title: Cognitive and Behavioral Symptoms in ALSFTD: Detection, Differentiation, and Progression.
abstract: Brief screening tools that detect and differentiate patients with amyotrophic lateral sclerosis and frontotemporal dementia (ALSFTD) from those more subtle cognitive or behavioral symptoms (ALS plus) and motor symptoms only (ALS pure) is pertinent in a clinical setting. The utility of 2 validated and data-driven tests (Mini-Addenbrooke's Cognitive Examination [M-ACE] and Motor Neuron Disease Behavioral Scale [MiND-B]) was investigated in 70 ALS patients (24 ALSFTD, 19 ALS plus, and 27 ALS pure). More than 90% of patients with ALSFTD scored at or below the cutoff on the M-ACE, whereas this was seen in only about 20% of ALS patients without dementia. The MiND-B differentiated between ALS pure and ALS plus diagnostic categories. Rasch modeling of M-ACE and MiND-B items revealed early cognitive (fluency, memory recall) and behavioral (apathy) symptoms in ALSFTD. The combined use of the M-ACE and MiND-B detects patients with ALSFTD, differentiates along the ALS continuum, and offers insight into the progression of nonmotor symptomatology in ALSFTD.
authors.affiliation: Brain and Mind Research Institute, Sydney, New South Wales, Australia Neuroscience Research Australia, Sydney, New South Wales, Australia ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, New South Wales, Australia sharpley.hsieh@sydney.edu.au.
authors.forename: Sharpley
authors.initials: S
authors.lastname: Hsieh
authors.affiliation: Brain and Mind Research Institute, Sydney, New South Wales, Australia.
authors.forename: Jashelle
authors.initials: J
authors.lastname: Caga
authors.affiliation: Neuroscience Research Australia, Sydney, New South Wales, Australia.
authors.forename: Felicity V C
authors.initials: FV
authors.lastname: Leslie
authors.affiliation: Neuroscience Research Australia, Sydney, New South Wales, Australia.
authors.forename: Marlene
authors.initials: M
authors.lastname: Shibata
authors.affiliation: Neuroscience Research Australia, Sydney, New South Wales, Australia.
authors.forename: Naomi
authors.initials: N
authors.lastname: Daveson
authors.affiliation: Neuroscience Research Australia, Sydney, New South Wales, Australia.
authors.forename: David
authors.initials: D
authors.lastname: Foxe
authors.affiliation: Brain and Mind Research Institute, Sydney, New South Wales, Australia.
authors.forename: Eleanor
authors.initials: E
authors.lastname: Ramsey
authors.affiliation: Departamento de Neurología Sur, Facultad de Medicina, Universidad de Chile, Santiago, Chile.
authors.forename: Patricia
authors.initials: P
authors.lastname: Lillo
authors.affiliation: Brain and Mind Research Institute, Sydney, New South Wales, Australia Neuroscience Research Australia, Sydney, New South Wales, Australia Prince of Wales Clinical School, University of New South Wales, Sydney, New South Wales, Australia.
authors.forename: Rebekah M
authors.initials: RM
authors.lastname: Ahmed
authors.affiliation: Brain and Mind Research Institute, Sydney, New South Wales, Australia Neuroscience Research Australia, Sydney, New South Wales, Australia Prince of Wales Clinical School, University of New South Wales, Sydney, New South Wales, Australia.
authors.forename: Emma
authors.initials: E
authors.lastname: Devenney
authors.affiliation: Neuroscience Research Australia, Sydney, New South Wales, Australia Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia.
authors.forename: James R
authors.initials: JR
authors.lastname: Burrell
authors.affiliation: Neuroscience Research Australia, Sydney, New South Wales, Australia ARC Centre of Excellence in Cognition and its Disorders, University of New South Wales, Sydney, New South Wales, Australia.
authors.forename: John R
authors.initials: JR
authors.lastname: Hodges
authors.affiliation: Brain and Mind Research Institute, Sydney, New South Wales, Australia.
authors.forename: Matthew C
authors.initials: MC
authors.lastname: Kiernan
authors.affiliation: Neuroscience Research Australia, Sydney, New South Wales, Australia Department of Psychiatry, Cambridge University, Cambridge, United Kingdom.
authors.forename: Eneida
authors.initials: E
authors.lastname: Mioshi
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000523:psychology; D001519:Behavior / Q000502:physiology; D001526:Behavioral Symptoms / Q000175:diagnosis* / Q000523:psychology; D003071:Cognition / Q000502:physiology; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology; D018450:Disease Progression; D005260:Female; D057180:Frontotemporal Dementia / Q000150:complications* / Q000523:psychology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D011594:Psychometrics / Q000295:instrumentation*; D011795:Surveys and Questionnaires / Q000592:standards*
keywords: amyotrophic lateral sclerosis; behavioral disturbance; cognitive screening; frontotemporal dementia