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

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

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

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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

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Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

DeJesus-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

Primary Progressive Aphasia

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The Determinants of Quality of Life of Nursing Home Residents with Young-Onset Dementia and the Differences between Dementia Subtypes

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

Motor symptoms in genetic frontotemporal dementia: developing a new module for clinical rating scales

Samra, K., MacDougall, A. M., Peakman, G., Bouzigues, A., Bocchetta, M., Cash, D. M., Greaves, C. V., Convery, R. S., Swieten, J. C. V., Jiskoot, L. C., et al. (2022) · Journal of Neurology · doi:10.1007/s00415-022-11442-y · Cited by 23

microRNA‐based predictor for diagnosis of frontotemporal dementia

Magen, I., Yacovzada, N., Warren, J. D., Heller, C., Swift, I., Bobeva, Y., Malaspina, A., Rohrer, J. D., Fratta, P., & Hornstein, E. (2023) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12916 · Cited by 12

Temporal course of cognitive and behavioural changes in motor neuron diseases

McHutchison, C. A., Wuu, J., McMillan, C. T., Rademakers, R., Statland, J. M., Wu, G., Rampersaud, E., Myers, J., Hernandez, J., Abrahams, S., et al. (2023) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2023-331697 · Cited by 18

OPTN p.Met468Arg and ATXN2 intermediate length polyQ extension in families with C9orf72 mediated amyotrophic lateral sclerosis and frontotemporal dementia

Farhan, S. M. K., Gendron, T. F., Petrucelli, L., Hegele, R. A., & Strong, M. J. (2017) · American Journal of Medical Genetics Part B Neuropsychiatric Genetics · doi:10.1002/ajmg.b.32606 · Cited by 18

Phosphorylation of Threonine 175 Tau in the Induction of Tau Pathology in Amyotrophic Lateral Sclerosis—Frontotemporal Spectrum Disorder (ALS-FTSD). A Review

Moszczynski, A. J., Hintermayer, M. A., & Strong, M. J. (2018) · Frontiers in Neuroscience · doi:10.3389/fnins.2018.00259 · Cited by 19

Cognitive and behavioral status in Japanese ALS patients: a multicenter study

Watanabe, Y., Raaphorst, J., Izumi, Y., Yoshino, H., Ito, S., Adachi, T., Takigawa, H., Masuda, M., Atsuta, N., Adachi, Y., et al. (2020) · Journal of Neurology · doi:10.1007/s00415-019-09655-9 · Cited by 18

Reliability and validity of the brief dimensional apathy scale

Radakovic, R., Gray, D., Dudley, K., Mioshi, E., Dick, D., Melchiorre, G., Gordon, H., Newton, J., Colville, S., Pal, S., et al. (2020) · Archives of Clinical Neuropsychology · doi:10.1093/arclin/acaa002 · Cited by 18

Plateaus in amyotrophic lateral sclerosis progression: results from a population‐based cohort

Vasta, R., D’Ovidio, F., Canosa, A., Manera, U., Torrieri, M. C., Grassano, M., Marchi, F. D., Mazzini, L., Moglia, C., Calvo, A., et al. (2020) · European Journal of Neurology · doi:10.1111/ene.14287 · Cited by 18

Longitudinal clinical and neuroanatomical correlates of memory impairment in motor neuron disease

Machts, J., Keute, M., Kaufmann, J., Schreiber, S., Kasper, E., Petri, S., Prudlo, J., Vielhaber, S., & Schoenfeld, M. A. (2020) · NeuroImage Clinical · doi:10.1016/j.nicl.2020.102545 · Cited by 18

Mapping cortical disease-burden at individual-level in frontotemporal dementia: implications for clinical care and pharmacological trials

McKenna, M. C., Tahedl, M., Lope, J., Chipika, R. H., Shing, S. L. H., Doherty, M. A., Hengeveld, J. C., Vajda, A., McLaughlin, R. L., Hardiman, O. M., et al. (2021) · Brain Imaging and Behavior · doi:10.1007/s11682-021-00523-7 · Cited by 18

Everyday life experiences of close relatives of people with amyotrophic lateral sclerosis (ALS) receiving home mechanical ventilation - a qualitative study.

