• Yasuhiro Watanabe, Emma Beeldman, Joost Raaphorst, Yuishin Izumi, Hiide Yoshino, Michihito Masuda, Naoki Atsuta, Satoru Ito, Tadashi Adachi, Yoshiki Adachi, Osamu Yokota, Masaya Oda, Ritsuko Hanashima, Mieko Ogino, Hiroo Ichikawa, Kazuko Hasegawa, Hideki Kimura, Toshio Shimizu, Ikuko Aiba, Hayato Yabe, Makoto Kanba, Kimiyoshi Kusumi, Tetsuya Aoki, Yu Hiroe, Hirohisa Watanabe, Kazutoshi Nishiyama, Masahiro Nomoto, Gen Sobue, Kenji Nakashima, and ALS-FTD-Q-J Research Group.
• Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan. Electronic address: yawatana@med.tottori-u.ac.jp.

AbstractAmyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) share common clinical, genetic and neuropathological features. Some ALS patients have behavioral/personality changes, which could result in significant obstacles in the care provided by family members and caregivers. An easy screening tool would contribute greatly to the evaluation of these symptoms. We translated the ALS-FTD-Questionnaire, developed in the Netherlands, into Japanese (ALS-FTD-Q-J) and examined the clinimetric properties (internal consistency, construct and clinical validity). Patients with ALS and/or behavioral variant FTD (bvFTD) were evaluated alongside healthy controls in this multicenter study. All ALS patients, regardless of bvFTD status, were further evaluated by the frontal behavioral inventory (FBI) and for frontal/executive function, cognition, anxiety/depression, and motor functions. Data from 146 subjects were analyzed: ALS (92), ALS-bvFTD (6), bvFTD (16), and healthy controls (32). The internal consistency of the ALS-FTD-Q-J was good (Cronbach α=0.92). The ALS-FTD-Q-J showed construct validity as it exhibited a high correlation with the FBI (r=0.79). However, correlations were moderate with anxiety/depression and low with cognitive scales, in contrast to the original report, i.e. a moderate correlation with cognition and a low correlation with anxiety/depression. The ALS-FTD-Q-J discriminated ALS patients from (ALS-)bvFTD patients and controls. Thus, the ALS-FTD-Q-J is useful for evaluating Japanese ALS/FTD patients. Copyright © 2016 Elsevier B.V. All rights reserved.

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