Morris, J. Clinical presentation and course of Alzheimer disease. In Terry, R, Katzman, R, Bick, K, Sisodia, S, eds. Alzheimer Disease. Philadelphia, PA: Lippincott Williams & Wilkins, 1999; pp. 11–24.Google Scholar

Moller, HJ, Graeber, MB. The case described by Alois Alzheimer in 1911. Historical and conceptual perspectives based on the clinical record and neurohistological sections. Eur Arch Psychiatry Clin Neurosci. 1998;248(3):111–122.Google ScholarPubMed

Braak, H, Thal, DR, Ghebremedhin, E, Del Tredici, K. Stages of the pathologic process in Alzheimer disease: age categories from 1 to 100 years. J Neuropathol Exp Neurol. 2011 Nov;70(11):960–969.10.1097/NEN.0b013e318232a379CrossRefGoogle ScholarPubMed

Wimo, A, Ali, G-C, Guerchet, M, et al. World Alzheimer Report 2015: The global impact of dementia: an analysis of prevalence, incidence, cost and trends. September 21, 2015 [cited 2021 Feb 9]. Available from: www.alzint.org/resource/world-alzheimer-report-2015/Google Scholar

Wolters, FJ, Chibnik, LB, Waziry, R, et al. Twenty-seven-year time trends in dementia incidence in Europe and the United States: the Alzheimer Cohorts Consortium. Neurology. 2020 Aug 4;95(5):e519–e531.10.1212/WNL.0000000000010022CrossRefGoogle ScholarPubMed

Chen, Y, Wilson, L, Kornak, J, et al. The costs of dementia subtypes to California Medicare fee-for-service, 2015. Alzheimers Dement. 2019 Jul;15(7):899–906.10.1016/j.jalz.2019.03.015CrossRefGoogle ScholarPubMed

Snyder, HM, Asthana, S, Bain, L, et al. Sex biology contributions to vulnerability to Alzheimer’s disease: a think tank convened by the Women’s Alzheimer’s Research Initiative. Alzheimers Dement. 2016 Nov;12(11):1186–1196.10.1016/j.jalz.2016.08.004CrossRefGoogle ScholarPubMed

Davis, EJ, Lobach, I, Dubal, DB. Female XX sex chromosomes increase survival and extend lifespan in aging mice. Aging Cell. 2019 Feb;18(1):e12871.10.1111/acel.12871CrossRefGoogle ScholarPubMed

Cannon-Albright, LA, Foster, NL, Schliep, K, et al. Relative risk for Alzheimer disease based on complete family history. Neurology. 2019 Apr 9;92(15):e1745–e1753.10.1212/WNL.0000000000007231CrossRefGoogle ScholarPubMed

Peloso, GM, Beiser, AS, Satizabal, CL, et al. Cardiovascular health, genetic risk, and risk of dementia in the Framingham Heart Study. Neurology. 2020 Sep 8;95(10):e1341–e1350.10.1212/WNL.0000000000010306CrossRefGoogle ScholarPubMed

Martínez-Lapiscina, EH, Clavero, P, Toledo, E, et al. Mediterranean diet improves cognition: the PREDIMED-NAVARRA randomised trial. J Neurol Neurosurg Psychiatry. 2013 Dec;84(12):1318–1325.10.1136/jnnp-2012-304792CrossRefGoogle ScholarPubMed

Iaccarino, L, La Joie, R, Lesman-Segev, OH, et al. Association between ambient air pollution and amyloid positron emission tomography positivity in older adults with cognitive impairment. JAMA Neurol. 2021 Feb 1;78(2):197–207.10.1001/jamaneurol.2020.3962CrossRefGoogle ScholarPubMed

Livingston, G, Huntley, J, Sommerlad, A, et al. Dementia prevention, intervention, and care: 2020 report of the Lancet Commission. Lancet. 2020 Aug 8;396(10248):413–446.10.1016/S0140-6736(20)30367-6CrossRefGoogle ScholarPubMed

Arenaza-Urquijo, EM, Landeau, B, La Joie, R, et al. Relationships between years of education and gray matter volume, metabolism and functional connectivity in healthy elders. NeuroImage. 2013 Dec;83:450–745.10.1016/j.neuroimage.2013.06.053CrossRefGoogle ScholarPubMed

Busatto, GF, de Gobbi Porto, FH, Faria, D de P, et al. In vivo imaging evidence of poor cognitive resilience to Alzheimer’s disease pathology in subjects with very low cognitive reserve from a low-middle income environment. Alzheimers Dement Amst Neth. 2020;12(1):e12122.Google ScholarPubMed

Kornblith, E, Peltz, CB, Xia, F, et al. Sex, race, and risk of dementia diagnosis after traumatic brain injury among older veterans. Neurology. 2020 Sep 29;95(13):e1768–e1775.10.1212/WNL.0000000000010617CrossRefGoogle ScholarPubMed

Holth, J, Patel, T, Holtzman, DM. Sleep in Alzheimer’s disease – beyond amyloid. Neurobiol Sleep Circadian Rhythms. 2017 Jan;2:4–14.10.1016/j.nbscr.2016.08.002CrossRefGoogle ScholarPubMed

Winer, JR, Mander, BA, Kumar, S, et al. Sleep disturbance forecasts β-amyloid accumulation across subsequent years. Curr Biol CB. 2020 Nov 2;30(21):4291–4298.e3.10.1016/j.cub.2020.08.017CrossRefGoogle ScholarPubMed

Ehrenberg, AJ, Suemoto, CK, França Resende, E de P, et al. Neuropathologic correlates of psychiatric symptoms in Alzheimer’s disease. J Alzheimers Dis. 2018 Oct 16;66(1):115–126.10.3233/JAD-180688CrossRefGoogle ScholarPubMed

Jack, CR, Bennett, DA, Blennow, K, et al. NIA-AA Research framework: toward a biological definition of Alzheimer’s disease. Alzheimers Dement. 2018 Apr;14(4):535–562.10.1016/j.jalz.2018.02.018CrossRefGoogle Scholar

Jansen, IE, Savage, JE, Watanabe, K, et al. Genome-wide meta-analysis identifies new loci and functional pathways influencing Alzheimer’s disease risk. Nat Genet. 2019 Mar;51(3):404–413.10.1038/s41588-018-0311-9CrossRefGoogle ScholarPubMed

Bateman, RJ, Xiong, C, Benzinger, TLS, et al. Clinical and biomarker changes in dominantly inherited Alzheimer’s disease. N Engl J Med. 2012 Aug 30;367(9):795–804.10.1056/NEJMoa1202753CrossRefGoogle ScholarPubMed

Serrano-Pozo, A, Das, S, Hyman, BT. APOE and Alzheimer’s disease: advances in genetics, pathophysiology, and therapeutic approaches. Lancet Neurol. 2021 Jan;20(1):68–80.10.1016/S1474-4422(20)30412-9CrossRefGoogle ScholarPubMed

Snyder, HM, Bain, LJ, Brickman, AM, et al. Further understanding the connection between Alzheimer’s disease and Down syndrome. Alzheimers Dement. 2020 Jul;16(7):1065–1077.10.1002/alz.12112CrossRefGoogle ScholarPubMed

Fortea, J, Vilaplana, E, Carmona-Iragui, M, et al. Clinical and biomarker changes of Alzheimer’s disease in adults with Down syndrome: a cross-sectional study. Lancet. 2020 Jun 27;395(10242):1988–1997.10.1016/S0140-6736(20)30689-9CrossRefGoogle ScholarPubMed

McKhann, G, Drachman, D, Folstein, M, et al. Clinical diagnosis of Alzheimer’s disease: report of the NINCDS-ADRDA Work Group under the auspices of Department of Health and Human Services Task Force on Alzheimer’s Disease. Neurology. 1984 Jul;34(7):939–944.10.1212/WNL.34.7.939CrossRefGoogle ScholarPubMed

Aisen, PS, Cummings, J, Jack, CR, et al. On the path to 2025: understanding the Alzheimer’s disease continuum. Alzheimers Res Ther. 2017 Aug 9;9(1):60.10.1186/s13195-017-0283-5CrossRefGoogle ScholarPubMed

Dubois, B, Hampel, H, Feldman, HH, et al. Preclinical Alzheimer’s disease: definition, natural history, and diagnostic criteria. Alzheimers Dement. 2016 Mar;12(3):292–323.10.1016/j.jalz.2016.02.002CrossRefGoogle ScholarPubMed

McKhann, GM, Knopman, DS, Chertkow, H, et al. The diagnosis of dementia due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease. Alzheimers Dement. 2011 May;7(3):263–269.10.1016/j.jalz.2011.03.005CrossRefGoogle ScholarPubMed

Albert, MS, DeKosky, ST, Dickson, D, et al. The diagnosis of mild cognitive impairment due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease. Alzheimers Dement. 2011 May;7(3):270–279.10.1016/j.jalz.2011.03.008CrossRefGoogle ScholarPubMed

Jack, CR, Knopman, DS, Jagust, WJ, et al. Hypothetical model of dynamic biomarkers of the Alzheimer’s pathological cascade. Lancet Neurol. 2010 Jan;9(1):119–128.10.1016/S1474-4422(09)70299-6CrossRefGoogle ScholarPubMed

van der Linde, RM, Dening, T, Stephan, BCM, et al. Longitudinal course of behavioural and psychological symptoms of dementia: systematic review. Br J Psychiatry J Ment Sci. 2016 Nov;209(5):366–377.10.1192/bjp.bp.114.148403CrossRefGoogle ScholarPubMed

Theofilas, P, Dunlop, S, Heinsen, H, Grinberg, LT. Turning on the light within: subcortical nuclei of the isodentritic core and their role in Alzheimer’s disease pathogenesis. J Alzheimers Dis. 2015 May 7;46(1):17–34.10.3233/JAD-142682CrossRefGoogle ScholarPubMed

Jacobs, HIL, Riphagen, JM, Ramakers, IHGB, Verhey, FRJ. Alzheimer’s disease pathology: pathways between central norepinephrine activity, memory, and neuropsychiatric symptoms. Mol Psychiatry. 2019 May 28;26(3):897–906.10.1038/s41380-019-0437-xCrossRefGoogle ScholarPubMed

Morris, JC. The Clinical Dementia Rating (CDR): current version and scoring rules. Neurology. 1993 Nov;43(11):2412–2414.10.1212/WNL.43.11.2412-aCrossRefGoogle ScholarPubMed

Horváth, A, Szűcs, A, Barcs, G, Noebels, JL, Kamondi, A. Epileptic seizures in Alzheimer disease: a review. Alzheimer Dis Assoc Disord. 2016 Jun;30(2):186–192.10.1097/WAD.0000000000000134CrossRefGoogle ScholarPubMed

Jack, CR, Wiste, HJ, Weigand, SD, et al. Predicting future rates of tau accumulation on PET. Brain J Neurol. 2020 Oct 1;143(10):3136–3150.10.1093/brain/awaa248CrossRefGoogle ScholarPubMed

Smits, LL, Pijnenburg, YAL, van der Vlies, AE, et al. Early onset APOE E4-negative Alzheimer’s disease patients show faster cognitive decline on non-memory domains. Eur Neuropsychopharmacol. 2015 Jul;25(7):1010–1017.10.1016/j.euroneuro.2015.03.014CrossRefGoogle ScholarPubMed

Bolton, CJ, Tam, JW. Differential involvement of the locus coeruleus in early- and late-onset Alzheimer’s disease: a potential mechanism of clinical differences? J Geriatr Psychiatry Neurol. 2022 Sep;35(5):733–739.10.1177/08919887211044755CrossRefGoogle ScholarPubMed

