title: The ALS-FTD-Q: a new screening tool for behavioral disturbances in ALS.
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.
  
  
  CONCLUSIONS
  The ALS-FTD-Q is a feasible and clinimetrically validated instrument for the screening of behavioral disturbances in ALS.
authors.affiliation: Department of Neurology, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands. j.raaphorst@amc.uva.nl
authors.forename: Joost
authors.initials: J
authors.lastname: Raaphorst
authors.forename: Emma
authors.initials: E
authors.lastname: Beeldman
authors.forename: Ben
authors.initials: B
authors.lastname: Schmand
authors.forename: Joris
authors.initials: J
authors.lastname: Berkhout
authors.forename: Wim H J P
authors.initials: WH
authors.lastname: Linssen
authors.forename: Leonard H
authors.initials: LH
authors.lastname: van den Berg
authors.forename: Yolande A
authors.initials: YA
authors.lastname: Pijnenburg
authors.forename: Hepke F
authors.initials: HF
authors.lastname: Grupstra
authors.forename: Janneke G
authors.initials: JG
authors.lastname: Weikamp
authors.forename: H Jurgen
authors.initials: HJ
authors.lastname: Schelhaas
authors.forename: Janne M
authors.initials: JM
authors.lastname: Papma
authors.forename: John C
authors.initials: JC
authors.lastname: van Swieten
authors.forename: Marianne
authors.initials: M
authors.lastname: de Visser
authors.forename: Rob J
authors.initials: RJ
authors.lastname: de Haan
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D005260:Female; D057180:Frontotemporal Dementia / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009135:Muscular Diseases / Q000175:diagnosis; D009483:Neuropsychological Tests; D011594:Psychometrics / Q000295:instrumentation*; D011795:Surveys and Questionnaires / Q000592:standards*