Use of the CDR® plus NACC FTLD in mild FTLD: Data from the ARTFL/LEFFTDS consortium

Miyagawa, Toji; Brushaber, Danielle; Syrjanen, Jeremy; Kremers, Walter; Fields, Julie; Forsberg, Leah K.; Heuer, Hilary W.; Knopman, David; Kornak, John; Boxer, Adam; Rosen, Howie; Boeve, Bradley; on Behalf of the ARTFL/LEFFTDS Consortium

doi:10.1016/j.jalz.2019.05.013

Cited by 92Dementia and Cognitive Impairment ResearchHistorical and Modern Theater StudiesAlzheimer's disease research and treatments

Use of the CDR® plus NACC FTLD in mild FTLD: Data from the ARTFL/LEFFTDS consortium

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Miyagawa, Toji, Brushaber, Danielle, Syrjanen, Jeremy, Kremers, Walter, Fields, Julie, Forsberg, Leah K., Heuer, Hilary W., Knopman, David +5 more·2020·Alzheimer's & Dementia

DOI 10.1016/j.jalz.2019.05.013PMC PMC6949373

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Abstract

Introduction: Behavior/Comportment/Personality (BEHAV) and Language (LANG) domains were added to the Clinical Dementia Rating (CDR®) for improving evaluation of patients with frontotemporal lobar degeneration (FTLD) (CDR® plus NACC FTLD). Methods: We analyzed the CDR® plus NACC FTLD among participants from the baseline visit of the Advancing Research and Treatment for Frontotemporal Lobar Degeneration/Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects Consortium. Results: The CDR® plus NACC FTLD was able to detect early symptoms in the mildly impaired participants who were rated as CDR® sum of boxes (CDR®‐SB) = 0. The CDR®‐SB was not sensitive, particularly in participants with mild nonfluent/agrammatic primary progressive aphasia. Participants with familial and sporadic behavioral variant FTD exhibited similar CDR® plus NACC FTLD profiles except that language impairment was more frequent in participants with mild sporadic behavioral variant FTD. Adding the BEHAV and/or LANG domains to the CDR®‐SB significantly enhanced discriminatory power in differentiating among the FTLD spectrum disorders. Discussion: The BEHAV and LANG domains enable the CDR® plus NACC FTLD to capture early symptomatology of FTLD.

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Use of newly developed psychometric measures for characterizing all stages of symptoms in familial FTD research will facilitate the discovery and validation of early FTD biomarkers.
“Use of newly developed psychometric measures for characterizing all stages of symptoms (e.g., FTLD‐Clinical Dementia Rating, Multidomain Impairment Rating) in familial FTD research will facilitate the discovery and validation of early FTD biomarkers. 51 , 127”
From New developments of biofluid‐based biomarkers for routine diagnosis and disease trajectories in frontotemporal dementia (2022) · cites this paper as [127]

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Samra, K., MacDougall, A. M., Bouzigues, A., Bocchetta, M., Cash, D. M., Greaves, C. V., Convery, R. S., Hardy, C. J. D., Swieten, J. C. V., Seelaar, H., et al. (2023) · Brain Communications · doi:10.1093/braincomms/fcad036 · Cited by 19 · see citing works
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 18 · see citing works
Examining associations between smartphone use and clinical severity in frontotemporal dementia: proof-of-concept study
Paolillo, E. W., Casaletto, K. B., Clark, A. L., Taylor, J. C., Heuer, H. W., Wise, A. B., Dhanam, S., Sanderson-Cimino, M., Saloner, R., Kramer, J. H., et al. (2024) · JMIR Aging · doi:10.2196/52831 · Cited by 16 · see citing works
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A latent variable approach to measuring bridging social capital and examining its association to older adults’ cognitive health
Peng, S., Roth, A. R., & Perry, B. L. (2021) · Social Neuroscience · doi:10.1080/17470919.2021.2001368 · Cited by 19 · see citing works
Language impairment in the genetic forms of behavioural variant frontotemporal dementia
Samra, K., MacDougall, A. M., Bouzigues, A., Bocchetta, M., Cash, D. M., Greaves, C. V., Convery, R. S., Swieten, J. C. V., Seelaar, H., Jiskoot, L. C., et al. (2022) · Journal of Neurology · doi:10.1007/s00415-022-11512-1 · Cited by 16 · see citing works
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 15 · see citing works
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 15 · see citing works
Cognitive dispersion and its functional relevance in behavioral variant frontotemporal dementia and prodromal behavioral variant frontotemporal dementia.
