title: Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria.
abstract: This article presents the revised consensus criteria for the diagnosis of frontotemporal dysfunction in amyotrophic lateral sclerosis (ALS) based on an international research workshop on frontotemporal dementia (FTD) and ALS held in London, Canada in June 2015. Since the publication of the Strong criteria, there have been considerable advances in the understanding of the neuropsychological profile of patients with ALS. Not only is the breadth and depth of neuropsychological findings broader than previously recognised - - including deficits in social cognition and language - but mixed deficits may also occur. Evidence now shows that the neuropsychological deficits in ALS are extremely heterogeneous, affecting over 50% of persons with ALS. When present, these deficits significantly and adversely impact patient survival. It is the recognition of this clinical heterogeneity in association with neuroimaging, genetic and neuropathological advances that has led to the current re-conceptualisation that neuropsychological deficits in ALS fall along a spectrum. These revised consensus criteria expand upon those of 2009 and embrace the concept of the frontotemporal spectrum disorder of ALS (ALS-FTSD).
authors.affiliation: a Department of Clinical Neurological Sciences, Schulich School of Medicine & Dentistry , London , Ontario , Canada.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Strong
authors.affiliation: b Department of Psychology, School of Philosophy, Psychology & Language Sciences , Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh , Edinburgh , UK.
authors.forename: Sharon
authors.initials: S
authors.lastname: Abrahams
authors.affiliation: c King's College London, Department of Psychology, Institute of Psychiatry, Psychology and Neuroscience , London , UK.
authors.forename: Laura H
authors.initials: LH
authors.lastname: Goldstein
authors.affiliation: d Forbes Norris MDA/ALS Research Centre, California Pacific Medical Centre , San Francisco , CA , USA.
authors.forename: Susan
authors.initials: S
authors.lastname: Woolley
authors.affiliation: e Western University , Schulich School of Medicine & Dentistry , London , ON , Canada.
authors.forename: Paula
authors.initials: P
authors.lastname: Mclaughlin
authors.affiliation: f Greater Manchester Neuroscience Centre , Salford Royal NHS Trust and University of Manchester , Manchester , UK.
authors.forename: Julie
authors.initials: J
authors.lastname: Snowden
authors.affiliation: g Faculty of Medicine and Health Sciences , University of East Anglia , Norwich , UK.
authors.forename: Eneida
authors.initials: E
authors.lastname: Mioshi
authors.affiliation: h Northwestern University , Roxelyn and Richard Pepper Department of Communication Sciences and Disorders , Evanston , IL , USA.
authors.forename: Angie
authors.initials: A
authors.lastname: Roberts-South
authors.affiliation: i Department of Neurology , University of Miami Miller School of Medicine , Miami , FL , USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Benatar
authors.affiliation: j Department of Neuropathology , Institute of Pathology, University of Debrecen , Debrecen , Hungary.
authors.forename: Tibor
authors.initials: T
authors.lastname: HortobáGyi
authors.affiliation: k Department of Neurology , Loma Linda University School of Medicine , Loma Linda , CA , USA.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Rosenfeld
authors.affiliation: l Department of Neurology and Laboratory Neuroscience - IRCCS Istituto Auxologico Italiano, Department of Pathophysiology and Transplantation , 'Dino Ferrari' Centre, Università degli Studi di Milano , Milan , Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Silani
authors.affiliation: m Sheffield Institute for Translational Neuroscience, Department of Neuroscience , The University of Sheffield , Sheffield , UK , and.
authors.forename: Paul G
authors.initials: PG
authors.lastname: Ince
authors.affiliation: n Nuffield Department of Clinical Neurosciences , University of Oxford , Oxford , UK.
authors.forename: Martin R
authors.initials: MR
authors.lastname: Turner
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000523:psychology*; D002170:Canada; D003937:Diagnosis, Differential; D057180:Frontotemporal Dementia / Q000175:diagnosis* / Q000523:psychology*; D006801:Humans; D007802:Language; D059906:Neuroimaging; D009483:Neuropsychological Tests; D012938:Social Perception; D016019:Survival Analysis
keywords: Amyotrophic lateral sclerosis; behaviour; cognition; frontotemporal dementia; genetics; neuropsychology