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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 16 (1970), S. 271-284 
    ISSN: 1432-0533
    Keywords: Medulloblastoma ; Electron Microscopy ; Arachnoid Sarcoma ; Lymphocyte-Like Cells ; Dark Cells ; Histogenesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung 9 Medulloblastome wurden elektronenmikroskopisch untersucht. Sie zeigten eine relativ einheitliche histologische und cytologische Struktur. Die Tumorzellen haben eine wechselnde Form, wenig Organellen und in der Regel zahlreiche cytoplasmatische Fortsätze. In Gewebspartikeln, die nach der vorherigen lichtmikroskopischen Untersuchung aus dem Tumorzentrum stammten, wurden keine Zellen mit glialer oder neuronaler Differenzierung beobachtet. Nur in den am Tumorrand gelegenen Infiltrationszonen, wo die Tumorgrenze nicht mehr sicher zu bestimmen war, fanden sich gliale oder neuronale Zellelemente. — Das Medulloblastom hat die wesentlichen morphologischen Kriterien der kleinzelligen Sarkome und des embryonalen Gewebes. Deren gemeinsames Charakteristicum, daß sie nämlich keine speziellen Differenzierungen besitzen, ist auch eine spezifische Eigenschaft des Medulloblastoms.
    Notes: Summary 9 medulloblastomas were investigated by electron microscopy. They all showed a rather unique histological and cytological structure. The cells were differently shaped, had in general many cytoplasmic processes and only few organells. Special differentiations which would have made tumour elements look like glial or neural cells were not observed when the tissue samples under investigation were carefully selected by light microscopical examination. Glial or neural elements were found only in infiltration zones where no clear distinction between pre-existing cerebellar and tumourous tissues could be made by conventional histological investigation. Medulloblastomas have essentially the same appearance as parvicellular sarcomas or embryonic tissues. Their common characteristic, viz. that they usually show no special differentiation, is also the most specific quality of medulloblastomas.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Leukodystrophy ; Very long chain fatty acids ; Phytanic acid ; Peroxisomal disease ; Adrenoleukodystrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Very long chain fatty acids (VLCFA) were found to be markedly increased and phytanic acid was borderline above normal in formalin-fixed brain white matter of case with an unusual type of familial leukodystrophy with epitheloid cells as described previously by Gullotta et al. [Neuropädiatrie (1970) 2: 173–186]. Increased VLCFA in brain clearly demonstrate that the patient had suffered from a peroxisomal disease. This diagnosis is corroborated by ultrastructural findings in brain showing typical lamellar inclusions. The particular type of peroxisomal disorder present in case (heterozygote of X-linked adrenoleukodystrophy?) remains speculative.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Muscular dystrophy ; Becker-type ; Benign X-linked ; Histopathology ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Muscle biopsies of two patients originally reported in the Göttingen family by Becker (1962) that formed the basis of separating a benign X-linked muscular dystrophy from the rapidly progressive Duchenne-type X-linked muscular dystrophy, revealed mild pathological changes in the younger patient and more advanced in the older one, consisting of increased spectra of fiber diameters, endomysial fibrosis, angulated fibers, pyknotic nuclear clumps and small groups of atrophic fibers. Essentially, both biopsies showed the same changes, but of different severity, possibly due to the differences in age and muscle biopsy sites. These changes were regarded “myopathic”, but a neurogenic component was suggested. Our observations accord well with those of a larger series (Bradley et al., 1978) where both electromyography and histopathology revealed a mixed “myopathic-neurogenic pattern” in patients with Becker-type dystrophy. Differential diagnostic aspects encompass Duchenne's muscular dystrophy, the other hereditary dystrophies and X-linked proximal spinal muscular atrophies. The precise nature of Becker-type muscular dystrophy requires morphological data on peripheral nerves, spinal roots and spinal cord anterior horn cells as well as sequential biopsy analysis to substantiate the primary site of pathology. However, on the basis of available data, it seems reasonable to suggest that the early changes of degeneration/regeneration which are accompanied by a markedly elevated CPK eventuate in the histopathologic and electromyographic patterns illustrated in these two patients with Beckertype dystrophy.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 86 (1993), S. 105-108 
    ISSN: 1432-0533
    Keywords: Pallido-luysio-nigral atrophy ; Motor neuron disease ; Ubiquitin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of motor neuron disease (MND) with pallido-luysio-nigral atrophy (PLNA) is reported. The 45-year-old male patient presented with lower motor neuron symptoms and signs of basal ganglia disturbance. He died after a progressive course of 7 months. Neuropathological examination revealed motor neuron loss at all spinal cord levels with sparing of Onuf's nucleus. Nerve cell loss and gliosis were also present in substantia nigra, globus pallidus, and subthalamic nucleus. The presence of ubiquitin-positive inclusions, a hallmark of most variants of MND, confirms this case as an example of MND. At immunoelectron microscopy the granules were distributed on filamentous material. The combination of clinically apparent PLNA with MND has only been described twice previously. The relationship of this syndrome to other forms of MND and its nosological placement are discussed.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Tissue Culture ; Electron Microscopy ; Enzyme Histochemistry ; Sympathetic Ganglioneuroblastoma ; Medulloblastoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The results of combined tissue culture, ultrastructural and enzyme histochemical investigations carried out on a sympathetic ganglioneuroblastoma are reported.In vitro a vigorous sprouting of newly formed neuritic processes was observed a few days after explantation. The enzymatic reactions for acethylcholinesterase were positivein situ as well asin vitro. The tumor elements showed the ultrastructural characteristics of nerve cells with many microtubuli and filaments, plenty of ribosomes, well developed endoplasmic reticulum. Dense bodies, corresponding to neurosecretion granula (catecholamines) were also observed. Synaptic structures were missing. The results of these investigations confirm that this kind of tumor is different from so-called medulloblastoma.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Craniopharyngioma ; Tissue Culture ; Electron Microscopy ; Enzyme Histochemistry ; Keratine ; Enamel ; Rosenthal Fibers
