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Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria
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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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“Cognition is also affected in a majority of cases at some point in the disease course (10).”
From Chronic Traumatic Encephalopathy Within an Amyotrophic Lateral Sclerosis Brain Bank Cohort (2018) · cites this paper as [10]
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“Notably, TGF-ß is upregulated in ALS (55), and aberrant Wnt signaling is associated with TDP43 mislocalization in cellular and animal models (56).”
From Nemo-like kinase disrupts nuclear import and drives TDP43 mislocalization in ALS (2025) · cites this paper as [55]
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NEUROPATHOLOGY OF NEURODEGENERATIVE DISEASES: A PRACTICAL GUIDE
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Metadata
- 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
- 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
- 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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