Graff-Radford, J, Yong, KXX, Apostolova, LG, et al. New insights into atypical Alzheimer’s disease in the era of biomarkers. Lancet Neurol. 2021 Mar;20(3):222–234.10.1016/S1474-4422(20)30440-3CrossRefGoogle ScholarPubMed

Petersen, C, Nolan, AL, de Paula França Resende, E, et al. Alzheimer’s disease clinical variants show distinct regional patterns of neurofibrillary tangle accumulation. Acta Neuropathol (Berl). 2019 Oct;138(4):597–612.10.1007/s00401-019-02036-6CrossRefGoogle ScholarPubMed

Murray, ME, Graff-Radford, NR, Ross, OA, et al. Neuropathologically defined subtypes of Alzheimer’s disease with distinct clinical characteristics: a retrospective study. Lancet Neurol. 2011 Sep;10(9):785–796.10.1016/S1474-4422(11)70156-9CrossRefGoogle ScholarPubMed

Ossenkoppele, R, Cohn-Sheehy, BI, La Joie, R, et al. Atrophy patterns in early clinical stages across distinct phenotypes of Alzheimer’s disease. Hum Brain Mapp. 2015 Nov;36(11):4421–4437.10.1002/hbm.22927CrossRefGoogle ScholarPubMed

Miller, ZA, Rosenberg, L, Santos-Santos, MA, et al. Prevalence of mathematical and visuospatial learning disabilities in patients with posterior cortical atrophy. JAMA Neurol. 2018 Jun 1;75(6):728–737.10.1001/jamaneurol.2018.0395CrossRefGoogle ScholarPubMed

Spina, S, La Joie, R, Petersen, C, et al. Comorbid neuropathological diagnoses in early versus late-onset Alzheimer’s disease. Brain. 2021 Aug 17;144(7):2186–2198.10.1093/brain/awab099CrossRefGoogle ScholarPubMed

Hyman, BT, Phelps, CH, Beach, TG, et al. National Institute on Aging-Alzheimer’s Association guidelines for the neuropathologic assessment of Alzheimer’s disease. Alzheimers Dement. 2012 Jan;8(1):1–13.10.1016/j.jalz.2011.10.007CrossRefGoogle ScholarPubMed

Thal, DR, Rüb, U, Orantes, M, Braak, H. Phases of A beta-deposition in the human brain and its relevance for the development of AD. Neurology. 2002 Jun 25;58(12):1791–1800.10.1212/WNL.58.12.1791CrossRefGoogle ScholarPubMed

Jack, CR Jr, Knopman, DS, Jagust, WJ, et al. Tracking pathophysiological processes in Alzheimer’s disease: an updated hypothetical model of dynamic biomarkers. Lancet Neurol. 2013 Feb;12(2):207–216.10.1016/S1474-4422(12)70291-0CrossRefGoogle ScholarPubMed

Jack CR, Jr, Andrews, JS, Beach, TG, Buracchio, T, Dunn, B, Graf, A, Hansson, O, Ho, C, Jagust, W, McDade, E, Molinuevo, JL, Okonkwo, OC, Pani, L, Rafii, MS, Scheltens, P, Siemers, E, Snyder, HM, Sperling, R, Teunissen, CE, Carrillo, MC. Revised criteria for diagnosis and staging of Alzheimer’s disease: Alzheimer’s Association Workgroup. Alzheimers Dement. 2024 Aug;20(8):5143-5169. doi: 10.1002/alz.13859. Epub 2024 Jun 27. PMID: 38934362; PMCID: PMC11350039.CrossRefGoogle Scholar

Dubois, B, Villain, N, Schneider, L, Fox, N, Campbell, N, Galasko, D, Kivipelto, M, Jessen, F, Hanseeuw, B, Boada, M, Barkhof, F, Nordberg, A, Froelich, L, Waldemar, G, Frederiksen, KS, Padovani, A, Planche, V, Rowe, C, Bejanin, A, Ibanez, A, Cappa S, Caramelli P, Nitrini R, Allegri R, Slachevsky A, de Souza LC, Bozoki A, Widera E, Blennow K, Ritchie C, Agronin M, Lopera F, Delano-Wood L, Bombois S, Levy R, Thambisetty M, Georges J, Jones DT, Lavretsky H, Schott J, Gatchel J, Swantek S, Newhouse P, Feldman HH, Frisoni GB. Alzheimer Disease as a Clinical-Biological Construct-An International Working Group Recommendation. JAMA Neurol. 2024 Dec 1;81(12):1304-1311. doi: 10.1001/jamaneurol.2024.3770. PMID: 39483064; PMCID: PMC12010406.CrossRefGoogle Scholar

Elahi, FM, Miller, BL. A clinicopathological approach to the diagnosis of dementia. Nat Rev Neurol 2017;13:457–476. https://doi.org/10.1038/nrneurol.2017.96.CrossRefGoogle Scholar

Rascovsky, K, Hodges, JR, Knopman, D, et al. Sensitivity of revised diagnostic criteria for the behavioral variant of frontotemporal dementia. Brain 2011;134:2456–2477. https://doi.org/10.1093/brain/awr179.CrossRefGoogle Scholar

Gorno-Tempini, ML, Hillis, AE, Weintraub, S, et al. Classification of primary progressive aphasia and its variants. Neurology 2011;76:1006–1014. https://doi.org/10.1212/WNL.0b013e31821103e6.CrossRefGoogle ScholarPubMed

Neary, D, Snowden, JS, Gustafson, L, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998;51:1546–1554. https://doi.org/10.1212/WNL.51.6.1546.CrossRefGoogle ScholarPubMed

Harris, JM, Gall, C, Thompson, JC, et al. Sensitivity and specificity of FTDC criteria for behavioral variant frontotemporal dementia. Neurology 2013;80:1881–1887. https://doi.org/10.1212/WNL.0b013e318292a342.CrossRefGoogle ScholarPubMed

Coyle-Gilchrist, ITS, Dick, KM, Patterson, K, et al. Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes. Neurology 2016;86:1736–1743.10.1212/WNL.0000000000002638CrossRefGoogle ScholarPubMed

Logroscino, G, Piccininni, M, Binetti, G, et al. Incidence of frontotemporal lobar degeneration in Italy: the Salento-Brescia Registry study. Neurology 2019;92:e2355–e2363.10.1212/WNL.0000000000007498CrossRefGoogle ScholarPubMed

Onyike, CU, Diehl-Schmid, J. The epidemiology of frontotemporal dementia. Int Rev Psychiatry 2013;25:130–137.10.3109/09540261.2013.776523CrossRefGoogle ScholarPubMed

Marelli, C, Gutierrez, L-A, Menjot de Champfleur, N, et al. Late-onset behavioral variant of frontotemporal lobar degeneration versus Alzheimer’s disease: interest of cerebrospinal fluid biomarker ratios. Alzheimers Dement (Amst) 2015;1:371–379.10.1016/j.dadm.2015.06.004CrossRefGoogle ScholarPubMed

Woolley, JD, Khan, BK, Murthy, NK, Miller, BL, Rankin, KP. The diagnostic challenge of psychiatric symptoms in neurodegenerative disease: rates of and risk factors for prior psychiatric diagnosis in patients with early neurodegenerative disease. J Clin Psychiatry 2011;72:126–133. https://doi.org/10.4088/JCP.10m06382oli.CrossRefGoogle ScholarPubMed

Seo, SW, Thibodeau, M-P, Perry, DC, et al. Early vs late age at onset frontotemporal dementia and frontotemporal lobar degeneration. Neurology 2018;90:e1047–e1056.10.1212/WNL.0000000000005163CrossRefGoogle ScholarPubMed

Murley, AG, Coyle-Gilchrist, I, Rouse, MA, et al. Redefining the multidimensional clinical phenotypes of frontotemporal lobar degeneration syndromes. Brain 2020;143:1555–1571. https://doi.org/10.1093/brain/awaa097.CrossRefGoogle ScholarPubMed

Kansal, K, Mareddy, M, Sloane, KL, et al. Survival in frontotemporal dementia phenotypes: a meta-analysis. Dement Geriatr Cogn Disord 2016;41:109–122. https://doi.org/10.1159/000443205.CrossRefGoogle ScholarPubMed

Agarwal, S, Ahmed, RM, D’Mello, M, et al. Predictors of survival and progression in behavioural variant frontotemporal dementia. Eur J Neurol 2019;26:774–779. https://doi.org/10.1111/ene.13887.CrossRefGoogle ScholarPubMed

Garcin, B, Lillo, P, Hornberger, M, et al. Determinants of survival in behavioral variant frontotemporal dementia. Neurology 2009;73:1656–1661.10.1212/WNL.0b013e3181c1dee7CrossRefGoogle ScholarPubMed

Caswell, C, McMillan, CT, Xie, SX, et al. Genetic predictors of survival in behavioral variant frontotemporal degeneration. Neurology 2019;93:e1707–e1714. https://doi.org/10.1212/WNL.0000000000008387.CrossRefGoogle ScholarPubMed

Mauvais-Jarvis, F, Bairey Merz, N, Barnes, PJ, et al. Sex and gender: modifiers of health, disease, and medicine. Lancet 2020;396:565–582. https://doi.org/10.1016/S0140-6736(20)31561-0.CrossRefGoogle ScholarPubMed

Perry, DC, Brown, JA, Possin, KL, et al. Clinicopathological correlations in behavioural variant frontotemporal dementia. Brain 2017;140:3329–3345. https://doi.org/10.1093/brain/awx254.CrossRefGoogle ScholarPubMed

O’Connor, CM, Landin-Romero, R, Clemson, L, et al. Behavioral-variant frontotemporal dementia: distinct phenotypes with unique functional profiles. Neurology 2017;89:570–577. https://doi.org/10.1212/WNL.0000000000004215.CrossRefGoogle ScholarPubMed

Illan-Gala, I, Montal, V, Borrego-Ecija, S, et al. Cortical microstructure in the behavioural variant of frontotemporal dementia: looking beyond atrophy. Brain 2019;142:1121–1133. https://doi.org/10.1093/brain/awz031.CrossRefGoogle ScholarPubMed

Spinelli, EG, Mandelli, ML, Miller, ZA, et al. Typical and atypical pathology in primary progressive aphasia variants. Ann Neurol 2017;81:430–443.10.1002/ana.24885CrossRefGoogle ScholarPubMed

Illán-Gala, I, Casaletto, KB, Borrego-Écija, S, et al. Sex differences in the behavioral variant of frontotemporal dementia: a new window to executive and behavioral reserve. Alzheimers Dement 2021 Aug;17(8):1329–1341. https://doi.org/1002/alz.12299.CrossRefGoogle ScholarPubMed

Miller, BL. Frontotemporal Dementia. Oxford University Press, 2014.10.1093/med/9780195380491.001.0001CrossRefGoogle ScholarPubMed

Karageorgiou, E, Miller, BL. Frontotemporal lobar degeneration: a clinical approach. Semin Neurol 2014;34:189–201.10.1055/s-0034-1381735CrossRefGoogle ScholarPubMed

Miller, BL, Seeley, WW, Mychack, P, et al. Neuroanatomy of the self: evidence from patients with frontotemporal dementia. Neurology 2001;57:817–821. https://doi.org/10.1212/wnl.57.5.817.CrossRefGoogle ScholarPubMed

Ducharme, S, Dols, A, Laforce, R, et al. Recommendations to distinguish behavioural variant frontotemporal dementia from psychiatric disorders. Brain 2020;143:1632–1650. https://doi.org/10.1093/brain/awaa018.CrossRefGoogle ScholarPubMed

