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

Michael J. Strong, Sharon Abrahams, Laura H. Goldstein, Susan Woolley, Paula Mclaughlin, Julie Snowden, Eneida Mioshi, Angie Roberts-South, Michael Benatar, Tibor HortobáGyi, Jeffrey Rosenfeld, Vincenzo Silani, Paul G. Ince, Martin R. Turner

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139 Citations (Scopus)

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).

Original languageEnglish
Pages (from-to)153-174
Number of pages22
JournalAmyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Volume18
Issue number3-4
DOIs
Publication statusPublished - Apr 3 2017

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Keywords

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

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Strong, M. J., Abrahams, S., Goldstein, L. H., Woolley, S., Mclaughlin, P., Snowden, J., Mioshi, E., Roberts-South, A., Benatar, M., HortobáGyi, T., Rosenfeld, J., Silani, V., Ince, P. G., & Turner, M. R. (2017). Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 18(3-4), 153-174. https://doi.org/10.1080/21678421.2016.1267768