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).

Keywords:

• Amyotrophic lateral sclerosis
• frontotemporal dementia
• neuropsychology
• cognition
• behaviour
• genetics

Acknowledgements

The consensus conference at which these criteria were formulated was generously supported by the ALS Society of Canada, the Michael Halls Endowment, and the Windsor-Essex County ALS Society. This paper also represents independent work part-funded (LHG) by the National Institute for Health Research (NIHR) Dementia Biomedical Research Unit at South London and Maudsley NHS Foundation Trust and King’s College London. The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health. MB is supported by CReATe (U54 NS092091), which is funded through collaboration between NCATS and NINDS.

Listing of conference attendees

Michael Strong (conference organiser and chair), Sharon Abrahams, Thomas Bak, Emma Beeldman, Steve Bell, Michael Benatar, Mervin Blair, Robert Bowser, Emanuele Buratti, Danae Campos-Melos, Marvin Chum, Kristy Coleman, Chris Crockford, Karen Dunkerley, Marwa Elamin, Sali Farhan, Elizabeth Finger, Claire Flaherty, Tania Gendron, Laura Goldstein, Rosanne Govaarts, Francois Gros-Louis, Murray Grossman, Tibor Hortobagyi, Sarah Jesso, Kalyani Kansal, Yasumasa Kokubo, Suzee Lee, Julia MacKinley, Paula McLaughlin, Eneida Mioshi, Hiroshi Mitsumoto, Jennifer Murphy, Catherine Lomen-Hoerth, Lindsay Oliver, Chiadi Onyike, JB Orange, Markus Otto, Erik Pioro, Joost Raaphorst, Angela Roberts, Janice Robertson, Jonathon Rohrer, Jeffrey Rosenfeld, Vincenzo Silani, Julie Snowden, Carmela Tartaglia, Tamara Tavares, Christine Vande Velde, Kathryn Volkening, Susan Woolley.

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.

Supplementary material available online