Cited by 909Amyotrophic Lateral Sclerosis ResearchDementia and Cognitive Impairment ResearchAlzheimer's disease research and treatments

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

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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, Turner, Martin R.·2017·Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

DOI 10.1080/21678421.2016.1267768PMC PMC7409990

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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).

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Authors: 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.
Affiliations: Western University (CA)
Euan MacDonald Centre for Motor Neuron Disease Research (GB)
King's College London (GB)
California Pacific Medical Center (US)
Salford Royal NHS Foundation Trust (GB)
University of East Anglia (GB)
Northwestern University, Roxelyn and Richard Pepper Department of Communication Sciences and Disorders, Evanston, IL, USA,
University of Miami (US)
University of Debrecen (HU)
Loma Linda University Medical Center (US)
University of Milan (IT)
University of Sheffield (GB)
University of Oxford (GB)
Corresponding author: Strong, Michael J.
ORCID: Strong, Michael J.
Abrahams, Sharon
Goldstein, Laura H.
Woolley, Susan
Mclaughlin, Paula
Snowden, Julie
Mioshi, Eneida
Benatar, Michael
HortobáGyi, Tibor
Rosenfeld, Jeffrey
Silani, Vincenzo
Ince, Paul G
Turner, Martin R.
Venue: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Publisher: Informa UK Limited
Published: 2017-01-05
Volume: 18
Issue: 3-4
Pages: 153-174
ISSN: 2167-8421 (ISSN-L)
2167-9223
DOI: 10.1080/21678421.2016.1267768
PMC: PMC7409990
OpenAlex: W2562172900
Cited by: 909
References: 194
Citations by year: 2026: 90 · 2025: 117 · 2024: 102 · 2023: 119 · 2022: 108 · 2021: 118 · 2020: 101 · 2019: 61 · 2018: 68 · 2017: 25
FWCI: 48.58
Type: journal article
Language: en
Open access: green
ueaeprints.uea.ac.uk
Locations: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration (publishedVersion)
PubMed (publishedVersion)
White Rose Research Online (University of Leeds, The University of Sheffield, University of York) (acceptedVersion)
UEA Digital Repository (University of East Anglia) (acceptedVersion)
PubMed Central (acceptedVersion)
University of Debrecen Electronic Archive (University of Debrecen) (submittedVersion)
Research Portal (King's College London) (submittedVersion, other-oa)
Research Explorer (The University of Manchester) (submittedVersion)
Edinburgh Research Explorer (University of Edinburgh) (submittedVersion, other-oa)
Funders: South London and Maudsley NHS Foundation Trust
National Institute for Health and Care Research
Motor Neurone Disease Association (TURNER/JAN13/944-795)
King's College London
Medical Research Council (MR/K026992/1, MR/K01014X/1)
National Institute of Neurological Disorders and Stroke (U54NS092091)
Topics: Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Keywords: amyotrophic lateral sclerosis, frontotemporal dementia, diagnostic criteria, neuropsychological deficits, social cognition, language deficits, cognitive heterogeneity, survival, consensus criteria
MeSH terms: Amyotrophic Lateral Sclerosis, Canada, Diagnosis, Differential, Humans, Language, Neuropsychological Tests, Social Perception, Survival Analysis, Frontotemporal Dementia, Neuroimaging

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

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