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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Semantic dementia with lower motor neuron disease showing FTLD-TDP type 3 pathology (sensu Mackenzie).
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Semantic dementia with lower motor neuron disease showing FTLD-TDP type 3 pathology (sensu Mackenzie).

机译：下运动神经元疾病的语义性痴呆，表现为FTLD-TDP 3型病理（sensu Mackenzie）。

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摘要

We describe a case of frontotemporal lobar degeneration with semantic dementia and lower motor neuron disease. A 63-year-old man presented with the full clinical picture of semantic dementia, including semantic anomia, surface alexia, lexical agraphia, associative agnosia, prosopagnosia and phonagnosia. Flaccid dysarthria, bulbar dysphagia and fasciculations developed 7 years after onset, followed by death within a year. The neuropathological examination showed heavy neuronal loss in the anterior temporal lobe cortex, dorsal vagal and hypoglossal nuclei and anterior horns of the spinal cord. Ubiquitin- and TDP-43-positive cytoplasmic inclusions were abundant in layer II of affected cortices and in granular cells of the hippocampal dentate gyrus, whereas dystrophic neurites were sparse and intranuclear inclusions absent. It is concluded that FTLD-TDP type 3 can be associated with semantic dementia and lower motor neuron disease in combination.
机译：我们描述了一例额颞叶变性伴语义性痴呆和下运动神经元疾病。一名63岁的男子呈现了语义性痴呆的完整临床图景，包括语义性失语，表面厌食症，词汇失语，联想性失语症，前瞻性失语症和语音失语症。弛缓性构音障碍，延髓吞咽困难和束缚发作于发病后7年，随后一年内死亡。神经病理学检查显示前颞叶皮质，背迷走神经和舌下核和脊髓前角神经元大量丢失。泛泛素和TDP-43阳性细胞质内含物在受影响皮层的II层和海马齿状回的颗粒细胞中丰富，而营养不良的神经突稀疏，没有核内内含物。结论是，FTLD-TDP 3型可与语义性痴呆和下运动神经元疾病相结合。

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