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  • 1
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Congenital cataracts facial dysmorphism neuropathy (CCFDN) syndrome (OMIM 604168) is an autosomal recessive developmental disorder that occurs in an endogamous group of Vlax Roma (Gypsies; refs. 1–3). We previously localized the gene associated with CCFDN to 18qter, where a conserved ...
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  • 2
    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.
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  • 3
    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.
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  • 4
    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.
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 99 (2000), S. 539-546 
    ISSN: 1432-0533
    Keywords: Key words Diabetic neuropathy ; Collagen ; Extracellular matrix ; Nerve regeneration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The pattern of collagenisation in peripheral nerve in diabetic polyneuropathy was examined in nerve biopsy specimens from patients with diabetic polyneuropathy in comparison with organ donor control nerves and disease controls (other neuropathies). There was increased endoneurial collagenisation both in the diabetic polyneuropathy cases and the disease controls, this predominantly involving types I and III. Type II collagen was not detected in organ donor control nerves or in the diabetic and the disease control nerves. There was a relative increase in type VI collagen in the endoneurium in the diabetic nerves immediately surrounding groups of Schwann cells. This was not a feature in the other neuropathies. The quantity of types IV, V and VI collagen was increased around the endoneurial microvessels in the diabetic patients and, to a lesser extent, in those with hereditary motor and sensory neuropathy (HMSN). Increased deposition of types IV and V collagen was observed in the perineurium in the diabetic nerves, the latter being most evident in the innermost lamellae where the amount of laminin was possibly also increased. The diameter of the general endoneurial collagen fibrils was greater in the diabetic nerves, although this was not more than in a disease control (HMSN). The collagen fibrils that were present within the basal laminal tubes that had surrounded degenerated myelinated fibres in the diabetic nerves, and those within the onion bulbs of the HMSN cases, were of the normal endoneurial calibre. The expression of laminin by Büngner bands in diabetic neuropathy did not differ from that in disease control nerves, nor were any differences detected for fibronectin. Whether the changes observed are important for the impaired regenerative capacity in diabetic neuropathy requires further investigation.
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  • 6
    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.
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 52 (1980), S. 1-6 
    ISSN: 1432-0533
    Keywords: Peripheral nerves ; Aging ; Pressure neuropathy ; Axonal glycogen bodies ; Polyglucosan bodies ; Hirano bodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Ultrastructural observations have been made on the tibial and plantar nerves of Wistar rats aged 18–24 months. Changes indicative of segmental demyelination and remyelination and axonal degeneration and regeneration were prominent in the plantar nerves. Both in the plantar and tibial nerves, but particularly in the former, axonal abnormalities were frequent. These included the occurrence of multiple intra-axonal vacuoles containing glycogen and polyglucosan bodies. Axonal sequestration by adaxonal Schwann cell processes was also increased. The Schwann cell cytoplasm in relation to this activity contained bundles of filaments with the ultrastructural features of Hirano bodies. The changes in the plantar nerves probably indicate a pressure neuropathy, but the possibility of a superimposed distal axonal degeneration related to aging cannot be excluded on the present evidence. Such changes must be taken into consideration in experimental studies performed on rats of this age.
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  • 8
    ISSN: 1432-0533
    Keywords: Experimental allergic neuritis ; Suppression ; Bovine dorsal root ; Lewis rat ; Resistance to reinduction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The injection of bovine dorsal root antigen in complete Freund's adjuvant can be used to produce experimental allergic neuritis (EAN) in rats. In this study attempts were made to prevent the development of the disease by prior injections of antigen. It was found that eight intradermal (i.d.) injections of antigen in either incomplete Freund's adjuvant or in saline failed to suppress EAN. A single intraperitoneal (i.p.) injection of antigen in saline produced only minimal protection against the disease. However, it was found that rats which had been given a primary course of EAN were subsequently completely unresponsive to a second injection of antigen.
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  • 9
    ISSN: 1432-0428
    Keywords: Diabetic vascular disease ; diabetes mellitus ; diabetic autonomie neuropathy ; vasomotor nerves ; arterioles ; morphometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A quantitative ultrastructural analysis was made of the terminal innervation of epineurial arterioles in the sural nerve of 6 diabetic and 6 nondiabetic patients of comparable age (mean±SD: 68 ±9 non-diabetic, 65±16 diabetic) with end stage peripheral vascular disease. The results demonstrated specific differences, identifiable morphometrically, in the pattern of innervation of epineurial vessels of diabetics compared with non-diabetics. The differences were: 1) in the diabetic group the proportion of perivascular axons found less than 7 μm from the nearest smooth muscle cell was significantly less than in the non-diabetic group (p 〈0.001); 2), the mean distance of the axons from their effector sites, the vascular smooth muscle cells, was nearly twice as far in the diabetic group compared with the nondiabetic group (p 〈0.05); and 3) the mean absolute number of axons less than 7 μ from the arteriole in the diabetic group was significantly less than in the non-diabetic group (p 〈0.01). These results demonstrate that the neuropathy associated with diabetes mellitus also involves the autonomic terminal innervation of some blood vessels. In addition, this neuropathy selectively affects the vasomotor nerves closer than 7 μm to the media.
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  • 10
    ISSN: 1432-0428
    Keywords: Diabetic vascular disease ; diabetes mellitus ; diabetic autonomic neuropathy ; vasomotor nerves ; arterioles ; morphometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A quantitative ultrastructural analysis was made of the terminal innervation of epineurial arterioles in the sural nerve of 6 diabetic and 6 nondiabetic patients of comparable age (mean ± SD: 68 ±9 non-diabetic, 65±16 diabetic) with end stage peripheral vascular disease. The results demonstrated specific differences, identifiable morphometrically, in the pattern of innervation of epineurial vessels of diabetics compared with non-diabetics. The differences were: 1) in the diabetic group the proportion of perivascular axons found less than 7 μm from the nearest smooth muscle cell was significantly less than in the non-diabetic group (p〈0.001); 2) the mean distance of the axons from their effector sites, the vascular smooth muscle cells, was nearly twice as far in the diabetic group compared with the nondiabetic group (p〈0.05); and 3) the mean absolute number of axons less than 7 μm from the arteriole in the diabetic group was significantly less than in the non-diabetic group (p〈0.01). These results demonstrate that the neuropathy associated with diabetes mellitus also involves the autonomic terminal innervation of some blood vessels. In addition, this neuropathy selectively affects the vasomotor nerves closer than 7 μm to the media.
    Type of Medium: Electronic Resource
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