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; Turner, Martin R.

doi:10.1080/21678421.2016.1267768

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

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 +6 more·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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Cognition is affected in a majority of ALS cases at some point in disease course.
“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]
The Discussion reports that more than 50% of ALS patients prospectively assessed had some form of frontotemporal disruption or dementia. That category includes behavioural dysfunction, so it does not establish that cognition specifically is affected in a majority, nor does the passage establish the claim’s ‘at some point in the disease course’ timing. (LLM, conf=0.86)
Experimental — accuracy not yet measured
TGF-ß is upregulated in ALS.
“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]
The Axis I discussion addresses ALS biomarkers such as neurofilaments, tau, and TDP-43, but does not report TGF-β upregulation. Searches for TGF-β and transforming growth factor beta in our copy found no passage addressing that claim; the copy is not marked as truncated. (LLM, conf=0.73)
Experimental — accuracy not yet measured

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Deficits in sentence expression in amyotrophic lateral sclerosis
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Ocular fixation instabilities in motor neurone disease
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Diagnostic criteria in amyotrophic lateral sclerosis
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Emotion processing deficits distinguish pure amyotrophic lateral sclerosis from frontotemporal dementia
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Mutational analysis of TBK1 in Taiwanese patients with amyotrophic lateral sclerosis
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Neural substrate of cognitive theory of mind impairment in amyotrophic lateral sclerosis
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Soluble Beta-Amyloid Precursor Protein Is Related to Disease Progression in Amyotrophic Lateral Sclerosis
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Executive dysfunction predicts social cognition impairment in amyotrophic lateral sclerosis
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Spectrum of Cognitive Impairment in Korean ALS Patients without Known Genetic Mutations
Oh, S., Park, A., Kim, H.-J., Oh, K., Choi, H., Kwon, M., Ki, C., Kim, H.-T., & Kim, S. H. (2014) · PLoS ONE · doi:10.1371/journal.pone.0087163 · Cited by 44 · see citing works
The Role ofAPOEin the Occurrence of Frontotemporal Dementia in Amyotrophic Lateral Sclerosis
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Language impairment in motor neurone disease
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The processing of actions and action-words in amyotrophic lateral sclerosis patients
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Deficits in concept formation in amyotrophic lateral sclerosis.
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Selective Attention and the Three-Process Memory Model for the Interpretation of Verbal Free Recall in Amyotrophic Lateral Sclerosis
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The UCSF screening exam effectively screens cognitive and behavioral impairment in patients with ALS
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Complex Cognitive Disruption in Motor Neuron Disease
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Impaired set-shifting in amyotrophic lateral sclerosis: An event-related potential study of executive function.
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Syntactic Comprehension in Patients with Amyotrophic Lateral Sclerosis
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New ALS‐Related Genes Expand the Spectrum Paradigm of Amyotrophic Lateral Sclerosis
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Semantic and nonfluent aphasic variants, secondarily associated with amyotrophic lateral sclerosis, are predominant frontotemporal lobar degeneration phenotypes in TBK1 carriers
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Profilin-1 mutations are rare in patients with amyotrophic lateral sclerosis and frontotemporal dementia
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Cognitive and Behavioral Symptoms in ALSFTD
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Syntactic comprehension deficits across the FTD-ALS continuum
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Frontal lobe-mediated behavioral changes in amyotrophic lateral sclerosis: Are they independent of physical disabilities?
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Amyotrophic lateral sclerosis patients show executive impairments on standard neuropsychological measures and an ecologically valid motor-free test of executive functions
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Longitudinal Changes in Discourse Prouction in Amyotrophic Lateral Sclerosis
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Transcultural validation of the ALS-CBS Cognitive Section for the Brazilian population
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Cognitive impairment in ALS patients and validation of the Spanish version of the ALS-CBS test
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Replication analysis of genetic variants on 17q11.2 and 9p21.2 with sporadic amyotrophic lateral sclerosis and Parkinson's disease in a Chinese population
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Amyotrophic lateral sclerosis and frontotemporal dementia
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Motor neuron disease associated with non‐fluent rapidly progressive aphasia: case report and review of the literature
da Rocha, A. J., Valério, B. C. O., Buainain, R. P., Ferraz, M. E., da Silva, C. J., Maia, A. C. M., de Andrade, V. P., & Lancellotti, C. L. P. (2007) · European Journal of Neurology · doi:10.1111/j.1468-1331.2007.01912.x · Cited by 12 · see citing works
Erratum to: Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD)
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Cortical Manifestations in Amyotrophic Lateral Sclerosis
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[87]Massman PJ, Sims J, Cooke N, Haverkamp LJ, Appel V, Appel SH. Prevalence and correlates of neuropsychological deficits in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 1996; 61: 450– 5. 8937336 PMC1074039

