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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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Cites
Rosen, D. R., Siddique, T., Patterson, D., Figlewicz, D. A., Sapp, P., Hentati, A., Donaldson, D., Goto, J., O'Regan, J. P., Deng, H.-X., et al. (1993) · Nature · doi:10.1038/362059a0 · Cited by 7,062
El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis
Brooks, B. R., Miller, R. G., Swash, M., & Munsat, T. L. (2000) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/146608200300079536 · Cited by 5,433
Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia
Rascovsky, K., Hodges, J. R., Knopman, D., Mendez, M. F., Kramer, J. H., Neuhaus, J., van Swieten, J. C., Seelaar, H., Dopper, E. G. P., Onyike, C. U., et al. (2011) · Brain · doi:10.1093/brain/awr179 · Cited by 5,221
Classification of primary progressive aphasia and its variants
Gorno-Tempini, M. L., Hillis, A. E., Weintraub, S., Kertesz, A., Mendez, M., Cappa, S. F., Ogar, J. M., Rohrer, J. D., Black, S., Boeve, B. F., et al. (2011) · Neurology · doi:10.1212/wnl.0b013e31821103e6 · Cited by 5,141
Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria
Neary, D., Snowden, J. S., Gustafson, L., Passant, U., Stuss, D., Black, S., Freedman, M., Kertesz, A., Robert, P. H., Albert, M., et al. (1998) · Neurology · doi:10.1212/wnl.51.6.1546 · Cited by 5,047
Self-citationDeJesus-Hernandez, M., Mackenzie, I. R., Boeve, B. F., Boxer, A. L., Baker, M., Rutherford, N. J., Nicholson, A. M., Finch, N. A., Flynn, H., Adamson, J., et al. (2011) · Neuron · doi:10.1016/j.neuron.2011.09.011 · Cited by 4,936
A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD
Renton, A. E., Majounie, E., Waite, A., Simón-Sánchez, J., Rollinson, S., Gibbs, J. R., Schymick, J. C., Laaksovirta, H., van Swieten, J. C., Myllykangas, L., et al. (2011) · Neuron · doi:10.1016/j.neuron.2011.09.010 · Cited by 4,409
Self-citationWeıntraub, S. (1990) · Archives of Neurology · doi:10.1001/archneur.1990.00530120075013 · Cited by 3,018
Appelhof, B., Bakker, C., den IJssel, J. C. L. V. D., Zwijsen, S. A., Smalbrugge, M., Verhey, F. R. J., de Vugt, M. E., Zuidema, S. U., & Koopmans, R. T. C. M. (2017) · Dementia and Geriatric Cognitive Disorders · doi:10.1159/000477087 · Cited by 2,774
TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
Sreedharan, J., Blair, I. P., Tripathi, V. B., Hu, X., Vance, C., Rogelj, B., Ackerley, S., Durnall, J. C., Williams, K. L., Buratti, E., et al. (2008) · Science · doi:10.1126/science.1154584 · Cited by 2,630
Research works printed but not linked: 3
- 10.1136/jnnp.61.5.450 · DOI
- Ince PG, 2015, Greenfield's Neuropathology, 817
- Le B,I, 2015, Neurobiol Aging, 36, 3116
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Baiardi, S., Quadalti, C., Mammana, A., Dellavalle, S., Zenesini, C., Sambati, L., Pantieri, R., Polischi, B., Romano, L., Suffritti, M., et al. (2022) · Alzheimer's Research & Therapy · doi:10.1186/s13195-022-01093-6 · Cited by 193
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Portelius, E., Olsson, B., Höglund, K., Cullen, N. C., Kvartsberg, H., Andréasson, U., Zetterberg, H., Sandelius, Å., Shaw, L. M. J., Lee, V. M. ‐., et al. (2018) · Acta Neuropathologica · doi:10.1007/s00401-018-1851-x · Cited by 171
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Abu‐Rumeileh, S., Steinacker, P., Polischi, B., Mammana, A., Bartoletti‐Stella, A., Oeckl, P., Baiardi, S., Zenesini, C., Huss, A. M., Cortelli, P., et al. (2019) · Alzheimer s Research & Therapy · doi:10.1186/s13195-019-0562-4 · Cited by 157
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Deshaies, J.-E., Shkreta, L., Moszczynski, A. J., Sidibé, H., Semmler, S., Fouillen, A., Bennett, E. R., Bekenstein, U., Destroismaisons, L., Toutant, J., et al. (2018) · Brain · doi:10.1093/brain/awy062 · Cited by 138
Self-citationLiguori, M., Nuzziello, N., Introna, A., Consiglio, A., Licciulli, F., D’Errico, E., Scarafino, A., Distaso, E., & Simone, I. L. (2018) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2018.00288 · Cited by 131
