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  • 11
    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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  • 12
    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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  • 13
    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: 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 under-line 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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  • 14
    Electronic Resource
    Electronic Resource
    Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL): a morphological study of a German family (1996)
    Springer
    Acta neuropathologica 92 (1996), S. 341-350 
    Details
    ISSN: 1432-0533
    Keywords: Key words Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) ; Hereditary multi-infarct dementia ; Skin biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized clinically by recurrent cerebral infarcts, subcortical dementia and pseudobulbar palsy, and morphologically by a granular degeneration of cerebral and, to a lesser degree, extracerebral blood vessels. We present morphological findings in a further German family affected by CADASIL. The index case showed the typical periodic acid-Schiff-positive granular degeneration of vascular smooth muscle cells (VSMC) in cerebral vessels, which did not react with antibodies against various immunoglobulins or complement factors. Ultrastructurally, granular osmiophilic material (GOM) covered the VSMC in different cerebral regions as well as in extracerebral organs (muscle, nerve, skin, small and large intestine, liver, kidney and heart). Skin biopsy samples from other family members of the last two generations also revealed GOM irrespective of the clinical symptomatology (CADASIL, migraine only or asymptomatic). Patients in the third generation had higher amounts of GOM in skin vessels than did asymptomatic or migraine patients in the fourth generation. We conclude that skin biopsy is a useful and less-invasive screening method for the differential diagnosis of CADASIL.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050528
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  • 15
    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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  • 16
    Electronic Resource
    Electronic Resource
    Dysembryoplastic neuroepithelial tumour of the cerebellum (1995)
    Springer
    Acta neuropathologica 89 (1995), S. 385-390 
    Details
    ISSN: 1432-0533
    Keywords: Key words Dysembryoplastic neuroepithelial tumour ; Cerebellum ; Cerebellar astrocytoma ; Granule neurons
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A case of dysembryoplastic neuroepithelial tumour of the cerebellum occurring in a 28-year-old woman is presented. The lesion extended from the cortex of the inferior vermis upwards into the white matter. Histologically, it exhibited areas of microcystic cerebellar astrocytoma and glial regions with hamartomatous blood vessels as well as areas with oligodendrocyte-like cells (OLC) with a delicate, fibrillary stroma lying in a mucinous, often microcystic matr ix. The OLC showed prominent rosette formation and immunohistochemical features suggesting neuronal, i.e. granule cell, differentiation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00309634
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  • 17
    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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    Paper (German National Licenses)
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  • 18
    Electronic Resource
    Electronic Resource
    Dysembryoplastic neuroepithelial tumour of the cerebellum (1995)
    Springer
    Acta neuropathologica 89 (1995), S. 385-390 
    Details
    ISSN: 1432-0533
    Keywords: Dysembryoplastic neuroepithelial tumour ; Cerebellum ; Cerebellar astrocytoma ; Granule neurons
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A case of dysembryoplastic neuroepithelial tumour of the cerebellum occurring in a 28-year-old woman is presented. The lesion extended from the cortex of the inferior vermis upwards into the white matter. Histologically, it exhibited areas of microcystic cerebellar astrocytoma and glial regions with hamartomatous blood vessels as well as areas with oligodendrocyte-like cells (OLC) with a delicate, fibrillary stroma lying in a mucinous, often microcystic matrix. The OLC showed prominent rosette formation and immunohistochemical features suggesting neuronal, i.e. granule cell, differentiation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00309634
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    Paper (German National Licenses)
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  • 19
    Electronic Resource
    Electronic Resource
    Medullomyoblastoma: a histological, immunohistochemical, ultrastructural and molecular genetic study (1998)
    Springer
    Acta neuropathologica 95 (1998), S. 205-212 
    Details
    ISSN: 1432-0533
    Keywords: Key words Medullomyoblastoma ; Medulloblastoma ; c-myc ; c-erb-B2 ; Allelic loss
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Medullomyoblastoma is a rare variant of medulloblastoma containing myoblastic elements. A 9-year-old boy developed a cerebellar syndrome and signs of increased intracranial pressure, the cause of which was a tumor of the cerebellar vermis measuring 7 × 4.5 × 4.5 cm. Morphologically the tumor largely consisted of a medulloblastoma component but displayed glial, myoblastic and ganglionic differentiation on light microscopic, immunohistochemical and ultrastructural examination. The non-enhancing rim of the tumor on magnetic resonance imaging showed extensive ganglionic differentiation. The tumor did not express bcl-2, c-myc, or c-erb-B2 oncoproteins and was negative for the p53 gene product. On molecular genetic studies, the tumor did not show allelic loss on chromosome loci, frequently altered in medulloblastomas, such as 17p, 1q and 9q.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050788
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  • 20
    Electronic Resource
    Electronic Resource
    Primary malignant rhabdoid tumours of the central nervous system: An immunohistochemical and ultrastructural study (1997)
    Springer
    Acta neurochirurgica 139 (1997), S. 961-969 
    Details
    ISSN: 0942-0940
    Keywords: Malignant rhabdoid tumour ; atypical teratoid/rhabdoid tumour
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Three cases of primary rhabdoid tumour of the CNS (RT-CNS) are presented. In case 1 a hemispheric tumour developed in a 10.5 months old girl, who survived for 6 months after incomplete resection, radio- and polychemotherapy. Case 2 was a 4 years and 8 months old boy with a large IIIrd ventricle tumour, who died of leptomeningeal tumour dissemination 7 months after diagnosis despite radiotherapy. In case 3 a pineal mass occurring in a 14 month old female was radioresistant and totally exstirpated. The child died due to tumour recurrence two months later. Autopsy examination revealed widespread leptomeningeal dissemination. All three cases fulfilled light and electron microscopic criteria of RT-CNS including abundant eosinophilic cytoplasm, vesicular nuclei with large nucleoli and conspicuous anti-vimentin positive filaments. Extensive immunohistochemical studies showed expression of epithelial (EMA, KL1), macrophage (alpha-1 antichymotrypsin), neuro-ectodermal (GFAP, NSE, β-tubulin III) and myogenic markers (desmin, actin). Different stress proteins (alpha-B crystallin, HSP70) were also expressed. Tumour cells showed a proliferation (MIB1) index of 28.4% (case 1) and 33.4% (case 2). From our study it can be concluded that RT-CNS reveals significant immuno-morphological heterogeneity thus supporting the view that it is not a specific pathological entity but merely a phenotypic appearance of different neoplasms, some of which are linked to primitive neuro-ectodermal tumours.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01411306
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