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成人起病的伴有轴突球状体和色素性胶质细胞的白质脑病（ALSP）中胼胝体优先变薄的病理基础。

Pathologic basis of the preferential thinning of thecorpus callosum in adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP).

作者信息

Kinoshita Michiaki, Oyanagi Kiyomitsu, Kondo Yasufumi, Ishizawa Keisuke, Ishihara Kenji, Yoshida Mari, Inoue Teruhiko, Mitsuyama Yoshio, Yoshida Kunihiro, Yamada Mitsunori, Sekijima Yoshiki, Ikeda Shu-Ichi

机构信息

Department of Neurology, Suwa Red Cross Hospital, 5-11-50 Kogandori, Suwa 392-8510, Japan.

Division of Neuropathology, Department of Brain Disease Research, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan.

出版信息

eNeurologicalSci. 2021 Jan 22;22:100310. doi: 10.1016/j.ensci.2021.100310. eCollection 2021 Mar.

DOI:10.1016/j.ensci.2021.100310
PMID:33553700
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7844436/
Abstract

BACKGROUND

Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is an early onset dementia characterized by axonal loss in the cerebral white matter with swollen axons (spheroids). It had been reported that the preferential thinning and "focal lesions" of the corpus callosum were observed on T2-weighted MRI in ALSP patients. The present study aimed to reveal the pathologic basis of them in relation to brain lesion staging (I ~ IV: Oyanagi et al. 2017).

METHODS

Seven autopsied brains of ALSP and five controls were neuropathologically examined.

RESULTS

Even at Stage I, corpus callosum body showed evident atrophy, and the atrophy advanced with stage progression. Spheroid size and density were maximal at Stage II in both centrum semiovale and corpus callosum body, but spheroids were larger in corpus callosum body than in centrum semiovale. Microglia in the body at Stage II had a larger cytoplasm than those in centrum semiovale. But spheroids and microglia in the "focal lesions" were identical with those of centrum semiovale.

CONCLUSION

Preferential thinning of corpus callosum was considered to be formed in relation to peculiar morphological alteration of microglia there in ALSP. Instead, "focal lesions" were formed in connection with the lesions in centrum semiovale.

摘要

背景

成人起病的轴索性球状体和色素性神经胶质细胞白质脑病（ALSP）是一种早发性痴呆，其特征为脑白质中轴突丢失并伴有轴突肿胀（球状体）。据报道，在ALSP患者的T2加权磁共振成像（MRI）上可观察到胼胝体的优先变薄和“局灶性病变”。本研究旨在揭示其与脑病变分期（I~IV期：Oyanagi等人，2017年）相关的病理基础。

方法

对7例ALSP尸检脑和5例对照进行神经病理学检查。

结果

即使在I期，胼胝体也显示出明显萎缩，且萎缩随分期进展而加重。在半卵圆中心和胼胝体中，球状体大小和密度在II期最大，但胼胝体中的球状体比半卵圆中心中的更大。II期胼胝体中的小胶质细胞比半卵圆中心中的小胶质细胞具有更大的细胞质。但“局灶性病变”中的球状体和小胶质细胞与半卵圆中心中的相同。

结论

ALSP中胼胝体的优先变薄被认为是与那里小胶质细胞的特殊形态改变有关而形成的。相反地，“局灶性病变”是与半卵圆中心的病变相关而形成的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/c8d1a2a5f1ee/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/f30bb40eb653/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/9d5da073a10e/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/f793477d06fa/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/81688f95bf29/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/6f0b4a0ee75e/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/c8d1a2a5f1ee/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/f30bb40eb653/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/9d5da073a10e/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/f793477d06fa/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/81688f95bf29/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/6f0b4a0ee75e/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb87/7844436/c8d1a2a5f1ee/gr6.jpg

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