Perry, DC, Datta, S, Sturm, VE et al. Reward deficits in behavioural variant frontotemporal dementia include insensitivity to negative stimuli. Brain 2017;140:3346–3356. https://doi.org/10.1093/brain/awx259.CrossRefGoogle ScholarPubMed

Liljegren, M, Naasan, G, Temlett, J, et al. Criminal behavior in frontotemporal dementia and Alzheimer disease. JAMA Neurol 2015;72:295–300.10.1001/jamaneurol.2014.3781CrossRefGoogle ScholarPubMed

Liljegren, M, Landqvist Waldö, M, Frizell Santillo, A, et al. Association of neuropathologically confirmed frontotemporal dementia and Alzheimer disease with criminal and socially inappropriate behavior in a Swedish cohort. JAMA Netw Open 2019;2:e190261. https://doi.org/10.1001/jamanetworkopen.2019.0261.CrossRefGoogle Scholar

Lansdall, CJ, Coyle-Gilchrist, ITS, Vázquez Rodríguez, P, et al. Prognostic importance of apathy in syndromes associated with frontotemporal lobar degeneration. Neurology 2019;92:e1547–e1557. https://doi.org/10.1212/WNL.0000000000007249.CrossRefGoogle ScholarPubMed

Perry, DC, Sturm, VE, Seeley, WW, et al. Anatomical correlates of reward-seeking behaviours in behavioural variant frontotemporal dementia. Brain 2014;137:1621–1626.10.1093/brain/awu075CrossRefGoogle ScholarPubMed

Piguet, O, Petersén, A, Yin Ka Lam, B, et al. Eating and hypothalamus changes in behavioral-variant frontotemporal dementia. Ann Neurol 2011;69:312–319.10.1002/ana.22244CrossRefGoogle ScholarPubMed

Ahmed, RM, Ke, YD, Vucic, S, et al. Physiological changes in neurodegeneration – mechanistic insights and clinical utility. Nat Rev Neurol 2018;14:259–271. https://doi.org/10.1038/nrneurol.2018.23.CrossRefGoogle ScholarPubMed

Ranasinghe, KG, Rankin, KP, Lobach, IV, et al. Cognition and neuropsychiatry in behavioral variant frontotemporal dementia by disease stage. Neurology 2016;86:6006–10. https://doi.org/10.1212/WNL.0000000000002373.CrossRefGoogle ScholarPubMed

Henry, JD, von Hippel, W, Molenberghs, P, Lee, T, Sachdev, PS. Clinical assessment of social cognitive function in neurological disorders. Nat Rev Neurol 2016;12:28–39.10.1038/nrneurol.2015.229CrossRefGoogle ScholarPubMed

Rowe, JB. Parkinsonism in frontotemporal dementias. Int Rev Neurobiol 2019;149:249–275. https://doi.org/10.1016/bs.irn.2019.10.012.Google ScholarPubMed

Baborie, A, Griffiths, TD, Jaros, E, et al. Frontotemporal dementia in elderly individuals. Arch Neurol 2012;69:1052–1060.10.1001/archneurol.2011.3323CrossRefGoogle ScholarPubMed

Illán-Gala, I, Falgàs, N, Friedberg, A, et al. Diagnostic utility of measuring cerebral atrophy in the behavioral variant of frontotemporal dementia and association with clinical deterioration. JAMA Netw Open. 2021;4(3):e211290. https://doi.org/10.1001/jamanetworkopen.2021.1290CrossRefGoogle Scholar

Harper, L, Fumagalli, G G, Barkhof, F, et al. MRI visual rating scales in the diagnosis of dementia: evaluation in 184 post-mortem confirmed cases. Brain 2016;139:1211–1225, https://doi.org/10.1093/brain/aww005CrossRefGoogle ScholarPubMed

McCarthy, J, Collins, DL, Ducharme, S. Morphometric MRI as a diagnostic biomarker of frontotemporal dementia: a systematic review to determine clinical applicability. NeuroImage Clin 2018;20:685–696. https://doi.org/10.1016/j.nicl.2018.08.028.CrossRefGoogle ScholarPubMed

Chételat, G, Arbizu, J, Barthel, H, et al. Amyloid-PET and 18F-FDG-PET in the diagnostic investigation of Alzheimer’s disease and other dementias. Lancet Neurol 2020;19:951–962. https://doi.org/10.1016/S1474-4422(20)30314-8.CrossRefGoogle Scholar

Zimmer, ER, Parent, MJ, Souza, DG, et al. [18F]FDG PET signal is driven by astroglial glutamate transport. Nat Neurosci 2017;20:393–395. https://doi.org/10.1038/nn.4492.CrossRefGoogle ScholarPubMed

Ranasinghe, KG, Rankin, KP, Pressman, PS, et al. Distinct subtypes of behavioral variant frontotemporal dementia based on patterns of network degeneration. JAMA Neurol 2016;73:1078. https://doi.org/10.1001/jamaneurol.2016.2016.CrossRefGoogle ScholarPubMed

Kertesz, A, McMonagle, P, Blair, M, Davidson, W, Munoz, DG. The evolution and pathology of frontotemporal dementia. Brain 2005;128:1996–2005. https://doi.org/10.1093/brain/awh598.CrossRefGoogle ScholarPubMed

Devenney, E, Swinn, T, Mioshi, E, et al. The behavioural variant frontotemporal dementia phenocopy syndrome is a distinct entity – evidence from a longitudinal study. BMC Neurol 2018;18:56. https://doi.org/10.1186/s12883-018-1060-1.CrossRefGoogle ScholarPubMed

Ossenkoppele, R, Pijnenburg, YAL, Perry, DC, et al. The behavioural/dysexecutive variant of Alzheimer’s disease: clinical, neuroimaging and pathological features. Brain 2015;138:2732–2749. https://doi.org/10.1093/brain/awv191.CrossRefGoogle ScholarPubMed

Meeter, LH, Kaat, LD, Rohrer, JD, van Swieten, JC. Imaging and fluid biomarkers in frontotemporal dementia. Nat Rev Neurol 2017;13:406–419. https://doi.org/10.1038/nrneurol.2017.75.CrossRefGoogle ScholarPubMed

Leuzy, A, Smith, R, Ossenkoppele, R, et al. Diagnostic performance of RO948 F 18 Tau positron emission tomography in the differentiation of Alzheimer disease from other neurodegenerative disorders. JAMA Neurol 2020;77:955–965. https://doi.org/10.1001/jamaneurol.2020.0989.CrossRefGoogle ScholarPubMed

Palmqvist, S, Janelidze, S, Quiroz, YT, et al. Discriminative accuracy of plasma phospho-tau217 for Alzheimer disease vs other neurodegenerative disorders. JAMA 2020;324:772. https://doi.org/10.1001/jama.2020.12134.CrossRefGoogle ScholarPubMed

Illán-Gala, I, Lleo, A, Karydas, A, et al. Plasma tau and neurofilament light in frontotemporal lobar degeneration and Alzheimer’s disease. Neurology 2020;96:e671–e683. https://doi.org/10.1212/WNL.0000000000011226.Google Scholar

van der Ende, EL, Meeter, LH, Poos, JM, et al. Serum neurofilament light chain in genetic frontotemporal dementia: a longitudinal, multicentre cohort study. Lancet Neurol 2019;18:1103–1111. https://doi.org/10.1016/S1474-4422(19)30354-0.CrossRefGoogle ScholarPubMed

Al Shweiki, MR, Steinacker, P, Oeckl, P, et al. Neurofilament light chain as a blood biomarker to differentiate psychiatric disorders from behavioural variant frontotemporal dementia. J Psychiatr Res 2019;113:137–140. https://doi.org/10.1016/j.jpsychires.2019.03.019.CrossRefGoogle ScholarPubMed

Whitwell, JL, Przybelski, SA, Weigand, SD, et al. Distinct anatomical subtypes of the behavioural variant of frontotemporal dementia: a cluster analysis study. Brain 2009;132:2932–2946. https://doi.org/10.1093/brain/awp232.CrossRefGoogle ScholarPubMed

Seeley, WW. Behavioral variant frontotemporal dementia. Continuum (Minneap Minn) 2019;25:76–100.10.1212/CON.0000000000000698CrossRefGoogle ScholarPubMed

Ulugut Erkoyun, H, Groot, C, Heilbron, R, et al. A clinical-radiological framework of the right temporal variant of frontotemporal dementia. Brain 2020;143:2831–2843. https://doi.org/10.1093/brain/awaa225.CrossRefGoogle ScholarPubMed

Illán-Gala, I, Montal, V, Pegueroles, J, et al. Cortical microstructure in the amyotrophic lateral sclerosis–frontotemporal dementia continuum. Neurology 2020;95:e2565–e2576. https://doi.org/10.1212/WNL.0000000000010727.CrossRefGoogle ScholarPubMed

Cortes-Vicente, E, Turon-Sans, J, Gelpi, E, et al. Distinct clinical features and outcomes in motor neuron disease associated with behavioural variant frontotemporal dementia. Dement Geriatr Cogn Disord 2018;45:220–231. https://doi.org/10.1159/000488528.CrossRefGoogle ScholarPubMed

Brown, RH, Al-Chalabi, A. Amyotrophic lateral sclerosis. N Engl J Med 2017;377:162–172. https://doi.org/10.1056/NEJMra1603471.CrossRefGoogle ScholarPubMed

Goldstein, LH, Abrahams, S. Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment. Lancet Neurol 2013;12:368–380. https://doi.org/10.1016/S1474-4422(13)70026-7.CrossRefGoogle ScholarPubMed

Höglinger, GU, Respondek, G, Stamelou, M, et al. Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria. Mov Disord 2017;32:853–864.10.1002/mds.26987CrossRefGoogle ScholarPubMed

Walker, Z, Possin, KL, Boeve, BF, Aarsland, D. Lewy body dementias. Lancet 2015;386:1683–1697.10.1016/S0140-6736(15)00462-6CrossRefGoogle ScholarPubMed

Bologna, M, Paparella, G, Fasano, A, Hallett, M, Berardelli, A. Evolving concepts on bradykinesia. Brain 2020;143:727–750. https://doi.org/10.1093/brain/awz344.CrossRefGoogle ScholarPubMed

Lesman-Segev, OH, Edwards, L, Rabinovici, GD. Chronic traumatic encephalopathy: a comparison with Alzheimer’s disease and frontotemporal dementia. Semin Neurol 2020;40:394–410. https://doi.org/10.1055/s-0040-1715134.Google ScholarPubMed

Wicklund, MR, Mokri, B, Drubach, DA, et al. Frontotemporal brain sagging syndrome: an SIH-like presentation mimicking FTD. Neurology 2011;76:1377–1382. https://doi.org/10.1212/WNL.0b013e3182166e42.CrossRefGoogle ScholarPubMed

Ducharme, S, Price, BH, Larvie, M, Dougherty, DD, Dickerson, BC. Clinical approach to the differential diagnosis between behavioral variant frontotemporal dementia and primary psychiatric disorders. Am J Psychiatry 2015;172:827–837.10.1176/appi.ajp.2015.14101248CrossRefGoogle Scholar

Ducharme, S, Pearl-Dowler, L, Gossink, F, et al. the frontotemporal dementia versus primary psychiatric disorder (FTD versus PPD) checklist: a bedside clinical tool to identify behavioral variant FTD in patients with late-onset behavioral changes. J Alzheimers Dis 2019;67:113–124. https://doi.org/10.3233/JAD-180839.CrossRefGoogle ScholarPubMed

Mackenzie, IRA, Neumann, M. Molecular neuropathology of frontotemporal dementia: insights into disease mechanisms from postmortem studies. J Neurochem 2016;138 Suppl 1:54–70.10.1111/jnc.13588CrossRefGoogle ScholarPubMed