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Dysregulation of MicroRNAs and Target Genes Networks in Peripheral Blood of Patients With Sporadic Amyotrophic Lateral Sclerosis.
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European Academy of Neurology ( EAN) guideline on the management of amyotrophic lateral sclerosis in collaboration with European Reference Network for Neuromuscular Diseases ( ERN EURO‐NMD)
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Frontotemporal Dementia, Where Do We Stand? A Narrative Review
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Lipid Peroxidation and Antioxidant Supplementation in Neurodegenerative Diseases: A Review of Human Studies
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Copper, Iron, and Manganese Toxicity in Neuropsychiatric Conditions
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Identification of GGC repeat expansion in the NOTCH2NLC gene in amyotrophic lateral sclerosis
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New directions in clinical trials for frontotemporal lobar degeneration: methods and outcome measures
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Development and assessment of the inter-rater and intra-rater reproducibility of a self-administration version of the ALSFRS-R
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The Impact of Cognitive and Behavioral Symptoms on ALS Patients and Their Caregivers
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Cerebrospinal Fluid Biomarkers in Patients with Frontotemporal Dementia Spectrum: A Single-Center Study
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Prognostic clinical and biological markers for amyotrophic lateral sclerosis disease progression: validation and implications for clinical trial design and analysis
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An overview of screening instruments for cognition and behavior in patients with ALS: selecting the appropriate tool for clinical practice
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Phosphorylated TDP-43 aggregates in peripheral motor nerves of patients with amyotrophic lateral sclerosis
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Telemedicine for Delivery of Care in Frontotemporal Lobar Degeneration During COVID-19 Pandemic: Results from Southern Italy
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Progression of cognitive and behavioural impairment in early amyotrophic lateral sclerosis
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The Study of Language in the Amyotrophic Lateral Sclerosis - Frontotemporal Spectrum Disorder: a Systematic Review of Findings and New Perspectives
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The Role of VCP Mutations in the Spectrum of Amyotrophic Lateral Sclerosis—Frontotemporal Dementia
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Cerebrospinal Fluid TAR DNA-Binding Protein 43 Combined with Tau Proteins as a Candidate Biomarker for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Spectrum Disorders
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Resting state fMRI correlates of Theory of Mind impairment in amyotrophic lateral sclerosis
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Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis
Strong, M. J., Grace, G. M., Freedman, M., Lomen-Hoerth, C., Woolley, S. C., Goldstein, L. H., Murphy, J. M., Shoesmith, C. L., Rosenfeld, J., Leigh, P. N., et al. (2009) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960802654364 · Cited by 543 · see citing works
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Neuropsychological impairment in amyotrophic lateral sclerosis–frontotemporal spectrum disorder
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Cognitive and behavioural criteria for ALS-FTD
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Cognition in amyotrophic lateral sclerosis
Murphy, J. M., Ahmed, F., & Lomen‐Hoerth, C. (2012) · Oxford University Press eBooks · doi:10.1093/med/9780199590674.003.0010 · Cited by 3 · see citing works

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 › Neurology › Amyotrophic Lateral Sclerosis Research
Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
Health Sciences › Medicine › Physiology › 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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