抗磷脂综合征患者伴运动神经元病的额颞叶痴呆：一例报告
Frontotemporal Dementia with Motor Neuron Disease in a Patient with Antiphospholipid Syndrome: A Case Report.
作者信息
Jeong Yoon-Cheol, Park Jin-Seok, Kim Seung-Hyun, Choi Hojin
机构信息
Department of Neurology, Hanyang University College of Medicine, Seoul, Korea.
出版信息
Dement Neurocogn Disord. 2016 Dec;15(4):165-169. doi: 10.12779/dnd.2016.15.4.165. Epub 2016 Dec 31.
BACKGROUND
Frontotemporal dementia (FTD) with motor neuron disease (MND) is a syndrome of progressive changes in behavior, language, muscle weakness and atrophy due to loss of function of neurons in the frontal and temporal lobes and in motor neurons. Etiology and pathogenesis of FTD with MND are still uncertain.
CASE REPORT
A 71-year-old man presented with a 2-year history of progressive muscle weakness and cognitive deficits. We diagnosed this patient as FTD with MND by neurological examination, electromyography, brain imaging and neuro-psychological evaluation. We also confirmed antiphospholipid syndrome (APS) in this patient as a way to rule out secondary causes of MND.
CONCLUSIONS
This was a very rare case of FTD with MND in APS. We should focus study on the possible role of autoimmune pathogenesis in FTD with MND.
背景
伴有运动神经元病（MND）的额颞叶痴呆（FTD）是一种由于额叶、颞叶及运动神经元功能丧失而导致行为、语言、肌肉无力和萎缩进行性改变的综合征。伴有MND的FTD的病因和发病机制仍不明确。
病例报告
一名71岁男性，有2年进行性肌肉无力和认知缺陷病史。通过神经检查、肌电图、脑成像和神经心理学评估，我们将该患者诊断为伴有MND的FTD。我们还确诊该患者患有抗磷脂综合征（APS），以排除MND的继发原因。
结论
这是一例非常罕见的APS合并伴有MND的FTD病例。我们应重点研究自身免疫发病机制在伴有MND的FTD中可能发挥的作用。
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