Visit NavNeuro at:

Episode 84 | Behavioral Variant Frontotemporal Dementia – With Dr. Bruce Miller


This episode is a discussion of behavioral variant frontotemporal dementia (bvFTD). We first talk about overlap and differences between the various FTD spectrum conditions. Next, we cover the following topics specific to bvFTD: neuropathology, genetics, and neuroimaging, phenocopy syndrome, the classic clinical and behavioral syndrome, cognitive profile, and treatment options.

apa-logo_white_screenThe International Neuropsychological Society is approved by the American Psychological Association to sponsor continuing education for psychologists. The International Neuropsychological Society maintains responsibility for this program and its content.

Bruce Miller
Instructor Credentials

Bruce L. Miller, MD, holds the A.W. and Mary Margaret Clausen Distinguished Professorship in Neurology at the University of California, San Francisco, directs the UCSF Memory and Aging Center and is a director of the Global Brain Health Institute. He is the principal investigator of the NIH-sponsored Alzheimer’s Disease Research Center and program project grant on frontotemporal dementia. In addition, he helps lead the Tau Consortium and the Bluefield Foundation, precision medicine collaborations focused on developing treatments for tauopathies and progranulin-mediated forms of frontotemporal dementia.

Miller is a behavioral neurologist who studies the underlying mechanisms of neurocognitive disorders and is a world-renowned expert in the diagnosis and management of dementia. Until recently, most cases of dementia were classified as Alzheimer’s disease with little awareness of the importance of non-Alzheimer dementias. Miller’s description of changes in behavior, language and emotion in the setting of aging have improved the separation of various neurodegenerative diseases from one another, in particular, Alzheimer’s disease from frontotemporal dementia. To support people after diagnosis, Miller has pioneered groundbreaking changes in the provision of care coordination for patients with dementia and their caregivers. His ongoing work includes overseeing a healthy aging study and an artist-in-residence program, both of which emphasize positive aspects of aging.

Miller has been featured in Fortune Magazine, the New York Times, 60 Minutes, the Charlie Rose Show and the PBS Newshour. He has authored more than 800 publications and written several books: The Human Frontal Lobes, The Behavioral Neurology of Dementia and Frontotemporal Dementia. He has received many awards including the Potamkin Award from the American Academy of Neurology, the Elliot Royer Award from the San Francisco Neurological community, the UCSF Academic Senate Distinction in Mentoring Award and the Robert A. Fishman Award and Lecture. In 2016, Miller was elected to the National Academy of Medicine.

