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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Neuroradiology 30 (1988), S. 421-425 
    ISSN: 1432-1920
    Keywords: CT-scanning ; Muscles ; Myopathy ; Neurogenic disorder ; Muscle biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The value of CT-examination of the muscles compared to histology was studied in a retrospective analysis of 30 patients with clinical suspicion of neuromuscular disorder. In the evaluation of the CT-results descriptive criteria were used. The histologic diagnosis came from needle-biopsies taken from the quadriceps muscle. Considering the whole group of neuromuscular disorders, CT has an overall accuracy of 84.8%, a positive predictive value of 95.5% and a negative predictive value of 63.6%. This makes the use of CT as a diagnostic tool in neuromuscular disorders a reliable examination technique. In patients with a polymyositis there is even a 100% correlation between CT findings and biopsy results. Discrepancy between the biopsy results of the quadriceps muscle and the CT findings is remarkable: the number of abnormal histological findings is twice the number of abnormal CT findings. Using the more proximal gluteal region as a biopsy site would have decreased this discrepancy and would therefore have given a better correlation between CT and histology. The choice of protocol in determining the levels to be scanned is of great importance in achieving good reproducability in follow-up CT examinations.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 0003-2670
    Keywords: Gas chromatography ; Mass spectrometry ; Methylmalonic acid ; Urine
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Polyglucosan bodies ; Muscle ; Nerve ; Lafora's disease ; Ageing
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The presence of polyglucosan bodies was studied in intramuscular motor nerves of 292 muscle biopsies. These biopsies were classified into five diagnostic categories and investigated for the presence of polyglucosan bodies in relation to age and sex. Their presence was nonspecific in patients over 20 years, the only correlation being with ageing. Under 20 years, their presence pointed to the diagnosis of Lafora's disease. In cases in which both a muscle biopsy and a sural nerve biopsy were performed, the former appeared to contain these polyglucosans more frequently.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Anatomy and embryology 179 (1989), S. 449-456 
    ISSN: 1432-0568
    Keywords: Corticospinal tract ; Development ; Myelination ; Pyramidal tract ; Anterograde tracing ; Electronmicroscopy ; Rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Horseradish-peroxidase was used to anterogradely label and thus to trace the growth of corticospinal axons in rats ranging in age from one day to six months. Three to eight HRP-gels were implanted in the left cerebral hemisphere of the cortex. In each spinal cord three levels were studied, the cervical intumescence (C5), the mid-thoracic region (T5) and the lumbar enlargement (L3). The methodology employed for the electron microscopic visualization of HRP has been described previously (Joosten et al. 1987a). The outgrowth of labelled unmyelinated corticospinal tract axons in the rat spinal cord primarily occurs during the first ten postnatal days. The outgrowth of the main weve of these fibres is preceded by a number of pathfinding axons, characterized by dilatations at their distal ends, the growth cones. By contrast, later appearing unmyelinated axons, which presumably grow along the pathfinding axons, do not exhibit such growth cones. The first labelled pioneer axons can be observed in the cervical intumescence at postnatal day one (P1), in the mid-thoracic region at day three (P3) and in the lumbar enlargement at day five (P5). Prior to the entrance of the axons, the prospective corticospinal area or the pre-arrival zone is composed of fascicles consisting of unlabelled, unmyelinated fibres surrounded by lucent amorphous structures. During the outgrowth phase of the corticospinal fibres some myelinated axons could be observed within the outgrowth area even before day 14. These axons, however, were never labelled. These findings strongly suggest that the outgrowth area, which is generally denoted as the pyramidal tract, contains other axons besides the corticospinal fibres (and glial cells). The process of myelination of the labelled corticospinal tract axons in the rat spinal cord starts rostrally (C5) at about day 14 and progresses caudally during the third and fourth postnatal weeks. Although myelination seems to be largely complete at day 28 at all three spinal cord levels, some labelled unmyelinated axons are still present in the adult stage.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Dysphagia 12 (1997), S. 63 -67 
    ISSN: 1432-0460
