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  • 1
    Electronic Resource
    Electronic Resource
    Familial amyotrophic lateral sclerosis with onset in bulbar sign, benign clinical course, and Bunina bodies: a clinical, genetic, and pathological study of a Japanese family (2000)
    Springer
    Acta neuropathologica 100 (2000), S. 603-607 
    Details
    ISSN: 1432-0533
    Keywords: Key words Bunina bodies ; Cu/Zn superoxide ; dismutase ; Familial amyotrophic lateral sclerosis ; Neuropathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a Japanese family with autosomal dominant adult-onset amyotrophic lateral sclerosis (FALS) with onset in the bulbar musculature, clinically benign course, absence of the Cu/Zn superoxide dismutase-1 (SOD 1) gene mutation, and many Bunina bodies, in addition to involvement of the upper and lower motor neurons. The proband was a Japanese woman who was 66 years old at the time of death. Family history disclosed five patients with FALS over three generations. She developed dysarthria at age 57, followed by dysphagia, muscle weakness of the upper extremities, and difficulty in respiration. She could walk without support until her death. The elder sister of the proband developed dysarthria at age 48 and died at age 58. A genetic study of the nephew of the proband showed the absence of a mutation in the SOD 1 gene. Neuropathological examination of the proband disclosed neuronal loss in the upper and lower motor neurons, and numerous Bunina bodies in the lower motor neurons without Lewy body-like inclusions or ubiquitin-immunoreactive neuronal inclusions. No degeneration of the Clarke’s column, middle root zone of the posterior column, or posterior spinocerebellar tract was present. Review of the literature revealed that only patients with FALS with a long survival period of over 5 years had pathological findings consistent with FALS with posterior column involvement. This study contributes to the elucidation of the clinicopathological heterogeneity of FALS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010000237
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  • 2
    Electronic Resource
    Electronic Resource
    An intelligent hybrid method to automatically detect and eliminate experimental measurement errors for linear elastic deformation fields (2000)
    Springer
    Experimental mechanics 40 (2000), S. 170-179 
    Details
    ISSN: 1741-2765
    Keywords: Hybrid experimental-numerical method ; intelligent measurement ; noise elimination ; variational principle ; moiré interferometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Notes: Abstract In a previous study, to minimize or eliminate the errors and noises associated with a full-field experimental measurement and subsequent fringe analysis such as moiré interferometry, the authors derived a variational principle minimizing the experimental measurement errors. Furthemore, on the basis of this variational principle, the authors developed an intelligent hybrid method. In several test simulations, the method has demonstrated the automatic detection and elimination of randomly incorporated errors into known correct finite element displacement fields. In this study, a fringe analysis method is developed together with the two-dimensional fast Fourier transform method. Then, experimentally recorded moiré fringe patterns are analyzed by the fringe analysis method. The conventional and intelligent hybrid analyses are carried out using the analyzed fringe information as input data. The present method verifies the automatic detection of experimental errors and noises, and the simultaneous automatic elimination of those experimental errors. This method also makes it possible to obtain a fairly smooth visualization of higher order information such as the stress and strain distributions.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02325043
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    Paper (German National Licenses)
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