Webber, T. A., Woods, S. P., Lorkiewicz, S. A., Yazbeck, H. W., Schultz, E. R., & Kiselica, A. M. (2024) · Neuropsychology · doi:10.1037/neu0000969 · Cited by 13 · see citing works
Quality of life and caregiver burden in familial frontotemporal lobar degeneration: Analyses of symptomatic and asymptomatic individuals within the LEFFTDS cohort
Gentry, M. T., Lapid, M. I., Syrjanen, J. A., Calvert, K. J., Hughes, S. R., Brushaber, D. E., Kremers, W. K., Bove, J., Brannelly, P., Coppola, G., et al. (2020) · Alzheimer s & Dementia · doi:10.1002/alz.12095 · Cited by 13 · see citing works
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Plasma lysosphingolipids in GRN-related diseases: Monitoring lysosomal dysfunction to track disease progression
Khrouf, W., Saracino, D., Rucheton, B., Houot, M., Clot, F., Rinaldi, D., Vítor, J., Huynh, M., Heng, E., Schlemmer, D., et al. (2023) · Neurobiology of Disease · doi:10.1016/j.nbd.2023.106108 · Cited by 11 · see citing works
The Frontotemporal Dementia Prevention Initiative: Linking Together Genetic Frontotemporal Dementia Cohort Studies
Rohrer, J. D., & Boxer, A. L. (2021) · Advances in experimental medicine and biology · doi:10.1007/978-3-030-51140-1_8 · Cited by 12 · see citing works
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Predictors of Care Home Admission and Survival Rate in Patients With Syndromes Associated With Frontotemporal Lobar Degeneration in Europe
Borroni, B., Tarantino, B., Graff, C., Krüger, J., Ludolph, A. C., Moreno, F., Otto, M., Rowe, J. B., Seelaar, H., Solje, E., et al. (2024) · Neurology · doi:10.1212/wnl.0000000000209793 · Cited by 11 · see citing works
Gene-Specific Effects on Brain Volume and Cognition of TMEM106B in Frontotemporal Lobar Degeneration
Vandebergh, M., Ramos, E. M., Corriveau‐Lecavalier, N., Ramanan, V. K., Kornak, J., Mester, C. T., Kolander, T. W., Brushaber, D. E., Staffaroni, A. M., Geschwind, D. H., et al. (2024) · Neurology · doi:10.1212/wnl.0000000000209832 · Cited by 11 · see citing works
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Association of Initial Side of Brain Atrophy With Clinical Features and Disease Progression in Patients With GRN Frontotemporal Dementia
Borrego‐Écija, S., Juncà‐Parella, J., Vandebergh, M., Pérez‐Millan, A., Balasa, M., Lladó, A., Bouzigues, A., Russell, L. L., Foster, P. H., Ferry‐Bolder, E., et al. (2024) · Neurology · doi:10.1212/wnl.0000000000209944 · Cited by 11 · see citing works
Functional Disabilities and Psychiatric Symptoms in Primary Progressive Aphasia
Morrow, C. B., Leoutsakos, J., & Onyike, C. U. (2021) · American Journal of Geriatric Psychiatry · doi:10.1016/j.jagp.2021.07.013 · Cited by 11 · see citing works
Biological basis and psychiatric symptoms in frontotemporal dementia
Mori, K., & Ikeda, M. (2022) · Psychiatry and Clinical Neurosciences · doi:10.1111/pcn.13375 · Cited by 11 · see citing works
Neuropsychiatric symptoms cluster and fluctuate over time in behavioral variant frontotemporal dementia
Morrow, C. B., Kamath, V. J., Dickerson, B. C., Eldaief, M. C., Rezaii, N., Wong, B., McGinnis, S. M., Darby, R., Staffaroni, A. M., Lapid, M. I., et al. (2025) · Psychiatry and Clinical Neurosciences · doi:10.1111/pcn.13810 · Cited by 10 · see citing works
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Development of a Speech-based Composite Score for Remotely Quantifying Language Changes in Frontotemporal Dementia