Winther, D., Lorenzen, C., & Dreyer, P. (2020) · Journal of Clinical Nursing · doi:10.1111/jocn.15239 · Cited by 17

Clinical features and progress in diagnosis and treatment of amyotrophic lateral sclerosis

Yuan, D., Jiang, S., & Xu, R. (2024) · Annals of Medicine · doi:10.1080/07853890.2024.2399962 · Cited by 17

Schwann Cells in Neuromuscular Disorders: A Spotlight on Amyotrophic Lateral Sclerosis

Moss, K. R., & Saxena, S. (2025) · Cells · doi:10.3390/cells14010047 · Cited by 17

Potential utility of plasma pTau217 for assessing amyloid and tau biomarker profiles

Shin, D., Jang, H., Yoo, H., Kim, K., Zetterberg, H., Blennow, K., González‐Ortiz, F., Ashton, N. J., Lee, E. H., Yun, J., et al. (2025) · European Journal of Nuclear Medicine and Molecular Imaging · doi:10.1007/s00259-025-07457-y · Cited by 18

Does embodied cognition allow a better management of neurological diseases? A review on the link between cognitive language processing and motor function

Naro, A., Maggio, M. G., Latella, D., Rosa, G. L., Sciarrone, F., Manuli, A., & Calabrò, R. S. (2021) · Applied Neuropsychology Adult · doi:10.1080/23279095.2021.1890595 · Cited by 19

Cognitive Profiles of Amyotrophic Lateral Sclerosis Differ in Resting-State Functional Connectivity: An fMRI Study

Temp, A. G. M., Dyrba, M., Büttner, C., Kasper, E., Machts, J., Kaufmann, J., Vielhaber, S., Teipel, S. J., & Prudlo, J. (2021) · Frontiers in Neuroscience · doi:10.3389/fnins.2021.682100 · Cited by 17

Behavioral and Cognitive Phenotypes of Patients With Amyotrophic Lateral Sclerosis Carrying SOD1 Variants

Bella, E. D., Bersano, E., Bruzzone, M. G., Gellera, C., Pensato, V., Lauria, G., & Consonni, M. (2022) · Neurology · doi:10.1212/wnl.0000000000201044 · Cited by 17

Assessment of pragmatic abilities and cognitive substrates (APACS) brief remote: a novel tool for the rapid and tele-evaluation of pragmatic skills in Italian

Bischetti, L., Pompei, C., Scalingi, B., Frau, F., Bosia, M., Arcara, G., & Bambini, V. (2023) · Language Resources and Evaluation · doi:10.1007/s10579-023-09667-y · Cited by 20

Acoustic Voice Analysis as a Useful Tool to Discriminate Different ALS Phenotypes

Milella, G., Sciancalepore, D., Cavallaro, G., Piccirilli, G., Nanni, A. G., Fraddosio, A., D’Errico, E., Paolicelli, D., Fiorella, M. L., & Simone, I. L. (2023) · Biomedicines · doi:10.3390/biomedicines11092439 · Cited by 17

Machine learning-based radiomics for amyotrophic lateral sclerosis diagnosis

Tafuri, B., Milella, G., Filardi, M., Giugno, A., Zoccolella, S., Tamburrino, L., Gnoni, V., Urso, D., Blasi, R. D., Nigro, S. L., et al. (2023) · Expert Systems with Applications · doi:10.1016/j.eswa.2023.122585 · Cited by 17

Multimodal MRI quantification of the common neurostructural bases within the FTD-ALS continuum

Crespi, C., Dodich, A., Cappa, S. F., Canessa, N., Iannaccone, S., Corbo, M., Lunetta, C., Falini, A., & Cerami, C. (2017) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2017.09.019 · Cited by 17

Patterns of Language Impairment in Early Amyotrophic Lateral Sclerosis

Pinto‐Grau, M., Donohoe, B., O’Connor, S. S., Murphy, L., Costello, E., Heverin, M., Vajda, A., Hardiman, O. M., & Pender, N. P. (2020) · Neurology Clinical Practice · doi:10.1212/cpj.0000000000001006 · Cited by 17

