title: Identifying behavioural changes in ALS: Validation of the Beaumont Behavioural Inventory (BBI).
abstract: OBJECTIVE
  Behavioural changes are an important part of amyotrophic lateral sclerosis (ALS). However, most tools do not account for the influence of motor impairment. Furthermore, they do not fully measure the broad range of behavioural changes specific to ALS. This study aimed to develop and validate an ALS specific behavioural inventory, the Beaumont Behavioural Inventory (BBI).
  
  
  METHODS
  The BBI was validated in a cohort of ALS patients (n = 85) and 78 age-, gender-, and education-matched controls. The scale was validated against the Frontal Systems Behaviour Scale (FrSBe) and The Frontal Assessment Battery (FAB) for convergent validity, and against other non-behavioural measures to assess discriminant validity. Reliability was assessed with Cronbach's alpha.
  
  
  RESULTS
  The instrument showed high internal consistency (Cronbach's alpha value =0.891). BBI scores highly correlated with the FrSBe and moderately with the FAB. However, the measure was independent from non-behavioural measures. Using a cut-off score of 7 for mild behavioural changes, the BBI displayed high sensitivity and specificity (87.9% and 78.85%, respectively). The cut-off score for moderate changes, consistent with a diagnosis of ALS-FTD, is set at 22.5, showing 90% sensitivity and 96% specificity.
  
  
  CONCLUSIONS
  The BBI is a sensitive and specific tool to assess the entire behavioural spectrum of ALS.
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.
authors.forename: Marwa
authors.initials: M
authors.lastname: Elamin
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.|b Department of Psychology , Beaumont Hospital , Dublin , Ireland.
authors.forename: Marta
authors.initials: M
authors.lastname: Pinto-Grau
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.|b Department of Psychology , Beaumont Hospital , Dublin , Ireland.
authors.forename: Tom
authors.initials: T
authors.lastname: Burke
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.
authors.forename: Peter
authors.initials: P
authors.lastname: Bede
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.
authors.forename: James
authors.initials: J
authors.lastname: Rooney
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.
authors.forename: Meabhdh
authors.initials: M
authors.lastname: O'Sullivan
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.|b Department of Psychology , Beaumont Hospital , Dublin , Ireland.
authors.forename: Katie
authors.initials: K
authors.lastname: Lonergan
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.
authors.forename: Emma
authors.initials: E
authors.lastname: Kirby
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.
authors.forename: Emma
authors.initials: E
authors.lastname: Quinlan
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.
authors.forename: Nadia
authors.initials: N
authors.lastname: Breen
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.
authors.forename: Alice
authors.initials: A
authors.lastname: Vajda
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.
authors.forename: Mark
authors.initials: M
authors.lastname: Heverin
authors.affiliation: b Department of Psychology , Beaumont Hospital , Dublin , Ireland.
authors.forename: Niall
authors.initials: N
authors.lastname: Pender
authors.affiliation: a Academic Unit of Neurology, Trinity Biomedical Sciences Institute , Trinity College Dublin , Ireland.
authors.forename: Orla
authors.initials: O
authors.lastname: Hardiman
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications*; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D008297:Male; D001523:Mental Disorders / Q000175:diagnosis* / Q000209:etiology*; D008875:Middle Aged; D009483:Neuropsychological Tests; D011594:Psychometrics / Q000379:methods*; D012372:ROC Curve; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires
keywords: ALS; BBI; behaviour; neuropsychology; validation