Mioshi, E, Kipps, CM, Dawson, K, et al. Activities of daily living in frontotemporal dementia and Alzheimer disease. Neurology 2007;68:2077–2084.10.1212/01.wnl.0000264897.13722.53CrossRefGoogle ScholarPubMed

Mioshi, E, Hsieh, S, Savage, S, Hornberger, M, Hodges, JR. Clinical staging and disease progression in frontotemporal dementia. Neurology 2010;74:1591–1597.10.1212/WNL.0b013e3181e04070CrossRefGoogle ScholarPubMed

Miyagawa, T, Brushaber, D, Syrjanen, J, et al. Utility of the global CDR® plus NACC FTLD rating and development of scoring rules: data from the ARTFL/LEFFTDS Consortium. Alzheimers Dement 2020;16:106–117. https://doi.org/10.1002/alz.12033.CrossRefGoogle ScholarPubMed

Rohrer, JD, Nicholas, JM, Cash, DM, et al. Presymptomatic cognitive and neuroanatomical changes in genetic frontotemporal dementia in the Genetic Frontotemporal dementia Initiative (GENFI) study: a cross-sectional analysis. Lancet Neurol 2015;14:253–262.10.1016/S1474-4422(14)70324-2CrossRefGoogle ScholarPubMed

Rosen, HJ, Boeve, BF, Boxer, AL. Tracking disease progression in familial and sporadic frontotemporal lobar degeneration: recent findings from ARTFL and LEFFTDS. Alzheimers Dement 2020;16:71–78. https://doi.org/10.1002/alz.12004.CrossRefGoogle ScholarPubMed

Staffaroni, AM, Ljubenkov, PA, Kornak, J, et al. Longitudinal multimodal imaging and clinical endpoints for frontotemporal dementia clinical trials. Brain 2019;142:443–459. https://doi.org/10.1093/brain/awy319.CrossRefGoogle ScholarPubMed

Damasio, A. R., & Geschwind, N. (1984). The neural basis of language. Annu Rev Neurosci, 7, 127–147. https://doi.org/10.1146/annurev.ne.07.030184.001015CrossRefGoogle ScholarPubMed

Dronkers, N. F., Plaisant, O., Iba-Zizen, M. T., et al. (2007). Paul Broca’s historic cases: high resolution MR imaging of the brains of Leborgne and Lelong. Brain, 130(Pt 5), Article Pt 5. https://doi.org/10.1093/brain/awm042CrossRefGoogle ScholarPubMed

Mesulam, M.-M., Thompson, C. K., Weintraub, S., et al. (2015). The Wernicke conundrum and the anatomy of language comprehension in primary progressive aphasia. Brain, 138(8), 2423–2437. https://doi.org/10.1093/brain/awv154CrossRefGoogle ScholarPubMed

Harrison, D. W. (2015). Functional cerebral systems theory: an integrated brain. In Harrison, D. W., ed. Brain Asymmetry and Neural Systems: Foundations in Clinical Neuroscience and Neuropsychology. Springer International; pp. 53–58. https://doi.org/10.1007/978-3-319-13069-9_6CrossRefGoogle Scholar

Hickok, G. (2022). The dual stream model of speech and language processing. In Hillis, A. E., Fridriksson, J., eds. Handbook of Clinical Neurology (vol. 185). Elsevier; pp. 57–69. https://doi.org/10.1016/B978-0-12-823384-9.00003-7Google Scholar

Mesulam, M. M. (1982). Slowly progressive aphasia without generalized dementia. Ann Neurol, 11(6), Article 6.10.1002/ana.410110607CrossRefGoogle ScholarPubMed

Pick, A. (1892). Uber die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Medizinische Wochenschrift, 17, 165–167.Google Scholar

Déjerine, J. J., & Sérieux, P. (1897). Un cas de surdité verbale pure, terminée par aphasie sensorielle, suivi d’autopsie. Paris.Google Scholar

Sasanuma, S., & Monoi, H. (1975). The syndrome of Gogi (word meaning) aphasia. Selective impairment of kanji processing. Neurology, 25(7), 627–632. https://doi.org/10.1212/wnl.25.7.627CrossRefGoogle ScholarPubMed

Warrington, E. K. (1975). The selective impairment of semantic memory. Quart J Exp Psychology, 27, 635–657.10.1080/14640747508400525CrossRefGoogle ScholarPubMed

Snowden, J. S., Goulding, P. J., & Neary, D. (1989). Semantic dementia: a form of circumscribed cerebral atrophy. Behavl Neurol, 2, 167–182.10.1155/1989/124043CrossRefGoogle Scholar

Grossman, M., Mickanin, J., Onishi, K., et al. (1996). Progressive non-fluent aphasia: language, cognitive and PET measures contrasted with probable Alzheimer’s disease. J Cog Neurosci, 8, 135–154.10.1162/jocn.1996.8.2.135CrossRefGoogle Scholar

Neary, D., Snowden, J. S., Gustafson, L., et al. (1998). Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria see comments. Neurology, 51(6), Article 6.10.1212/WNL.51.6.1546CrossRefGoogle Scholar

Gorno-Tempini, M. L., Dronkers, N. F., Rankin, K. P., et al. (2004). Cognition and anatomy in three variants of primary progressive aphasia. Ann Neurol, 55(3), Article 3.10.1002/ana.10825CrossRefGoogle ScholarPubMed

Gorno-Tempini, M. L., Hillis, A. E., Weintraub, S., et al. (2011). Classification of primary progressive aphasia and its variants. Neurology, 76(11), 1006–1014. https://doi.org/10.1212/WNL.0b013e31821103e6CrossRefGoogle ScholarPubMed

Mesulam, M. M. (2001). Primary progressive aphasia. Ann Neurol, 49(4), Article 4.10.1002/ana.91CrossRefGoogle ScholarPubMed

Tastevin, M., Lavoie, M., de la Sablonnière, J., et al. (2021). Survival in the three common variants of primary progressive aphasia: a retrospective study in a tertiary memory clinic. Brain Sci, 11(9), Article 9. https://doi.org/10.3390/brainsci11091113CrossRefGoogle Scholar

Coyle-Gilchrist, I. T. S., Dick, K. M., Patterson, K., et al. (2016). Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes. Neurology, 86(18), 1736–1743. https://doi.org/10.1212/WNL.0000000000002638CrossRefGoogle ScholarPubMed

Hendriks, S., Peetoom, K., Bakker, C., et al. (2021). Global prevalence of young-onset dementia: a systematic review and meta-analysis. JAMA Neurol, 78(9), 1080–1090. https://doi.org/10.1001/jamaneurol.2021.2161CrossRefGoogle ScholarPubMed

Rohrer, J. D., Guerreiro, R., Vandrovcova, J., et al. (2009). The heritability and genetics of frontotemporal lobar degeneration. Neurology, 73(18), Article 18.10.1212/WNL.0b013e3181bf997aCrossRefGoogle ScholarPubMed

Hodges, J. R., Patterson, K., Oxbury, S., et al. (1992). Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy. Brain, 115(Pt 6), Article Pt 6.Google ScholarPubMed

Seeley, W. W., Bauer, A. M., Miller, B. L., et al. (2005). The natural history of temporal variant frontotemporal dementia. Neurology, 64(8), Article 8.10.1212/01.WNL.0000158425.46019.5CCrossRefGoogle ScholarPubMed

Younes, K., Borghesani, V., Montembeault, M., et al. (2022). Right temporal degeneration and socioemotional semantics: semantic behavioural variant frontotemporal dementia. Brain, 145(11), 4080–4096. https://doi.org/10.1093/brain/awac217CrossRefGoogle ScholarPubMed

Kertesz, A., Jesso, S., Harciarek, M., et al. (2010). What is semantic dementia?: a cohort study of diagnostic features and clinical boundaries. Arch Neurol, 67(4), 483–489. https://doi.org/10.1001/archneurol.2010.55CrossRefGoogle ScholarPubMed

Van den Bosch, A., Content, A., Daelemans, W., et al. (1994). Measuring the complexity of writing systems*. J Quant Linguist, 1(3), 178–188. https://doi.org/10.1080/09296179408590015CrossRefGoogle Scholar

Patterson, K., & Hodges, J. R. (1992). Deterioration of word meaning: implications for reading. Neuropsychologia, 30(12), Article 12.10.1016/0028-3932(92)90096-5CrossRefGoogle ScholarPubMed

Irish, M., Bunk, S., Tu, S., et al. (2016). Preservation of episodic memory in semantic dementia: the importance of regions beyond the medial temporal lobes. Neuropsychologia, 81, 50–60. https://doi.org/10.1016/j.neuropsychologia.2015.12.005CrossRefGoogle ScholarPubMed

Chan, D., Fox, N. C., Scahill, R. I., et al. (2001). Patterns of temporal lobe atrophy in semantic dementia and Alzheimer’s disease. Ann Neurol, 49(4), 433–442.10.1002/ana.92CrossRefGoogle ScholarPubMed

Galton, C. J., Patterson, K., Xuereb, J. H., et al. (2000). Atypical and typical presentations of Alzheimer’s disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases. Brain, 123(Pt 3), 484–498.10.1093/brain/123.3.484CrossRefGoogle ScholarPubMed

Rosen, H. J., Gorno-Tempini, M. L., Goldman, W. P., et al. (2002). Patterns of brain atrophy in frontotemporal dementia and semantic dementia. Neurology, 58(2), Article 2.10.1212/WNL.58.2.198CrossRefGoogle ScholarPubMed

Miller, Z. A., Mandelli, M. L., Rankin, K. P., et al. (2013). Handedness and language learning disability differentially distribute in progressive aphasia variants. Brain, 136(11), 3461–3473. https://doi.org/10.1093/brain/awt242CrossRefGoogle ScholarPubMed

Miller, Z. A., Hinkley, L. B., Herman, A., et al. (2015). Anomalous functional language lateralization in semantic variant PPA. Neurology, 84(2), 204–206. https://doi.org/10.1212/WNL.0000000000001131CrossRefGoogle ScholarPubMed

Miller, Z., Hinkley, L., Bogley, R., et al. (2022). Anomalous language lateralization in semantic dementia: a neurodevelopmental hypothesis (P8-3.003). Neurology, 98(18 Suppl). https://n.neurology.org/content/98/18_Supplement/372710.1212/WNL.98.18_supplement.3727CrossRefGoogle Scholar

Bocchetta, M., Iglesias Espinosa, M. del M., Lashley, T., et al. (2020). In vivo staging of frontotemporal lobar degeneration TDP-43 type C pathology. Alzheimers Res Ther, 12(1), 34. https://doi.org/10.1186/s13195-020-00600-xCrossRefGoogle ScholarPubMed

Patterson, K., Nestor, P. J., & Rogers, T. T. (2007). Where do you know what you know? The representation of semantic knowledge in the human brain. Nat Rev Neurosci, 8(12), Article 12. https://doi.org/10.1038/nrn2277CrossRefGoogle Scholar

Migliaccio, R., Boutet, C., Valabregue, R., et al. (2016). The brain network of naming: a lesson from primary progressive aphasia. PLoS One, 11(2), e0148707. https://doi.org/10.1371/journal.pone.0148707CrossRefGoogle ScholarPubMed

Bocchetta, M., Iglesias, J. E., Russell, L. L., et al. (2019). Segmentation of medial temporal subregions reveals early right-sided involvement in semantic variant PPA. Alzheimers Res The, 11(1), 41. https://doi.org/10.1186/s13195-019-0489-9CrossRefGoogle ScholarPubMed