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Electron optical and enzyme histochemical investigations carried out on four craniopharyngiomas and their tissue cultures demonstrated that the tumour elements are keratinizing epithelial cells, plenty of tonofilaments, glycogen granules, mitochondria and desmosomes. Their ultrastructural and histochemical characteristics are the same in every part of the tumour (solid; cystic; “adamantinoma-like”). In the keratinizing cells, the reactions for non-specific esterases were high positive. The ultrastructural characteristics of the tumour cells grownin vitro are the same as thosein situ; the cells remain attached to one another by desmosomes and retain their capacity to produce keratine. This therefore seems to be a primary characteristic of the tumour cells and not a secondary dysmetabolic disturbance. Calcium was found onlyin situ. That the tumour cells may produce enamelin situ seems to be possible, but it could not be confirmed with certainty. The glial proliferation which is always presentin situ, is reactive and not neoplastic; thein vitro new built cell colonies consist only of epithelial elements.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 85 (1993), S. 199-204 
    ISSN: 1432-0533
    Keywords: Desmoplastic infantile ganglioglioma ; Ganglioglioma ; Desmoplasia ; Gliofibroma ; Neurofilament polypeptides
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two supratentorial desmoplastic gangliogliomas arising in a 15-year-old boy and a 25-year-old man are reported. Both tumors reached the brain surface and exhibited large cysts. They showed intense desmoplasia and tumor cells of astrocytic and ganglionic differentiation. In one case the ganglionic nature was only demonstrable by immunohistochemistry. Such neoplasms can no longer be regarded as exclusively infantile brain tumors.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: KeyWordsInfantile multiple system atrophy Ubiquitin ; Neuronal intranuclear hyaline inclusion disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report presents a case of infantile multiple system atrophy with probably autosomal recessive inheritance. The female patient developed generalized muscular hypotonia, myoclonias and tonic-clonic seizures at the age of 8 months, followed by gradual development of choreoathetotic hyperkinesia and increasing psychomotor retardation. Metabolic disease was ruled out and the child died of aspiration pneumonia at the age of 5 years. General autopsy was unremarkable, but neuropathological examination showed degeneration of cerebellum, inferior olives, medial thalamus, Clarke's nucleus, anterior horn cells, corticospinal, spinocerebellar tracts, and posterior columns. Immunohistochemically many neurons contained intranuclear and intracytoplasmic ubiquitin-positive inclusions, which did not contain neurofilament or tau epitopes and ultrastructurally consisted of granulofilamentous material. We tentatively classify this case as a form of infantile multiple system atrophy linked to neuronal intranuclear hyaline inclusion disease.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0533
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Electron microscopic and enzyme histochemical investigations were carried out on a group of spongioblastomas and astrocytomas and their tissue cultures. Both neoplastic cell populations are ultrastructurally identical, but show differences in the late stages of cultivation,viz. in the modality of degeneration. The cytoplasm of “spongioblasts” becomes progressively overloaded with filaments, floccular material and fine granular osmiophilic masses, which leads to cell necrosis. These osmiophilic masses correspond to Rosenthal fibers of light microscopy. In some cells, before Rosenthal fibers appear, the granular ground substance is thickened, particularly close to the Golgi apparatus and the ribosomes are increased in number. At times, some mitochondria show an abnormal dense homogeneous matrix. This peculiar cell degeneration seems to be not the consequence of a simple overproduction of filaments, but a manifold cellular disorder akin to a storage dystrophy.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 79 (1990), S. 680-685 
    ISSN: 1432-0533
    Keywords: Down's syndrome ; Alzheimer's disease ; Hippocampus ; Golgi method ; Dendritic spines
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Samples of the hippocampus of four patients with Down's syndrome [two men aged 35 and 36 years with no evidence of Alzheimer's disease (AD) and two patients aged 47 and 55 years with associated AD] were obtained at post mortem and processed according to the rapid Golgi method. A significant reduction in the number of dendritic spines (DS) was found in the apical (middle, distal and oblique segments) and basilar (thick and thin segments) dendritic arbors of CA1 and CA2–3 pyramidal neurons in patients with Down's syndrome and no AD when compared to age-matched controls. An additional decrease of DS in every segment occurred in Down's patients with associated AD when compared to agematched controls and Down's patients with no AD. In Down's syndrome (either associated or not to AD) thin basilar dendrites were the most severely involved; in AD patients CA1 pyramids were more severely affected than pyramidal neurons of the CA2–3 subfield.
    Type of Medium: Electronic Resource
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