前頭側頭葉変性症への対応と支援

Coping and Supporting Patients and Families of Frontotemporal Lobar Degeneration

前頭側頭葉変性症（FTLD）は，行動障害のため既存の介護サービスが利用しづらく，比較的若年者に多いため，経済的支援の必要性も高い。臨床サブタイプのうち，行動異常型前頭側頭型認知症と意味性認知症は2015年に指定難病となった。本論では，指定難病の利用を含めた支援，特徴的な症候への対応とケアについて述べる。最後に進行中のFTLDに関するコホート研究であるFTLD-Jの現状を紹介する。

Abstract

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.