Thompson, S. A., Patterson, K., & Hodges, J. R. (2003). Left/right asymmetry of atrophy in semantic dementia: behavioral-cognitive implications. Neurology, 61(9), Article 9.10.1212/01.WNL.0000091868.28557.B8CrossRefGoogle ScholarPubMed

Henry, M. L., Wilson, S. M., Ogar, J. M., et al. (2014). Neuropsychological, behavioral, and anatomical evolution in right temporal variant frontotemporal dementia: a longitudinal and post-mortem single case analysis. Neurocase, 20(1), 10.1080/13554794.2012.732089. https://doi.org/10.1080/13554794.2012.732089CrossRefGoogle ScholarPubMed

Guo, C. C., Gorno-Tempini, M. L., Gesierich, B., et al. (2013). Anterior temporal lobe degeneration produces widespread network-driven dysfunction. Brain, 136(Pt 10), Article Pt 10. https://doi.org/10.1093/brain/awt222CrossRefGoogle ScholarPubMed

Schwab, S., Afyouni, S., Chen, Y., et al. (2020). Functional connectivity alterations of the temporal lobe and hippocampus in semantic dementia and Alzheimer’s disease. J Alzheimers Dis, 76(4), 1461–1475. https://doi.org/10.3233/JAD-191113CrossRefGoogle ScholarPubMed

Goldman, J. S., Farmer, J. M., Van Deerlin, V. M., et al. (2004). Frontotemporal dementia: genetics and genetic counseling dilemmas. Neurologist, 10(5), Article 5.10.1097/01.nrl.0000138735.48533.26CrossRefGoogle ScholarPubMed

Pickering-Brown, S. M., Rollinson, S., Du Plessis, D., et al. (2008). Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: comparison with patients with MAPT and no known mutations. Brain, 131(Pt 3), Article Pt 3.10.1093/brain/awm331CrossRefGoogle ScholarPubMed

Benajiba, L., Le Ber, I., Camuzat, A., et al. (2009). TARDBP mutations in motoneuron disease with frontotemporal lobar degeneration. Ann Neurol, 65(4), 470–473. https://doi.org/10.1002/ana.21612CrossRefGoogle ScholarPubMed

Caroppo, P., Camuzat, A., De Septenville, A., et al. (2015). Semantic and nonfluent aphasic variants, secondarily associated with amyotrophic lateral sclerosis, are predominant frontotemporal lobar degeneration phenotypes in TBK1 carriers. Alzheimers Dement, 1(4), 481–486. https://doi.org/10.1016/j.dadm.2015.10.002Google ScholarPubMed

Snowden, J. S., Rollinson, S., Thompson, J. C., et al. (2012). Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations. Brain, 135(3), 693–708. https://doi.org/10.1093/brain/awr355CrossRefGoogle ScholarPubMed

Borroni, B., Ferrari, F., Galimberti, D., et al. (2014). Heterozygous TREM2 mutations in frontotemporal dementia. Neurobiol Aging, 35(4), 934.e7–934.e10. https://doi.org/10.1016/j.neurobiolaging.2013.09.017CrossRefGoogle ScholarPubMed

Van Schoor, E., Vandenbulcke, M., Bercier, V., et al. (2022). frontotemporal lobar degeneration case with an N-Terminal Tuba4a mutation exhibits reduced TUBA4A levels in the brain and TDP-43 pathology. Biomolecules, 12(3), 440. https://doi.org/10.3390/biom12030440CrossRefGoogle ScholarPubMed

Pellerin, D., Ellezam, B., Korathanakhun, P., et al. (2020). multisystem proteinopathy associated with a VCP G156S mutation in a French Canadian family. Can J Neurol Sci, 47(3), 412–415. https://doi.org/10.1017/cjn.2020.25CrossRefGoogle Scholar

Bergeron, D., Gorno-Tempini, M. L., Rabinovici, G. D., et al. (2018). Prevalence of amyloid-β pathology in distinct variants of primary progressive aphasia. Ann Neurol, 84(5), Article 5. https://doi.org/10.1002/ana.25333CrossRefGoogle ScholarPubMed

Spinelli, E. G., Mandelli, M. L., Miller, Z. A., et al. (2017). Typical and atypical pathology in primary progressive aphasia variants: pathology in PPA Variants. Ann Neurol, 81(3), Article 3. https://doi.org/10.1002/ana.24885CrossRefGoogle Scholar

Diggs, R. Neylan, K., Allen, I. E. et al. (2019). Getting to the root cause: an increased prevalence of stem careers in frontotemporal dementia. Poster presented at the Alzheimer’s Association International Conference, 2019, Los Angeles, CA. https://alz-journals.onlinelibrary.wiley.com/doi/full/10.1016/j.jalz.2019.06.4088CrossRefGoogle Scholar

Miller, Z. A., Rankin, K. P., Graff-Radford, N. R., et al. (2013). TDP-43 frontotemporal lobar degeneration and autoimmune disease. J Neurol Neurosurg Psychiatry, 84(9), 956–962. https://doi.org/10.1136/jnnp-2012-304644CrossRefGoogle ScholarPubMed

Miller, Z. A., Sturm, V. E., Camsari, G. B., et al. (2016). Increased prevalence of autoimmune disease within C9 and FTD/MND cohorts. Neurol Neuroimmunol Neuroinflamm, 3(6), Article 6. https://doi.org/10.1212/NXI.0000000000000301CrossRefGoogle ScholarPubMed

Illán-Gala, I., Lorca-Puls, D. L., Ezzes, Z., et al. (2024). Clinical dimensions along the progressive nonfluent variant primary progressive aphasia spectrum. Brain, 147(4), 1511–1525. https://doi.org/10.1101/2023.04.18.23288702CrossRefGoogle Scholar

Josephs, K. A., Duffy, J. R., Strand, E. A., et al. (2013). Syndromes dominated by apraxia of speech show distinct characteristics from agrammatic PPA. Neurology, 81(4), Article 4. https://doi.org/10.1212/WNL.0b013e31829c5ed5CrossRefGoogle ScholarPubMed

Tetzloff, K. A., Duffy, J. R., Clark, H. M., et al. (2019). Progressive agrammatic aphasia without apraxia of speech as a distinct syndrome. Brain, 142(8), 2466–2482. https://doi.org/10.1093/brain/awz157CrossRefGoogle ScholarPubMed

Wilson, S., Henry, M., Besbri, M., et al. (2010). Connected speech production in three variants of primary progressive aphasia. Brain, 133(Pt 7), 2069–2088.10.1093/brain/awq129CrossRefGoogle ScholarPubMed

Thompson, C. K., & Mack, J. E. (2014). Grammatical impairments in PPA. Aphasiology, 28(8–9), 1018–1037. https://doi.org/10.1080/02687038.2014.912744CrossRefGoogle ScholarPubMed

Josephs, K. A., Duffy, J. R., Strand, E. A., et al. (2006). Clinicopathologic and imaging correlates of progressive aphasia and apraxia of speech. Brain, 129(Pt 6), Article Pt 6. https://doi.org/10.1093/brain/awl078CrossRefGoogle ScholarPubMed

Foxe, D., Irish, M., Hu, A., et al. (2021). Longitudinal cognitive and functional changes in primary progressive aphasia. J Neurol, 268(5), 1951–1961. https://doi.org/10.1007/s00415-020-10382-9CrossRefGoogle ScholarPubMed

Goodglass, H., & Kaplan, E. (1983). Boston Diagnostic Aphasia Examination (BDAE). Lea and Febiger. Distributed by Psychological Assessment Resources, Odessa, FL.Google Scholar

Kertesz, A. (1980). Western Aphasia Battery. University of Western Ontario Press.Google Scholar

Ogar, J. M., Dronkers, N. F., Brambati, S. M., et al. (2007). Progressive nonfluent aphasia and its characteristic motor speech deficits. Alzheimer Dis Assoc Disord, 21(4), Article 4. https://doi.org/10.1097/WAD.0b013e31815d19feCrossRefGoogle ScholarPubMed

Ash, S., McMillan, C., Gunawardena, D., et al. (2010). Speech errors in progressive non-fluent aphasia. Brain Lang, 113(1), Article 1. https://doi.org/10.1016/j.bandl.2009.12.001CrossRefGoogle ScholarPubMed

Wertz, R. T., LaPointe, L. L., & Rosenbek, J. C. (1984). Apraxia of Speech: The Disorders and Its Management. Grune and Stratton.Google Scholar

Utianski, R. L., Duffy, J. R., Clark, H. M., et al. (2018). Prosodic and phonetic subtypes of primary progressive apraxia of speech. Brain Lang, 184, 54–65. https://doi.org/10.1016/j.bandl.2018.06.004CrossRefGoogle ScholarPubMed

Thompson, C. K., Lukic, S., King, M. C., et al. (2012). Verb and noun deficits in stroke-induced and primary progressive aphasia: the Northwestern Naming Battery. Aphasiology, 26(5), 632–655. https://doi.org/10.1080/02687038.2012.676852CrossRefGoogle ScholarPubMed

Rohrer, J. D., Rossor, M. N., & Warren, J. D. (2010). Syndromes of nonfluent primary progressive aphasia: a clinical and neurolinguistic analysis. Neurology, 75(7), Article 7. https://doi.org/10.1212/WNL.0b013e3181ed9c6bCrossRefGoogle ScholarPubMed

Rosen, H. J., Allison, S. C., Ogar, J. M., et al. (2006). Behavioral features in semantic dementia versus other forms of progressive aphasias. Neurology, 67(10), Article 10.10.1212/01.wnl.0000247630.29222.34CrossRefGoogle Scholar

Mandelli, M. L., Vitali, P., Santos, M., et al. (2016). Two insular regions are differentially involved in behavioral variant FTD and nonfluent/agrammatic variant PPA. Cortex, 74, 149–157. doi: 10.1016/j.cortex.2015.10.012.CrossRefGoogle ScholarPubMed

Mandelli, M. L., Vilaplana, E., Brown, J. A., et al. (2016). Healthy brain connectivity predicts atrophy progression in non-fluent variant of primary progressive aphasia. Brain, 139(Pt 10), 2778–2791. doi: 10.1093/brain/aww195.CrossRefGoogle ScholarPubMed

Tetzloff, K. A., Duffy, J. R., Clark, H. M., et al. (2018). Longitudinal structural and molecular neuroimaging in agrammatic primary progressive aphasia. Brain, 141(1), 302–317. https://doi.org/10.1093/brain/awx293CrossRefGoogle ScholarPubMed

Catani, M., Mesulam, M. M., Jakobsen, E., et al. (2013). A novel frontal pathway underlies verbal fluency in primary progressive aphasia. Brain, 136(Pt 8), Article Pt 8. https://doi.org/10.1093/brain/awt163CrossRefGoogle ScholarPubMed

Mandelli, M. L., Caverzasi, E., Binney, R. J., et al. (2014). Frontal white matter tracts sustaining speech production in primary progressive aphasia. J Neurosci, 34(29), 9754–9767. https://doi.org/10.1523/JNEUROSCI.3464-13.2014CrossRefGoogle ScholarPubMed

Galantucci, S., Tartaglia, M. C., Wilson, S. M., et al. (2011). White matter damage in primary progressive aphasias: a diffusion tensor tractography study. Brain, 134(Pt 10), Article Pt 10. https://doi.org/10.1093/brain/awr099CrossRefGoogle ScholarPubMed

Grossman, M., Powers, J., Ash, S., et al. (2013). Disruption of large-scale neural networks in non-fluent/agrammatic variant primary progressive aphasia associated with frontotemporal degeneration pathology. Brain Lang, 127(2), 106–120. https://doi.org/10.1016/j.bandl.2012.10.005CrossRefGoogle ScholarPubMed

