Memory and Aging Center, Department of Neurology, University of California, San Francisco, USA.
Adv Exp Med Biol. 2021;1281:297-310. doi: 10.1007/978-3-030-51140-1_18.
While behavioral variant frontotemporal dementia (bvFTD) and primary progressive aphasia (PPA) remain unrelenting and universally fatal conditions, there is a framework for supportive treatment in patients diagnosed with these frontotemporal dementia (FTD) syndromes and the larger spectrum of clinical syndromes associated with frontotemporal lobar degeneration (FTLD) pathology on autopsy. A managing physician has an important role in weighing therapeutic options, organizing caregiver support, and framing long-term expectations for patients and caregivers. Additionally, a dedicated neurologist may assist patients and caregivers in navigating a growing range of FTD research, including exciting opportunities in clinical therapeutic trials. This chapter will review current therapeutic options for patients with bvFTD and PPA and detail the landscape of potential new disease-modifying therapies targeting the pathophysiology or FTLD.
虽然行为变异型额颞叶痴呆（bvFTD）和原发性进行性失语症（PPA）仍然是无情且普遍致命的疾病，但对于被诊断患有这些额颞叶痴呆（FTD）综合征以及在尸检中与额颞叶变性（FTLD）病理相关的更大范围临床综合征的患者，存在一种支持性治疗框架。主治医生在权衡治疗选择、组织护理人员支持以及为患者和护理人员规划长期预期方面发挥着重要作用。此外，专门的神经科医生可以帮助患者和护理人员了解越来越多的 FTD 研究，包括临床治疗试验中的令人兴奋的机会。本章将回顾目前 bvFTD 和 PPA 患者的治疗选择，并详细介绍针对病理生理学或 FTLD 的潜在新型疾病修饰治疗方法的前景。
