Kim Eun-Joo, Park Kyung-Won, Lee Jae-Hong, Choi SeongHye, Jeong Jee H, Yoon Soo Jin, Kim Byeong C, Kwon Jay C, Ku Bon D, Kim Seung Hyun, Choi Byung-Ok, Na Duk L
Department of Neurology, Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute, Seoul, Korea.
Department of Neurology, Dong-A Medical Center, Dong-A University College of Medicine, Busan, Seoul, Korea.
Dement Geriatr Cogn Dis Extra. 2014 Jul 10;4(2):242-51. doi: 10.1159/000360278. eCollection 2014 May.
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.
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.
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.
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.
我们通过韩国痴呆临床研究中心(CREDOS)-额颞叶痴呆(FTD)登记处,调查了额颞叶痴呆(FTD)的人口统计学、临床和神经心理学特征。
从韩国16家神经科诊所连续招募了200例FTD患者,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进行评估。
在我们的登记处中,78例患者被分类为行为变异型FTD(bvFTD),70例为语义性痴呆(SD),33例为进行性非流利性失语(PNFA),8例为运动神经元病加综合征(MND-plus)。痴呆语言变异型患者比bvFTD患者年龄更大。四个亚组之间在性别比例、病程或教育程度方面没有差异。总体而言,bvFTD患者在认知测试中的表现明显更好。与bvFTD和SD相比,PNFA中运动症状的发生率更高,行为症状的发生率更低。SD的整体语言指数明显低于bvFTD和PNFA。MND-plus组在所有认知领域的表现均比其他所有组差。
四种亚型的神经心理学、行为、运动和语言特征与其他系列研究中的特征相当。然而,在我们的登记处中,SD的比例(37.0%)与bvFTD的比例(41.3%)相似,高于其他系列研究。