title: Screening for cognitive dysfunction in ALS: validation of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) using age and education adjusted normative data.
abstract: BACKGROUND
  Cognitive and behavioural changes are an important aspect in Amyotrophic Lateral Sclerosis (ALS). The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) briefly assesses these changes in ALS.
  
  
  OBJECTIVE
  To validate the ECAS against a standardised neuropsychological battery and assess its sensitivity and specificity using age and education adjusted cut-off scores.
  
  
  METHODS
  30 incident ALS cases were assessed on both, ECAS and neuropsychological battery. Age and education adjusted cut-off scores were created from a sample of 82 healthy controls.
  
  
  RESULTS
  ECAS composite scores (Total, ALS Specific and Non-Specific) were highly correlated with battery composite scores. High correlations were also observed between ECAS and full battery cognitive domains and subtests. The ECAS Total, ALS Specific and Non-Specific scores were highly sensitive to cognitive impairment. ECAS ALS-Specific cognitive domains also evidenced high sensitivity. Individual subtest sensitivity was medium to low, suggesting that caution should be used when interpreting these scores. Low positive predictive values indicated the presence of false positives.
  
  
  CONCLUSIONS
  Psychometric properties of the ECAS using age and education adjusted norms indicate that the ECAS, when used as an overall measure of cognitive decline, is highly sensitive. Further comprehensive assessment is required for patients that present as impaired on the ECAS.
authors.affiliation: a Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin , Ireland.|b Department of Psychology , Beaumont Hospital , Dublin , Ireland , and.
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 , and.
authors.forename: Tom
authors.initials: T
authors.lastname: Burke
authors.affiliation: a Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin , Ireland.|b Department of Psychology , Beaumont Hospital , Dublin , Ireland , and.
authors.forename: Katie
authors.initials: K
authors.lastname: Lonergan
authors.affiliation: c University of Surrey , Guildford , United Kingdom.
authors.forename: Caroline
authors.initials: C
authors.lastname: McHugh
authors.affiliation: a Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin , Ireland.
authors.forename: Iain
authors.initials: I
authors.lastname: Mays
authors.affiliation: a Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin , Ireland.
authors.forename: Caoifa
authors.initials: C
authors.lastname: Madden
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: 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.
authors.forename: Orla
authors.initials: O
authors.lastname: Hardiman
authors.affiliation: a Academic Unit of Neurology , Trinity Biomedical Sciences Institute, Trinity College Dublin , Ireland.|b Department of Psychology , Beaumont Hospital , Dublin , Ireland , and.
authors.forename: Niall
authors.initials: N
authors.lastname: Pender
mesh_terms: D000367:Age Factors; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications*; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology*; D004185:Disability Evaluation; D004522:Educational Status; D005260:Female; D006801:Humans; D008297:Male; D008403:Mass Screening; D001523:Mental Disorders / Q000175:diagnosis* / Q000209:etiology*; D008875:Middle Aged; D009483:Neuropsychological Tests; D011237:Predictive Value of Tests; D012016:Reference Values; D012680:Sensitivity and Specificity
keywords: ALS; Cognition; ECAS; screen; standardisation; validation