Robin, J., Xu, M., Kaufman, L. D., Simpson, W. S., McCaughey, S. M., Tatton, N. A., Wolfus, C., & Ward, M. E. (2023) · Cognitive and Behavioral Neurology · doi:10.1097/wnn.0000000000000356 · Cited by 9 · see citing works
Efficacy and safety of blood derivatives therapy in Alzheimer’s disease: a systematic review and meta-analysis
Fei, Z., Pan, B., Pei, R., Chen, Z., Du, X., Cao, H., & Li, C. (2022) · Systematic Reviews · doi:10.1186/s13643-022-02115-y · Cited by 8 · see citing works
Extending the phenotypic spectrum assessed by the CDR plus NACC FTLD in genetic frontotemporal dementia
Samra, K., Peakman, G., MacDougall, A. M., Bouzigues, A., Greaves, C. V., Convery, R. S., Swieten, J. C. V., Jiskoot, L. C., Seelaar, H., Moreno, F., et al. (2024) · Alzheimer s & Dementia Diagnosis Assessment & Disease Monitoring · doi:10.1002/dad2.12571 · Cited by 8 · see citing works
Multiparametric MRI-based biomarkers in the non-fluent and semantic variants of primary progressive aphasia
Michelutti, M., Huppertz, H., Volkmann, H., Anderl‐Straub, S., Urso, D., Tafuri, B., Nigro, S. L., Manganotti, P., Werner, L., Lombardi, J., et al. (2025) · Journal of Neurology · doi:10.1007/s00415-025-13215-9 · Cited by 7 · see citing works
Preclinical and Prodromal Frontotemporal Dementia: Challenges and Opportunities
Palacino, F., Manganotti, P., & Benussi, A. (2025) · International Journal of Translational Medicine · doi:10.3390/ijtm5040052 · Cited by 7 · see citing works
Hyperorality in Frontotemporal Dementia: Cognitive and Psychiatric Symptom Profiles in Early-Stage Disease
Morrow, C. B., Chaney, G.-A. S., Capuzzi, D., Bakker, A., Onyike, C. U., & Kamath, V. J. (2022) · Journal of Alzheimer s Disease · doi:10.3233/jad-220443 · Cited by 7 · see citing works
Brain Metabolic Profile in Presymptomatic GRN Carriers Throughout a 5-Year Follow-up
Saracino, D., Sellami, L., Boniface, H., Houot, M., Pélégrini‐Issac, M., Funkiewiez, A., Rinaldi, D., Locatelli, M., Azuar, C., Causse-Lemercier, V., et al. (2022) · Neurology · doi:10.1212/wnl.0000000000201439 · Cited by 7 · see citing works
Network Connectivity Alterations across the MAPT Mutation Clinical Spectrum
Zhang, L., Flagan, T., Häkkinen, S., Chu, S. A., Brown, J. A., Lee, A. J., Pasquini, L., Mandelli, M. L., Gorno‐Tempini, M. L., Sturm, V. E., et al. (2023) · Annals of Neurology · doi:10.1002/ana.26738 · Cited by 7 · see citing works
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How can we define the presymptomatic C9orf72 disease in 2022? An overview on the current definitions of preclinical and prodromal phases
Saracino, D., & Ber, I. L. (2022) · Revue Neurologique · doi:10.1016/j.neurol.2022.03.007 · Cited by 6 · see citing works
Sex Hormone-Binding Globulin (SHBG) in Cerebrospinal Fluid Does Not Discriminate between the Main FTLD Pathological Subtypes but Correlates with Cognitive Decline in FTLD Tauopathies
del Campo, M., Pijnenburg, Y. A. L., Chen‐Plotkin, A. S., Irwin, D. J., Grossman, M., Twaalfhoven, H. A. M., Hu, W. T., Meeter, L. H. H., Swieten, J. C. V., Vermunt, L., et al. (2021) · Biomolecules · doi:10.3390/biom11101484 · Cited by 6 · see citing works
The ScreeLing: Detecting Semantic, Phonological, and Syntactic Deficits in the Clinical Subtypes of Frontotemporal and Alzheimer’s Dementia
Jiskoot, L. C., Poos, J. M., van Boven, K., de Boer, L., Giannini, L., Satoer, D., Visch-Brink, E. G., van Hemmen, J., Franzen, S., Pijnenburg, Y. A. L., et al. (2023) · Assessment · doi:10.1177/10731911231154512 · Cited by 5 · see citing works
