Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria

Strong, Michael J., Abrahams, Sharon, Goldstein, Laura H., Woolley, Susan, Mclaughlin, Paula, Snowden, Julie, Mioshi, Eneida, Roberts-South, Angie, Benatar, Michael, HortobáGyi, Tibor, Rosenfeld, Jeffrey, Silani, Vincenzo, Ince, Paul G and Turner, Martin R. (2017) Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 18 (3-4). pp. 153-174. ISSN 2167-8421

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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).

Item Type: Article
Uncontrolled Keywords: amyotrophic lateral sclerosis,frontotemporal dementia,neuropsychology,cognition,behaviour,genetics
Faculty \ School: Faculty of Medicine and Health Sciences > School of Health Sciences
Related URLs:
Depositing User: Pure Connector
Date Deposited: 04 Feb 2017 03:27
Last Modified: 22 Jul 2020 01:22
URI: https://ueaeprints.uea.ac.uk/id/eprint/62302
DOI: 10.1080/21678421.2016.1267768

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