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Broad, R. J., Gabel, M. C., Dowell, N. G., Schwartzman, D. J., Seth, A. K., Zhang, H., Alexander, D. C., Cercignani, M., & Leigh, P. N. (2019) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2018-318830 · Cited by 105
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Riva, N., Domi, T., Pozzi, L., Lunetta, C., Schito, P., Spinelli, E. G., Cabras, S., Matteoni, E., Consonni, M., Bella, E. D., et al. (2024) · Journal of Neurology · doi:10.1007/s00415-024-12435-9 · Cited by 74
Cognitive and behavioural changes in PLS and PMA:challenging the concept of restricted phenotypes
de Vries, B. S., Rustemeijer, L. M. M., Bakker, L. A., Schröder, C. D., Veldink, J. H., van den Berg, L. H., Nijboer, T. C. W., & van Es, M. A. (2018) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2018-318788 · Cited by 67
The impact of cognitive and behavioral impairment in amyotrophic lateral sclerosis
Huynh, W., Ahmed, R. M., Mahoney, C., Nguyen, C., Tu, S., Caga, J., Loh, P., Lin, C. S.-Y., & Kiernan, M. C. (2020) · Expert Review of Neurotherapeutics · doi:10.1080/14737175.2020.1727740 · Cited by 66
Cognitive and Behavioral Manifestations in ALS: Beyond Motor System Involvement
Rusina, R., Vandenberghe, R. R., & Bruffaerts, R. (2021) · Diagnostics · doi:10.3390/diagnostics11040624 · Cited by 66
Elevated Plasma Phosphorylated Tau 181 in Amyotrophic Lateral Sclerosis
Cousins, K. A. Q., Shaw, L. M. J., Shellikeri, S., Dratch, L., Rosario, L., Elman, L. B., Quinn, C. C., Amado, D. A., Wolk, D. A., Tropea, T. F., et al. (2022) · Annals of Neurology · doi:10.1002/ana.26462 · Cited by 66
Multidimensional apathy and executive dysfunction in amyotrophic lateral sclerosis
Radaković, R., Stephenson, L. J., Newton, J. L., Crockford, C., Swingler, R. J., Chandran, S., & Abrahams, S. (2017) · Cortex · doi:10.1016/j.cortex.2017.06.023 · Cited by 66
Self-citationThe Impact of Cognitive and Behavioral Symptoms on ALS Patients and Their Caregivers
Caga, J., Hsieh, S., Lillo, P., Dudley, K., & Mioshi, E. (2019) · Frontiers in Neurology · doi:10.3389/fneur.2019.00192 · Cited by 67
Self-citationAbu‐Rumeileh, S., Mometto, N., Bartoletti‐Stella, A., Polischi, B., Oppi, F., Poda, R., Maserati, M. S., Cortelli, P., Liguori, R., Capellari, S., et al. (2018) · Journal of Alzheimer s Disease · doi:10.3233/jad-180409 · Cited by 69
Current State and Future Directions in the Diagnosis of Amyotrophic Lateral Sclerosis
Vidovic, M., Müschen, L. H., Brakemeier, S., Machetanz, G., Naumann, M., & Castro‐Gomez, S. (2023) · Cells · doi:10.3390/cells12050736 · Cited by 73
The path to biomarker-based diagnostic criteria for the spectrum of neurodegenerative diseases
Baldacci, F., Mazzucchi, S., Vecchia, A. D., Giampietri, L., Giannini, N., Koronyo‐Hamaoui, M., Ceravolo, R., Siciliano, G., Bonuccelli, U., Elahi, F. M., et al. (2020) · Expert Review of Molecular Diagnostics · doi:10.1080/14737159.2020.1731306 · Cited by 65
Resting-state EEG reveals four subphenotypes of amyotrophic lateral sclerosis
Dukic, S., McMackin, R., Costello, E., Metzger, M., Buxó, T., Fasano, A., Chipika, R. H., Pinto‐Grau, M., Schuster, C., Hammond, M., et al. (2021) · Brain · doi:10.1093/brain/awab322 · Cited by 63
Beeldman, E., Raaphorst, J., Twennaar, M. K., Govaarts, R., Pijnenburg, Y. A. L., de Haan, R. J., de Visser, M., & Schmand, B. A. (2018) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2017-317459 · Cited by 63
Gold Coast diagnostic criteria: Implications for ALS diagnosis and clinical trial enrollment
Vucic, S., Ferguson, T. A., Cummings, C., Hotchkin, M. T., Genge, A. L., Glanzman, R., Roet, K. C. D., Cudkowicz, M., & Kiernan, M. C. (2021) · Muscle & Nerve · doi:10.1002/mus.27392 · Cited by 66
Mild motor impairment as prodromal state in amyotrophic lateral sclerosis: a new diagnostic entity
Benatar, M. G., Granit, V., Andersen, P. M., Grignon, A., McHutchison, C. A., Cosentino, S., Malaspina, A., & Wuu, J. (2022) · Brain · doi:10.1093/brain/awac185 · Cited by 62