Prodromal language impairment in genetic frontotemporal dementia within the GENFI cohort
Samra, K., MacDougall, A. M., Bouzigues, A., Bocchetta, M., Cash, D. M., Greaves, C. V., Convery, R. S., Swieten, J. C. V., Jiskoot, L. C., Seelaar, H., et al. (2023) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2023.120711 · Cited by 5 · see citing works
Longitudinal changes in qualitative aspects of semantic fluency in presymptomatic and prodromal genetic frontotemporal dementia
Jiskoot, L. C., van den Berg, E., Laenen, S. A. A. M., Poos, J. M., Giannini, L., Satoer, D., van Hemmen, J., Pijnenburg, Y. A. L., Vonk, J. M. J., & Seelaar, H. (2023) · Journal of Neurology · doi:10.1007/s00415-023-11845-5 · Cited by 5 · see citing works
The role of bilingualism on functional decline and neurodegeneration in distinct ADRD clinical syndromes
Tablante, J., Casaletto, K., VandeVrede, L., Mamuyac, E., Gao, L., Esteban, J. S., Guevarra, A., Johnson, J., Rios, M., Martinez, L., et al. (2025) · Alzheimer s & Dementia · doi:10.1002/alz.70991 · Cited by 4 · see citing works
Portuguese version of the CDR plus NACC FTLD: Validation studies
Lima, M., Tábuas‐Pereira, M., Durães, J., Faustino, P., Simões, M. R., Kukull, W. A., Knopman, D. S., & Santana, I. J. (2022) · Alzheimer s & Dementia Diagnosis Assessment & Disease Monitoring · doi:10.1002/dad2.12355 · Cited by 4 · see citing works
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Clinical Trial Development in Frontotemporal Lobar Degeneration
Ljubenkov, P. A., & Boxer, A. L. (2022) · Cambridge University Press eBooks · doi:10.1017/9781108975759.019 · Cited by 3 · see citing works
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Mini social cognition and emotional assessment : Diagnostic performance and neural correlates in behavioural‐variant frontotemporal dementia
Doğdu, C., Upadhyay, N., Frommann, I., Kleineidam, L., Johnen, A., Stark, M., Roeske, S., Spottke, A. E., Gamez, A., Petzold, G. C., et al. (2025) · Journal of Neuropsychology · doi:10.1111/jnp.70000 · Cited by 2 · see citing works
Cortical microstructure in familial frontotemporal dementia associated with MAPT, GRN, and C9orf72 pathogenic variants: Looking beyond atrophy
Wang, L., Cen, S., Zhao, L., Tang, J., Xu, P., Quan, P., & Ding, W. (2025) · The Journal of Prevention of Alzheimer s Disease · doi:10.1016/j.tjpad.2025.100306 · Cited by 2 · see citing works
MRI ‐ DTI Biomarkers Along the Continuum of Behavioral Variant Frontotemporal Dementia
Michelutti, M., Huppertz, H., Anderl‐Straub, S., Volkmann, H., Urso, D., Tafuri, B., Nigro, S. L., Manganotti, P., Rosenbohm, A., Ludolph, A. C., et al. (2025) · European Journal of Neurology · doi:10.1111/ene.70438 · Cited by 2 · see citing works
Individualized Atrophy‐Based Prediction of Dementia Progression in Familial Frontotemporal Lobar Degeneration With Bayesian Linear Mixed‐Effects Modeling
Dutt, S., Leichter, D., Cobigo, Y., Wolf, A. A., Kornak, J., Clark, A. L., Russell, L. L., Bouzigues, A., Cash, D. M., Bocchetta, M., et al. (2026) · Annals of Neurology · doi:10.1002/ana.78167 · Cited by 2 · see citing works
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Utility of the Japanese version of the Clinical Dementia Rating® plus National Alzheimer's Coordinating Centre Behaviour and Language Domains for sporadic cases of frontotemporal dementia in Japan
