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  • Remyelination  (2)
  • Adrenomyeloneuropathy  (1)
  • Alternating hemiparesis  (1)
  • Beta-galactosidase deficiency  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 45 (1979), S. 43-45 
    ISSN: 1432-0533
    Keywords: Cerebrotendinous xanthomatosis ; Segmental demyelination ; Remyelination ; Sural nerve ; Onion bubl
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In a case of cerebrotendinous xanthomatosis (CTX), confirmed biochemically and histologically, quantitative histological studies of the biopsied sural nerve revealed significantly higher incidence of de- and remyelination and onion bulb than in controls. The density of total myelinated fibers fell within the range of controls, although the density of large myelinated fibers seemed to be slightly decreased. It was suggested that the preferential involvement of the myelin sheath and Schwann cell may exist in CTX.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Multiple sclerosis ; Schwann cell ; Remyelination ; Glial fibrillary acidic protein ; Immunocytochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary To extend earlier observations on Schwann cell remyelination in multiple sclerosis (MS) lesions (Itoyama et al. 1983) we immunostained spinal cord sections from eight Japanese MS patients with antiserum to Po glycoprotein, a major constituent of peripheral nervous system (PNS) myelin, myelin basic protein (MBP), and glial fibrillary acidic protein (GFAP). Spinal cord sections from six of the eight Japanese MS patients contained large clusters of peripheral myelin sheaths with anti-Po immunoreactivity. In lesions found in four of the six patients, thousands of Po-stained PNS myelin sheaths were present. Necrosis was prominent in these lesions which included more than half of the spinal cord's transverse area. The number and density of regenerating myelin sheaths of peripheral origin were much greater than we observed in MS spinal cord lesions of white people (Itoyama et al. 1983). Anti-GFAP immunoreactivity was present in most brain and spinal cord lesions. However, the areas in lesions that contained large groups of PNS myelin sheaths lacked anti-GFAP immunoreactivity. Our data suggest that spinal MS lesions that are large, severely demyelinated, and partially necrotic may contain factors that inhibit fibrous astrogliosis. These factors, other substances in the large lesions and/or the lack of astrocytic scarring could then promote Schwann cell invasion, multiplication, and remyelination of surviving axons.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Neuroradiology 28 (1986), S. 284-284 
    ISSN: 1432-1920
    Keywords: Alternating hemiparesis ; Neuro-Behçet's disease ; Thin-cut ; CT slices
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1459
    Keywords: Adrenoleukodystrophy ; Adrenomyeloneuropathy ; Fatty acids ; High-performance liquid chromatography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Mittels Hochleistungs-Flüssigkeitschromatographie wurden durch Analyse der Fettsäuren in Sphingomyelien Adrenoleukodystrophien und Adrenomyeloneuropathien untersucht. Die oben erwähnte analytische Methode erwies sich als einfach, empfindlich und exakt. Bei der Adrenoleukodystrophie und bei Patienten mit Adrenomyeloneuropathie wurden sehr lange Fettsäureketten (C26:0, C25:0 und C24:0 auf der Basis von C22:0) sowohl in Plasma und den Erythrozytenmembranen als auch in Gesamtblutproben als vermehrt erkannt. Auch in Kulturen von Hautfibroblasten von Sphingomyelien wurde eine Zunahme der langkettigen Fettsäuren nachgewiesen. In einem Fall eines heterozygoten Adrenoleukodystrophiepatienten wurden Werte die zwischen jenen der homozygoten und der Kontrollfälle lagen gefunden. Somit ist die Hochleistungs-Flüssigkeitschromatographie eine brauchbare Methode, um eine Zunahme langkettiger Fettsäuren zwecks Diagnose einer Adrenoleukodystrophie oder einer Adrenomyeloneuropathie festzustellen.
    Notes: Summary Using high-performance liquid chromatography, adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AMN) were diagnosed by the analysis of fatty acids in sphingomyelin. The analytical method was simple, sensitive and accurate. In ALD and AMN patients, very long chain fatty acids (C26:0, C25:0 and C24:0 on the base of C22:0) were elevated not only in plasma and erythrocyte membranes but also in whole blood samples. An increase of long chain fatty acids was also shown in sphingomyelin of cultured skin fibroblasts. One heterozygote for ALD showed intermediate values between homozygotes and controls. Thus, high-performance liquid chromatography is a valuable method to detect increased long chain fatty acids for the diagnosis of ALD or AMN.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 233 (1986), S. 306-308 
    ISSN: 1432-1459
    Keywords: Adrenoleukodystrophy ; Beta-galactosidase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A patient with adrenoleukodystrophy and his mother, a carrier, showed an elevated ratio of very long-chain fatty acids to long-chain fatty acids and decreased beta-galactosidase activity. Other lysosomal enzyme activities were normal except for the borderline level of arylsulfatase-A activity. However, the father and other patients with variant forms of adrenoleukodystrophy showed normal beta-galactosidase and other lysosomal enzyme activities.
    Type of Medium: Electronic Resource
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