Josephs, K. A., Duffy, J. R., Strand, E. A., et al. (2012). Characterizing a neurodegenerative syndrome: primary progressive apraxia of speech. Brain, 135(Pt 5), Article Pt 5. https://doi.org/10.1093/brain/aws032CrossRefGoogle ScholarPubMed

Mandelli, M. L., Welch, A. E., Vilaplana, E., et al. (2018). Altered topology of the functional speech production network in non-fluent/agrammatic variant of PPA. Cortex, 108, 252–264. https://doi.org/10.1016/j.cortex.2018.08.002CrossRefGoogle ScholarPubMed

Wilson, S. M., Dronkers, N. F., Ogar, J. M., et al. (2010). Neural correlates of syntactic processing in the nonfluent variant of primary progressive aphasia. J Neurosci, 30(50), Article 50. https://doi.org/10.1523/JNEUROSCI.2547-10.2010CrossRefGoogle ScholarPubMed

Gorno-Tempini, M. L., Ogar, J. M., Brambati, S. M., et al. (2006). Anatomical correlates of early mutism in progressive nonfluent aphasia. Neurology, 67(10), Article 10. https://doi.org/10.1212/01.wnl.0000237038.55627.5bCrossRefGoogle ScholarPubMed

Santos-Santos, M. A., Rabinovici, G. D., Iaccarino, L., et al. (2018). Rates of amyloid imaging positivity in patients with primary progressive aphasia. JAMA Neurol, 75(3), Article 3. https://doi.org/10.1001/jamaneurol.2017.4309CrossRefGoogle ScholarPubMed

Josephs, K. A., Martin, P. R., Botha, H., et al. (2018). [18F]AV-1451 tau-PET and primary progressive aphasia. Ann Neurol, 83(3), 599–611. https://doi.org/10.1002/ana.25183CrossRefGoogle ScholarPubMed

Schaeverbeke, J., Evenepoel, C., Declercq, L., et al. (2018). Distinct [18F]THK5351 binding patterns in primary progressive aphasia variants. Eur J Nucl Med Mol Imaging, 45(13), 2342–2357. https://doi.org/10.1007/s00259-018-4075-3CrossRefGoogle ScholarPubMed

Yoon, C. W., Jeong, H. J., Seo, S., et al. (2018). 18F-THK5351 PET imaging in nonfluent-agrammatic variant primary progressive aphasia. Dement Neurocog Disord, 17(3), 110–119. https://doi.org/10.12779/dnd.2018.17.3.110CrossRefGoogle ScholarPubMed

Lee, H., Seo, S., Lee, S.-Y., J., et al. (2018). 18F.-THK5351 PET imaging in patients with semantic variant primary progressive aphasia. Alzheimer Dis Assoc Disord, 32(1), 62. https://doi.org/10.1097/WAD.0000000000000216CrossRefGoogle ScholarPubMed

Makaretz, S. J., Quimby, M., Collins, J., et al. (2018). Flortaucipir tau PET imaging in semantic variant primary progressive aphasia. J Neuro lNeurosurg Psychiatry, 89(10), 1024–1031. https://doi.org/10.1136/jnnp-2017-316409CrossRefGoogle ScholarPubMed

Deleon, J., & Miller, B. L. (2018). Frontotemporal dementia. In Geschwind, D. H., Paulson, H. L., Klein, C., eds. Handbook of Clinical Neurology (vol. 148). Elsevier; pp. 409–430. https://doi.org/10.1016/B978-0-444-64076-5.00027-2Google Scholar

Santos-Santos, M. A., Mandelli, M. L., Binney, R. J., et al. (2016). Features of patients with nonfluent/agrammatic primary progressive aphasia with underlying progressive supranuclear palsy pathology or corticobasal degeneration. JAMA Neurol, 73(6), 733–742. https://doi.org/10.1001/jamaneurol.2016.0412CrossRefGoogle ScholarPubMed

Rohrer, J. D., Lashley, T., Schott, J. M., et al. (2011). Clinical and neuroanatomical signatures of tissue pathology in frontotemporal lobar degeneration. Brain, 134(Pt 9), Article Pt 9. https://doi.org/10.1093/brain/awr198CrossRefGoogle ScholarPubMed

Caso, F., Gesierich, B., Henry, M., et al. (2013). Nonfluent/agrammatic PPA with in-vivo cortical amyloidosis and Pick’s disease pathology. Behav Neurol, 26(1–2), Article 1–2. https://doi.org/10.3233/BEN-2012-120255CrossRefGoogle ScholarPubMed

Miller, Z., Bogley, R., Miller, C., et al. (2021). Increased prevalence of developmental stuttering across lvPPA, nfvPPA, and CBS spectrum degenerative disorders (2205). Neurology, 96(15 Suppl). https://n.neurology.org/content/96/15_Supplement/220510.1212/WNL.96.15_supplement.2205CrossRefGoogle Scholar

Weintraub, S., Rubin, N. P., & Mesulam, M.-M. (1990). Primary progressive aphasia: longitudinal course, neuropsychological profile, and language features. Arch Neurol, 47, 1329–1335.10.1001/archneur.1990.00530120075013CrossRefGoogle ScholarPubMed

Conca, F., Esposito, V., Giusto, G., et al. (2022). Characterization of the logopenic variant of primary progressive aphasia: a systematic review and meta-analysis. Ageing Res Rev, 82, 101760. https://doi.org/10.1016/j.arr.2022.101760CrossRefGoogle ScholarPubMed

Chare, L., Hodges, J. R., Leyton, C. E., et al. (2014). New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications. J Neurol Neurosurg Psychiatry, 85(8), Article 8. https://doi.org/10.1136/jnnp-2013-306948CrossRefGoogle ScholarPubMed

Catricalà, E., Santi, G. C., Polito, C., et al. (2022). Comprehensive qualitative characterization of linguistic performance profiles in primary progressive aphasia: a multivariate study with FDG-PET. Neurobiol Aging, 120, 137–148. https://doi.org/10.1016/j.neurobiolaging.2022.09.001CrossRefGoogle ScholarPubMed

Rohrer, J. D., Ridgway, G. R., Crutch, S. J., et al. (2010). Progressive logopenic/phonological aphasia: erosion of the language network. NeuroImage, 49(1), 984–993. https://doi.org/10.1016/j.neuroimage.2009.08.002CrossRefGoogle ScholarPubMed

Basaglia-Pappas, S., Laurent, B., Getenet, J.-C., et al. (2023). Executive profile of the logopenic variant of primary progressive aphasia: comparison with the semantic and non-fluent variants and Alzheimer’s disease. Brain Sci, 13(3), Article 3. https://doi.org/10.3390/brainsci13030406CrossRefGoogle ScholarPubMed

Leyton, C. E., Hsieh, S., Mioshi, E., et al. (2013). Cognitive decline in logopenic aphasia: more than losing words. Neurology, 80(10), Article 10. https://doi.org/10.1212/WNL.0b013e318285c15bCrossRefGoogle ScholarPubMed

Leyton, C. E., Landin-Romero, R., Liang, C. T., et al. (2019). Correlates of anomia in non-semantic variants of primary progressive aphasia converge over time. Cortex, 120, 201–211. https://doi.org/10.1016/j.cortex.2019.06.008CrossRefGoogle ScholarPubMed

Haley, K. L., Jacks, A., Jarrett, J., et al. (2021). Speech metrics and samples that differentiate between nonfluent/agrammatic and logopenic variants of primary progressive aphasia. JSLHR, 64(3), Article 3. https://doi.org/10.1044/2020_JSLHR-20-00445Google ScholarPubMed

Sajjadi, S. A., Patterson, K., Arnold, R. J., et al. (2012). Primary progressive aphasia: a tale of two syndromes and the rest. Neurology, 78(21), Article 21. https://doi.org/10.1212/WNL.0b013e3182574f79CrossRefGoogle ScholarPubMed

Tippett, D. C. (2020). Classification of primary progressive aphasia: challenges and complexities. F1000Research, 9, 64. https://doi.org/10.12688/f1000research.21184.1CrossRefGoogle ScholarPubMed

Kaplan, E. F., Goodglass, H., & Weintraub, S. (1983). The Boston Naming Test. Lea & Febiger.Google Scholar

Mack, J. E., Chandler, S. D., Meltzer-Asscher, A., et al. (2015). What do pauses in narrative production reveal about the nature of word retrieval deficits in PPA? Neuropsychologia, 77, 211–222. https://doi.org/10.1016/j.neuropsychologia.2015.08.019CrossRefGoogle ScholarPubMed

Beeson, P. M., Rising, K., Kim, E. S., et al. (2010). A treatment sequence for phonological alexia/agraphia. J Speech Lang Hear Res, 53(2), Article 2. https://doi.org/10.1044/1092-4388(2009/08-0229)CrossRefGoogle ScholarPubMed

Teichmann, M., Kas, A., Boutet, C., et al. (2013). Deciphering logopenic primary progressive aphasia: a clinical, imaging and biomarker investigation. Brain, 136(Pt 11), Article Pt 11. https://doi.org/10.1093/brain/awt266CrossRefGoogle ScholarPubMed

Gorno-Tempini, M. L., Brambati, S. M., Ginex, V., et al. (2008). The logopenic/phonological variant of primary progressive aphasia. Neurology, 71(16), 1227–1234. https://doi.org/10.1212/01.wnl.0000320506.79811.daCrossRefGoogle ScholarPubMed

Migliaccio, R., Agosta, F., Possin, K. L., et al. (2012). White matter atrophy in Alzheimer’s disease variants. Alzheimers Dement, 8(5 Suppl), Article 5 Suppl. https://doi.org/10.1016/j.jalz.2012.04.010CrossRefGoogle ScholarPubMed

Whitwell, J. L., Jones, D. T., Duffy, J. R., et al. (2014). Working memory and language network dysfunctions in logopenic aphasia: a task-free fMRI comparison with Alzheimer’s dementia. Neurobiol Aging, 36(3),1245–1252. https://doi.org/10.1016/j.neurobiolaging.2014.12.013CrossRefGoogle ScholarPubMed

Tu, S., Leyton, C. E., Hodges, J. R., et al. (2015). Divergent longitudinal propagation of white matter degradation in logopenic and semantic variants of primary progressive aphasia. J Alzheimers Dis, 49(3), Article 3. https://doi.org/10.3233/JAD-150626CrossRefGoogle Scholar

Rogalski, E. J., Rademaker, A., Harrison, T. M., et al. (2011). ApoE E4 is a susceptibility factor in amnestic but not aphasic dementias. Alzheimer Dis Assoc Disorde, 25(2), Article 2. https://doi.org/10.1097/WAD.0b013e318201f249CrossRefGoogle Scholar

Munoz, D. G., Woulfe, J., & Kertesz, A. (2007). Argyrophilic thorny astrocyte clusters in association with Alzheimer’s disease pathology in possible primary progressive aphasia. Acta Neuropathol, 114(4), Article 4. https://doi.org/10.1007/s00401-007-0266-xCrossRefGoogle ScholarPubMed

Miller, Z. A., Spina, S., Pakvasa, M., et al. (2019). Cortical developmental abnormalities in logopenic variant primary progressive aphasia with dyslexia. Brain Commun, 1(1). https://doi.org/10.1093/braincomms/fcz027CrossRefGoogle ScholarPubMed

Ramos, E. M., Dokuru, D. R., Van Berlo, V., et al. (2019). Genetic screen in a large series of patients with primary progressive aphasia. Alzheimers & Dement, 15(4), 553–560. https://doi.org/10.1016/j.jalz.2018.10.009CrossRefGoogle Scholar

