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  • Electronic Resource  (32)
  • 2005-2009  (2)
  • 1995-1999  (19)
  • 1985-1989  (11)
  • 11
    ISSN: 1434-5196
    Keywords: Key words Internal cerebral vein thrombosis ; Sinovenous thrombosis ; Colitis ulcerosa ; Trauma ; Schlüsselwörter Hirnvenenthrombose ; Sinusthrombose ; Trauma ; Colitis ulcerosa
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine , Law
    Description / Table of Contents: Zusammenfassung Venöse Abflußstörungen des Gehirns werden mit unterschiedlicher Häufigkeit im Obduktionsgut aufgeführt. Neben entzündlichen Prozessen, hämorheologischen Störungen und Neoplasien kommen bei der forensischen Begutachtung traumatische Ursachen für die Genese von Thrombosen in Frage. Berichtet wird über eine 14 Jahre alt gewordene Schülerin, die 2 1/2 Tage nach einer tätlichen Auseinandersetzung verstarb. Anamnestisch wurde von einer Colitis ulcerosa berichtet. Initial waren weder äußere Verletzungen noch neurologische Auffälligkeiten erkennbar. In der Nacht wurde sie komatös in der Klinik aufgenommen. Die kranielle computertomographische Untersuchung zeigte eine zunehmende intraventrikuläre und intrazerebrale Blutung, Zeichen der venösen Stauung und ein Hirnödem. Die Patientin verstarb im dissoziierten Hirntod. Neuropathologisch fand sich eine in Organisation stehende Thrombose des Sinus sagittalis superior, die sich in die inneren Hirnvenen fortsetzte und die durch die entzündliche Darmerkrankung verursacht war. Eine traumatische Genese war somit auszuschließen.
    Notes: Abstract Thromboses of the cerebral veins which occur at a frequency of 3.75 to 9.3% in autopsy material are caused by local or systemic inflammatory processes, hematological disorders, neoplasms or traumatic brain damage. We report on a 14-year-old girl who suffered from colitis ulcerosa during the last 4 years of life. Immediately after a brawl with a schoolboy she complained of headache and vertigo, but no physical abnormality was detected by the physician. She was admitted to the hospital after becoming comatose during the following night. On neurological examination she was comatous with a decerebrate state, the pupils were small and non-reactive to light, divergent ocular bulbi and bilateral extensor plantar responses. A cerebral computer tomogramm showed a cerebral edema and a hemorrhage in the left basal ganglia and both cerebral ventricles which subsequently increased to the right cerebral ganglia, the corpus callosum and the right cerebral hemisphere. Despite antiedematous therapy the girl died on the second day after the brawl. A neuropathology examination revealed thrombosis of the superior sagittal sinus extending to the internal cerebral veins and a bilateral hemorrhagic infarction of the thalamus and the basal ganglia with intraventricular hemorrhage. As the thrombus in the sagittal sinus showed signs of organisation, a postraumatic origin alone is improbable and a hemorrheological disorder due to colitis ulcerosa seemed to be the main cause.
    Type of Medium: Electronic Resource
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  • 12
    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
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  • 13
    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
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  • 14
    ISSN: 1432-0533
    Keywords: Key words Frontotemporal dementia ; Dementia of ; frontal lobe type ; Pick’s disease ; Motor neuron disease ; with dementia ; Pick bodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Histological and immunohistochemical findings in 20 cases of frontotemporal dementias – 8 cases of dementia of frontal lobe type (DFT), 7 cases of Pick’s disease (PD), and 5 cases of motor neuron disease with dementia (MND/D) – are presented. Common features of all three syndromes were: frontotemporal atrophy, involvement of subcortical nuclei, and swollen chromatolytic cells. Ubiquitin (Ub)-positive and tau-negative inclusions in cortical, hippocampal, and motor neurons were found in MND/D and DFT cases, suggesting a common pathogenesis of MND/D and DFT. MND/D showed the same cytoskeletal alterations in motor nuclei as MND without dementia: Bunina bodies and skein-like, Ub-positive inclusions. DFT differed from PD in the preponderance of histopathological changes in upper cortical layers, the sparseness of chromatolytic cells, and the absence of tau-positive Pick bodies (PBs). There were, however, two transitional cases showing Pick-type histology but no PBs, thus linking DFT and PD. PBs expressed chromogranin B and secretoneurin strongly, but chromogranin A only weakly. They were negative for the 70-kDa heat-shock protein, metallothionein, and glutathione-S-transferase.