Inducible expression of human C9ORF72 36× G4C2 hexanucleotide repeats is sufficient to cause RAN translation and rapid muscular atrophy in mice

Riemslagh, F. W., van der Toorn, E. C., Verhagen, R. F. M., Maas, A., Bosman, L. W. J., Hukema, R. K., & Willemsen, R. (2021) · Disease Models & Mechanisms · doi:10.1242/dmm.044842 · Cited by 16

Italian adaptation of the Beaumont Behavioral Inventory (BBI): psychometric properties and clinical usability

Iazzolino, B., Pain, D., Peotta, L., Aiello, E. N., Gallucci, M., Radici, A., Palumbo, F., Canosa, A., Moglia, C., Calvo, A., et al. (2021) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2021.1946085 · Cited by 17

Language impairment in motor neuron disease phenotypes different from classical amyotrophic lateral sclerosis: a review

Sbrollini, B., Preti, A. N., Zago, S., Papagno, C., Appollonio, I. M., & Aiello, E. N. (2021) · Aphasiology · doi:10.1080/02687038.2021.1959017 · Cited by 17

Cognition and motor phenotypes in ALS: a retrospective study

Aiello, E. N., Pain, D., Radici, A., Aktipi, K. M., Sideri, R., Appollonio, I. M., & Mora, G. (2022) · Neurological Sciences · doi:10.1007/s10072-022-06157-x · Cited by 17

Is Telomere Length Shortening a Risk Factor for Neurodegenerative Disorders?

Yu, H.-J., & Koh, S. (2022) · Dementia and Neurocognitive Disorders · doi:10.12779/dnd.2022.21.3.83 · Cited by 17

Prefrontal cortical thickness in motor neuron disease

Machts, J., Cárdenas‐Blanco, A., Acosta‐Cabronero, J., Kaufmann, J., Loewe, K., Kasper, E., Schuster, C., Prudlo, J., Vielhaber, S., & Nestor, P. J. (2018) · NeuroImage Clinical · doi:10.1016/j.nicl.2018.03.002 · Cited by 17

The Edinburgh Cognitive and Behavioral ALS screen: relationship to age, education, IQ and the Addenbrooke’s Cognitive Examination-III

Valenzuela, M. M. D. I., Bak, T. H., Pal, S., & Abrahams, S. (2018) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2018.1491601 · Cited by 17

Improved detection of RNA foci in C9orf72 amyotrophic lateral sclerosis post-mortem tissue using BaseScope™ shows a lack of association with cognitive dysfunction

Mehta, A. R., Selvaraj, B. T., Barton, S. K., McDade, K., Abrahams, S., Chandran, S., Smith, C., & Gregory, J. M. (2020) · Brain Communications · doi:10.1093/braincomms/fcaa009 · Cited by 17

Examining empathy deficits across familial forms of frontotemporal dementia within the GENFI cohort

Foster, P. H., Russell, L. L., Peakman, G., Convery, R. S., Bouzigues, A., Greaves, C. V., Bocchetta, M., Cash, D. M., van Swieten, J. C., Jiskoot, L. C., et al. (2022) · Cortex · doi:10.1016/j.cortex.2022.01.012 · Cited by 16

Sensory Dysfunction in ALS and Other Motor Neuron Diseases: Clinical Relevance, Histopathology, Neurophysiology, and Insights from Neuroimaging

Kleinerová, J., Chipika, R. H., Tan, E. L., Yunusova, Y., Marchand‐Pauvert, V., Kassubek, J., Pradat, P., & Bede, P. (2025) · Biomedicines · doi:10.3390/biomedicines13030559 · Cited by 19

Lung volume recruitment improves volitional airway clearance in amyotrophic lateral sclerosis

Cleary, S., Misiaszek, J. E., Wheeler, S., Kalra, S., Genuis, S. K., & Johnston, W. S. W. (2021) · Muscle & Nerve · doi:10.1002/mus.27417 · Cited by 16

Clinicoanatomical substrates of selfish behaviour in amyotrophic lateral sclerosis – An observational cohort study

