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Clinical and Neuropsychological Characteristics of a Nationwide Hospital-Based Registry of Frontotemporal Dementia Patients in Korea: A CREDOS-FTD Study

机译:基于全国性医院的额颞痴呆患者注册系统的临床和神经心理特征:一项CREDOS-FTD研究

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Background: We investigated the demographic, clinical, and neuropsychological characteristics of frontotemporal dementia (FTD) from the Clinical Research Center for Dementia of South Korea (CREDOS)-FTD registry. Methods: A total of 200 consecutive patients with FTD recruited from 16 neurological clinics in Korea were evaluated by cognitive and functional assessments, a screening test for aphasia, behavioral questionnaires, motor assessments, and brain MRI or PET. Results: In our registry, 78 patients were classified as having been diagnosed with behavioral-variant FTD (bvFTD), 70 with semantic dementia (SD), 33 with progressive nonfluent aphasia (PNFA), and 8 with motor neuron disease plus syndrome (MND-plus). The patients with language variants of dementia were older than those with bvFTD. There were no differences in sex ratio, duration of illness, or level of education among the four subgroups. Overall, the patients with bvFTD showed a significantly better performance in cognitive tests. A higher frequency of motor symptoms and a lower frequency of behavioral symptoms were found in PNFA than in bvFTD and SD. The Global Language Index was significantly lower in SD than in bvFTD and PNFA. The MND-plus group had a poorer performance than all the others in all cognitive domains. Conclusion: The neuropsychological, behavioral, motor, and language characteristics of the four subtypes are comparable with those from other series. However, the proportion of SD (37.0%), which was similar to that of bvFTD (41.3%), was higher in our registry than in other series.
机译:背景:我们调查了韩国痴呆临床研究中心(CREDOS)-FTD注册中心的额颞叶痴呆(FTD)的人口统计学,临床和神经心理学特征。方法:通过认知和功能评估,失语症筛查测试,行为问卷,运动评估以及脑部MRI或PET,对韩国16家神经病学诊所招募的200名连续性FTD患者进行了评估。结果:在我们的注册表中,将78例患者诊断为行为变异性FTD(bvFTD),70例语义痴呆(SD),33例进行性非流利性失语症(PNFA)和8例运动神经元疾病加综合征(MND) -加)。患有痴呆症语言变异的患者比患有bvFTD的患者年龄更大。四个亚组之间的性别比例,疾病持续时间或受教育程度没有差异。总体而言,bvFTD患者在认知测试中表现出明显更好的表现。与bvFTD和SD中相比,PNFA中发现较高的运动症状频率和较低的行为症状频率。 SD的全球语言指数显着低于bvFTD和PNFA。 MND-plus组在所有认知领域的表现均较其他组差。结论:这四个亚型的神经心理,行为,运动和语言特征可与其他系列的特征相媲美。但是,我们的注册表中SD的比例(37.0%)与bvFTD的比例(41.3%)相似,高于其他系列。

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