Taomoto, D., Sato, S., Kanemoto, H., Suzuki, M., Hirakawa, N., Takasaki, A., Akimoto, M., Satake, Y., Koizumi, F., Yoshiyama, K., et al. (2023) · Psychogeriatrics · doi:10.1111/psyg.13072 · Cited by 2 · see citing works
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Impairments in knowledge of social norms in presymptomatic, prodromal, and symptomatic frontotemporal dementia
de Boer, L., van den Berg, E., Poos, J. M., Klop, W., Giannini, L., Houwer, J. F. H. D., Seelaar, H., & Jiskoot, L. C. (2024) · Alzheimer s & Dementia Diagnosis Assessment & Disease Monitoring · doi:10.1002/dad2.12630 · Cited by 2 · see citing works
Loss of brainstem white matter predicts onset and motor neuron symptoms in C9orf72 expansion carriers: a GENFI study
Pérez‐Millan, A., Borrego‐Écija, S., Swieten, J. C. V., Jiskoot, L. C., Moreno, F., Laforce, R. J., Graff, C., Masellis, M., Tartaglia, M. C., Rowe, J. B., et al. (2022) · Journal of Neurology · doi:10.1007/s00415-022-11435-x · Cited by 2 · see citing works
Integrating a motor domain enhances disease severity scales in an FTD‐ALS spectrum cohort
Snyder, A. G., Samra, K., Wu, T., Russell, L. L., Farren, J., Crook, J., Haselhuhn, T., Porter, K., Szabo, M. P., Duckett, A., et al. (2025) · Alzheimer s & Dementia · doi:10.1002/alz.70786 · Cited by 1 · see citing works
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Social vulnerability shapes deep clinical phenotypes and brain health in aging and dementia across Latin America
Farombi, T., Azzi, T. D. S., Legaz, A., Aguillón, D., Ávila‐Funes, J. A., Behrens, M. I., Bruno, M., Cardona, J. F., Custodio, N., Delgado, C., et al. (2026) · Alzheimer s & Dementia · doi:10.1002/alz.71534 · Cited by 2 · see citing works
Theme 11 - Cognitive and Psychological Assessment and Support
(2023) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2023.2260203 · Cited by 1 · see citing works
Semantic knowledge of social norms in frontotemporal dementia patients with either predominant frontal or temporal lobe atrophy
Alsemari, A., Osher, J., & Cobb, R. E. (2024) · Applied Neuropsychology Adult · doi:10.1080/23279095.2024.2447041 · Cited by 1 · see citing works
Hyperorality in Frontotemporal Dementia: How Psychiatric and Neural Correlates Change During the Disease Course
Morrow, C. B., Onyike, C. U., Pantelyat, A. Y., Smith, G. S., Leoutsakos, J., Faria, A. V., Graff-Radford, N. R., Darby, R. R., Ghoshal, N., Staffaroni, A. M., et al. (2025) · Journal of Neuropsychiatry · doi:10.1176/appi.neuropsych.20240134 · Cited by 1 · see citing works
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Development and validation of the TabCAT-EXAMINER: tablet-based executive functioning factor score for research and clinical trialsPreprint
Sanderson‐Cimino, M. E., Possin, K. L., Mungas, D., Paolillo, E. W., Asken, B. M., Tsoy, E., Jarrott, S., Cobigo, Y., Saloner, R., Casaletto, K. B., et al. (2024) · medRxiv · doi:10.1101/2024.10.23.24315997 · Cited by 1 · see citing works
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Hyperorality in Frontotemporal Dementia: How Psychiatric and Neural Correlates Change Across the Disease CoursePreprint
Morrow, C. B., Onyike, C. U., Pantelyat, A. Y., Smith, G. S., Leoutsakos, J., Faria, A. V., Graff‐Radford, N. R., Darby, R. R., Ghoshal, N., Staffaroni, A. M., et al. (2024) · medRxiv · doi:10.1101/2024.02.19.24302699 · Cited by 1 · see citing works
