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  • 1
    ISSN: 1432-0533
    Keywords: Peripheral nerve ; Morphometry ; Diabetes mellitus ; Hypomyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The relative changes in the growth and maturation of axon size and myelin thickness were studied in the medial plantar division of the tibial nerve in the lower leg and in the motor branches of the tibial nerve to the calf muscles in rats in which diabetes mellitus had been induced with streptozotocin at the time of weaning. Observations were made at 6 weeks and 3, 6, 9 and 12 months of diabetes for comparison with age-matched controls. Similar changes were observed in both nerves. Growth in body weight and skeletal growth was severely retarded from the time of induction of diabetes but at the 6-week stage axon size was not reduced, suggesting that neural growth may initially be relatively protected. At later stages axon size was consistently reduced in the diabetic animals as compared with the controls and showed an absolute reduction at 12 months, as compared with 9 months, that was greater than in the controls. Myelin thickness became reduced earlier and was more severely affected than axon size so that the fibers were relatively hypomyelinated. The myelin changes were greater in larger than in smaller fibers. The index of circularity of axons was reduced in the diabetic nerves. These results show that induction of diabetes in prepubertal rats produces effects on peripheral nerve fibers which differ from those resulting from diabetes induced in adult animals. The effects also differ between large and small nerve fibres. These observations may explain some of the disparate findings obstained in previous studies on experimental diabetes in rats.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Friedreich's ataxia ; Sensory neuropathy ; Distal axonopathy ; Hypomyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Observations have been made on a patient with Friedreich's ataxia who died 52 years after the onset of symptoms. The pathology of the brain and spinal cord was typical of this disorder. Apart from loss of dorsal root ganglion cells, severe loss of secondary sensory neurons was observed, including the nucleus dorsalis in the spinal cord, the spinal and principal trigeminal nuclei and, in particular, the mesencephalic trigeminal nucleus in the brain stem. Morphometric studies on the first sacral nerve root and on the sural nerve at levels from midthigh to ankle revealed a distally accentuated axonal loss that predominantly affected larger myelinated nerve fibres. Regenerative activity was seen, mainly in the spinal root and proximally in the sural nerve. Relative myelin thickness, assessed by g ratios, tended to be reduced. As teased fibre studies showed only limited evidence of demyelination/remyelination and of axonal regeneration, this therefore suggests the presence of hypomyelination. The results confirm the presence of a distal axonopathy and provide no evidence that this is preceded by axonal atrophy.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 62 (1984), S. 316-323 
    ISSN: 1432-0533
    Keywords: Experimental allergic neuritis ; Macrophage function ; Silica blockade
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The selective toxicity of silica dust for macrophages has been used to assess the role of these cells in experimental allergic neuritis (EAN). Inbred Lewis rats were inoculated with bovine dorsal roots in Freund's complete adjuvant (day 0). In two experiments, animals received 200 mg of silica dust in 1 cm3 of saline intraperitoneally (IP) at days 8 and 16. In another two experiments, animals received IP silica at days 3, 7, and 11. Control animals received 1 cm3 saline IP at corresponding times. Regular clinical assessment showed that in animals treated on days 8 and 16 there was a significant delay in the time taken to reach their maximum degree of illness. This delay was not seen in the animals treated on days 3, 7, and 11. Neither of the injection regimes reduced the final maximum severity of the disease. In three experiments recovery of the treated and control animals occurred concurrently, hence the duration of the disease was reduced in the animals treated at days 8 and 16. However, in one group of animals given silica at days 3, 7 and 11, there was a delay in the time taken to recover from the most severe phase of the disease but thereafter the treated animals improved more quickly to reach their best grade at the same time as the controls. If the silica blockade of macrophages is to be effective in delaying the onset of EAN, the timing of injections is critical.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 59 (1983), S. 262-268 
    ISSN: 1432-0533
    Keywords: Experimental allergic neuritis ; Cyclosporin-A
