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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
    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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  • 4
    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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  • 5
    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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  • 6
    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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  • 7
    ISSN: 1432-0428
    Keywords: Diabetic neuropathy ; hereditary motor and sensory neuropathy ; sural nerve ; endoneurial capillaries ; basal lamina
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Nerve biopsies were obtained from 27 patients with diabetic neuropathy. All had a symmetric distal sensory and autonomic neuropathy or a purely sensory neuropathy. Mean age was 39.8 years (range 23–57 years). Two patients had Type 2 (non-insulin-dependent) diabetes mellitus and the remainder Type 1 (insulin-dependent) diabetes. Morphometric observations on endoneurial capillaries were compared with results from organ donor control cases and from patients with type 1 hereditary motor and sensory neuropathy. The area of the lumen of the capillaries did not differ between the three groups. The area occupied by the capillary endothelial cells in transverse section and the number of endothelial cell nuclei were increased both in the patients with diabetic neuropathy and hereditary motor and sensory neuropathy, as was the thickness of the surrounding basal laminal zone. ‘Closure’ of endoneurial capillaries in diabetic neuropathy, reported in another study, was not confirmed. Capillary density and nearest-neighbour distances were similar in the diabetic and organ donor control cases. Capillary density was reduced in the patients with hereditary motor and sensory neuropathy, this being related to increased fascicular area consequent upon the presence of hypertrophic changes. The presence of thickening of the pericapillary basal laminal zone and endothelial cell hyperplasia both in diabetic and hereditary motor and sensory neuropathy, the latter being a neuropathy in which a vascular basis can be discounted, makes it difficult to use such changes as an argument favouring a vascular cause for diabetic neuropathy. There were differences in the basal laminal zone between the diabetic and hereditary motor and sensory neuropathy cases suggesting that the reduplicated basal lamina was more persistent in the diabetic patients.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0428
    Keywords: Streptozotocin diabetes ; blood-nerve barrier ; non-enzymatic glycation ; serum proteins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The permeation of native and non-enzymatically glycated albumin and immunoglobulin G into the endoneurium of the sciatic nerve of rats was examined in acute experiments. Low amounts of native albumin entered both in control and streptozotocin-diabetic animals with no significant difference between them. The entry of glycated albumin was significantly greater both in control and diabetic rats, especially in the former. Permeation of native and glycated immunoglobulin G was not detectable over the time course of the experiment. It is concluded that glycation of albumin enhances its permeation into the nerve. This may be relevant to the increased amounts of endoneurial albumin that are detectable in human diabetic neuropathy.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 11 (1968), S. 93-102 
    ISSN: 1432-0533
    Keywords: Diabetic Neuropathy ; Hypertrophic Changes ; Nerve Biopsy ; Electron Microscopy ; Segmental Demyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Beobachtungen an Nervenbiopsien bei 10 aufeinanderfolgenden Patienten mit diabetischer Neuropathie wurden unternommen. 1 Patient wies die für eine hypertrophische Neuropathie typischen licht-und elektronenmikroskopischen Veränderungen auf. 5 zeigten typische hypertrophische Veränderungen, die aber nur bei elektronenmikroskopischer Untersuchung sichtbar waren; bei weiteren wurden ähnliche geringe Veränderungen entdeckt. Es wird angenommen, daß diese Veränderungen durch segmentale Demyelinisation verursacht wurden.
    Notes: Summary Observations have been made on 10 consecutive nerve biopsies from patients with diabetic neuropathy. 1 patient showed the typical appearances of hypertrophic neuropathy on light and electron microscopy. 5 displayed typical hypertrophic changes visible only on electron microscopy and minor abnormalities of a similar nature were seen in 2 others. It was considered that they were likely to have resulted from recurrent segmental demyelination.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 14 (1969), S. 237-249 
    ISSN: 1432-0533
    Keywords: Electron Microscopy ; Wallerian Degeneration ; Nodal Changes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über ultrastrukturelle Veränderungen in den Ranvierschen Knoten des N.suralis der Ratte im Laufe der Wallerschen Degeneration berichtet. Die Untersuchungen erfolgten 12 und 120 Std nach einer örtlichen Quetschungsverletzung. Die ersten bemerkbaren Veränderungen finden sich im Axon. Nodale und paranodale Anhäufungen von Mitochondrien, multivesikulären und lamellären Körpern, wie auch kleinen blasen- und röhrenartigen Bildungen sind teilweise in den Knoten sichtbar und am deutlichsten nach 24–36 Std erkennbar. Gleichzeitig erfolgt eine Aufsplitterung der Neurofilamente und Neurotubuli, die ihre Liniengestaltung verlieren und sich zusammenballen. Die Zone der erhöhten Dichte gerade unterhalb des nodalen Axolemmas bleibt erhalten. Veränderungen im Myelin beginnen etwas später und bestehen in einem vesikulären Verfall der Endomyelinlamellen und einer Trennung der Endomyelinschlaufen vom Axolemma durch Schwannzellenfortsätze. Dieser Vorgang schien mit einem Zurückziehen des Myelins vom Knoten im Zusammenhang zu stehen. Schwannzellenfortsätze erstrecken sich auch so weit, daß sie das nodale Axon bedecken, wobei sie die nodalen Schwannzellenfortsätze vom Axolemma trennen. Das Endstadium ist die Unterbrechung des nodalen Axons und die Verschmelzung der Myelinenden als Teil der Ovoidbildung.
    Notes: Summary Observations are reported on the ultrastructural alterations at the nodes of Ranvier in the rat sural nerve during the course of Wallerian degeneration. These were examined between 12 and 120 hours after a localized crush injury. The earliest detectable changes are in the axon. Nodal and paranodal accumulations of mitochondria, multivesicular bodies, lamellar bodies and small vesicular and tubular profiles are seen at a proportion of the nodes and are most evident at 24–36 hours. Concomitantly with this, the neurofilaments and neurotubulus fragment, lose their alignment and clump together. The zone of increased density just beneath the nodal axolemma is preserved. Changes in the myelin begin slightly later and consist of vesicular breakdown of the terminal myelin lamellae, and separation of the terminal myelin loops from the axolemma by Schwann cell processes. The latter event appeared to be associated with retraction of the myelin from the node. Schwann cell processes also extend to cover the nodal axon, separating the Schwann cell nodal processes from the axolemma. The final stage is the interruption of the nodal axon and the fusion of the ends of the myelin as part of ovoid formation.
    Type of Medium: Electronic Resource
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