Lulé, D. E., Michels, S., Finsel, J., Braak, H., Tredici, K. D., Strobel, J., Beer, A. J., Uttner, I., Müller, H., Kassubek, J., et al. (2021) · Cortex · doi:10.1016/j.cortex.2021.11.009 · Cited by 16

Pathophysiological Underpinnings of Extra-Motor Neurodegeneration in Amyotrophic Lateral Sclerosis: New Insights From Biomarker Studies

Reyes‐Leiva, D., Dols‐Icardo, O., Sirisi, S., Cortés‐Vicente, E., Turón‐Sans, J., de Luna, N., Blesa, R., Belbin, O., Montal, V., Alcolea, D. A., et al. (2022) · Frontiers in Neurology · doi:10.3389/fneur.2021.750543 · Cited by 18

Cognition in the course of ALS—a meta-analysis

Finsel, J., Uttner, I., Vázquez, C., Ludolph, A. C., & Lulé, D. E. (2022) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2022.2101379 · Cited by 16

The Role of TAR DNA Binding Protein 43 (TDP-43) as a CandiDate Biomarker of Amyotrophic Lateral Sclerosis: A Systematic Review and Meta-Analysis

Gambino, C. M., Ciaccio, A. M., Sasso, B. L., Giglio, R. V., Vidali, M., Agnello, L., & Ciaccio, M. (2023) · Diagnostics · doi:10.3390/diagnostics13030416 · Cited by 17

Association of Copresence of Pathogenic Variants Related to Amyotrophic Lateral Sclerosis and Prognosis

Chiò, A., Moglia, C., Canosa, A., Manera, U., Grassano, M., Vasta, R., Palumbo, F., Gallone, S., Brunetti, M., Barberis, M., et al. (2023) · Neurology · doi:10.1212/wnl.0000000000207367 · Cited by 16

Identifying and Diagnosing TDP-43 Neurodegenerative Diseases in Psychiatry

Ducharme, S., Pijnenburg, Y. A. L., Rohrer, J. D., Huey, E. D., Finger, E. C., & Tatton, N. A. (2023) · American Journal of Geriatric Psychiatry · doi:10.1016/j.jagp.2023.08.017 · Cited by 16

Distinct neuronal circuits mediate cortical hyperexcitability in amyotrophic lateral sclerosis

Pavey, N., Hannaford, A. P., van den Bos, M. A. J., Kiernan, M. C., Menon, P., & Vucic, S. (2024) · Brain · doi:10.1093/brain/awae049 · Cited by 18

Clinical features and incidence trends of amyotrophic lateral sclerosis in Navarre, Spain, 2007–2018: a population-based study

Jericó, I., Beiras, I. E., Pagola, I., Torné, L., Galbete, A., Osinaga, J. D., & Vicente, E. F. (2021) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2021.1891249 · Cited by 16

Dysgraphic features in motor neuron disease: a review

Aiello, E. N., Feroldi, S., Preti, A. N., Zago, S., & Appollonio, I. M. (2021) · Aphasiology · doi:10.1080/02687038.2021.1942774 · Cited by 16

Upper motor neuron dysfunction is associated with the presence of behavioural impairment in patients with amyotrophic lateral sclerosis

Maranzano, A., Poletti, B., Solca, F., Torre, S., Colombo, E., Farè, M., Ferrucci, R., Carelli, L., Verde, F., Morelli, C., et al. (2022) · European Journal of Neurology · doi:10.1111/ene.15243 · Cited by 16

Primary progressive aphasia and motor neuron disease: A review

Aiello, E. N., Feroldi, S., Luca, G. D., Guidotti, L., Arrigoni, E., Appollonio, I. M., Solca, F., Carelli, L., Poletti, B., Verde, F., et al. (2022) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2022.1003792 · Cited by 19

The vulnerability of motor and frontal cortex-dependent behaviors in mice expressing ALS-linked mutation in TDP-43

Wong, P., Ho, W. Y., Yen, Y.-C., Sanford, E., & Ling, S. (2020) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2020.03.019 · Cited by 16

Neuropathology of Speech Network Distinguishes Bulbar From Nonbulbar Amyotrophic Lateral Sclerosis

Shellikeri, S., Keith, J. L., Black, S. E., Zinman, L. H., & Yunusova, Y. (2019) · Journal of Neuropathology & Experimental Neurology · doi:10.1093/jnen/nlz130 · Cited by 15