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Language Impairment in the Genetic Forms of Behavioural Variant Frontotemporal DementiaPreprint
Samra, K., MacDougall, A. M., Bouzigues, A., Bocchetta, M., Cash, D. M., Greaves, C. V., Convery, R. S., Swieten, J. C. V., Jiskoot, L. C., Moreno, F., et al. (2022) · Research Square · doi:10.21203/rs.3.rs-1416934/v1
Symptomatic progression of frontotemporal dementia with the TARDBP I383V variant
Pressman, P., Carter, D. J., Ramos, E. M., Molden, J., Smith, K., Dino, F., McMillan, C. T., Irwin, D. J., Rascovsky, K., Ghoshal, N., et al. (2024) · Neurocase · doi:10.1080/13554794.2024.2354540
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Additional file 1 of An MRI-based strategy for differentiation of frontotemporal dementia and Alzheimer’s disease
Yu, Q., Mai, Y., Ruan, Y., Luo, Y., Zhao, L., Fang, W., Cao, Z., Li, Y., Liao, W., Xiao, S., et al. (2021) · Figshare · doi:10.6084/m9.figshare.13566108
Additional file 1 of Prodromal frontotemporal dementia: clinical features and predictors of progression
Benussi, A., Ashton, N. J., Karikari, T. K., Alberici, A., Saraceno, C., Ghidoni, R., Benussi, L., Zetterberg, H., Blennow, K., & Borroni, B. (2021) · Figshare · doi:10.6084/m9.figshare.17020881
Additional file 1 of Association of cortical and subcortical microstructure with disease severity: impact on cognitive decline and language impairments in frontotemporal lobar degeneration
Ding, W., Ren, P., Yi, L., Si, Y., Yang, F., Li, Z., Bao, H., Yan, S., Zhang, X., Li, S., et al. (2023) · Figshare · doi:10.6084/m9.figshare.22620649
Additional file 1 of The Revised Self-Monitoring Scale detects early impairment of social cognition in genetic frontotemporal dementia within the GENFI cohort
Franklin, H., Russell, L. L., Peakman, G., Greaves, C. V., Bocchetta, M., Nicholas, J. M., Poos, J. M., Convery, R. S., Cash, D. M., Swieten, J. C. V., et al. (2021) · Figshare · doi:10.6084/m9.figshare.14966272
Additional file 1 of Elevated CSF and plasma complement proteins in genetic frontotemporal dementia: results from the GENFI study
van der Ende, E. L., Heller, C., Sogorb‐Esteve, A., Swift, I. J., McFall, D. J., Peakman, G., Bouzigues, A., Poos, J. M., Jiskoot, L. C., Panman, J. L., et al. (2022) · Figshare · doi:10.6084/m9.figshare.20968112
Additional file 2 of An MRI-based strategy for differentiation of frontotemporal dementia and Alzheimer’s disease
Yu, Q., Mai, Y., Ruan, Y., Luo, Y., Zhao, L., Fang, W., Cao, Z., Li, Y., Liao, W., Xiao, S., et al. (2021) · Figshare · doi:10.6084/m9.figshare.13566111
Unveiling discrepancies in depression detection among persons with dementia: A comparative analysis of caregiver and self-report
Ortiz, B. L., Scheffler, A. W., Callahan, P. M., Shdo, S. M., Rijpma, M. G., Antoniou, R., Hirst, R. B., Brown, L. M., Rabinovici, G. D., Rosen, H. J., et al. (2026) · Journal of Alzheimer s Disease · doi:10.1177/13872877261422511
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Plasma Biomarkers and Clinical Outcomes in Early-Onset Dementia
Jang, H., Lee, S. I., Kim, H. J., Jung, N., Zetterberg, H., Blennow, K., Apostolova, L. G., Moon, S. Y., Kim, E., L on behalf of the CKD-FIX Study Investigators, et al. (2026) · JAMA Network Open · doi:10.1001/jamanetworkopen.2026.9687
Cortical free-water imaging in familial frontotemporal dementia associated with MAPT, GRN, and C9orf72 pathogenic variantsPreprint
Wu, W., Wu, H., Zhang, L., Dai, P., Ding, W., Tang, X., Zhang, T., & Shao, Z. (2026) · Research Square · doi:10.21203/rs.3.rs-9677636/v1
