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Abstract
Objective: The assessment of behavioral disturbances in amyotrophic lateral sclerosis (ALS) is important because of the overlap with the behavioral variant of frontotemporal dementia (ALS-bvFTD). Motor symptoms and dysarthria are not taken into account in currently used behavioral questionnaires. We examined the clinimetric properties of a new behavioral questionnaire for patients with ALS (Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire [ALS-FTD-Q]).
Methods: In addition to other clinimetric properties, we examined reliability, clinical validity, and construct validity of the ALS-FTD-Q, using data from patients with ALS (n = 103), ALS-bvFTD (n = 10), bvFTD (n = 25), muscle disease control subjects (n = 39), and control subjects (n = 31). Construct validity of the ALS-FTD-Q was assessed using the Frontal Systems Behavior scale (FrSBe), Frontal Behavioral Inventory (FBI), Hospital Anxiety and Depression Scale, ALS Functional Rating Scale−Revised, Frontal Assessment Battery, Mini-Mental State Examination, and a fluency index. In addition, the point prevalence of behavioral disturbances according to the ALS-FTD-Q was compared with those obtained with the FrSBe and FBI.
Results: The internal consistency of the ALS-FTD-Q was good (Cronbach α = 0.92). The ALS-FTD-Q showed construct validity because it correlated highly with other behavioral measures (r = 0.80 and 0.79), moderately with measures of frontal functions and global cognitive functioning (r = 0.37; r = 0.32), and poorly with anxiety/depression and motor impairment (r = 0.18 for both). The ALS-FTD-Q discriminated between patients with ALS-bvFTD, patients with ALS, and control subjects. The point prevalence of behavioral disturbances in patients with ALS measured with the ALS-FTD-Q was lower than that for the FrSBe and FBI.
Conclusion: The ALS-FTD-Q is a feasible and clinimetrically validated instrument for the screening of behavioral disturbances in ALS.
GLOSSARY
- ALS=
- amyotrophic lateral sclerosis;
- ALSFRS-R =
- ALS Functional Rating Scale−Revised;
- ALS-FTD-Q =
- Amyotrophic Lateral Sclerosis-Frontotemporal Dementia−Questionnaire;
- bvFTD =
- behavioral variant of frontotemporal dementia;
- FAB =
- Frontal Assessment Battery;
- FBI =
- Frontal Behavioral Inventory;
- FrSBe =
- Frontal Systems Behavior scale;
- HADS =
- Hospital Anxiety and Depression Scale;
- MMSE =
- Mini-Mental State Examination
Footnotes
Supplemental data at www.neurology.org
- Received November 20, 2011.
- Accepted May 8, 2012.
- Copyright © 2012 by AAN Enterprises, Inc.
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