Cognitive and behavioral involvement in ALS has been known for more than a century

Zago, S., Lorusso, L., Aiello, E. N., Ugolini, M., Poletti, B., Ticozzi, N., & Silani, V. (2022) · Neurological Sciences · doi:10.1007/s10072-022-06340-0 · Cited by 15

Mild behavioral impairment as a potential marker of predementia risk states in motor neuron diseases

Ferraro, P. M., Gervino, E., Maria, E. D., Meo, G., Ponzano, M., Pardini, M., Signori, A., Schenone, A. E., Roccatagliata, L., & Caponnetto, C. (2022) · European Journal of Neurology · doi:10.1111/ene.15570 · Cited by 14

Motor cortex functional connectivity is associated with underlying neurochemistry in ALS

Dey, A., Luk, C., Ishaque, A. H., Ta, D., Srivastava, O., Krebs, D., Seres, P., Hanstock, C. C., Beaulieu, C., Korngut, L. W., et al. (2022) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2022-329993 · Cited by 15

Clinical and genetic characteristics of amyotrophic lateral sclerosis patients with ANXA11 variants

Sung, W., Nahm, M., Lim, S. M., Noh, M., Lee, S.-G., Hwang, S.-M., Kim, Y. H., Park, J., Oh, K., Ki, C., et al. (2022) · Brain Communications · doi:10.1093/braincomms/fcac299 · Cited by 15

Amyloid-Beta Co-Pathology Is a Major Determinant of the Elevated Plasma GFAP Values in Amyotrophic Lateral Sclerosis

Mastrangelo, A., Vacchiano, V., Zenesini, C., Ruggeri, E., Baiardi, S., Cherici, A., Avoni, P., Polischi, B., Santoro, F., Capellari, S., et al. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms241813976 · Cited by 16

Quality of life, cognitive and behavioural impairment in people with motor neuron disease: a systematic review

Radaković, R., Radakovic, C., Abrahams, S., Simmons, Z., & Carroll, A. (2024) · Quality of Life Research · doi:10.1007/s11136-024-03611-5 · Cited by 15

High Frequency of Cognitive and Behavioral Impairment in Amyotrophic Lateral Sclerosis Patients with SOD1 Pathogenic Variants

Calvo, A., Moglia, C., Canosa, A., Manera, U., Vasta, R., Grassano, M., Daviddi, M., Mattei, F. D., Matteoni, E., Gallone, S., et al. (2024) · Annals of Neurology · doi:10.1002/ana.26928 · Cited by 15

Cerebral degeneration in amyotrophic lateral sclerosis

Srivastava, O., Hanstock, C. C., Chenji, S., Mah, D., Eurich, D. T., Ta, D., Seres, P., Luk, C., Zinman, L. H., Abrahão, A., et al. (2019) · Neurology Clinical Practice · doi:10.1212/cpj.0000000000000674 · Cited by 15

Papez Circuit Gray Matter and Episodic Memory in Amyotrophic Lateral Sclerosis and Behavioural Variant Frontotemporal Dementia

Bueno, A. P. A., de Souza, L. C., Pinaya, W. H. L., Teixeira, A. L., Prado, L. D. G. R., Caramelli, P., Hornberger, M., & Sato, J. R. (2020) · Brain Imaging and Behavior · doi:10.1007/s11682-020-00307-5 · Cited by 15

Brain metabolic correlates of apathy in amyotrophic lateral sclerosis: An 18F‐FDG‐positron emission tomography stud

Canosa, A., Vacchiano, V., D’Ovidio, F., Calvo, A., Moglia, C., Manera, U., Vasta, R., Liguori, R., Arena, V., Grassano, M., et al. (2020) · European Journal of Neurology · doi:10.1111/ene.14637 · Cited by 15

Retinal Spheroids and Axon Pathology Identified in Amyotrophic Lateral Sclerosis

Sharma, K., Amin, M. A. M., Gupta, N., Zinman, L. H., Zhou, X., Irving, H. M., & Yücel, Y. H. (2020) · Investigative Ophthalmology & Visual Science · doi:10.1167/iovs.61.13.30 · Cited by 15

