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伴有铁沉积的苍白球黑质路易体变性:三例尸检病例研究

Pallidonigroluysian degeneration with iron deposition: a study of three autopsy cases.

作者信息

Kawai J, Sasahara M, Hazama F, Kuno S, Komure O, Nomura S, Yamaguchi M

机构信息

Department of Pathology, Shiga University of Medical Science, Otsu, Japan.

出版信息

Acta Neuropathol. 1993;86(6):609-16. doi: 10.1007/BF00294300.

Abstract

In the basal ganglia of three autopsy cases of pallidonigroluysian degeneration, we found marked iron deposition, a finding which has not been mentioned previously in the literature. Besides severe astrogliosis and neuronal loss in the pallidum, Luysian body and nigra, granular deposits of brown pigments were found in the neuropil, microglias, oligodendrocytes and astrocytes in three such the nuclei and the striatum. These brown pigments proved histochemically to be iron. Our histochemical semiquantitative study showed a significantly stronger reaction for iron in the degenerated nuclei in these three cases than in control cases comprising non-degenerative and the other degenerative diseases. Quantitative study with inductively coupled emission spectrometry also demonstrated a markedly higher iron content in the globus pallidus and the striatum in comparison with the control cases. The possibility is discussed that iron deposition plays a role in generating the lesions of pallidonigroluysian degeneration.

摘要

在三例苍白球黑质路易体变性尸检病例的基底神经节中,我们发现了明显的铁沉积,这一发现此前在文献中未曾提及。除苍白球、路易体和黑质出现严重的星形胶质细胞增生和神经元丢失外,在这三个核团及纹状体的神经纤维网、小胶质细胞、少突胶质细胞和星形胶质细胞中发现了棕色色素颗粒沉积。组织化学证明这些棕色色素为铁。我们的组织化学半定量研究显示,这三例病例中退变核团的铁反应明显强于包括非退行性和其他退行性疾病的对照病例。电感耦合发射光谱定量研究也表明,与对照病例相比,苍白球和纹状体中的铁含量明显更高。文中讨论了铁沉积在苍白球黑质路易体变性病变形成中起作用的可能性。

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