Impact of awareness of genetic status on cognitive, behavioral, and neuropsychiatric outcomes in genetic frontotemporal dementia
de Boer, L., Reichard, B., Poos, J. M., Houwer, J. F. H. D., Swartenbroekx, T., Aro, O. A., Rajicic, A., Brouwer, A., Dopper, E. G. P., Kaat, L. D., et al. (2026) · Journal of Neurology · doi:10.1007/s00415-026-13892-0
Diagnosis and management of frontotemporal dementia: a narrative review
Cenacchi, V., Lombardo, T., Palacino, F., Cattaruzza, T., Manganotti, P., & Benussi, A. (2026) · Neurodegenerative Disease Management · doi:10.1080/17582024.2026.2687481 · Cited by 1 · see citing works
The ILLUMINATE natural history study in colony-stimulating factor 1 receptor-related adult-onset leukoencephalopathy with axonal spheroids and pigmented glia
Lynch, D. S., Wade, C., Schöls, L. J., Hayer, S. N., Gelfand, J. M., Köhler, W., Bergner, C., de Souza, P. V. S., Finger, E. C., Wolf, N. I., et al. (2026) · Brain Communications · doi:10.1093/braincomms/fcag271
Blood-based multimodal biomarker models for differentiating early-onset Alzheimer’s disease from frontotemporal dementia: a longitudinal study of early-onset dementia and family members (LEAF) study
Kwon, H. S., Moon, S. Y., Hwang, M., Kim, H. J., Lee, S. M., Jung, N., Jang, H., Baek, J. M., Kim, M.-J., Han, M., et al. (2026) · Journal of Neurology · doi:10.1007/s00415-026-13996-7
The value of brain age as a transdiagnostic biomarker of neurodegenerationPreprint
Bos, L., van Nederpelt, D. R., Cole, J. H., Jasperse, B., Wink, A. M., Tranfa, M., Strijbis, E. M. M., Killestein, J., Uitdehaag, B. M. J., Barkhof, F., et al. (2026) · medRxiv · doi:10.64898/2026.07.21.26358569
TDP-43–Associated Neurodegenerative Disease Conceptualization and Integrated Staging
Benatar, M., Barmada, S., Jicha, G. A., Lagier‐Tourenne, C., Gregory, J., Al‐Chalabi, A., Boeve, B. F., Jack, C. R., Seeley, W. W., Maragakis, N. J., et al. (2026) · JAMA Neurology · doi:10.1001/jamaneurol.2026.2812
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Longitudinal functional network connectivity changes across the clinical stages of C9orf72 hexanucleotide repeat expansion carriers
Zhang, L., Häkkinen, S., Jung, Y., Mandelli, M. L., Leichter, D., Onyike, C. U., Rojas, J. C., Lombardi, J., Gorno‐Tempini, M. L., Yokoyama, J. S., et al. (2026) · Alzheimer s & Dementia · doi:10.1002/alz.71744
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Utility of the FTLD-CDR in the mild FTLD: Data from the ARTFL/LEFFTDS Consortium (P3.1-001)
Miyagawa, T., Brushaber, D. E., Dever, R., Fields, J. A., Forsberg, L. K., Gavrilova, R. H., Heuer, H. W., Knopman, D. S., Kornak, J., Kramer, J. H., et al. (2019) · Neurology · doi:10.1212/wnl.92.15_supplement.p3.1-001
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Utility of the global CDR® plus NACC FTLD rating and development of scoring rules: Data from the ARTFL/LEFFTDS Consortium
Miyagawa, T., Brushaber, D. E., Syrjanen, J. A., Kremers, W. K., Fields, J. A., Forsberg, L. K., Heuer, H. W., Knopman, D. S., Kornak, J., Boxer, A. L., et al. (2020) · Alzheimer s & Dementia · doi:10.1002/alz.12033 · Cited by 162 · see citing works
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Portuguese version of the CDR plus NACC FTLD: Validation studies
Lima, M., Tábuas‐Pereira, M., Durães, J., Faustino, P., Simões, M. R., Kukull, W. A., Knopman, D. S., & Santana, I. J. (2022) · Alzheimer s & Dementia Diagnosis Assessment & Disease Monitoring · doi:10.1002/dad2.12355 · Cited by 4 · see citing works
