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  • 1
    ISSN: 1432-0533
    Keywords: Connatal Pelizaeus-Merzbacher disease ; Congenital stridor ; X-linked inheritance ; Psychomotor retardation ; Quantified cerebral morphology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two maternal cousins are described with the connatal form of Pelizaeus-Merzbacher Disease (PMD) and congenital stridor. Study of brain biopsy material confirms the diagnosis of PMD. The neuropathological findings are suggestive for the transitional form of this disease. Quantitative morphology gives support to the hypothesis that PMD is a disturbance in maturation of neurons and in myelin formation rather than an active degenerative process. The hereditary transmission is most consistent with a sex-linked recessive pattern. Different X-linked signs seem combined in the presented cases.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 80 (1990), S. 432-438 
    ISSN: 1432-0533
    Keywords: Brain ; drug effects ; Abnormalities, drug-induced ; Pregnancy ; Animals, drug effects ; Methylmercury
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The effects of organic mercury compounds on the development of the brain are well known since the exposure of people at a large scale to methylmercury in the Minamata Bay area and in Iraq. The neuropathological examination of the brains of children prenatally exposed revealed dysplasia of the cerebral and cerebellar cortex, neuronal ectopia and several other developmental disturbances. In this experimental study we examined developmental mechanisms involved in methylmercury-induced cerebral anomalies. By examining the fetuses soon after treatment we concentrated in the initial effects of the treatment. The pregnant rats were given 10 mg/kg methylmercury chloride i.p. on day 18. Already at 2 h after administration mitochondrial degeneration occurred in the endothelium of the cerebral capillaries. Subsequently hemorrhages developed interfering with the cellular arrangement in the ventricular zone, with neuronal migration in the intermediate zone and with the development of the cortical cytoarchitecture. Macrophages and cavities appeared in the hemorrhagic areas. It is suggested that the abnormalities seen in the experiments can be considered as the initial methylmercury-induced effects which, in combination with various other toxic effects, ultimately result in the anomalies that have been observed in the brains of children prenatally exposed to methylmercury.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Archives of toxicology 47 (1981), S. 25-37 
    ISSN: 1432-0738
    Keywords: Rabbit ; Foetal brain ; Cyst ; Artefact ; Fixation methods
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Reports have appeared in the literature on brain cysts in rabbit foetuses. This paper reports on investigations carried out to assess whether “cystic dilatation” is a malformation or an artefact. The results show that “cystic dilatation” arises by splitting of the pia-arachnoid membrane leading to a space between the skull and the neural tissue which is lined by the pial layer on its interior aspect and the arachnoid on its exterior aspect. The evidence presented indicates that “cystic dilatation” is a fixation-induced artefact. In addition, the presence of several artificial tissue clefts and spaces in preserved rabbit foetal brains is reported.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1920
    Keywords: Huntington's disease ; Computed tomography ; Magnetic resonance imaging ; Single photon emission computed tomography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A Turkish family with Huntington's disease documented on CT, MRI and SPECT is reported. Whereas in clinically definite cases CT and MRI are of limited value and SPECT does not add anything of value, in one asymptomatic subject SPECT showed moderate caudate nucleus hypoperfusion, underlining the hypothesis that SPECT may have a role in predicting Huntington's disease.
    Type of Medium: Electronic Resource
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