    Type of Medium: Electronic Resource
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  • 15
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 89 (1995), S. 385-390 
    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
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  • 16
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 89 (1995), S. 385-390 
    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
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  • 17
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 73 (1987), S. 349-356 
    ISSN: 1432-0533
    Keywords: Blood-brain barrier ; Cortex ; Lanthanum tracer ; Potassium ; Ion-selective micro-electrode
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Using lanthanum nitrate as a tracer, the permeability of rat cortical blood vessels was investigated during regional hypothermia of the cerebral cortex by electron microscopy. The concentration of K+ ions in the extracellular space of the cortex was determined using an ion-selective micro-electrode. Only at temperatures below about 7°C was an extravasation of tracer observed in a number of cortical capillaries and arterioles, where some of the tight junctions became widened and permeable. In a few cases penetration of the tracer into vacuoles of phagocytosing pericytes or macrophages was found. The extravasation of the tracer could mainly be observed in vessels on and below the cortical surface, less often in deeper cortical parts. At the same degree of hypothermia, the K+ concentration in the extracellular space increased, reaching a maximal value of ca. 6 mmol/l at a depth of about 200 μm. As could be shown in one previous experiment, the K+ concentration reached normal values again after rewarming to 37°C for 15 min, but an extravasation of the tracer was still observed. The role of the endothelial tight junctions and of the astrocytes together with the pericytes (or macrophages) is discussed regarding their importance for the effectiveness of the blood-brain barrier system.
    Type of Medium: Electronic Resource
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  • 18
    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
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  • 19
    ISSN: 1432-119X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract  Fibroblast growth factor 2 (FGF-2), which occurs in various isoforms both species and tissue specifically, regulates cell proliferation and differentiation via a dual receptor system consisting of heparan sulphate proteoglycans and receptor tyrosine kinases (FGFRs). This study demonstrates for the first time the distribution pattern of FGF-2 and the receptors FGFR 1–4 in the normal seminiferous epithelium of adult men. In western blot analyses, the polyclonal antibody, anti-FGF-2, shows two immunoreactive bands at 18 and 24 kDa. On paraffin sections, positive immunoreaction occurs within the cytoplasm of spermatogonia. The distribution pattern of the polyclonal anti-FGFR 1–4 antibodies is as follows: anti-FGFR-1 (one 68-kDa band) stains nuclei and cytoplasm of spermatogonia; anti-FGFR-3 (five bands at 68, 78, 105, 125 and 145 kDa) stains the nuclei of all germ cells except those of elongated spermatids; and anti-FGFR-4 (one 48-kDa band) stains the cytoplasm of primary pachytene spermatocytes. We were unable to demonstrate FGFR-2 immunoreactivity either in western blot analysis or on paraffin sections. This distribution pattern suggests that FGF-2 in spermatogonia is involved in the autocrine and paracrine regulation of the proliferation and differentiation of spermatogonia and spermatocytes via the receptors FGFR-1, FGFR-3 and FGFR-4.
    Type of Medium: Electronic Resource
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  • 20
    Electronic Resource
    Electronic Resource
    Springer
    Der Pathologe 17 (1996), S. 213-218 
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Riesenaxonale Neuropathie ; Mikrofilamentallagerung ; Key words Giant axonal neuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We report a sporadic case of giant axonal neuropathy (GAN) starting in a 5-year-old boy with gait disturbance. At the age of 10 years he showed signs of a sensomotoric polyneuropathy and central neurological symptoms: intention tremor, nystagmus and visual disturbance. The boy also had curly hair. Sural nerve biopsy showed many giant axons with accumulation of microfilaments, which were also found in cytoplasma of Schwann cells, endothelial cells and fibrocytes. Similar changes were detected in endothelial cells of a muscle biopsy, which additionally displayed neurogenic atrophy and target fibers. Immunohistochemically, desmin, vimentin, heat shock protein 70, and ubiquitin were not detectable in giant axons, which, however, contained neurofilament protein 68, 200 and β -tubulin. Our case shows that careful ultrastructural examination of a muscle biopsy may point to the diagnosis of GAN.
    Notes: Zusammenfassung Wir berichten über einen sporadischen Fall von Riesenaxonneuropathie (RAN), die bei einem 5 jährigen Jungen mit einer progredienten Gangstörung begann. Auffällig waren außerdem stumpfe, gekräuselte Haare. Im Alter von 10 Jahren bestanden eine sensomotorische Polyneuropathie und zentral-neurologische Symptome: Visusminderung, sakkadierte Blickfolge und Intentionstremor. Die Biopsie des N. suralis bestätigt das Vorliegen einer Neuropathie und zeigt viele Risenaxone mit ultrastruktureller Anhäufung von Mikrofilamenten. Diese sind auch im Zytoplasma der Schwann-Zellen, Endothelzellen und Fibrozyten zu erkennen. Auch intramuskuläre Endothelzellen enthalten gleichartige Veränderungen; darüber hinaus ist im Muskel ein neurogenes Gewebsbild ausgebildet. Immunhistochemisch exprimieren die Axone Neurofilamentprotein 200 und 68 sowie β -Tubulin, jedoch weder Desmin, Vimentin, Heat-shock-Protein 70 oder Ubiquitin. Der Fall illustriert, daß die ultrastrukturelle Untersuchung einer Muskelbiopsie diagnostische Hinweise für eine RAN liefern kann.
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