Mori Kohji, Sato Shunsuke, Miyawaki Eiko, Ikeda Manabu
Department of Psychiatry, Osaka University Graduate School of Medicine.
Brain Nerve. 2020 Jun;72(6):623-632. doi: 10.11477/mf.1416201572.
Frontotemporal lobar degeneration (FTLD) is a comprehensive term encompassing a group of clinically overlapping but heterogeneous conditions with selective frontal and temporal lobar neurodegeneration. Among the three clinical subtypes of FTLD, behavioral variant frontotemporal dementia (bvFTD) and semantic dementia (SD) were specified as "designated intractable diseases" by the Japanese Ministry of Health, Labor and Welfare in 2015. Under this designation system, relatively young patient with bvFTD or SD of certain severities can receive partial financial support from the Japanese local government. Here we introduce financial and social support for FTLD, including utilization of the designated intractable disease system and other support available in Japan. In addition, we describe the methods for coping and care of prominent symptoms in FTLD. Lastly, we address the current status of a nationwide Japanese FTLD study cohort named FTLD-J.
额颞叶变性(FTLD)是一个综合性术语,涵盖了一组临床症状重叠但异质性的疾病,其具有选择性的额叶和颞叶神经变性。在FTLD的三种临床亚型中,行为变异型额颞叶痴呆(bvFTD)和语义性痴呆(SD)在2015年被日本厚生劳动省指定为“特定难治性疾病”。在这一指定体系下,患有特定严重程度的bvFTD或SD的相对年轻患者可获得日本地方政府的部分财政支持。在此,我们介绍针对FTLD的财政和社会支持,包括特定难治性疾病体系的利用及日本现有的其他支持。此外,我们描述了应对FTLD突出症状的方法及护理措施。最后,我们阐述了一个名为FTLD-J的日本全国性FTLD研究队列的现状。