Abstract Details

Title Can Clinical Features Predict Tau Pathology in Patients with Behavioral Variant Frontotemporal Dementia (bvFTD)?

Topic Aging and Dementia

Presentation(s) P05 - (-)

Poster/Presentation
Number 101

Objective

Background BACKGROUND: Although characterized by behavior changes and frontotemporal atrophy, bvFTD is pathologically heterogeneous. With the development of tau agents for the treatment of FTD, it is increasingly important to establish underlying tau pathology during life. Therefore, we attempted to identify early clinical predictors of tau pathology in a multi-site sample of autopsy-confirmed bvFTD patients.

Design/Methods DESIGN/METHODS: 146 cases from the International bvFTD Criteria Consortium (FTDC) database were included. Cases met Possible bvFTD criteria at presentation and had FTLD pathology at autopsy. Chi-squared analyses compared early behavioral and cognitive features in tau positive (tau+) and tau negative (non-tau) cases.

Results RESULTS: 56 cases (38%) were classified as tau+ and 90 (62%) as non-tau. A higher proportion of non-tau cases presented with a neuropsychological profile consistent with bvFTD (i.e., executive/generation deficits with relative sparing of memory and visuospatial functions: tau+=69%, non-tau=84%, X2=4.1, p<.05). This may be driven in part by a high frequency of memory (31%) and visuospatial dysfunction (31%) in patients with tau genetic mutations (n=13). No significant group differences were observed for gender distribution (tau+=62% male, non-tau=56% male), age-at-onset (tau+=56yrs, non-tau=57yrs), or initial MMSE scores (tau+=20.3, non-tau=22.8). Frequency rates of core behavioral symptoms did not differ between groups: early disinhibition (tau+=86%, non-tau=86%), apathy/inertia (tau+=93%, non-tau=89%), loss of empathy (tau+=79%, non-tau=87%), compulsive/perseverative behaviors (tau+=81%, non-tau=76%), or hyperorality (tau+=68%, non-tau=70%). Groups did not differ in the frequency of extrapyramidal symptoms or additional language impairments.

Conclusions CONCLUSIONS: Our findings suggest that prediction of tau pathology in bvFTD may be difficult based on demographic, clinical or behavioral features alone. Accurate prediction of tau pathology in bvFTD may ultimately depend on more detailed cognitive/behavioral testing coupled with imaging and biofluid biomarkers.

