Shi Zhihong, Liu Shuai, Wang Ying, Liu Shuling, Han Tong, Cai Li, Zhou Yuying, Gao Shuo, Ji Yong
Tianjin Key Laboratory of Cerebrovascular and Neurodegenerative Diseases, Tianjin Dementia Institute Department of Neurology, Tianjin Huanhu Hospital PET-CT Center, General Hospital of Tianjin Medical University Department of Neuroimaging, Tianjin Huanhu Hospital, Tianjin, China.
Medicine (Baltimore). 2017 Sep;96(37):e7948. doi: 10.1097/MD.0000000000007948.
The aim of the study was to obtain an overview of the clinical and neuroimaging features of Chinese patients with subtypes of frontotemporal lobe degeneration (FTLD).We evaluated the demographic features, clinical presentation, and lobe atrophy depicted by magnetic resonance imaging (MRI) in 133 patients with FTLD. Two positron emission tomography (PET) scans were performed at baseline: [C]Pittsburgh compound B PET to assess amyloid-β plaque load and [F]fluorodeoxyglucose (FDG) PET to assess glucose metabolism.The behavioral variant of FTD (bvFTD) was the most common subtype (67.7%) of FTLD. The percentages of progressive nonfluent aphasia (PNFA) and semantic dementia (SD) were similar. Cerebral lobe atrophy was seen in 87.7% of the cases. The Activities of Daily Living scale, Mini-Mental State Examination, and Montreal Cognitive Assessment scores were significantly correlated with the degree of overall atrophy. The severity of abnormal behavior was correlated with right anterior and right posterior temporal atrophy scores. The overall atrophy scores and atrophy score in the left temporal region were related to cognitive outcomes and Activities of Daily Living scores. Most of the bvFTD patients presented symmetric/asymmetric hypometabolism in the bilateral temporal cortex, frontal cortex, anterior cingulate cortex, insula, and caudate nucleus. All the PNFA patients presented left dominant hypometabolism in the frontal cortex. All the SD patients presented left dominant hypometabolism in the anterior temporal cortex.FTLD is not rare in cognitive clinics, and the ratios of subtypes in Chinese patients are similar to other ethnic groups. Overall atrophy scores, determined by MRI, were related to the severity of cognitive dysfunction and deficits in Activities of Daily Living. Patterns of hypometabolism, determined by [F]FDG PET, were more specific to subtypes of FTLD and may help provide differential diagnoses of variants of FTLD.
本研究旨在全面了解中国额颞叶变性（FTLD）各亚型患者的临床及神经影像学特征。我们评估了133例FTLD患者的人口统计学特征、临床表现以及磁共振成像（MRI）显示的脑叶萎缩情况。在基线时进行了两次正电子发射断层扫描（PET）：[C]匹兹堡化合物B PET用于评估淀粉样β斑块负荷，[F]氟脱氧葡萄糖（FDG）PET用于评估葡萄糖代谢。
行为变异型额颞叶痴呆（bvFTD）是FTLD最常见的亚型（67.7%）。进行性非流利性失语（PNFA）和语义性痴呆（SD）的比例相似。87.7%的病例出现脑叶萎缩。日常生活活动量表、简易精神状态检查表和蒙特利尔认知评估得分与总体萎缩程度显著相关。异常行为的严重程度与右侧前颞叶和右侧后颞叶萎缩得分相关。左侧颞叶区域的总体萎缩得分和萎缩得分与认知结果及日常生活活动得分相关。大多数bvFTD患者在双侧颞叶皮质、额叶皮质、前扣带回皮质、岛叶和尾状核呈现对称/不对称代谢减低。所有PNFA患者在额叶皮质呈现左侧优势代谢减低。所有SD患者在颞叶前部皮质呈现左侧优势代谢减低。
FTLD在认知门诊并不罕见，中国患者各亚型的比例与其他种族群体相似。MRI确定的总体萎缩得分与认知功能障碍的严重程度及日常生活活动缺陷相关。[F]FDG PET确定的代谢减低模式对FTLD各亚型更具特异性，可能有助于对FTLD不同变异型进行鉴别诊断。
