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迟发性散发性进行性皮质下胶质增生症。

Late-onset sporadic progressive subcortical gliosis.

作者信息

Lanska D J, Markesbery W R, Cochran E, Bennett D, Lanska M J, Cohen M

机构信息

Department of Neurology and The Sanders-Brown Center on Aging, University of Kentucky Medical Center, Lexington, USA.

出版信息

J Neurol Sci. 1998 May 7;157(2):143-7. doi: 10.1016/s0022-510x(98)00063-x.

DOI:10.1016/s0022-510x(98)00063-x
PMID:9619636
Abstract

We report two sporadic cases of progressive subcortical gliosis (PSG) with onset after age 60. The presentation included slowly progressive dementia with memory loss, geographic disorientation, and personality change. Both were diagnosed clinically as Alzheimer's disease (AD) and both met NINCDS-ADRDA criteria for probable AD. Autopsy revealed generalized atrophy, predominantly involving the white matter of the frontal and temporal lobes. Microscopically, prominent fibrillary astrocytosis was present in the subcortical white matter and in the subpial and deep layers of the overlying cerebral cortex. Mild cortical neuron loss accompanied the gliosis, but no myelin loss was evident. Amyloid deposits and neuronal cytoskeletal inclusions were absent.

摘要

我们报告了两例60岁以后发病的散发性进行性皮质下胶质增生(PSG)病例。临床表现为伴有记忆力减退、空间定向障碍和人格改变的缓慢进展性痴呆。两例患者临床均诊断为阿尔茨海默病(AD),且均符合NINCDS-ADRDA可能AD的标准。尸检显示广泛性萎缩,主要累及额叶和颞叶白质。显微镜下,皮质下白质以及覆盖其上的大脑皮质软膜下和深层可见显著的纤维性星形细胞增生。胶质增生伴有轻度皮质神经元丢失,但未见明显髓鞘丢失。无淀粉样沉积物和神经元细胞骨架包涵体。

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