Rohrer, J. D., Warren, J. D., Omar, R., et al. (2008). Parietal lobe deficits in frontotemporal lobar degeneration caused by a mutation in the progranulin gene. Arch Neuroly, 65(4), 506–513. https://doi.org/10.1001/archneur.65.4.506CrossRefGoogle ScholarPubMed

Saracino, D., Ferrieux, S., Noguès-Lassiaille, M., et al. (2021). Primary progressive aphasia associated with GRN mutations: new insights into the nonamyloid logopenic variant. Neurology, 9 7(1), e88–e102. https://doi.org/10.1212/WNL.0000000000012174Google Scholar

Momota, Y., Konishi, M., Takahata, K., et al. (2022). Case report: non-Alzheimer’s disease tauopathy with logopenic variant primary progressive aphasia diagnosed using amyloid and tau PET. Front Neurol, 13,1049113. https://doi.org/10.3389/fneur.2022.1049113CrossRefGoogle ScholarPubMed

Rogalski, E., Johnson, N., Weintraub, S., et al. (2008). Increased frequency of learning disability in patients with primary progressive aphasia and their first-degree relatives. Arch Neurol, 65(2), Article 2. https://doi.org/10.1001/archneurol.2007.34CrossRefGoogle ScholarPubMed

Miller, Z. A., Rosenberg, L., Santos-Santos, M. A., et al. (2018). Prevalence of mathematical and visuospatial learning disabilities in patients with posterior cortical atrophy. JAMA Neurol, 75(6), 728–737. https://doi.org/10.1001/jamaneurol.2018.0395CrossRefGoogle ScholarPubMed

Harris, J. M., Gall, C., Thompson, J. C., et al. (2013). Classification and pathology of primary progressive aphasia. Neurology, 81(21), 1832–1839. https://doi.org/10.1212/01.wnl.0000436070.28137.7bCrossRefGoogle ScholarPubMed

Leyton, C. E., Villemagne, V. L., Savage, S., et al. (2011). Subtypes of progressive aphasia: application of the International Consensus Criteria and validation using beta-amyloid imaging. Brain, 134(Pt 10), Article Pt 10. https://doi.org/10.1093/brain/awr216CrossRefGoogle ScholarPubMed

Wicklund, M. R., Duffy, J. R., Strand, E. A., et al. (2014). Quantitative application of the primary progressive aphasia consensus criteria. Neurology, 82(13), 1119–1126. https://doi.org/10.1212/WNL.0000000000000261CrossRefGoogle ScholarPubMed

Leyton, C. E., Hodges, J. R., Piguet, O., et al. (2017). Common and divergent neural correlates of anomia in amnestic and logopenic presentations of Alzheimer’s disease. Cortex, 86, 45–54. https://doi.org/10.1016/j.cortex.2016.10.019CrossRefGoogle ScholarPubMed

Reilly, J., Peelle, J. E., Antonucci, S. M., et al. (2011). Anomia as a marker of distinct semantic memory impairments in Alzheimer’s disease and semantic dementia. Neuropsychology, 25(4), 413–426. https://doi.org/10.1037/a0022738CrossRefGoogle ScholarPubMed

Leyton, C. E., Savage, S., Irish, M., et al. (2014). Verbal repetition in primary progressive aphasia and Alzheimer’s disease. J Alzheimers Dis, 41(2), Article 2. https://doi.org/10.3233/JAD-132468CrossRefGoogle ScholarPubMed

Rohrer, J. D., Crutch, S. J., Warrington, E. K., et al. (2010). Progranulin-associated primary progressive aphasia: a distinct phenotype? Neuropsychologia, 48(1), 288–297. https://doi.org/10.1016/j.neuropsychologia.2009.09.017CrossRefGoogle ScholarPubMed

Rogalski, E. J., & Mesulam, M. M. (2009). Clinical trajectories and biological features of primary progressive aphasia (PPA). Curr Alzheimer Res, 6(4), Article 4.10.2174/156720509788929264CrossRefGoogle ScholarPubMed

Boschi, V., Catricalà, E., Consonni, M., et al. (2017). connected speech in neurodegenerative language disorders: a review. Front Psychol, 8, 269. https://doi.org/10.3389/fpsyg.2017.00269CrossRefGoogle ScholarPubMed

Miller, B. L., Cummings, J., Mishkin, F., et al. (1998). Emergence of artistic talent in frontotemporal dementia. Neurology, 51(4), Article 4.10.1212/WNL.51.4.978CrossRefGoogle ScholarPubMed

Viskontas, I. V., Boxer, A. L., Fesenko, J., et al. (2011). Visual search patterns in semantic dementia show paradoxical facilitation of binding processes. Neuropsychologia, 49(3), 468–478. https://doi.org/10.1016/j.neuropsychologia.2010.12.039CrossRefGoogle ScholarPubMed

Tee, B. L., Watson Pereira, C., Lukic, S., et al. (2022). Neuroanatomical correlations of visuospatial processing in primary progressive aphasia. Brain Commun, 4(2), fcac060. https://doi.org/10.1093/braincomms/fcac060CrossRefGoogle ScholarPubMed

Binney, R. J., Henry, M. L., Babiak, M., et al. (2016). Reading words and other people: a comparison of exception word, familiar face and affect processing in the left and right temporal variants of primary progressive aphasia. Cortex, 82, 147–163. https://doi.org/10.1016/j.cortex.2016.05.014CrossRefGoogle ScholarPubMed

Omar, R., Rohrer, J. D., Hailstone, J. C., et al. (2011). Structural neuroanatomy of face processing in frontotemporal lobar degeneration. J Neurol Neurosurg Psychiatry, 82(12), Article 12. https://doi.org/10.1136/jnnp.2010.227983CrossRefGoogle ScholarPubMed

Perry, R. J., Rosen, H. R., Kramer, J. H., et al. (2001). Hemispheric dominance for emotions, empathy and social behaviour: evidence from right and left handers with frontotemporal dementia. Neurocase, 7(2), Article 2.Google ScholarPubMed

Kumfor, F., & Piguet, O. (2012). Disturbance of emotion processing in frontotemporal dementia: a synthesis of cognitive and neuroimaging findings. Neuropsychol Rev, 22(3), 280–297. https://doi.org/10.1007/s11065-012-9201-6CrossRefGoogle ScholarPubMed

Rosen, H. J., Perry, R. J., Murphy, J., et al. (2002). Emotion comprehension in the temporal variant of frontotemporal dementia. Brain, 125(Pt 10), Article Pt 10.10.1093/brain/awf225CrossRefGoogle ScholarPubMed

Pascual, B., Funk, Q., Zanotti-Fregonara, P., et al. (2021). Neuroinflammation in semantic variant primary progressive aphasia. J Nucl Med, 62(supplement 1), 101–101.Google Scholar

Zhang, J. (2015). Mapping neuroinflammation in frontotemporal dementia with molecular PET imaging. J Neuroinflamm, 12(1), 108. https://doi.org/10.1186/s12974-015-0236-5CrossRefGoogle ScholarPubMed

Volkmer, A., Rogalski, E., Henry, M., et al. (2020). Speech and language therapy approaches to managing primary progressive aphasia. Pract Neurol, 20(2), Article 2. https://doi.org/10.1136/practneurol-2018-001921CrossRefGoogle ScholarPubMed

Gervits, F., Ash, S., Coslett, H. B., et al. (2016). Transcranial direct current stimulation for the treatment of primary progressive aphasia: an open-label pilot study. Brain Lang, 162, 35–41. https://doi.org/10.1016/j.bandl.2016.05.007CrossRefGoogle ScholarPubMed

Pytel, V., Cabrera-Martín, M. N., Delgado-Álvarez, A., et al. (2021). Personalized Repetitive Transcranial Magnetic Stimulation for Primary Progressive Aphasia. J Alzheimers Dis, 84(1), 151–167. https://doi.org/10.3233/JAD-210566CrossRefGoogle ScholarPubMed

Rebeiz, JJ, Kolodny, EH, Richardson, EP Jr. Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol. 1968 Jan;18(1):20–33.10.1001/archneur.1968.00470310034003CrossRefGoogle ScholarPubMed

Gibb, WR, Luthert, PJ, Marsden, CD. Corticobasal degeneration. Brain. 1989 Oct;112 (Pt 5):1171–1192.10.1093/brain/112.5.1171CrossRefGoogle ScholarPubMed

Riley, DE, Lang, AE, Lewis, A, et al. Cortical-basal ganglionic degeneration. Neurology. 1990 Aug;40(8):1203–1212.10.1212/WNL.40.8.1203CrossRefGoogle ScholarPubMed

Constantinidis, J, Richard, J, Tissot, R. Pick’s disease. Histological and clinical correlations. Eur Neurol. 1974;11(4):208–217.10.1159/000114320CrossRefGoogle ScholarPubMed

Lee, SE, Rabinovici, GD, Mayo, MC, et al. Clinicopathological correlations in corticobasal degeneration. Ann Neurol. 2011 Aug;70(2):327–340.10.1002/ana.22424CrossRefGoogle ScholarPubMed

Vanvoorst, WA, Greenaway, MC, Boeve, BF, et al. Neuropsychological findings in clinically atypical autopsy confirmed corticobasal degeneration and progressive supranuclear palsy. Parkinsonism Relat Disord. 2008;14(4):376–378.10.1016/j.parkreldis.2007.09.006CrossRefGoogle ScholarPubMed

Grimes, DA, Lang, AE, Bergeron, CB. Dementia as the most common presentation of cortical-basal ganglionic degeneration. Neurology. 1999 Dec 10;53(9):1969–1674.10.1212/WNL.53.9.1969CrossRefGoogle Scholar

Murray, R, Neumann, M, Forman, MS, et al. Cognitive and motor assessment in autopsy-proven corticobasal degeneration. Neurology. 2007 Apr 17;68(16):1274–1283.10.1212/01.wnl.0000259519.78480.c3CrossRefGoogle ScholarPubMed

Kertesz, A, McMonagle, P, Blair, M, Davidson, W, Munoz, DG. The evolution and pathology of frontotemporal dementia. Brain. 2005 Sep;128(Pt 9):1996–2005.10.1093/brain/awh598CrossRefGoogle ScholarPubMed

Geda, YE, Boeve, BF, Negash, S, et al. Neuropsychiatric features in 36 pathologically confirmed cases of corticobasal degeneration. J Neuropsychiatry Clin Neurosci. 2007 Winter;19(1):77–80.10.1176/jnp.2007.19.1.77CrossRefGoogle ScholarPubMed

Togasaki, DM, Tanner, CM. Epidemiologic aspects. Adv Neurol. 2000;82:53–59.Google ScholarPubMed

Morimatsu, M, Negoro, K. [Provisional diagnostic criteria of corticobasal degeneration (CBD) and the survey of patients with CBD in Japan]. Rinsho Shinkeigaku. 2002 Nov;42(11):1150–1153.Google ScholarPubMed

Wenning, GK, Litvan, I, Jankovic, J, et al. Natural history and survival of 14 patients with corticobasal degeneration confirmed at postmortem examination. J Neurol Neurosurg Psychiatry. 1998 Feb;64(2):184–189.10.1136/jnnp.64.2.184CrossRefGoogle ScholarPubMed

Armstrong, MJ, Litvan, I, Lang, AE, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013 Jan 29;80(5):496–503.10.1212/WNL.0b013e31827f0fd1CrossRefGoogle ScholarPubMed