Validation of The Edinburgh cognitive and behavioural ALS screen (ECAS) in behavioural variant frontotemporal dementia and Alzheimer’s disease

Valenzuela, M. M. D. I., Bak, T. H., Thompson, H. E., Colville, S., Pal, S., & Abrahams, S. (2021) · International Journal of Geriatric Psychiatry · doi:10.1002/gps.5566 · Cited by 15

Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort

Peakman, G., Russell, L. L., Convery, R. S., Nicholas, J. M., Swieten, J. C. V., Jiskoot, L. C., Moreno, F., Sánchez‐Valle, R., Laforce, R. J., Graff, C., et al. (2021) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2021-326868 · Cited by 20

Social cognition in the FTLD spectrum: evidence from MRI

Magno, M. A., Canu, E., Filippi, M., & Agosta, F. (2021) · Journal of Neurology · doi:10.1007/s00415-021-10892-0 · Cited by 17

The diagnostic value of the Italian version of the Edinburgh Cognitive and Behavioral ALS Screen (ECAS)

Aiello, E. N., Iazzolino, B., Pain, D., Peotta, L., Palumbo, F., Radici, A., Canosa, A., Moglia, C., Calvo, A., Mora, G., et al. (2022) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2022.2051552 · Cited by 15

Targeting TNFα produced by astrocytes expressing amyotrophic lateral sclerosis‐linked mutant fused in sarcoma prevents neurodegeneration and motor dysfunction in mice

Jensen, B. K., McAvoy, K. J., Heinsinger, N. M., Lepore, A. C., Ilieva, H. S., Haeusler, A. R., Trotti, D., & Pasinelli, P. (2022) · Glia · doi:10.1002/glia.24183 · Cited by 14

Alteration of interoceptive sensitivity: expanding the spectrum of behavioural disorders in amyotrophic lateral sclerosis

Moretta, P., Spisto, M., Ausiello, F. P., Iodice, R., Lucia, N. D., Santangelo, G., Trojano, L., Salvatore, E., & Dubbioso, R. (2022) · Neurological Sciences · doi:10.1007/s10072-022-06231-4 · Cited by 15

Neuropsychiatric symptoms in genetic frontotemporal dementia: developing a new module for Clinical Rating Scales

Samra, K., MacDougall, A. M., Peakman, G., Bouzigues, A., Bocchetta, M., Cash, D. M., Greaves, C. V., Convery, R. S., Swieten, J. C. V., Jiskoot, L. C., et al. (2023) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2022-330152 · Cited by 23

Cognitive Syndromes and C9orf72 Mutation Are Not Related to Cerebellar Degeneration in Amyotrophic Lateral Sclerosis

Consonni, M., Bella, E. D., Nigri, A., Pinardi, C., Demichelis, G., Porcu, L., Gellera, C., Pensato, V., Cappa, S. F., Bruzzone, M. G., et al. (2019) · Frontiers in Neuroscience · doi:10.3389/fnins.2019.00440 · Cited by 15

Synergistic toxicity in an in vivo model of neurodegeneration through the co-expression of human TDP-43M337V and tauT175D protein

Moszczynski, A. J., Harvey, M., Fulcher, N., de Oliveira, C., McCunn, P., Donison, N., Bartha, R., Schmid, S., Strong, M. J., & Volkening, K. (2019) · Acta Neuropathologica Communications · doi:10.1186/s40478-019-0816-1 · Cited by 15

Developments in the assessment of non-motor disease progression in amyotrophic lateral sclerosis

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CSF Heavy Neurofilament May Discriminate and Predict Motor Neuron Diseases with Upper Motor Neuron Involvement

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TMEM106B Acts as a Modifier of Cognitive and Motor Functions in Amyotrophic Lateral Sclerosis

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Misdiagnosis of amyotrophic lateral sclerosis in clinical practice in Europe and the USA: a patient chart review and physician survey

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Structural and Functional Brain Network Connectivity at Different King's Stages in Patients With Amyotrophic Lateral Sclerosis

