Rehabilitation and Prophylaxis of Anomia in Primary Progressive Aphasia

描述(申请人提供)：这项研究的目标是纠正原发性进行性失语症(PPA)患者的单词查找问题，并延缓单词查找障碍的进一步进展。目前的方法是新颖的，因为它包含一个预防成分，在这个成分中，我们试图加强保持功能的神经连接，使它们更能抵抗随着疾病的进展而退化。虽然这项研究针对的是单词查找问题，但成功的结果将对其他旨在预防或逆转痴呆症认知功能下降的研究来说是个好兆头。PPA患者的图片命名刚刚开始出现下降迹象，将在两种情况下进行图片命名练习：观看图片并重复名称；查看带有书面名称的图片，以及阅读和书写名称。在这些条件下训练的图片的命名将在训练后的三个时间间隔与研究前测试但从未训练过的图片的命名进行比较。将使用包含训练项目的场景描述任务来评估概括性。据预测，将图片与其书写的名字配对，再加上写名字的机动任务，将产生比简单的观看图片和重复名字的练习更强的以后为图片命名的能力。还预测了对其他样本和其他任务情景的推广。此外，我们将对患者的大脑进行成像，以确定脑萎缩模式是否可以用于预测治疗反应。 公共卫生相关性：原发性进行性失语症是一种进行性语言处理障碍。在原发性进行性失语症的早期阶段，当患者仍然生活在家里，在正常的家庭环境下运作时，正常生活的最大挑战之一是沟通困难，部分原因是无法使用适当的词语。这项研究的目标是成功地延迟或补救此类单词查找问题，这将对原发性进行性失语患者的生活质量以及他们的家人和照顾者产生重大影响。

DESCRIPTION (provided by applicant): The goal of this study is to remediate word-finding problems in patients with Primary Progressive Aphasia (PPA) and to delay the further progression of word-finding impairment. The current approach is novel in that it contains a prophylaxis component in which we attempt to strengthen neural connections that remain functional, making them more resistant to degradation as the disease progresses. While the study is specific in its targeting of word-finding problems, a successful outcome would bode well for other studies aimed at prevention or reversal of declining cognitive functions in dementia. Patients with PPA whose naming of pictures has just begun to show signs of decline will receive practice with picture naming in two conditions: viewing the picture and repeating the name; and viewing the picture with its written name, plus reading and writing the name. Naming of pictures trained in each of these conditions will be compared, at three time intervals post-training, with naming of pictures tested before the study but never trained. A scene-description task containing trained items will be used to assess generalization. It is predicted that the pairing of the picture with its written name, combined with the motor task of writing the name, will result in a greater ability to name the picture at a later date than simple practice viewing the picture and repeating the name. Generalization to other exemplars and to other task situations is also predicted. In addition, we will image the brains of the patients to determine whether patterns of brain atrophy can be used to predict treatment response. PUBLIC HEALTH RELEVANCE: Primary progressive aphasia is a progressive disorder of language processing. During the early stages of primary progressive aphasia, when patients are still living at home and functioning within the normal family setting, one of the greatest challenges to normal living is difficulty communicating caused in part by the inability to access the appropriate words. The successful delay or remediation of such word finding problems - the goal of the proposed study - would have significant consequences for the quality of life of patients with primary progressive aphasia, and for their families and caregivers.