus, this study clearly demonstrated that torpedoes are formed in association with the loss of DNNs, suggesting that they may occur in consequence of synaptic disconnection of Purkinje cells from DNNs.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0533
    Keywords: Key words Paired helical filament ; Neurofibrillary ; tangles ; Scanning electron microscopy ; Alzheimer’s ; disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Neurofibrillary tangles (NFTs) have been ultrastructually studied by various methods, leading to several three-dimensional models of paired helical filaments (PHFs). In this study, we present the scanning electron microscopic findings of NFTs in an autopsy case of Alzheimer’s disease and clarify the three-dimensional structures of NFTs. NFTs were clearly defined in freeze-cracked nerve cells and consisted of two types of filamentous structures, straight and helical filaments. Straight filaments measured from 20 to 25 nm in diameter and had a smooth surface. They were slightly bent but mostly straight with no constrictions. One type of straight filaments ran in a bundle in the same direction, another was intertwined to each other. Most of the helical profiles of filaments usually measured about 28 nm in diameter, with a distance of 100 nm between periodic constrictions. They seemed to consist of a pair of isodiametric filaments of 10 nm in diameter. In addition, two unusual types of helical filaments were occasionally observed. One comprised thick filaments of about 38 nm in diameter, with a distance of 100 nm between constrictions; these helical filaments appeared to consist of two or more strands. The other comprised thin helical filaments of about 20 nm in diameter and regularly constricted at an interval of 50 nm. All types of the helical filaments examined in this case were leotropic. This result supports a protofilament model of PHFs. Scanning electron microscopy using the freeze-cracked and maceration method is a useful and simple method for three-dimensional observation of the filamentous structures in NFTs.
    Type of Medium: Electronic Resource
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