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Giving Breath to Motor Neurons: Noninvasive Mechanical Ventilation Slows Disease Progression in Amyotrophic Lateral Sclerosis

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Identifying Mild Behavioral and Neurocognitive Impairment in Amyotrophic Lateral Sclerosis ( MBNI ‐ ALS ) Provides Key Prognostic Insights

Spisto, M., Moretta, P., Senerchia, G., Iuzzolino, V. V., Aruta, L., Salvatore, E., Santangelo, G., Trojano, L., & Dubbioso, R. (2025) · European Journal of Neurology · doi:10.1111/ene.70171 · Cited by 14

Deficits in Emotion Recognition as Markers of Frontal Behavioral Dysfunction in Amyotrophic Lateral Sclerosis

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Potential of the Cardiovascular Drug Levosimendan in the Management of Amyotrophic Lateral Sclerosis: An Overview of a Working Hypothesis

Al‐Chalabi, A., Heunks, L. M. A., Papp, Z., & Pollesello, P. (2019) · Journal of Cardiovascular Pharmacology · doi:10.1097/fjc.0000000000000728 · Cited by 13

Radiogenomics of C9orf72 Expansion Carriers Reveals Global Transposable Element Derepression and Enables Prediction of Thalamic Atrophy and Clinical Impairment

Bonham, L. W., Geier, E. G., Sirkis, D. W., Leong, J. K., Ramos, E. M., Wang, Q., Karydas, A., Lee, S. E., Sturm, V. E., Sawyer, R. P., et al. (2022) · Journal of Neuroscience · doi:10.1523/jneurosci.1448-22.2022 · Cited by 15

The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) in frontotemporal dementia

Saxon, J. A., Thompson, J. C., Harris, J., Ealing, J., Hamdalla, H. H. M., Chaouch, A., Young, C. A., Blackburn, D. J., Majeed, T., Gall, C., et al. (2020) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2020.1797090 · Cited by 15

Plasma Uric Acid Helps Predict Cognitive Impairment in Patients With Amyotrophic Lateral Sclerosis

Tang, J., Yang, Y., Gong, Z., Li, Z., Huang, L., Ding, F., Liu, M., & Zhang, M. (2021) · Frontiers in Neurology · doi:10.3389/fneur.2021.789840 · Cited by 13

Cognitive, behavioral, and brain functional connectivity correlates of fatigue in amyotrophic lateral sclerosis

Trojsi, F., Nardo, F. D., D’Alvano, G., Passaniti, C., Sharbafshaaer, M., Canale, F., Russo, A., Silvestro, M., Lavorgna, L., Cirillo, M., et al. (2023) · Brain and Behavior · doi:10.1002/brb3.2931 · Cited by 13

Neuronal dysfunction caused by FUSR521G promotes ALS-associated phenotypes that are attenuated by NF-κB inhibition

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Cognitive reserve in ALS: the role of occupational skills and requirements

Rhodes, E., Alfa, S., Jin, H. A., Massimo, L. M., Elman, L. B., Amado, D. A., Baer, M., Quinn, C. C., & McMillan, C. T. (2024) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2024.2336113 · Cited by 12

Amyotrophic lateral sclerosis; clinical features, differential diagnosis and pathology

van Es, M. A. (2024) · International review of neurobiology · doi:10.1016/bs.irn.2024.04.011 · Cited by 15

Circulating endocannabinoidome signatures of disease activity in amyotrophic lateral sclerosis

Dubbioso, R., Iannotti, F. A., Senerchia, G., Verde, R., Iuzzolino, V. V., Spisto, M., Fasolino, I., Manganelli, F., Marzo, V. D., & Piscitelli, F. (2024) · European Journal of Neurology · doi:10.1111/ene.16400 · Cited by 12

TDP-43 seeding activity in the olfactory mucosa of patients with amyotrophic lateral sclerosis

Vizziello, M., Dellarole, I. L., Ciullini, A., Pascuzzo, R., Lombardo, A., Bellandi, F., Celauro, L., Battipaglia, C., Ciusani, E., Rizzo, A., et al. (2025) · Molecular Neurodegeneration · doi:10.1186/s13024-025-00833-0 · Cited by 13

