CSF abnormalities in patients with aceruloplasminemia

被引：35

作者：

Miyajima, H

Fujimoto, M

Kohno, S

Kaneko, E

Gitlin, JD

机构：

[1] Hamamatsu Univ Sch Med, Dept Med 1, Hamamatsu, Shizuoka 4313192, Japan

[2] Washington Univ, Sch Med, Edward Mallinckrodt Dept Pediat, St Louis, MO 63110 USA

来源：

NEUROLOGY | 1998年 / 51卷 / 04期

关键词：

D O I：

10.1212/WNL.51.4.1188

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Aceruloplasminemia is a disorder of iron metabolism characterized by degeneration of the retina and basal ganglia. CSF from affected patients showed a threefold increased iron concentration that was associated with increased superoxide dismutase activity and lipid peroxidation products. These findings support the hypothesis that iron-mediated lipid peroxidation contributes to neurodegeneration in patients with aceruloplasminemia. Such measurements may have value in assessing disease progression as well as the results of iron chelation and other therapeutic interventions.

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页码：1188 / 1190

页数：3

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