Inflammatory glial activation in the brain of a patient with hereditary sensory neuropathy type 1 with deafness and dementia
Inflammatory glial activation in the brain of a patient with hereditary sensory neuropathy type 1 with deafness and dementia
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DOI：
10.1016/j.neulet.2004.06.030
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发表时间：
2004-09-09
影响因子：
2.5
通讯作者：
Maeda, K
中科院分区：
文献类型：
--
作者：
Hojo, K;Kawamata, T;Maeda, K
对1例伴有感音神经性耳聋和早发性痴呆的遗传性感觉神经病1型（HSN-1）患者的脑进行了神经病理学研究。大脑新皮质、海马和基底节广泛的神经元变性是其临床特征。在海马、额叶、顶叶和枕叶，神经元丢失伴残留神经元气球样变是显著的。这些区域的神经元变性伴有轴突营养不良和胶质反应，如小胶质细胞增生和星形细胞增生，然而，仅基底节、脑干和小脑中的胶质反应突出，伴有轻度神经元丢失。这些结果表明，广泛的神经元变性可能被炎症过程加速，包括与耳聋和痴呆相关的HSN-1患者脑中的神经胶质活化。(C)2004爱思唯尔爱尔兰有限公司保留所有权利。
The brain of a patient with hereditary sensory neuropathy type 1 (HSN-1) associated with sensorineural deafness and early-onset dementia was neuropathologically investigated. Widespread neutronal degeneration in cerebral neocortex, hippocampus and basal ganglia was revealed, accounting for the clinical features. Loss of neurons with ballooning of residual neurons was remarkable in the hippocampus and frontal, parietal, and occipital lobes. Neuronal degeneration in these regions was accompanied by axonal dystrophy and glial reactions such as microgliosis and astrocytosis, however, only glial responses were prominent in the basal ganglia, brain-stem and cerebellum with mild neuronal loss. These results indicate that the widespread neuronal degeneration may be accelerated by inflammatory processes including glial activation in the brain of a patient with HSN-1 associated with deafness and dementia. (C) 2004 Elsevier Ireland Ltd. All rights reserved.