The capacity to consent to treatment in amyotrophic lateral sclerosis: a preliminary report

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Non-Motor Symptoms of Amyotrophic Lateral Sclerosis: A Multi-Faceted Disorder

Nash, Y., & Sitty, M. (2021) · Journal of Neuromuscular Diseases · doi:10.3233/jnd-210632 · Cited by 13

Harnessing the Potential of Human Pluripotent Stem Cell-Derived Motor Neurons for Drug Discovery in Amyotrophic Lateral Sclerosis: From the Clinic to the Laboratory and Back to the Patient

Lamas, N. J., & Roybon, L. (2021) · Frontiers in Drug Discovery · doi:10.3389/fddsv.2021.773424 · Cited by 12

Parkin beyond Parkinson’s Disease—A Functional Meaning of Parkin Downregulation in TDP-43 Proteinopathies

Gawęda-Walerych, K., Sitek, E. J., Narożańska, E., & Buratti, E. (2021) · Cells · doi:10.3390/cells10123389 · Cited by 13

A Systematic Review of Caregiver Coping Strategies in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

Caga, J., Kiernan, M., & Piguet, O. (2021) · Journal of Geriatric Psychiatry and Neurology · doi:10.1177/08919887211060016 · Cited by 11

Reliable change indices for the Italian Edinburgh Cognitive and Behavioral ALS Screen (ECAS)

Aiello, E. N., Solca, F., Torre, S., Carelli, L., Monti, A., Ferrucci, R., Verde, F., Ticozzi, N., Silani, V., & Poletti, B. (2022) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2022.2134801 · Cited by 13

Diagnostic properties of the Italian ECAS Carer Interview (ECAS-CI)

Poletti, B., Aiello, E. N., Solca, F., Torre, S., Carelli, L., Ferrucci, R., Verde, F., Ticozzi, N., & Silani, V. (2022) · Neurological Sciences · doi:10.1007/s10072-022-06505-x · Cited by 13

The quality of life and depression in primary caregivers of patients with amyotrophic lateral sclerosis is affected by patient-related and culture-specific conditions

Maksymowicz-Śliwińska, A., Lulé, D. E., Nieporęcki, K., Ciećwierska, K., Ludolph, A. C., & Kuźma‐Kozakiewicz, M. (2022) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2022.2126322 · Cited by 13

Association of the risk factor UNC13A with survival and upper motor neuron involvement in amyotrophic lateral sclerosis

Manini, A., Casiraghi, V., Brusati, A., Maranzano, A., Gentile, F., Colombo, E., Bonetti, R., Peverelli, S., Invernizzi, S., Gentilini, D., et al. (2023) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2023.1067954 · Cited by 13

Development, reliability, validity, and acceptability of the remote administration of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS)

Gray, D., Lesley, R., Mayberry, E. J., Williams, L., McHutchison, C. A., Newton, J. L., Pal, S., Chandran, S., MacPherson, S. E., Abrahams, S., et al. (2023) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2023.2278512 · Cited by 13

The neural network basis of altered decision‐making in patients with amyotrophic lateral sclerosis

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Comparative diagnosis interest of NfL and pNfH in CSF and plasma in a context of FTD–ALS spectrum

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CSF Ubiquitin Levels Are Higher in Alzheimer’s Disease than in Frontotemporal Dementia and Reflect the Molecular Subtype in Prion Disease

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Brain structural and perfusion changes in amyotrophic lateral sclerosis-frontotemporal dementia patients with cognitive and motor onset: a preliminary study

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Cognitive and behavioural but not motor impairment increases brain age in amyotrophic lateral sclerosis

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Clinical and Metabolic Signature of UNC13A rs12608932 Variant in Amyotrophic Lateral Sclerosis

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Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis

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Neuropsychological impairment in amyotrophic lateral sclerosis–frontotemporal spectrum disorder

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Cognitive and behavioural criteria for ALS-FTD

Hardiman, O. M. (2023) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2023.120981

Expanding the neuropsychological phenotype of ALS: Preliminary findings

Fiamingo, G., Diamanti, L., Collesi, M., Ballante, E., & Cappa, S. F. (2021) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2021.119397

Cognition in amyotrophic lateral sclerosis

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