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  • 1
    ISSN: 1432-0533
    Keywords: Key words Diabetic neuropathy ; Axonal regeneration ; Nerve growth factor receptors ; Schwann cells ; Basal lamina
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Observations were made on myelinated fibre regeneration in diabetic sensory polyneuropathy assessed in sural nerve biopsy specimens. These confirmed that regenerative clusters initially develop within abnormally persistent Schwann cell basal laminal tubes. The number of regenerating fibres, identified by light microscopy, was found to decline in proportion to the reduction in total myelinated fibre density. The relative number of regenerating fibres was significantly greater in patients with insulin-dependent as compared with those with non-insulin-dependent diabetes after correction for age. There was a slight negative correlation between the relative proportion of regenerating fibres and age, but this was not statistically significant. The progressive reduction in the number of regenerating fibres with declining total fibre density indicates that axonal regeneration fails with advancing neuropathy. The production of nerve growth factor (NGF) and NGF receptors by denervated Schwann cells is likely to be important for axonal regeneration. To investigate whether the failure of axonal regeneration could be related to a lack of NGF receptor production by Schwann cells, we examined the expression of p75 NGF receptors by Büngner bands immunocytochemically. In comparison with other types of peripheral neuropathy, p75 NGF receptor expression appeared to take place normally. It is concluded that failure of axonal regeneration constitutes an important component in diabetic neuropathy. Its explanation requires further investigation.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Key words Diabetic polyneuropathy ; Nodes of ; Ranvier ; Paranodes ; Axoglial dysjunction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Observations have been made on the structure of the paranodal region at nodes of Ranvier in the sural nerve of patients with diabetic sensory polyneuropathy. The structure of the paranodes was examined with particular attention to the definition and assessment of axoglial dysjunction, which has been claimed to be a characteristic feature of both human and experimental diabetic neuropathy and which has been related to paranodal swelling. In the present series of cases it was not possible to confirm that axoglial dysjunction is a distinctive feature of diabetic polyneuropathy in fibres not undergoing active demyelination or wallerian-type degeneration, neither was excessive paranodal enlargement found.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Peripheral neuropathy ; Hypomyelination ; Dysmorphism ; Cataracts
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Observations have been made on the peripheral nerve changes in four patients, ranging in age from 4 to 32 years, with the congenital cataracts facial dysmorphism neuropathy syndrome. Myelinated fibre density was within normal limits. The salient abnormality was diffuse hypomyelination which, in the older patients, was associated with demyelination and then axonal degeneration. These findings could be correlated with the relative preservation of sensory action potential amplitude despite markedly reduced nerve conduction velocity. Unmyelinated axon density was preserved. The morphological observations suggest the operation of a developmental process affecting myelination with a later superimposed degenerative disorder.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Diabetic neuropathy ; Axonal regeneration ; Nerve growth factor receptors ; Schwann cells ; Basal lamina
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Observations were made on myelinated fibre regeneration in diabetic sensory polyneuropathy assessed in sural nerve biopsy specimens. These confirmed that regenerative clusters initially develop within abnormally persistent Schwann cell basal laminal tubes. The number of regenerating fibres, identified by light microscopy, was found to decline in proportion to the reduction in total myelinated fibre density. The relative number of regenerating fibres was significantly greater in patients with insulin-dependent as compared with those with non-insulin-dependent diabetes after correction for age. There was a slight negative correlation between the relative proportion of regenerating fibres and age, but this was not statistically significant. The progressive reduction in the number of regenerating fibres with declining total fibre density indicates that axonal regeneration fails with advancing neuropathy. The production of nerve growth factor (NGF) and NGF receptors by denervated Schwann cells is likely to be important for axonal regeneration. To investigate whether the failure of axonal regeneration could be related to a lack of NGF receptor production by Schwann cells, we examined the expression of p75 NGF receptors by Büngner bands immunocytochemically. In comparison with other types of peripheral neuropathy, p75 NGF receptor expression appeared to take place normally. It is concluded that failure of axonal regeneration constitutes an important component in diabetic neuropathy. Its explanation requires further investigation.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Diabetologia 40 (1997), S. B74 
    ISSN: 1432-0428
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 6
    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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  • 7
    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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  • 8
    ISSN: 1432-1459
    Keywords: Optic neuropathy ; Sensory peripheral neuropathy ; Cuban epidemic ; Nutritional deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An epidemic of bilateral optic neuropathy and painful sensory neuropathy occurred in Cuba in 1991–1993. Over 45 000 individuals were stated to have been affected. We report a clinical study on 25 patients seen in Cuba in 1993–1994. Affected patients showed either bilateral optic neuropathy with caecocentral scotomata or a distal predominantly sensory neuropathy sometimes associated with deafness, or a combination of both optic and peripheral sensory neuropathy. The nature of the epidemic is discussed. The clinical features in patients with confirmed neurological deficits were consistent with a diagnosis of Strachan's syndrome, probably related to nutritional deficiency. Other patients with similar symptoms showed no evidence either of optic or peripheral neuropathy and were considered to represent disease mimicry on a psychoneurotic basis.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 245 (1998), S. 159-165 
    ISSN: 1432-1459
    Keywords: Key words Proximal diabetic ; neuropathy ; Diabetic amyotrophy ; Epineurial microvasculitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Amongst the focal and multifocal neuropathies that are associated with diabetes mellitus one of the most common is a proximal predominantly motor lower limb neuropathy. Recent evidence has indicated that, at least in a proportion of cases, this may have an inflammatory basis. We have examined a consecutive series of 15 cases of proximal diabetic neuropathy (diabetic amyotrophy). These were characterized by proximal pain and asymmetric proximal or generalized lower limb muscle weakness, associated in some cases with radicular sensory involvement. Two-thirds of the patients had an accompanying distal symmetric sensory polyneuropathy. Biopsy of the intermediate cutaneous nerve of the thigh, a sensory branch of the femoral nerve, showed epineurial microvasculitis in 3 patients and nonvasculitic epineurial inflammatory infiltrates in another case. In a further case, microvasculitis was found in both in the sural nerve and a quadriceps muscle biopsy specimen. The detection of inflammatory changes appeared to be correlated with the occurrence of sensory radicular involvement. Whether similar changes are present in muscle nerves in this predominantly motor syndrome requires further study. Nevertheless, the present observations confirm the view that secondary vasculitic or other inflammatory reactions may contribute to some forms of diabetic neuropathy.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1459
    Keywords: Key words Charcot-Marie-Tooth ; type 1B ; Hereditary motor and ; sensory neuropathy type 1B ; Myelin ; protein zero mutation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have identified a new point mutation in the myelin protein zero (P0) gene in two genetically identical twins with a demyelinating neuropathy. The G to A transition at nucleotide position 382 caused an aspartic acid to asparagine substitution in exon 3. Moreover, we found clear clinical differences which were most evident at an early age. These observations suggest that the expression of this P0 mutation may be susceptible to external, non-genetic influences that may act early in the course of the disease to alter the phenotype.
    Type of Medium: Electronic Resource
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