Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2017; 18: 153–174 RESEARCH ARTICLE Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria MICHAEL J. STRONG 1 , SHARON ABRAHAMS 2 , LAURA H. GOLDSTEIN 3 , SUSAN WOOLLEY 4 , PAULA MCLAUGHLIN 5 , JULIE SNOWDEN 6 , ENEIDA MIOSHI 7 , ANGIE ROBERTS-SOUTH 8 , MICHAEL BENATAR 9 , TIBOR HORTOBA ´ GYI 10 , JEFFREY ROSENFELD 11 , VINCENZO SILANI 12 , PAUL G INCE 13 & MARTIN R. TURNER 14 1 Department of Clinical Neurological Sciences, Schulich School of Medicine & Dentistry, London, Ontario, Canada, 2 Department of Psychology, School of Philosophy, Psychology & Language Sciences, Euan MacDonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh, UK, 3 King’s College London, Department of Psychology, Institute of Psychiatry, Psychology and Neuroscience, London, UK, 4 Forbes Norris MDA/ALS Research Centre, California Pacific Medical Centre, San Francisco, CA, USA, 5 Western University, Schulich School of Medicine & Dentistry, London, ON, Canada, 6 Greater Manchester Neuroscience Centre, Salford Royal NHS Trust and University of Manchester, Manchester, UK, 7 Faculty of Medicine and Health Sciences, University of East Anglia, Norwich, UK, 8 Northwestern University, Roxelyn and Richard Pepper Department of Communication Sciences and Disorders, Evanston, IL, USA, 9 Department of Neurology, University of Miami Miller School of Medicine, Miami, FL, USA, 10 Department of Neuropathology, Institute of Pathology, University of Debrecen, Debrecen, Hungary, 11 Department of Neurology, Loma Linda University School of Medicine, Loma Linda, CA, USA, 12 Department of Neurology and Laboratory Neuroscience - IRCCS Istituto Auxologico Italiano, Department of Pathophysiology and Transplantation, ‘Dino Ferrari’ Centre, Universita ` degli Studi di Milano, Milan, Italy, 13 Sheffield Institute for Translational Neuroscience, Department of Neuroscience, The University of Sheffield, Sheffield, UK, and 14 Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK 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). KEYWORDS: Amyotrophic lateral sclerosis, frontotemporal dementia, neuropsychology, cognition, behaviour, genetics Introduction While the core feature of amyotrophic lateral scler- osis (ALS) is a relentless loss of motor function leading to paralysis and ultimately death, the awareness that it can be associated with one or more features of frontotemporal dysfunction has gained increasing acceptance (1). This in part can be traced to the development of international Correspondence: Michael J Strong, MD, Room C7-120, UH-LHSC, 339 Windermere Road, London, Ontario, N6A 5A5. Tel: 519-663-3874. Fax: 519- 663-3609. E-mail: [email protected] (Received 22 October 2016; revised 15 November 2016; accepted 18 November 2016) ISSN 2167-8421 print/ISSN 2167-9223 online ß 2016 World Federation of Neurology on behalf of the Research Group on Motor Neuron Diseases DOI: 10.1080/21678421.2016.1267768
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