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Experimental allergic neuritis (EAN) was induced in guinea pigs and rats and treated with Cyclosporin-A (Cy-A). When Cy-A was given prophylactically for 1 month from the time of induction of the disease, it prevented the development of EAN during the course of its administration. When Cy-A was given therapeutically after the onset of neurological signs, it effectively prevented further deterioration. This effect was more marked after 3 weeks' treatment than after only 1 week's treatment. In both regimens, when dosing with Cy-A ceased there was a latent period before clinical signs of EAN developed. This latent period is similar to that seen in the development of EAN in normal control animals and is probably due to the continued presence of antigen at the injection sites. After primary treatment of EAN with Cy-A, animals that relapsed did not respond to further treatment with Cy-A. Histological examination revealed that the nature of the EAN lesions in both groups of animals given Cy-A were not as severe as those seen in control animals. Despite these observations, there was no statistically significant difference between the maximum clinical grades reached by animals in any one group. These experiments suggest that T-cells are important in the development of EAN and that Cy-A interferes with this process by suppressing T-helper cells. They also show that it is possible to influence favourably the course of immune mediated neurological disease.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 53 (1981), S. 257-265 
    ISSN: 1432-0533
    Keywords: Experimental diabetes ; Skeletal growth ; Nerve fibre maturation ; Diabetic neuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Observations were made between the ages of 2 and 12 months on rats made diabetic with streptozotocin at the age of 1 month, and compared with the findings in age-matched controls. Tibial length and body weight in the control animals increased progressively over the period examined, the growth rate being more rapid in the initial stages. Both of these parameters were consistently less in the diabetic animals over the whole of the observation period. Myelinated fibre numbers and diameters were measured in the tibial and plantar nerves. In the tibial nerve, fibre diameter did not differ between the diabetic and control animals up until 4 months of age; thereafter it changed little in the diabetic animals, but continued to increase in the controls. The findings in the medial plantar nerve were more difficult to analyse but showed comparable although less pronounced changes; fibre diameter may be have diminished in the diabetic nerves after 6 months. Teased fibre studies demonstrated few abnormalities in the tibial nerve, either in the control or the diabetic rats. In the lateral plantar nerves, there was a significant excess of axonal degeneration and regeneration in the diabetic nerves. It was concluded that diabetes impairs growth in nerve fibre diameter, but only after 4 months of age. Before then, no growth retardation is obvious, despite the fact that tibial length and body weight are less. This suggests that the peripheral nervous system may be protected against growth retardation during the early part of the postnatal growth period. The significance of the axonal degeneration in the plantar nerves is uncertain, but it may represent either an increased vulnerability of diabetic nerve to compression injury or, less probably, a distal axonopathy related to the diabetic state.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Peripheral nerve morphometry ; Axons ; Myelin ; Growth changes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Morphometric observations have been made on the medial plantar division of the tibial nerve (MPD) and on the motor branches of the tibial nerve to the calf muscles (MBC) in rats ranging in age from weaning (3 weeks) to 12 months. Axon size, assessed by measurements of circumference and cross-sectional area, increased rapidly until 3 months with further slight increases between 3 and 9 months and a slight fall between 9 and 12 months. Axon size distributions were unimodal throughout in the MPD but bimodal for the MBC except at 3 weeks. Distributions of myelin thickness were bimodal throughout for both nerves. Scatter plots of g ratios (axon diameter: total fibre diameter) confirmed the presence of two fibre populations: a group of small fibres with relatively thin myelin sheaths, and a group of larger fibres within which sheath thickness was relatively less on the larger than on the smaller axons. These two fibres populations were less easily separable in the MBC than in the MPD nerves. These results document morphometrically the normal growth changes in the rat tibial nerve and also provide control data for the analysis of the effects of experimental procedures on the growth and maturation of peripheral nerve fibres.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 81 (1991), S. 354-358 
    ISSN: 1432-0533