Self-citationBenatar, M., Macklin, E. A., Malaspina, A., Rogers, M.-L., Hornstein, E., Lombardi, V., Renfrey, D., Shepheard, S., Magen, I., Cohen, Y., et al. (2024) · eBioMedicine · doi:10.1016/j.ebiom.2024.105323 · Cited by 61
Self-citationGosselt, I. K., Nijboer, T. C. W., & van Es, M. A. (2020) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2020.1732424 · Cited by 64
Dubbioso, R., Provitera, V., Pacella, D., Santoro, L., Manganelli, F., & Nolano, M. (2023) · Journal of Neurology · doi:10.1007/s00415-023-11832-w · Cited by 60
Ahmed, R. M., Highton‐Williamson, E., Caga, J., Thornton, N. L. R., Ramsey, E., Zoing, M. C., Kim, W. S., Halliday, G. M., Piguet, O., Hodges, J. R., et al. (2017) · Journal of Alzheimer s Disease · doi:10.3233/jad-170660 · Cited by 60
Cognitive phenotypes of sequential staging in amyotrophic lateral sclerosis
Lulé, D. E., Böhm, S., Müller, H., Aho-Özhan, H. E. A., Keller, J., Gorges, M., Loose, M., Weishaupt, J. H., Uttner, I., Pinkhardt, E. H., et al. (2018) · Cortex · doi:10.1016/j.cortex.2018.01.004 · Cited by 60
Self-citationRiva, N., Gentile, F., Cerri, F., Gallia, F., Podini, P., Dina, G., Falzone, Y., Fazio, R., Lunetta, C., Calvo, A., et al. (2021) · Brain · doi:10.1093/brain/awab285 · Cited by 59
Multidimensional apathy: evidence from neurodegenerative disease
Radakovic, R., & Abrahams, S. (2018) · Current Opinion in Behavioral Sciences · doi:10.1016/j.cobeha.2017.12.022 · Cited by 57
Self-citationCapozzo, R., Zoccolella, S., Frisullo, M. E., Barone, R., Dell’Abate, M. T., Barulli, M. R., Musio, M., Accogli, M., & Logroscino, G. (2020) · Journal of Alzheimer s Disease · doi:10.3233/jad-200589 · Cited by 57
Diagnostics of Amyotrophic Lateral Sclerosis: Up to Date
Štětkářová, I., & Ehler, E. (2021) · Diagnostics · doi:10.3390/diagnostics11020231 · Cited by 59
Benatar, M. G., Wuu, J., Huey, E. D., McMillan, C. T., Petersen, R. C., Postuma, R. B., McHutchison, C. A., Dratch, L., Arias, J. J., Crawley, A., et al. (2024) · Nature Reviews Neurology · doi:10.1038/s41582-024-00961-z · Cited by 57
Self-citationProgression of cognitive and behavioural impairment in early amyotrophic lateral sclerosis
Beeldman, E., Govaarts, R., de Visser, M., Twennaar, M. K., van der Kooi, A. J., van den Berg, L. H., Veldink, J. H., Pijnenburg, Y. A. L., de Haan, R. J., Schmand, B. A., et al. (2020) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2020-322992 · Cited by 57
Capozzo, R., Zoccolella, S., Musio, M., Barone, R., Accogli, M., & Logroscino, G. (2020) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2020.1773502 · Cited by 57
Falzone, Y., Domi, T., Mandelli, A., Pozzi, L., Schito, P., Russo, T., Barbieri, A., Fazio, R., Volonté, M. A., Magnani, G., et al. (2022) · European Journal of Neurology · doi:10.1111/ene.15321 · Cited by 58
Pinto‐Grau, M., Hardiman, O. M., & Pender, N. P. (2018) · Neuropsychology Review · doi:10.1007/s11065-018-9375-7 · Cited by 56
The Role of VCP Mutations in the Spectrum of Amyotrophic Lateral Sclerosis—Frontotemporal Dementia
Scarian, E., Fiamingo, G., Diamanti, L., Palmieri, I., Gagliardi, S., & Pansarasa, O. (2022) · Frontiers in Neurology · doi:10.3389/fneur.2022.841394 · Cited by 56
Bourbouli, M., Rentzos, M., Bougea, A. M., Zouvelou, V., Constantinides, V. C., Zaganas, I. V., Evdokimidis, I., Kapaki, E. N., & Paraskevas, G. P. (2017) · Dementia and Geriatric Cognitive Disorders · doi:10.1159/000478979 · Cited by 55
Longitudinal diffusion imaging across the C9orf72 clinical spectrum
Floeter, M. K., Danielian, L. E., Braun, L. E., & Wu, T. (2018) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2017-316799 · Cited by 51
Resting state fMRI correlates of Theory of Mind impairment in amyotrophic lateral sclerosis
Trojsi, F., Nardo, F. D., Santangelo, G., Siciliano, M., Femiano, C., Passaniti, C., Caiazzo, G., Fratello, M., Cirillo, M., Monsurrò, M. R., et al. (2017) · Cortex · doi:10.1016/j.cortex.2017.09.016 · Cited by 54
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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