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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 75 (1987), S. 8-15 
    ISSN: 1432-0533
    Keywords: Choline acetyltransferase ; Lewy body ; Parkinson's disease ; Somatostatin ; Thioflavine S
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Post-mortem pathological and biochemical studies are reported on six patients with progressive dementia. The characteristic pathological finding was neurofilament-containing cytoplasmic inclusions in cortical and subcortical neurons. The clinical and pathological findings were consistent with so-called diffuse Lewy body disease. The patients had variable changes of the Alzheimer type, with five of six patients displaying “plaques only” Alzheimer's changes. Biochemical studies showed profound decreases in neocortical choline acetyltransferase (ChAT) activities that correlated with marked neuronal loss in the basal nucleus of Meynert. ChAT activities were normal in the hippocampus in three patients who also had no significant Alzheimer type hippocampal changes. All patients had decreased cortical somatostatin-like immunoreactivity. Our observations suggest that dementia in diffuse Lewy body disease bears biochemical similarities to Alzheimer's disease, in that biochemical markers for both intrinsic cortical neurons and ascending cholinergic neurons are affected.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 56 (1982), S. 75-77 
    ISSN: 1432-0533
    Keywords: Bielschowsky bodies ; Lafora body ; Status marmoratus ; Polyglucosan ; Anoxic encephalopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Intraneuronal inclusions, consisting of polyglucosan and having histochemical and ultrastructural features identical to Lafora body of familial myoclonic epilepsy (Unverrict-Lafora disease), have been found restricted to the lateral pallidum in five patients. Two of these patients were also found to have status marmoratus of the basal ganglia. These lateral pallidal inclusions have been named after Bielschowsky, their original discoverer. We report two additional patients with status marmoratus and Bielschowsky bodies and suggest that these two conditions are frequent concomitant phenomena arising independently from a common cause.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 61 (1983), S. 36-42 
    ISSN: 1432-0533
    Keywords: Astrocytes ; Retina ; Optic nerve
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The neuropathologic and ophthalmopathologic findings in a 53/4-year-old boy with Alexander's disease are reported. Light- and electron-microscopic and immunohistochemical studies revealed that (1) the granular osmiophilic deposits (GOD) in Alexander's disease accumulate mainly in astrocytic processes to form Rosenthal fibers, (2) the Bergmann glia are different in this regard and accumulate the deposits primarily in their perikarya, (3) the Müller cells of retina (which closely resemble astrocytes) do not accumulate GOD, (4) the deposits are also not present in other glial cells and glial-like cells such as pituicytes and pineocytes, (5) the deposits are sparse in the retrobulbar optic nerves, and (6) the peroxidase-antiperoxidase and immunofluorescence studies did not demonstrate glial fibrillary acidic protein (GFAP), albumin, immunoglobulins, or fibrinogen in the astrocytic deposits. The differential deposition of GOD in various cytoplasmic regions of astrocytes in different areas of central nervous system (CNS) suggests that astrocyte metabolism may not be uniform throughout the brain. Attention to this point may prove helpful in understanding the pathogenesis of the deposits in Alexander's disease. The absence of immunohistochemically demonstrable plasma proteins and GFAP in the astrocytic GOD indicates that the latter have an origin different from plasma proteins and glial filaments. Alternatively, the deposits may be derived from these proteins, but their antigenicity has since been altered.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 57 (1982), S. 165-170 
    ISSN: 1432-0533
    Keywords: Dendrites ; Dystrophic dendrites ; Golgi methods ; Ischemia-anoxia malnutrition ; Purkinje cells ; Secretory diarrhea
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A $$6{\raise0.5ex\hbox{$\scriptstyle 1$}\kern-0.1em/\kern-0.15em\lower0.25ex\hbox{$\scriptstyle 2$}}$$ month old male infant presented a week after his birth with secretory diarrhea of unknown etiology. He was sustained by central hyperalimentation for the rest of his life, and treated for presumed sepsis with a wide variety of antibiotics. The brain showed vacuolation in the diencephalic nuclei and white matter of the brain stem. There were also many clusters of enlarged Purkinje cell dendrites in the molecular layer. In Golgi preparations the primary and secondary dendrites showed segmental swellings and absent tertiary branchlets. The swellings were due to remarkable accumulations of mitochondria. The pathogenesis of the dendritic changes is discussed, and ‘dying back’ phenomenon is proposed to explain the changes.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 77 (1988), S. 219-223 
    ISSN: 1432-0533
