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  • 1
    ISSN: 1432-1920
    Keywords: Key words Papilloma, choroid plexus ; Tumour seeding
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A suprasellar location of a benign choroid plexus papilloma is reported. Local recurrence within the fourth ventricle was also present, 8 years after apparently complete removal. Imaging and histological findings were similar to those of the initial lesion. At surgery, the suprasellar lesion had no connection with the ventricular system. Seeding of choroid plexus papillomas is discussed, and the pertinent literature reviewed.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Neuroradiology 40 (1998), S. 743-747 
    ISSN: 1432-1920
    Keywords: Key words Meningioma ; Spine ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Meningiomas are rare tumours in children and lumbar lesions are exceptional. We report a clear-cell meningioma (CCM) of the cauda equina in a 10-year-old girl. The tumour was diagnosed by MRI, showing an enhancing intradural mass extending from L1 to L4. Pathology and immunohistochemical study demonstrated a CCM. The patient had a recurrence 6 months after the operation requiring further surgery. CCM are rare lesions, characterised by abundant cytoplasmic glycogen particles. Complete surgical removal is necessary because, despite their benign histological appearance, CCM are potentially aggressive and may recur, spread locally and even metastasize.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 75 (1987), S. 104-108 
    ISSN: 1432-0533
    Keywords: Motor neuron disease ; Parkinsonism ; Dementia ; Lewy body
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinicopathological finding in a 49-year-old man who presented multisystemic neurological degenerative disease are reported. The patient presented, at the age of 36, distal upper limb amyotrophy and 8 years later pyramidal signs with fasciculations. In his last year of life, he suffered extrapyramidal hypertonus and mental deterioration. Neuropathological examination showed anterior spinal root and cerebral atrophy. Myelin pallor was mild and predominated on posterior cords. Anterior horn neuronal loss was noted in the spinal cord as well as Alzheimer-type changes in the brain. Inclusion bodies consistent with Lewy bodies were diffusely apparent. The peculiar clinical progression in this case and the extension of neuropathological lesions with inclusion bodies mainly in the substantia nigra and cerebral cortex are an interesting subject of discussion.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Castleman disease ; Monoclonal gammopathy ; Myelin ; Peripheral neuropathy ; POEMS syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Mechanisms of peripheral neuropathies in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome are poorly understood. A peripheral nerve biopsy was performed in 22 patients. Of these 9 had histological features of Castleman's disease on lymph node biopsies, and 19 had a monoclonal lambda light chain in their serum. Certain nerve fragments were paraffin embedded, others were frozen and studied by direct immuno-fluorescence, and others were fixed for ultrastructural examination. Paraffin-embedded fragments did not show any amyloid deposits, and at direct immunofluorescence there was no immunoglobulin fixation. At ultrastructural examination, features of uncompacted myelin lamellae (UML) were present in 19 patients, and their frequency varied from 1% to 16% of myelinated fibres. Up to now UML have been reported only in 7 patients with POEMS syndrome in the literature. UML have also been noticed in a few cases of inflammatory demyelinating polyradiculoneuritis and inherited tendency to pressure palsy.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Key words     Primitive neuroepithelial tumor ; Desmoplastic small cell tumor ; Brain tumor of infancy ; Immunocytochemistry ; Genetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract      We describe a case of a desmoplastic brain tumor which was initially resected from the right fronto-temporal region in a 2 year-old boy. This nodular, calcified tumor was vascularized by the internal carotid artery and the middle meningeal artery branches. Grossly, it contained several mucoid cysts. Light microscopy showed cords or nests of small cuboidal cells surrounded by a loose connective tissue and desmoplasic areas containing fibers and spindle cells. The cuboidal cells expressed epithelial, neuronal and neuroendocrine markers. Some foci of spindle cells showed glial differentiation. The tumor recurred 16 months later and displayed some characteristics of the small cell neuroepithelial component, mitoses being conspicuous. Electron microscopy revealed undifferentiated clear cells, some containing neurosecretory granules. Karyotyping demonstrated the following formula: 〈 15 〉 46, t(8;11) (q13; q11). The chromosome 11 breakpoint was different from that described in Ewing's sarcoma. This isolated translocation has not been previously reported to our knowledge. These unusual features lead us to report this case and to discuss its pathogenesis.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Key words: Castleman disease – Monoclonal gammopathy – Myelin – Peripheral neuropathy – POEMS syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Mechanisms of peripheral neuropathies in polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome are poorly understood. A peripheral nerve biopsy was performed in 22 patients. Of these 9 had histological features of Castleman's disease on lymph node biopsies, and 19 had a monoclonal lambda light chain in their serum. Certain nerve fragments were paraffin embedded, others were frozen and studied by direct immunofluorescence, and others were fixed for ultrastructural examination. Paraffin-embedded fragments did not show any amyloid deposits, and at direct immunofluorescence there was no immunoglobulin fixation. At ultrastructural examination, features of uncompacted myelin lamellae (UML) were present in 19 patients, and their frequency varied from 1% to 16% of myelinated fibres. Up to now UML have been reported only in 7 patients with POEMS syndrome in the literature. UML have also been noticed in a few cases of inflammatory demyelinating polyradiculoneuritis and inherited tendency to pressure palsy.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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  • 7
    ISSN: 1432-0533
    Keywords: Primitive neuroepithelial tumor ; Desmoplastic small cell tumor ; Brain tumor of infancy Immunocytochemistry ; Genetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a case of a desmoplastic brain tumor which was initially resected from the right fronto-temporal region in a 2 year-old boy. This nodular, calcified tumor was vascularized by the internal carotid artery and the middle meningeal artery branches. Grossly, it contained several mucoid cysts. Light microscopy showed cords or nests of small cuboidal cells surrounded by a loose connective tissue and desmoplasic areas containing fibers and spindle cells. The cuboidal cells expressed epithelial, neuronal and neuroendocrine markers. Some foci of spindle cells showed glial differentiation. The tumor recurred 16 months later and displayed some characteristics of the small cell neuroepithelial component, mitoses being conspicuous. Electron microscopy revealed undifferentiated clear cells, some containing neurosecretory granules. Karyotyping demonstrated the following formula: 〈 15 〉 46, t(8;11) (a13; q11). The chromosome 11 breakpoint was different from that described in Ewing's sarcoma. This isolated translocation has not been previously reported to our knowledge. These unusual features lead us to report this case and to discuss its pathogenesis.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neurochirurgica 140 (1998), S. 905-911 
    ISSN: 0942-0940
    Keywords: Keywords: Endodermal tissue; intracranial cyst; neurenteric cyst; posterior fossa.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary  Neurenteric cysts are cystic lesions lined by a columnar epithelium of endodermal derivation. They are rarely located in the central nervous system. We describe the case history of two recurrent neurenteric cysts located within the posterior fossa: one in the 4th ventricle and the second in the cerebellopontine angle. The literature is reviewed and the follow-up and recurrence of such cysts are discussed.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 22 (1999), S. 187-188 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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