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  • 11
    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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  • 12
    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.
    Type of Medium: Electronic Resource
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  • 13
    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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  • 14
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 11 (1995), S. 610-615 
    ISSN: 1433-0350
    Keywords: Nemaline myopathy ; Cardiac involvement ; Nervous system involvement ; Ubiquitin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Nemaline myopathy belongs to the group of congenital non-progressive myopathies; however, in rare cases death occurs in early infancy. We report two cases of rapidly fatal nemaline myopathy. The first patient, who died at the age of 26 months, showed atrophy of type 1 fibers containing numerous rods in biopsy sections. Biopsy of the second patient, who had died at the age of 5 months, revealed severe maturational arrest and myopathy, but rods were so rare that diagnosis could only be made at the ultrastructural level. Autopsy of both patients showed that atrophy of type 1 fibers and maturational arrest had disappeared in the very same muscles; rods had moved to a central position in the first and significantly increased in number in the second case. Diaphragma muscles contained abundant amounts of rods in both cases. The cardiac musculature showed a few rods only in the first patient, who had developed heart insufficiency 11 months prior to death. Immunohistochemical analysis showed that rods did not contain desmin or ubiquitin.
    Type of Medium: Electronic Resource
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  • 15
    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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  • 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
    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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  • 18
    ISSN: 1434-6052
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract The behavior of the proton magnetic form factor is studied within the modified hard scattering picture, which takes into account gluonic radiative corrections in terms of transverse separations. We parallel the analysis given previously by Li and make apparent a serious objection. The appropriate cut-off needed to render the form-factor calculation finite is both detailed and analyzed by considering different cut-off prescriptions. The use of the maximum interquark separation as a common infrared cut-off in the Sudakov suppression factor is proposed, since it avoids difficulties with the α s -singularrities and yields a proton form factor insensitive to the inclusion of the soft region which therefore can be confidently attributed to perturbative QCD. Results are presented for a variety of proton wave functions including also their intrinsic transverse momentum. It turns out that the perturbative contribution, although theoretically self-consistent forQ 2 larger than about 6 GeV2 to 10 GeV2, is too small compared to the data.
    Type of Medium: Electronic Resource
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  • 19
    Electronic Resource
    Electronic Resource
    Springer
    Cell & tissue research 288 (1997), S. 539-544 
    ISSN: 1432-0878
    Keywords: Key words: Testis ; hsp60 ; Infertility ; Spermatogonia ; Immunohistochemistry ; Human
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract. The immunohistochemical localization of heat-shock protein 60 (hsp60) was investigated in testicular biopsies obtained from 121 adult men with disturbed fertility. In normal unaffected tubules, hsp60 immunoreactivity was localized to spermatogonia, primary spermatocytes and Sertoli cells. In spermatogonia, cytosolic and mitochondrial labelling could be differentiated. In general, the number of stained spermatogonia decreased with the loss of spermatogenic function. A significant (P〈0.01) reduction of stained spermatogonia was observed in testes with maturation arrest of spermatogenesis at the level of primary spermatocytes (30.2±21.6%) compared with testes exhibiting normal spermatogenesis. In addition, the decrease in the score correlated significantly with the diminution of cytosolic hsp60 immmunolabelling (coefficient r=0.25, P=0.03). There was a significant difference (P〈0.01) in the percentage of cytosolic-stained spermatogonia in testes with a score equal to or greater than 5 (14.7±9.8%) and a score less than 5 (8.9±6.9%). These observations suggest that a low level of hsp60 expression in spermatogonia may lead to a different pattern of protection, which in turn could be involved in low spermatogenic efficiency.
    Type of Medium: Electronic Resource
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