Topics Covered
  • Neuropathology
  • Genetics and Neuroimaging
  • Phenocopy Syndrome
  • The Classic Clinical and Behavioral Syndrome
  • Cognitive Profile
  • Treatment Options
Educational Objectives
  • Describe typical neuropathology and neuroimaging findings in behavioral variant FTD.
  • List and explain behavioral variant FTD clinical syndrome and cognitive profile.
  • Apply knowledge from this podcast episode in clinical evaluations, including interviewing, test selection, diagnosis, recommendations, and feedback.
Target Audience
  • Introductory
  • Date Available: 2021-12-01
  • You may obtain CE for this podcast at any time.
Offered for CE
  • Yes
  • Members $20
  • Non-Members $25
Refund Policy
  • This podcast is not eligible for refunds
CE Credits
  • 1.5 Credit(s)
  • None
  • Bang, J., Spina, S., & Miller, B. L. (2015). Frontotemporal dementia. The Lancet, 386(10004), 1672-1682.
  • Deleon, J., & Miller, B. L. (2018). Frontotemporal dementia. Handbook of Clinical Neurology, 148, 409-430.
  • Ducharme, S., Price, B. H., Larvie, M., Dougherty, D. D., & Dickerson, B. C. (2015, Sep 1). Clinical Approach to the Differential Diagnosis Between Behavioral Variant Frontotemporal Dementia and Primary Psychiatric Disorders. American Journal of Psychiatry, 172(9), 827-837.
  • Ferrari, R., Hernandez, D. G., Nalls, M. A., Rohrer, J. D., Ramasamy, A., Kwok, J. B. J., Dobson-Stone, C., Brooks, W. S., Schofield, P. R., Halliday, G. M., Hodges, J. R., Piguet, O., Bartley, L., Thompson, E., Haan, E., Hernández, I., Ruiz, A., Boada, M., Borroni, B., Padovani, A., Cruchaga, … Momeni, P. (2014). Frontotemporal dementia and its subtypes: a genome-wide association study. The Lancet Neurology, 13(7), 686-699.
  • Ferrari, R., Hernandez, D. G., Nalls, M. A., Rohrer, J. D., Ramasamy, A., Kwok, J. B. J., Dobson-Stone, C., Brooks, W. S., Schofield, P. R., Halliday, G. M., Hodges, J. R., Piguet, O., Bartley, L., Thompson, E., Haan, E., Hernández, I., Ruiz, A., Boada, M., Borroni, B., Padovani, A., … Momeni, P. (2014). Frontotemporal dementia and its subtypes: a genome-wide association study. The Lancet Neurology, 13(7), 686-699.
  • Ferrari, R., Kapogiannis, D., Huey, E. D., & Momeni, P. (2011, May). FTD and ALS: a tale of two diseases. Current Alzheimer Research, 8(3), 273-294.
  • Ghetti, B., Buratti, E., Boeve, B., Rademakers, R. (Eds.) (2021) Frontotemporal Dementias: Emerging Milestones of the 21st Century, Springer
  • Gitlin, L. N., Winter, L., Vause Earland, T., Adel Herge, E., Chernett, N. L., Piersol, C. V., & Burke, J. P. (2009, Jun). The Tailored Activity Program to reduce behavioral symptoms in individuals with dementia: feasibility, acceptability, and replication potential. Gerontologist, 49(3), 428-439.
  • Heuer, H. W., Wang, P., Rascovsky, K., Wolf, A., Appleby, B., Bove, J., Bordelon, Y., Brannelly, P., Brushaber, D. E., Caso, C., Coppola, G., Dickerson, B., Dickinson, S., Domoto-Reilly, K., Faber, K., Ferrall, J., Fields, J., Fishman, A., Fong, J., … Consortia, L. (2020, Jan). Comparison of sporadic and familial behavioral variant frontotemporal dementia (FTD) in a North American cohort. Alzheimers & Dementia, 16(1), 60-70.
  • Hua, A. Y., Chen, K. H., Brown, C. L., Lwi, S. J., Casey, J. J., Rosen, H. J., Miller, B. L., & Levenson, R. W. (2019, Dec 30). Physiological, behavioral and subjective sadness reactivity in frontotemporal dementia subtypes. Social Cognitive and Affective Neuroscience, 14(12), 1453-1465.
  • Illán-Gala, I., Casaletto, K. B., Borrego-Écija, S., Arenaza-Urquijo, E. M., Wolf, A., Cobigo, Y., Goh, S. Y. M., Staffaroni, A. M., Alcolea, D., Fortea, J., Blesa, R., Clarimon, J., Iulita, M. F., Brugulat-Serrat, A., Lladó, A., Grinberg, L. T., Possin, K., Rankin, K. P., Kramer, … Rosen, H. J. (2021). Sex differences in the behavioral variant of frontotemporal dementia: A new window to executive and behavioral reserve. Alzheimer’s & Dementia, 17(8), 1329-1341.
