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  • 1
    ISSN: 1432-0533
    Keywords: Myotonic dystrophy ; Dorsal raphe nucleus ; Superior central nucleus ; Hypersomnia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A quantitative study of neurons in the dorsal raphe nucleus (DRN) and the superior central nucleus (SCN) was performed in seven patients with myotonic dystrophy (MyD), five of whom showed hypersomnia, and in eight age-matched controls. The densities of neurons in the DRN and the SCN were significantly lower in MyD patients with hypersomnia than in MyD patients without hypersomnia and control subjects. There was an appreciable positive correlation in the density of neurons between the DRN and the SCN in all MyD patients. These data suggest that the neuronal loss of the DRN and the SCN is associated with the presence of hypersomnia in MyD.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Myotonic dystrophy ; Intracytoplasmic inclusion bodies ; Marinesco bodies ; Thalamus ; Substantia nigra
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Intracytoplasmic inclusion bodies of the thalamus and the substantia nigra, and Marinesco bodies have been studied in four patients with myotonic dystrophy (MyD), eight patients with other neurological diseases (control A), and eight patients without neurological diseases (control B). The percentages of the affected cells were calculated by dividing the number of neurons including intracytoplasmic inclusion bodies of the thalamus and the substantia nigra, and Marinesco bodies, by the total cell count in these respective regions. Statistical analyses were performed with regard to the frequency of these bodies by using Student'st test. There was a significantly higher incidence of intracytoplasmic inclusion bodies of the thalamus (13.2% versus 0.7%,P〈0.001) and the substantia nigra (20.4% versus 2.7%,P〈0.001), and Marinesco bodies (37.4% versus 4.1%,P〈0.001) in patients with MyD than in controls A and B. From our observations, it is suggested that the presence with a high frequency, in combination, of these bodies is not an incidental finding but may have an intimate and important relationship with the pathogenesis of MyD, and may be a conspicuous and diagnostically important feature of MyD.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 247 (2000), S. 633-635 
    ISSN: 1432-1459
    Keywords: Key words Amyloid β protein ; Amyotrophic lateral sclerosis ; Skin ; Alzheimer's disease ; Enzyme-linked immunosorbet assay
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Distinct vascular and periadnexal immunoreactivity have been observed for amyloid b protein (Aβ) in skin from patients with amyotrophic lateral sclerosis (ALS). We used an enzyme-linked immunosorbent assay to make a more quantitative comparison of Aβ concentrations between ALS patients and controls. The insoluble fractions of skin samples from ALS patients contained significantly higher Aβ concentrations per milligram protein than those from controls. Various alterations in extracellular matrix components have been reported to occur in the skin of patients with ALS, and several matrix constituents have been shown to affect processing and aggregation of Aβ in human brain. Taking these previous findings together with those of the present study, our observations suggest that changes in extracellular matrix in skin of ALS patients may facilitate aggregation and deposition of Aβ.
    Type of Medium: Electronic Resource
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