心脏与大脑:一种新的关联——一个“可能患有额颞叶痴呆和心脏传导阻滞”的家庭报告

S. Chandra, P. Mailankody, Manisha Gupta
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引用次数: 0

摘要

为5/10,类别流利度为4,音位流利度为零。注意力测试显示出明显的分散注意力,但她可以接受采访。最近的记忆受到严重影响,远程受到中度影响。在视觉空间测试中,患者甚至不能画出简单的图表,但没有确定的诊断。根据《精神疾病诊断与统计手册》第五版标准,考虑了可能的额颞叶痴呆。心脏评估显示有起搏器节律。回声显示通过右心房和右心室可以看到起搏器。超声心动图显示左心室舒张功能障碍,射血分数为58%。PET扫描显示额颞叶代谢低下,磁共振成像显示额颞叶萎缩[图1]。然而,我们无法进行基于体素的形态测量来证明任何特定的岛叶区域体积损失。她的父亲、姑姑、姐姐、哥哥和她自己在49岁至53岁时因心脏传导阻滞而使用起搏器[图2]。此外,据她丈夫告知,他们都有记忆和行为问题,但受影响的成员无法进行详细评估。一个姐姐患有败血症,享年55岁。这表明额颞叶痴呆(FTD)和心脏传导阻滞在这个家族中有着强烈的相关性,这是一种主要的遗传模式。这个家族可能指向FTD的一个新基因型。
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Heart and brain: A new association – Report of a family with “probable frontotemporal dementia and heart block”
was 5/10, category fluency was 4, and phonemic fluency was nil. Attention testing showed significant distractibility, but she could be interviewed. Recent memory was severely affected, and remote was moderately affected. In visuospatial testing, the patient could not draw even simple diagrams, but no definite agnosias were identified. Probable frontotemporal dementia was considered applying the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition Criteria. Cardiac evaluation showed a pacemaker rhythm. Echo showed a pacemaker seen through the right atrium and right ventricle. Echo cardiography revealed left ventricular diastolic dysfunction with Ejection fraction of 58%. PET scan showed frontotemporal hypometabolism, and magnetic resonance imaging showed frontotemporal atrophy [Figure 1]. However, we could not do voxel‐based morphometry to demonstrate any specific insular region volume loss. Her father, aunt, sister, brother, and herself were on pacemaker at ages from 49 to 53 years for heart block [Figure 2]. They all in addition had memory and behavior problems as informed by her husband, but the affected members were not available for detailed evaluation. One sister had sepsis and died at 55 years. This suggests a strong association of frontotemporal dementia (FTD) and heart block in a dominantly inherited pattern in this family. This family might point to a new genotype of FTD.
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