  • Karageorgiou, E., & Miller, B. L. (2014, Apr). Frontotemporal lobar degeneration: a clinical approach. Seminars in Neurology, 34(2), 189-201.
  • Krueger, C. E., Dean, D. L., Rosen, H. J., Halabi, C., Weiner, M., Miller, B. L., & Kramer, J. H. (2010, Jan-Mar). Longitudinal rates of lobar atrophy in frontotemporal dementia, semantic dementia, and Alzheimer’s disease. Alzheimer Disease and Associated Disorders, 24(1), 43-48.
  • Lanata, S. C., & Miller, B. L. (2016, May). The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry. Journal of Neurology, Neurosurgery, and Psychiatry, 87(5), 501-511.
  • Liljegren, M., Naasan, G., Temlett, J., Perry, D. C., Rankin, K. P., Merrilees, J., Grinberg, L. T., Seeley, W. W., Englund, E., & Miller, B. L. (2015, Mar). Criminal behavior in frontotemporal dementia and Alzheimer disease. JAMA Neurology, 72(3), 295-300.
  • Liu, A. J., Staffaroni, A. M., Rojas-Martinez, J. C., Olney, N. T., Alquezar-Burillo, C., Ljubenkov, P. A., La Joie, R., Fong, J. C., Taylor, J., Karydas, A., Ramos, E. M., Coppola, G., Boxer, A. L., Rabinovici, G. D., Miller, B. L., & Kao, A. W. (2020). Association of Cognitive and Behavioral Features Between Adults With Tuberous Sclerosis and Frontotemporal Dementia. JAMA Neurology, 77(3), 358-366.
  • Ljubenkov, P. A., Staffaroni, A. M., Rojas, J. C., Allen, I. E., Wang, P., Heuer, H., Karydas, A., Kornak, J., Cobigo, Y., Seeley, W. W., Grinberg, L. T., Spina, S., Fagan, A. M., Jerome, G., Knopman, D., Boeve, B. F., Dickerson, B. C., Kramer, J., Miller, … Rosen, H. J. (2018, Oct). Cerebrospinal fluid biomarkers predict frontotemporal dementia trajectory. Annals of Clinical and Translational Neurology, 5(10), 1250-1263.
  • Merrilees, J., Klapper, J., Murphy, J., Lomen-Hoerth, C., & Miller, B. L. (2010, May 3). Cognitive and behavioral challenges in caring for patients with frontotemporal dementia and amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, 11(3), 298-302.
  • O’Connor, C. M., Clemson, L., Brodaty, H., Jeon, Y. H., Mioshi, E., & Gitlin, L. N. (2014, May). Use of the Tailored Activities Program to reduce neuropsychiatric behaviors in dementia: an Australian protocol for a randomized trial to evaluate its effectiveness. International Psychogeriatrics, 26(5), 857-869.
  • O’Connor, C. M., Clemson, L., Brodaty, H., Low, L. F., Jeon, Y. H., Gitlin, L. N., Piguet, O., & Mioshi, E. (2019, Feb). The tailored activity program (TAP) to address behavioral disturbances in frontotemporal dementia: a feasibility and pilot study. Disability and rehabilitation, 41(3), 299-310.
  • Olney, N. T., Spina, S., & Miller, B. L. (2017, May). Frontotemporal Dementia. Neurologic Clinics, 35(2), 339-374.
  • Perry, D. C., Brown, J. A., Possin, K. L., Datta, S., Trujillo, A., Radke, A., Karydas, A., Kornak, J., Sias, A. C., Rabinovici, G. D., Gorno-Tempini, M. L., Boxer, A. L., De May, M., Rankin, K. P., Sturm, V. E., Lee, S. E., Matthews, B. R., Kao, A. W., Vossel, … Seeley, W. W. (2017, Dec 1). Clinicopathological correlations in behavioural variant frontotemporal dementia. Brain, 140(12), 3329-3345.
  • Perry, D. C., Sturm, V. E., Seeley, W. W., Miller, B. L., Kramer, J. H., & Rosen, H. J. (2014, Jun). Anatomical correlates of reward-seeking behaviours in behavioural variant frontotemporal dementia. Brain, 137(Pt 6), 1621-1626.
  • Possin, K., Laluz, V., Alcantar, O., Miller, B., & Kramer, J. (2010, 10/01). Distinct Neuroanatomical Substrates and Cognitive Mechanisms of Figure Copy Performance in Alzheimer’s Disease and Behavioral Variant Frontotemporal Dementia. Neuropsychologia, 49, 43-48.
  • Pressman, P. S., & Miller, B. L. (2014, Apr 1). Diagnosis and management of behavioral variant frontotemporal dementia. Biological Psychiatry, 75(7), 574-581.
  • Ramanan, S., Bertoux, M., Flanagan, E., Irish, M., Piguet, O., Hodges, J. R., & Hornberger, M. (2017, Jan). Longitudinal Executive Function and Episodic Memory Profiles in Behavioral-Variant Frontotemporal Dementia and Alzheimer’s Disease. Journal of the International Neuropsychological Society, 23(1), 34-43.
