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  • 1
    Electronic Resource
    Electronic Resource
    Unusual orthochromatic leukodystrophy with epitheloid cells (Norman-Gullotta): increase of very long chain fatty acids in brain discloses a peroxisomal disorder (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 187-189 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00334888
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  • 2
    Electronic Resource
    Electronic Resource
    Motor neuron disease with pallido-luysio-nigral atrophy (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 105-108 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00454908
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  • 3
    Electronic Resource
    Electronic Resource
    Desmoplastic ganglioglioma: report of two non-infantile cases (1993)
    Springer
    Acta neuropathologica 85 (1993), S. 199-204 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00227768
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  • 4
    Electronic Resource
    Electronic Resource
    Infantile multiple system atrophy with cytoplasmic and intranuclear glioneuronal inclusions (1994)
    Springer
    Acta neuropathologica 87 (1994), S. 642-647 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00293326
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  • 5
    Electronic Resource
    Electronic Resource
    Down's dyndrome and Alzheimer's disease: dendritic spine counts in the hippocampus (1990)
    Springer
    Acta neuropathologica 79 (1990), S. 680-685 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00294247
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  • 6
    Electronic Resource
    Electronic Resource
    Infantile multiple system atrophy with cytoplasmic and intranuclear glioneuronal inclusions (1994)
    Springer
    Acta neuropathologica 87 (1994), S. 642-647 
    Details
    ISSN: 1432-0533
    Keywords: Infantile multiple system atrophy ; Ubiquitin ; Neuronal intraunclear 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
    URL: http://dx.doi.org/10.1007/BF00293326
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    Paper (German National Licenses)
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  • 7
    Electronic Resource
    Electronic Resource
    Ocular findings in Walker-Warburg syndrome (1993)
    Springer
    Child's nervous system 9 (1993), S. 418-420 
    Details
    ISSN: 1433-0350
    Keywords: Walker-Warburg syndrome ; Ophthalmological findings ; Retinal dysplasia ; Rieger's anomaly ; Peters' anomaly ; Pseudoglioma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Ocular symptoms are frequently observed in Walker-Warburg (WWS) and associated syndromes. The majority of patients present with malformations of the anterior segment and severe retinal dysplasia. We report on the findings in a female patient with WWS who died at the age of 9 months. Major ocular findings were: severe iridocorneal malformation, a membran-like structure of the lens and funnel-shaped retinal dysplasia. The retina presented various grades of differentiation with rosettes and atypical sequences of cells, e.g. ganglion cells intermingled between granular layers. The anterior part of the retina presented as a primitive homogeneous layer with a cell-free space that might be interpreted as the primary optic ventricle. This finding suggests that we are dealing with a primary dysplastic non-attachment rather than a real detachment of the retina in WWS. The malformation of the anterior segment was not typical of the Peters' anomaly, as usually described in WWS, but of Rieger's syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00306196
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  • 8
    Electronic Resource
    Electronic Resource
    Pleomorphic pineocytoma with extensive neuronal differentiation: report of two cases (1994)
    Springer
    Acta neuropathologica 88 (1994), S. 448-453 
    Details
    ISSN: 1432-0533
    Keywords: Key words Pineal parenchymal tumors ; Pineocytoma ; Pineoblastoma ; Central neurocytoma ; Neuronal differentiation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two pineal parenchymal tumors are presented, arising in a 54-year-old man and a 72-year-old woman; respectively. They showed isomorphic, cellular areas of small cells, often with characteristic pineocytomatous rosettes, and of medium-sized cells, as well as less cellular regions with highly pleomorphic, often ganglioid large cells. Immunohistochemistry disclosed extensive neuronal differentiation. There was intense positivity for neurofilament protein and microtubule-associated protein 2 in the pleomorphic areas and more variable expression in the isomorphic regions. Diffuse synaptophysin positivity was seen, accentuated along the borders of pleomorphic cells and in the rosettes, as well as diffuse interstitial and/or cytoplasmic expression of neuron-specific enolase, PGP 9.5 and tau. β-Tubulin III was detected in most cells and slight positivity was found in the rosettes. Expression of glial fibrillary acidic protein, however, was restricted to resident astrocytes and an interstitial network of processes. These neuronally differentiated pleomorphic pineocytomas underline the broad histomorphological spectrum of pineal parenchymal tumors.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00389497
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  • 9
    Electronic Resource
    Electronic Resource
    Immunohistochemistry in childhood brain tumors: what are the facts? (1990)
    Springer
    Child's nervous system 6 (1990), S. 118-122 
    Details
    ISSN: 1433-0350
    Keywords: Immunohistochemistry ; Brain tumors in childhood
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In the past, contradictory results have been reported concerning the specificity of neuronal or glial cell markers. However, we have investigated this aspect in a large group of more than 550 brain tumors (among them 60 medulloblastomas). These contradictions can easily be explained by considering two basic facts. First, every neoplastic cell population, especially in embryonic tumors, diffusely infiltrates the brain tissue: non-neoplastic cells, intermingled with tumor cells, can therefore give rise to immunohistochemical and histogenetic misinterpretations. Second, different cell markers can be expressed by one and the same cell (e.g., GFAP, NSE, vimentin), making nosological interpretation of the tumor difficult, impossible, or at best rather subjective. Clear-cut marker positivity is mostly found in the differentiated tumors for which the nosological classification is already clear by the usual histological methods. Only synaptophysin seems to be a reliable marker for neurogenic cells. In embryonic brain tumors (so-called PNET), no correlations between the presence of a given cell marker and the biological behavior of the tumor have so far been detected.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00308484
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  • 10
    Electronic Resource
    Electronic Resource
    Summary and conclusions (1993)
    Springer
    Child's nervous system 9 (1993), S. 421-421 
    Details
    ISSN: 1433-0350
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00306197
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