Bugiani, O, Murrell, JR, Giaccone, G, et al. Frontotemporal dementia and corticobasal degeneration in a family with a P301S mutation in tau. J Neuropathol Exp Neurol. 1999 Jun;58(6):667–677.10.1097/00005072-199906000-00011CrossRefGoogle Scholar

Spillantini, MG, Yoshida, H, Rizzini, C, 9 et al. A novel tau mutation (N296 N) in familial dementia with swollen achromatic neurons and corticobasal inclusion bodies. Ann Neurol. 2000 Dec;48(6):939–943.10.1002/1531-8249(200012)48:6<939::AID-ANA17>3.0.CO;2-13.0.CO;2-1>CrossRefGoogle Scholar

Rossi, G, Marelli, C, Farina, L, et al. The G389 R mutation in the MAPT gene presenting as sporadic corticobasal syndrome. Mov Disord. 2008 Apr 30;23(6):892–895.10.1002/mds.21970CrossRefGoogle Scholar

Yu, CE, Bird, TD, Bekris, LM, et al. The spectrum of mutations in progranulin: a collaborative study screening 545 cases of neurodegeneration. Arch Neurol. 2010 Feb;67(2):161–170.10.1001/archneurol.2009.328CrossRefGoogle ScholarPubMed

Rohrer, JD, Beck, J, Warren, JD, et al. Corticobasal syndrome associated with a novel 1048_1049insG progranulin mutation. J Neurol Neurosurg Psychiatry. 2009 Nov;80(11):1297–1298.10.1136/jnnp.2008.169383CrossRefGoogle ScholarPubMed

Masellis, M, Momeni, P, Meschino, W, et al. Novel splicing mutation in the progranulin gene causing familial corticobasal syndrome. Brain. 2006 Nov;129(Pt 11):3115–3123.10.1093/brain/awl276CrossRefGoogle ScholarPubMed

Conrad, C, Amano, N, Andreadis, A, et al. Differences in a dinucleotide repeat polymorphism in the tau gene between Caucasian and Japanese populations: implication for progressive supranuclear palsy. Neurosci Lett. 1998 Jul 3;250(2):135–137.10.1016/S0304-3940(98)00417-0CrossRefGoogle Scholar

Evans, W, Fung, HC, Steele, J, et al. The tau H2 haplotype is almost exclusively Caucasian in origin. Neurosci Lett. 2004 Oct 21;369(3):183–185.10.1016/j.neulet.2004.05.119CrossRefGoogle ScholarPubMed

Baker, M, Litvan, I, Houlden, H, et al. Association of an extended haplotype in the tau gene with progressive supranuclear palsy. Hum Mol Genet. 1999 Apr;8(4):711–715.10.1093/hmg/8.4.711CrossRefGoogle ScholarPubMed

Houlden, H, Baker, M, Morris, HR, et al. Corticobasal degeneration and progressive supranuclear palsy share a common tau haplotype. Neurology. 2001 Jun 26;56(12):1702–1706.10.1212/WNL.56.12.1702CrossRefGoogle Scholar

Verpillat, P, Camuzat, A, Hannequin, D, et al. Association between the extended tau haplotype and frontotemporal dementia. Arch Neurol. 2002 Jun;59(6):935–939.10.1001/archneur.59.6.935CrossRefGoogle ScholarPubMed

Hughes, A, Mann, D, Pickering-Brown, S. Tau haplotype frequency in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Exp Neurol. 2003 May;181(1):12–16.10.1016/S0014-4886(03)00024-4CrossRefGoogle ScholarPubMed

Sobrido, MJ, Abu-Khalil, A, Weintraub, S, et al. Possible association of the tau H1/H1 genotype with primary progressive aphasia. Neurology. 2003 Mar 11;60(5):862–864.10.1212/01.WNL.0000049473.36612.F2CrossRefGoogle ScholarPubMed

Kouri, N, Ross, OA, Dombroski, B, et al. Genome-wide association study of corticobasal degeneration identifies risk variants shared with progressive supranuclear palsy. Nat Commun. 2015 Jun 16;6:7247.10.1038/ncomms8247CrossRefGoogle ScholarPubMed

Kertesz, A, Martinez-Lage, P, Davidson, W, Munoz, DG. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology. 2000 Nov 14;55(9):1368–1375.10.1212/WNL.55.9.1368CrossRefGoogle ScholarPubMed

Graham, NL, Bak, TH, Hodges, JR. Corticobasal degeneration as a cognitive disorder. Mov Disord. 2003 Nov;18(11):1224–1232.10.1002/mds.10536CrossRefGoogle ScholarPubMed

Pillon, B, Blin, J, Vidailhet, M, et al. The neuropsychological pattern of corticobasal degeneration: comparison with progressive supranuclear palsy and Alzheimer’s disease. Neurology. 1995 Aug;45(8):1477–1483.10.1212/WNL.45.8.1477CrossRefGoogle ScholarPubMed

Belfor, N, Amici, S, Boxer, AL, et al. Clinical and neuropsychological features of corticobasal degeneration. Mech Ageing Dev. 2006 Feb;127(2):203–207.10.1016/j.mad.2005.09.013CrossRefGoogle ScholarPubMed

Rascovsky, K, Hodges, JR, Knopman, D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain. Aug 2;2011;134(Pt 9):2456–2477.10.1093/brain/awr179CrossRefGoogle ScholarPubMed

Gorno-Tempini, ML, Hillis, AE, Weintraub, S, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011 Mar 15;76(11):1006–1014.10.1212/WNL.0b013e31821103e6CrossRefGoogle ScholarPubMed

Boeve, BF, Maraganore, DM, Parisi, JE, et al. Pathologic heterogeneity in clinically diagnosed corticobasal degeneration. Neurology. 1999 Sep 11;53(4):795–800.10.1212/WNL.53.4.795CrossRefGoogle ScholarPubMed

Josephs, KA, Dickson, DW. Diagnostic accuracy of progressive supranuclear palsy in the Society for Progressive Supranuclear Palsy brain bank. Mov Disord. 2003 Sep;18(9):1018–1026.10.1002/mds.10488CrossRefGoogle Scholar

Josephs, KA, Petersen, RC, Knopman, DS, et al. Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology. 2006 Jan 10;66(1):41–48.10.1212/01.wnl.0000191307.69661.c3CrossRefGoogle ScholarPubMed

Rohrer, JD, Geser, F, Zhou, J, et al. TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia. Neurology. 2010 Dec 14;75(24):2204–2211.10.1212/WNL.0b013e318202038cCrossRefGoogle ScholarPubMed

Spinelli, EG, Mandelli, ML, Miller, ZA, et al. Typical and atypical pathology in primary progressive aphasia variants. Ann Neurol. 2017 Mar 20;81(3):430–443.10.1002/ana.24885CrossRefGoogle ScholarPubMed

Wakabayashi, K, Oyanagi, K, Makifuchi, T, et al. Corticobasal degeneration: etiopathological significance of the cytoskeletal alterations. Acta Neuropathol. 1994;87(6):545–553.10.1007/BF00293314CrossRefGoogle ScholarPubMed

Buee Scherrer, V, Hof, PR, Buee, L, et al. Hyperphosphorylated tau proteins differentiate corticobasal degeneration and Pick’s disease. Acta Neuropathol. 1996;91(4):351–359.10.1007/s004010050436CrossRefGoogle ScholarPubMed

Grundke-Iqbal, I, Iqbal, K, Quinlan, M, et al. Microtubule-associated protein tau. A component of Alzheimer paired helical filaments. J Biol Chem. 1986 May 5;261(13):6084–6089.10.1016/S0021-9258(17)38495-8CrossRefGoogle ScholarPubMed

Witman, GB, Cleveland, DW, Weingarten, MD, Kirschner, MW. Tubulin requires tau for growth onto microtubule initiating sites. Proc Natl Acad Sci U S A. 1976 Nov;73(11):4070–4074.10.1073/pnas.73.11.4070CrossRefGoogle ScholarPubMed

Cairns, NJ, Bigio, EH, Mackenzie, IR, et al. Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol. 2007 Jul;114(1):5–22.10.1007/s00401-007-0237-2CrossRefGoogle Scholar

Dickson, DW, Bergeron, C, Chin, SS, et al. Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol. 2002 Nov;61(11):935–946.10.1093/jnen/61.11.935CrossRefGoogle ScholarPubMed

Komori, T, Arai, N, Oda, M, et al. Astrocytic plaques and tufts of abnormal fibers do not coexist in corticobasal degeneration and progressive supranuclear palsy. Acta Neuropathol. 1998 Oct;96(4):401–840.10.1007/s004010050911CrossRefGoogle Scholar

Feany, MB, Dickson, DW. Widespread cytoskeletal pathology characterizes corticobasal degeneration. Am J Pathol. 1995 Jun;146(6):1388–1396.Google ScholarPubMed

Litvan, I, Agid, Y, Goetz, C, et al. Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study. Neurology. 1997 Jan;48(1):119–125.10.1212/WNL.48.1.119CrossRefGoogle ScholarPubMed

Mackenzie, IR, Neumann, M, Bigio, EH, et al. Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol. 2010 Jan;119(1):1–4.10.1007/s00401-009-0612-2CrossRefGoogle ScholarPubMed

Josephs, KA, Duffy, JR, Strand, EA, et al. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain. 2006 Jun;129(Pt 6):1385–1398.10.1093/brain/awl078CrossRefGoogle ScholarPubMed

Ling, H, O’Sullivan, SS, Holton, JL, et al. Does corticobasal degeneration exist? A clinicopathological re-evaluation. Brain. 2010 Jul;133(Pt 7):2045–2057.10.1093/brain/awq123CrossRefGoogle ScholarPubMed

Hu, WT, Rippon, GW, Boeve, BF, et al. Alzheimer’s disease and corticobasal degeneration presenting as corticobasal syndrome. Mov Disord. 2009 Jul 15;24(9):1375–1379.10.1002/mds.22574CrossRefGoogle ScholarPubMed

Shelley, BP, Hodges, JR, Kipps, CM, Xuereb, JH, Bak, TH. Is the pathology of corticobasal syndrome predictable in life? Mov Disord. 2009 Aug 15;24(11):1593–1599.10.1002/mds.22558CrossRefGoogle Scholar

Sha, SJ, Ghosh, PM, Lee, SE, et al. Predicting amyloid status in corticobasal syndrome using modified clinical criteria, magnetic resonance imaging and fluorodeoxyglucose positron emission tomography. Alzheimers Res Ther. 2015;7(1):8.10.1186/s13195-014-0093-yCrossRefGoogle ScholarPubMed

Steele, JC, Richardson, JC, Olszewski, J. Progressive supranuclear palsy. a heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol. 1964 Apr;10:333–359.10.1001/archneur.1964.00460160003001CrossRefGoogle ScholarPubMed

Chambers, CB, Lee, JM, Troncoso, JC, Reich, S, Muma, NA. Overexpression of four-repeat tau mRNA isoforms in progressive supranuclear palsy but not in Alzheimer’s disease. Ann Neurol. 1999 Sep;46(3):325–332.10.1002/1531-8249(199909)46:3<325::AID-ANA8>3.0.CO;2-V3.0.CO;2-V>CrossRefGoogle Scholar

Steele, JC. Progressive supranuclear palsy. Brain. 1972;95(4):693–704.Google ScholarPubMed

David, NJ, Mackey, EA, Smith, JL. Further observations in progressive supranuclear palsy. Neurology. 1968 Apr;18(4):349–356.10.1212/WNL.18.4.349CrossRefGoogle ScholarPubMed

Litvan, I, Agid, Y, Calne, D, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology. 1996 Jul;47(1):1–9.10.1212/WNL.47.1.1CrossRefGoogle ScholarPubMed