Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Columbia University Irving Medical Center, New York, NY, USA.
The Association for Frontotemporal Degeneration, King of Prussia, PA, USA.
J Geriatr Psychiatry Neurol. 2023 May;36(3):201-214. doi: 10.1177/08919887221119976. Epub 2022 Aug 9.
Frontotemporal degeneration (FTD) is an umbrella term encompassing a range of rare neurodegenerative disorders that cause progressive declines in cognition, behavior, and personality. Hearing directly from individuals living with FTD and their care partners is critical in optimizing care, identifying meaningful clinical trial endpoints, and improving research recruitment and retention. The current paper presents a subset of data from the FTD Insights Survey, chronicling the diagnostic journey, symptoms, and the impact of FTD on distress, quality of life, and independence, in the mild to moderate stages of the disease. Survey respondents included 219 individuals diagnosed with FTD and 437 current care partners, representing a range of FTD diagnoses. Around half of survey respondents reported seeing three or more doctors before an FTD diagnosis was given, and a range of prior diagnoses were noted. Most frequently endorsed symptoms tended to be consistent with clinical characteristics of the specific diagnosis, though there was significant variability in symptoms reported within diagnostic categories as well as considerable overlap in symptoms between diagnostic categories. Cognitive and language symptoms of FTD were generally most distressing to the person diagnosed, and a loss of independence was endorsed as affecting quality of life. The distinct perspectives of diagnosed persons and care partners regarding disease impact differed notably for bvFTD/Pick's disease. Participating independently in a range of activities, within the home, outside the home, and with other people, were reported as challenging for people living with FTD, underscoring the degree to which the lives of these individuals are affected even at the mild and moderate stages of disease. Overall, by heeding the perspectives of those living with FTD, we can begin to design more meaningful research studies, provide better care, and develop therapies that improve quality of life.
额颞叶变性（FTD）是一个涵盖一系列罕见神经退行性疾病的总称，这些疾病会导致认知、行为和个性的逐渐衰退。直接听取患有 FTD 的个体及其护理伙伴的意见对于优化护理、确定有意义的临床试验终点以及改善研究招募和保留至关重要。本文介绍了 FTD 见解调查的一部分数据，该调查记录了疾病的轻度至中度阶段的诊断过程、症状以及 FTD 对困扰、生活质量和独立性的影响。调查对象包括 219 名被诊断患有 FTD 的人和 437 名现任护理伙伴，代表了一系列的 FTD 诊断。大约一半的调查对象报告说，在被诊断出 FTD 之前，他们看了三个或更多的医生，并且注意到了一系列的先前诊断。最常被认可的症状往往与特定诊断的临床特征一致，但在报告的症状在诊断类别内存在很大差异，以及在诊断类别之间存在相当大的症状重叠。FTD 的认知和语言症状通常对被诊断者最困扰，并且丧失独立性被认为会影响生活质量。被诊断者和护理伙伴对疾病影响的不同观点在 bvFTD/Pick 病中尤为明显。参与一系列在家中、在家外和与他人一起的活动，被认为对患有 FTD 的人具有挑战性，这突出表明即使在疾病的轻度和中度阶段，这些人的生活也受到了很大的影响。总的来说，通过倾听患有 FTD 的人的观点，我们可以开始设计更有意义的研究，提供更好的护理，并开发改善生活质量的疗法。
