[EEG in infantile neuroaxonal dystrophy and Hallervorden-Spatz disease].

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    ABSTRACT: Dynamic studies of iron metabolism in brain are generally unavailable despite the fact that a number of neurologic conditions are associated with excessive accumulation of iron in central nervous tissue. Cortical non-neuronal (glial) cultures were prepared from fetal mouse brain. After 13 days the cultures were exposed to radiolabeled iron. Brisk and linear total iron uptake and ferritin iron uptake occurred over 4 hours. When methylamine or ammonium chloride was added, (both known inhibitors of transferrin iron release because of their lysosomotropic properties), total iron uptake was diminished. Further studies indicated that methylamine inhibits glial cell ferritin iron incorporation. Glial cell iron transport is similar to previously reported neuronal cell iron transport (1) but glial cell iron uptake proceeds at a faster rate and is more susceptible to the inhibition of certain lysosomotropic agents. The data reinforces the likelihood that iron uptake by nervous tissues is transferrin-mediated.
    Neurochemical Research 01/1986; 10(12):1635-44. · 2.13 Impact Factor
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    ABSTRACT: An autopsy case is reported which revealed not only clinical and neuropathological features of progressive supranuclear palsy, but also the presence of large numbers of Lewy bodies in the brain stem nuclei and cerebral cortex. This case seems to be progressive supranuclear palsy with Lewy bodies distributed as in Parkinson's disease. Such case has not been previously reported.
    Acta Neuropathologica 02/1986; 71(3-4):344-6. · 9.73 Impact Factor
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    ABSTRACT: Primary torsion dystonia is an idiopathic movement disorder presumably caused by abnormal function of the basal ganglia. The disorder may be inherited either as an autosomal dominant, autosomal recessive, or X-linked recessive trait. At least six forms of autosomal dominant torsion dystonia can be distinguished clinically. Linkage analysis in one form of autosomal dominant torsion dystonia permits the assignment of a "torsion dystonia locus" to the long arm of chromosome 9.
    Human Genetics 02/1990; 84(2):107-15. · 4.63 Impact Factor