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Utility of the Japanese version of the Clinical Dementia Rating® plus National Alzheimer's Coordinating Centre Behaviour and Language Domains for sporadic cases of frontotemporal dementia in Japan
Taomoto, D., Sato, S., Kanemoto, H., Suzuki, M., Hirakawa, N., Takasaki, A., Akimoto, M., Satake, Y., Koizumi, F., Yoshiyama, K., et al. (2023) · Psychogeriatrics · doi:10.1111/psyg.13072 · Cited by 2 · see citing works
Self-citation
Utility of the Spanish version of the FTLD-modified CDR in the diagnosis and staging in frontotemporal lobar degeneration
Russo, G., Russo, M. J., Buyatti, D., Chrem, P., Bagnati, P., Suarez, M. F., Campos, J., Cohen, G., Amengual, A., Allegri, R., et al. (2014) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2014.06.024 · Cited by 19 · see citing works
Self-citation

Authors: Miyagawa, T., Brushaber, D., Syrjanen, J., Kremers, W., Fields, J., Forsberg, L. K., Heuer, H. W., Knopman, D., Kornak, J., Boxer, A., Rosen, H., Boeve, B., & on Behalf of the ARTFL/LEFFTDS Consortium
Affiliations: Mayo Clinic (US)
University of California, San Francisco (US)
Corresponding author: Boeve, Bradley
ORCID: Miyagawa, Toji
Brushaber, Danielle
Syrjanen, Jeremy
Kremers, Walter
Fields, Julie
Forsberg, Leah K.
Heuer, Hilary W.
Knopman, David
Kornak, John
Boxer, Adam
Boeve, Bradley
Venue: Alzheimer's & Dementia
Publisher: Wiley
Published: 2020-01
Volume: 16
Issue: 1
Pages: 79-90
ISSN: 1552-5260 (ISSN-L)
1552-5279
DOI: 10.1016/j.jalz.2019.05.013
PMC: PMC6949373
OpenAlex: W2970934901
Cited by: 92
References: 34
Citations by year: 2026: 12 · 2025: 10 · 2024: 14 · 2023: 13 · 2022: 16 · 2021: 21 · 2020: 4 · 2019: 2
FWCI: 8.56
Type: journal article
Language: en
Open access: green
europepmc.org
Licence: onlinelibrary.wiley.com/termsAndConditions#vor (vor, 5 d)
Locations: Alzheimer s & Dementia (publishedVersion)
PubMed (publishedVersion)
Europe PMC (PubMed Central) (acceptedVersion)
PubMed Central (acceptedVersion)
eScholarship (California Digital Library) (submittedVersion)
Funders: National Institutes of Health (U01 AG045390)
Centers for Disease Control and Prevention
Bristol-Myers Squibb
Avid Radiopharmaceuticals
Novartis
Pfizer
Wyeth
Huntington's Disease Society of America
New York State Department of Health (1510130358)
Biogen
AbbVie
Eli Lilly and Company
AstraZeneca
Roche
Genentech
Canadian Institutes of Health Research
Alzheimer Society of B.C.
BrightFocus Foundation
Alzheimer's Drug Discovery Foundation
Eisai
University of California, San Francisco
Parkinson Study Group
Parkinsonfonden
Michael J. Fox Foundation for Parkinson's Research
Janssen Pharmaceuticals
Merck
Alzheimer's Association
Topics: Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
Social Sciences › Psychology › Clinical Psychology › Historical and Modern Theater Studies
Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
Keywords: frontotemporal lobar degeneration
MeSH terms: Mental Status and Dementia Tests, Adult, Aged, Behavior, Cross-Sectional Studies, Female, Humans, Language, Middle Aged, Personality, Frontotemporal Lobar Degeneration, Frontotemporal Dementia

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Use of the CDR® plus NACC FTLD in mild FTLD: Data from the ARTFL/LEFFTDS consortium

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