    Keywords: Key words: Pharyngeal retention — Pharyngeal shortening — Tongue driving force — Hypopharyngeal suction pump — Pharyngeal contraction — Deglutition —Deglutition disorders.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. This study examines possible quantifiable causes of postdeglutition pharyngeal retention in the elderly. Manofluorography and computer processing of video images are performed. Retention in the valleculae and in the piriform sinuses is associated with a markedly reduced pharyngeal shortening, a low tongue driving force (TDF), and a diminished amplitude of the pharyngeal contraction. There is no relationship with the hypopharyngeal suction pump (HSP). Retention limited to the valleculae is associated with a low TDF, and retention restricted to the piriform sinuses is accompanied by a reduced pharyngeal shortening.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Dejerine-Sottas Polyneuropathy ; Onion-Bulbs ; Demyelination and Remyelination ; Basement Membranes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The microscopic and electron microscopic findings in sural nerve biopsies of a brother and sister with chronic polyneuropathy of the Dejerine-Sottas type resemble those reported by Lyon (1969). A genetically determined variant appears to be involved. The relationship between this syndrome and onion-bulb neuropathies is discussed. It seems unlikely that only segmental demyelination and remyelination is responsible for onionbulb formation. The relationship between axons in the layers of onion-bulbs and the central fibre is discussed, and between basement membranes and Schwann cell processes and the central fibre. Significant quantitative evidence of axon degeneration and regeneration after primary axon degeneration is pointed out. Earlier probable cases in the literature are reviewed.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-119X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary A double electronmicroscopical (EM) staining was developed which enabled the ultrastructural localization of cell adhesion molecules on the outer axonal membrane of horseradish peroxidase (HRP)-labelled axons in the developing central nervous system (CNS). HRP was used to anterogradely trace outgrowing corticospinal tract (CST) axons in ten-day-old rats. After visualization of HRP using tetramethylbenzidine (TMB) as a chromogen and ammoniumheptamolybdate (AHM) as a stabilizer at pH 6.0 as described previously (Joosten et al. 1987, J Histochem Cytochem 35:623–626) an additional diaminobenzindine (DAB)-Ni incubation was carried out for further stabilization. Subsequently a pre-embedding immunoperoxidase (DAB) staining was executed for detection of cell adhesion molecule L1. Using this procedure anterogradely HRP-labelled CST axons were recognizable by a granular black TMB-AHM-DABNi reaction product at the light microscopic (LM) level, which clearly contrasts to the relatively homogeneous brown L1-immunostaining. Electronmicroscopically HRP-labelled CST axons were characterized by the presence of an intracellular crystaloid TMB-AHM-DABNi reaction product which made identification of CST axons rather easy, whereas the L1-DAB precipitate could be noted on the outer axonal membrane of the HRP-labelled CST axons, marking the presence of the L1 cell adhesion molecule. In addition the procedure described in this report preserves ultrastructural details of developing neural tissue. In conclusion, the method presented can be employed in combined HRP-tracing and immunohistochemical electronmicroscopic studies.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: Dysautonomia ; Hypertrophic Neuropathy ; Sural Nerve Biopsy ; Syndrome of Multiple Mucosal Neuromas
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Sural nerve findings in a patient with the features of the syndrome of multiple mucosal neuromas are described. Hypertrophy with onion-bulb formation without signs of segmental de- and re-myelination or axonal de- and regeneration of myelinated fibers was found. A chronic process of de- and regeneration of unmyelinated fibers was found together with the clinical findings of a dysautonomia. This process was thought to be responsible for the hypertrophy and onion-bulb formation. These findings are in accordance with partial documentation on this subject described in some other patients with this syndrome.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0533
    Keywords: Adult metachromatic leukodystrophy ; Brain ; Peripheral nerve ; Kidney ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The fine structural characteristics of storage products in peripheral nerve, kidney and cerebral white matter, from a case of adult metachromatic leukodystrophy are described. There were pronounced differences from the fine structural aspects in late infantile cases. A large proportion of the inclusions did not exhibit a unit membrane. An hypothesis is proposed to clarify the delayed manifestation of this type of metachromatic leukodystrophy until adulthood.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0533
    Keywords: Lymphocyte ; Juvenile amaurotic idiocy ; Ceroid lipofuscinosis ; Vacuolar contents ; Electron microscopy ; Sural nerve ; Muscle ; Rectum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Lymphocytes of the peripheral blood of 31 patients with juvenile amaurotic idiocy (juvenile form of ceroid lipofuscinosis) were examined with the electron microscope. In all cases, intracytoplasmic clear vacuoles were present, containing round hollow, fingerprint and highly electron dense structures. The combination of these structures, not necessarily in one and the same vacuole, was considered to be highly indicative for the diagnosis of juvenile amaurotic idiocy. In addition to these three structures, parallel tubular inclusion bodies, rectilinear profiles and rodshaped structures were found but in a number of the cases. The parallel tubular inclusion bodies were not regarded as having any diagnostic significance.
    Type of Medium: Electronic Resource
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