    Keywords: Meningioma ; Folliculo-stellate cell ; Pituitary neoplasm ; Electron microscopy ; Immunochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A tumor arising in the pituitary fossa and having some of the histological and ultrastructural features of a recently described tumor, purportedly originating from the folliculo-stellate cells of the anterior pituitary, is presented. The results of our ultrastructural and immunohistochemical studies, however, favored a meningeal origin and suggested that the neoplasm was most likely a secretory meningioma.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Neurons ; Caudate ; Substantia nigra ; Inclusions ; Actin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The composition of the eosinophilic inclusions of the large neurons in the human caudate nucleus and of neurons in the substantia nigra was investigated by immunocytochemical methods. Sections of caudate nucleus and substantia nigra were stained using a peroxidase-anti-peroxidase method with antisera to actin, to neurofilament proteins, and to a crude CNS microtubule fraction, the last of which reacts with neurofibrillary tangles. Of the several antisera, only the anti-actin antiserum gave positive results, indicating that these inclusions, composed of regularly arranged filaments, are highly ordered aggregates of actin polymers.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 67 (1985), S. 326-328 
    ISSN: 1432-0533
    Keywords: Anencephaly ; Immunoreactive keratinin ; Foreign body inflammation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Keratinous material, in the form of epithelial squames often with foreign body reaction and dense gliosis, was demonstrated in all of ten cases of anencephaly by a sensitive and specific immunohistochemical stain. In some cases keratin was not recognized on routine histological stains.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 71 (1986), S. 38-45 
    ISSN: 1432-0533
    Keywords: Alzheimer's disease ; Pick bodies ; Neurofibrillary degeneration ; Thioflavine-S ; Immunocytochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Pick body-like inclusions are described in the granular neurons of the dentate fascia in Alzheimer's disease. The inclusions are round, argyrophilic and stained by thioflavine-S. Immunocytochemically they contain antigenic determinants of neurofilaments and of Alzheimer neurofibrillary tangles. Ultrastructurally they are composed primarily of 15–18 nm straight filaments similar to the neurofibrillary pathology of progressive supranuclear palsy and Pick's disease. The dentate fascia inclusions, as well as cerebellar plaques but not amyloid angiopathy, are found most frequently in association with severe neurofibrillary degeneration.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 79 (1990), S. 692-696 
    ISSN: 1432-0533
    Keywords: Arthrogryposis multiplex congenita ; Spinal cord dysplasia ; Hypoplasia, posterior spinal roots ; Spinal root ganglia, rudimentary ; Dysplasia, inferior olivary nuclei
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report a male infant with arthrogryposis multiplex congenita (AMC) who survived for 19 weeks following birth at 36 weeks gestational age. No heritable or acquired cause of neuromuscular disease was found. He manifested joint contractures of upper and lower extremities, diffuse hypotonia requiring ventilatory support, and areflexia; the general examination also showed facial dysmorphisms, and an ichthyotic rash. Pathological examination of the brain and spinal cord revealed severe hypoplasia of dorsal roots and posterior columns, nondecussation of pyramidal tracts, and anterior horns of an unusual configuration: the brain was normal, and the cerebellum contained Purkinje cell heterotopias. Muscle spindles could not be identified. To our knowledge, these spinal cord abnormalities in association with AMC have not been previously reported, thus raising interesting speculations about the possible role of such abnormalities in the pathogenesis of AMC.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 81 (1991), S. 287-295 
    ISSN: 1432-0533
    Keywords: Striatonigral degeneration ; Olivopontocerebellar atrophy ; Pick's disease ; Ubiquitinated bodies ; Glial/Oligodendroglial cytoplasmic inclusions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 75-year-old woman with parkinsonism plus was found at autopsy to have striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA) and intracytoplasmic neuronal inclusions, mostly confined to the hippocampus and pontine nuclei. These inclusions were intensely argyrophilic, ubiquitinated and expressed variable immunoreactivity for neurofilament but not for tau-1 and Alz 50 proteins. Ultrastructurally, they were formed of skeins of intermediate filaments averaging 11 nm in diameter. They were considered to represent Pick bodies. There was no cortical atrophy, gliosis or sponginess. To our knowledge, SND and OPCA in association with Pick's disease has not been previously reported. In addition, intracytoplasmic oligodendroglial inclusions were present in the deeper layers of the cortex, especially the pericentral gyri, the striatum and the white matter of certain regions of the cerebral hemispheres, as well as in the cerebellum. These inclusions which have been previously reported in multisystem atrophy, had to be distinguished from cortical Lewy bodies, Pick bodies, and the nonspecific ubiquitinated bodies in the white matter of the aged brain, mainly by their topographical distribution and immunostaining properties.
    Type of Medium: Electronic Resource
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