    Keywords: Peripheral neuropathy ; Chediak-Higashi syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical features of a brother and sister with the Chediak-Higashi syndrome (CHS) are reported. Both showed evidence of a sensory neuropathy associated with central nervous system involvement. Nerve conduction studies indicated an “axonal” neuropathy. Sural nerve biopsy in the brother demonstrated a loss of myelinated nerve fibres, particularly those of larger size, and of unmyelinated axons. In contradistinction to some previous reports, giant lysosomes in Schwann cells were not observed and there were no inflammatory changes. Electron microscopy and teased-fibre studies showed no evidence of demyelination. It is concluded that the neuropathy of CHS is of axonal type. Its mechanism remains obscure.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 88 (1994), S. 426-432 
    ISSN: 1432-0533
    Keywords: Diabetic neuropathy ; Perineurium Basal lamina ; Endoneurial capillaries
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Measurements were made of the thickness of the basal lamina of perineurial cells in the sural nerve in a series of patients with diabetic neuropathy and compared with a group of patients with type I hereditary motor and sensory neuropathy (HMSN) and with organ donor control cases. The thickness was significantly greater in the diabetic patients as compared both with the HMSN cases and the organ donor controls. This was most obvious for the intermediate layers of the perineurium. Perineurial basal laminal thickness was only slightly greater in the HMSN cases than in the organ donor controls and the difference was not statistically significant. The thickening of the perineurial cell basal laminae was compared with the thickening of the basal laminal zone around the endoneurial microvessels. No significant correlation was found either for the diabetic neuropathy or HMSN cases or for the organ donor controls. As had been observed previously, the basal laminal zone around the endoneurial capillaries was of increased thickness both in the diabetic neuropathy and the HMSN cases and, although it was greater for the diabetic neuropathy patients, the difference was not statistically significant. Taken together, these findings indicate that the thickening of the basal lamina of the perineurial cells in a more characteristic feature of diabetic neuropathy than is thickening of the basal laminal zone around the endoneurial capillaries. The results suggest that the causative mechanisms are likely to differ, a conclusion supported by the morphological appearances: the basal laminal thickening around the perineurial cells is uniform, whereas that around the capillaries consists of basal laminal reduplication. Atrophy and necrosis of perineurial cells were observed in patients with diabetic neuropathy but rarely in the cases with HMSN and not in the organ donor cases. This may be similar to the degeneration of endoneurial fibroblasts that has been described as a non-specific finding in neuropathies.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0533
    Keywords: Peripheral nerve ; Human diabetic polyneuropathy ; Perineurium ; Tight junctions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Perineurial cells in the human sural nerve possess tight junctions which in freeze-fracture replicas are seen to be composed of networks of branching and anastomosing P face strands and E face grooves. Isolated circular tight junctions (maculae occludentes) may represent attachment devices between adjacent perineurial lamellae. At the overlapping margins of the cells, a beltlike tight junction (zonula occludens) encircles the cells and is believed to comprise a paracellular diffusion barrier. As the permeability of the perineurium has been found to be altered in diabetic polyneuropathy, the zonulae occludentes have been studied. In freeze-fracture replicas from cases of diabetic polyneuropathy a mixed population of structurally normal and abnormal junctions was observed. In some, the strands were abnormally curved with reduced numbers of intersections, the intervening plasma membrane displaying prominent P face concavities and E face convexities. At other sites, the junctions were severely disorganized and represented by fragmented and isolated strands with few intersections and numerous free ends. These abnormalities resemble changes that have been produced experimentally in epithelial tight junctions by osmotic damage. The possibility is considered that similar mechanisms could result in the alterations of the perineurial tight junctions in diabetic polyneuropathy and account for its impaired permeability barrier properties.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 63 (1984), S. 319-329 
    ISSN: 1432-0533
    Keywords: Peripheral nerve myelin ; Myelin periodicity ; Myelin structure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The occurrence of myelin with an unusually large periodicity has been noted in a variety of human and animal diseases by many authors. It has also proved possible to create regular alterations in periodicity by various treatments of fresh unfixed nerve. We have quantified the changes found in material from a variety of sources and conclude that they are compatible with the occurrence of physicochemical changes in the myelin membranes, leading to overhydration.
    Type of Medium: Electronic Resource
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