  • Ranasinghe, K. G., Rankin, K. P., Lobach, I. V., Kramer, J. H., Sturm, V. E., Bettcher, B. M., Possin, K., Christine You, S., Lamarre, A. K., Shany-Ur, T., Stephens, M. L., Perry, D. C., Lee, S. E., Miller, Z. A., Gorno-Tempini, M. L., Rosen, H. J., Boxer, A., Seeley, W. W., Rabinovici, G. D., … Miller, B. L. (2016, Feb 16). Cognition and neuropsychiatry in behavioral variant frontotemporal dementia by disease stage. Neurology, 86(7), 600-610.
  • Ranasinghe, K. G., Rankin, K. P., Pressman, P. S., Perry, D. C., Lobach, I. V., Seeley, W. W., Coppola, G., Karydas, A. M., Grinberg, L. T., Shany-Ur, T., Lee, S. E., Rabinovici, G. D., Rosen, H. J., Gorno-Tempini, M. L., Boxer, A. L., Miller, Z. A., Chiong, W., DeMay, M., Kramer, J. H., Possin, … & Miller, B. L. (2016, Sep 1). Distinct Subtypes of Behavioral Variant Frontotemporal Dementia Based on Patterns of Network Degeneration. JAMA Neurology, 73(9), 1078-1088.
  • Rascovsky, K., Hodges, J. R., Knopman, D., Mendez, M. F., Kramer, J. H., Neuhaus, J., van Swieten, J. C., Seelaar, H., Dopper, E. G., Onyike, C. U., Hillis, A. E., Josephs, K. A., Boeve, B. F., Kertesz, A., Seeley, W. W., Rankin, K. P., Johnson, J. K., Gorno-Tempini, M. L., Rosen, H., Prioleau-Latham, C. E., … Miller, B. L. (2011, Sep). Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain, 134(Pt 9), 2456-2477.
  • Rohrer, J. D. (2012, Mar). Structural brain imaging in frontotemporal dementia. Biochim Biophys Acta, 1822(3), 325-332.
  • Rohrer, J. D., Geser, F., Zhou, J., Gennatas, E. D., Sidhu, M., Trojanowski, J. Q., Dearmond, S. J., Miller, B. L., & Seeley, W. W. (2010, Dec 14). TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia. Neurology, 75(24), 2204-2211.
  • Seo, S. W., Thibodeau, M. P., Perry, D. C., Hua, A., Sidhu, M., Sible, I., Vargas, J. N. S., Gaus, S. E., Rabinovici, G. D., Rankin, K. D., Boxer, A. L., Kramer, J. H., Rosen, H. J., Gorno-Tempini, M. L., Grinberg, L. T., Huang, E. J., DeArmond, S. J., Trojanowski, J. Q., Miller, B. L., & Seeley, W. W. (2018, Mar 20). Early vs late age at onset frontotemporal dementia and frontotemporal lobar degeneration. Neurology, 90(12), e1047-e1056.
  • Staffaroni, A. M., Bajorek, L., Casaletto, K. B., Cobigo, Y., Goh, S. M., Wolf, A., Heuer, H. W., Elahi, F. M., Ljubenkov, P. A., Dever, R., Kornak, J., Appleby, B., Bove, J., Bordelon, Y., Brannelly, P., Brushaber, D., Caso, C., Coppola, G., Dheel, C., … Rosen, H. J. (2020, Jan). Assessment of executive function declines in presymptomatic and mildly symptomatic familial frontotemporal dementia: NIH-EXAMINER as a potential clinical trial endpoint. Alzheimers & Dementia: the journal of the Alzheimer’s Association, 16(1), 11-21.
  • Staffaroni, A. M., Ljubenkov, P. A., Kornak, J., Cobigo, Y., Datta, S., Marx, G., Walters, S. M., Chiang, K., Olney, N., Elahi, F. M., Knopman, D. S., Dickerson, B. C., Boeve, B. F., Gorno-Tempini, M. L., Spina, S., Grinberg, L. T., Seeley, W. W., Miller, B. L., Kramer, J. H., … Rosen, H. J. (2019). Longitudinal multimodal imaging and clinical endpoints for frontotemporal dementia clinical trials. Brain, 142(2), 443-459.
  • Takeda, A., Sturm, V. E., Rankin, K. P., Ketelle, R., Miller, B. L., & Perry, D. C. (2019, Jul-Sep). Relationship Turmoil and Emotional Empathy in Frontotemporal Dementia. Alzheimer Disease & Associated Disorders, 33(3), 260-265.
  • Valente, E. S., Caramelli, P., Gambogi, L. B., Mariano, L. I., Guimaraes, H. C., Teixeira, A. L., & de Souza, L. C. (2019, Apr 1). Phenocopy syndrome of behavioral variant frontotemporal dementia: a systematic review. Alzheimers Research and Therapy, 11(1), 30.
  • Younes, K., & Miller, B. L. (2020, 2020/06/01/). Frontotemporal Dementia: Neuropathology, Genetics, Neuroimaging, and Treatments. Psychiatric Clinics of North America, 43(2), 331-344.
  • Younes, K., & Miller, B. L. (2020, 2020/06/01/). Neuropsychiatric Aspects of Frontotemporal Dementia. Psychiatric Clinics of North America, 43(2), 345-360.