Highlights
- •We established a screening tool for behavioral symptoms for Japanese ALS/FTD cases.
- •The tool was efficacious for Japanese patients same as the original English version.
- •Combining other measures, the tool revealed some characteristics of Japanese ALS/FTD.
Abstract
Amyotrophic 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.
Keywords
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Article info
Publication history
Published online: May 18, 2016
Accepted:
May 17,
2016
Received in revised form:
April 18,
2016
Received:
January 19,
2016
Identification
Copyright
© 2016 Elsevier B.V. All rights reserved.