Authors/Disclosures
Katya Rascovsky (University of Pennsylvania Perelman School of Medicine) PRESENTER	No disclosure on file
	No disclosure on file
David S. Knopman, MD, FAAN (Mayo Clinic)	Dr. Knopman has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for DIAN TU study. The institution of Dr. Knopman has received research support from NIH.
Mario F. Mendez, MD, PhD, FAAN (VA Greater Los Angeles Healthcare System and UCLA)	Dr. Mendez has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Medical ��ɫ�� Speakers' Bureau. Dr. Mendez has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for UpToDate. The institution of Dr. Mendez has received research support from NIH. Dr. Mendez has received publishing royalties from a publication relating to health care.
Joel Kramer, PhD (UCSF Medical Center)	The institution of Dr. Kramer has received research support from tau consortium. Dr. Kramer has received publishing royalties from a publication relating to health care.
	No disclosure on file
John Van Swieten, MD	Dr. Van Swieten has nothing to disclose.
Harro Seelaar, MD, PhD (Erasmus University Medical Center)	Dr. Seelaar has nothing to disclose.
	No disclosure on file
Chiadi U. Onyike, MD (Johns Hopkins University SOM)	Dr. Onyike has received personal compensation for serving as an employee of Rainwater Foundation. Dr. Onyike has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Acadia Pharmaceuticals . Dr. Onyike has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Reata Pharmaceuticals. Dr. Onyike has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Goodell, DeVries, Leech & Dann. The institution of Dr. Onyike has received research support from Alector.
Stuart Peltz	No disclosure on file
Argye E. Hillis, MD, MA (Johns Hopkins Hospital)	Dr. Hillis has received personal compensation for serving as an employee of Johns Hopkins University. Dr. Hillis has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for American Heart Association. The institution of Dr. Hillis has received research support from NIH. Dr. Hillis has received publishing royalties from a publication relating to health care. Dr. Hillis has received personal compensation in the range of $500-$4,999 for serving as a NIDCD Council Member with NIH.
Keith A. Josephs, Jr., MD, FAAN (Mayo Clinic)	Dr. Josephs has nothing to disclose.
Bradley F. Boeve, MD, FAAN (Mayo Clinic)	Dr. Boeve has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Rainwater Charitable Foundation. The institution of Dr. Boeve has received research support from Alector. The institution of Dr. Boeve has received research support from EIP Pharma. The institution of Dr. Boeve has received research support from Transposon. The institution of Dr. Boeve has received research support from Cognition Therapeutics. Dr. Boeve has received publishing royalties from a publication relating to health care.
Andrew Kertesz, MD, FAAN (St Joseph's Hospital)	No disclosure on file
William W. Seeley, MD	Dr. Seeley has received personal compensation in the range of $500-$4,999 for serving as a Consultant for GLG Council. Dr. Seeley has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Guidepoint Global Consulting. Dr. Seeley has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BridgeBio. Dr. Seeley has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Seeley has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Lyterian Therapeutics. The institution of Dr. Seeley has received research support from NIH. The institution of Dr. Seeley has received research support from Rainwater Charitable Foundation. The institution of Dr. Seeley has received research support from Bluefield Project to Cure FTD. The institution of Dr. Seeley has received research support from Chan-Zuckerberg Initiative.
Katherine P. Rankin, PhD (University of California San Francisco)	The institution of Dr. Rankin has received research support from Marcus Foundation.
Julene K. Johnson	No disclosure on file
Maria Luisa Gorno Tempini, MD, PhD (UCSF Memory and Aging Center)	The institution of Dr. Gorno Tempini has received research support from the NIH.
Howard J. Rosen, MD (UCSF)	Dr. Rosen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eli Lilly . Dr. Rosen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alector. The institution of Dr. Rosen has received research support from NIH. The institution of Dr. Rosen has received research support from State of CA. Dr. Rosen has a non-compensated relationship as a Consultant with Prevail Therapeutics that is relevant to AAN interests or activities. Dr. Rosen has a non-compensated relationship as a consultant with Alchemab that is relevant to AAN interests or activities.
	No disclosure on file
	No disclosure on file
Christopher Kipps, MD, MBBS (Southampton University Hospitals NHS Foundation Trust)	No disclosure on file
	No disclosure on file
	No disclosure on file
Jonathan D. Rohrer (Dementia Research Centre)	Dr. Rohrer has nothing to disclose.
Martin N. Rossor, MD, FAAN (University College London)	No disclosure on file
	No disclosure on file
	No disclosure on file
Douglas Galasko, MD (Dept. of Neurosciences, UCSD)	Dr. Galasko has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Eisai. Dr. Galasko has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eli Lilly, Inc. Dr. Galasko has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche Diagnostics. Dr. Galasko has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cognition THeraprutics. Dr. Galasko has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Artery Therapeutics.
David P. Salmon, PhD (Univ of Calif-San Diego)	Dr. Salmon has nothing to disclose.
Sandra E. Black, MD, FAAN (Sunnybrook Health Science Center)	Dr. Black has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Hoffmann-La Roche. Dr. Black has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Black has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Hoffmann-La Roche. Dr. Black has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Black has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Eisai Limited . Dr. Black has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Eli Lilly. Dr. Black has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen. The institution of Dr. Black has received research support from Hoffmann-La Roche. The institution of Dr. Black has received research support from Biogen. The institution of Dr. Black has received research support from GE Healthcare. The institution of Dr. Black has received research support from Eli Lilly. The institution of Dr. Black has received research support from Genentech. The institution of Dr. Black has received research support from NovoNordisk. The institution of Dr. Black has received research support from UCB Biopharma. The institution of Dr. Black has received research support from Alkahest Inc. The institution of Dr. Black has received research support from University of Southern California - AHEAD 3-45 Study.
M. M. Mesulam, MD, FAAN (Cogn Neur & Alzheimer Center, NW Univ)	Dr. Mesulam has received publishing royalties from a publication relating to health care.
Sandra Weintraub, PhD, FAAN (Northwestern Mesulam Center for Cognitive Neurology and Alzheimer'S Disease)	Dr. Weintraub has nothing to disclose.
Bradford Dickerson, MD, FAAN	Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Acadia. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arkuda. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eisai. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lantheus. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lilly. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novo Nordisk. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Dr. Dickerson has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arkuda. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Acadia. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Lilly. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. Dr. Dickerson has received publishing royalties from a publication relating to health care. Dr. Dickerson has received publishing royalties from a publication relating to health care.
	No disclosure on file
Florence Pasquier, MD	Dr. Pasquier has nothing to disclose.
	No disclosure on file
	No disclosure on file
Yolande Pijnenburg (VU University Medical Center)	No disclosure on file
Tiffany W. Chow, MD, FAAN (Alector)	Dr. Chow has received personal compensation for serving as an employee of Alector, Inc.. Dr. Chow has stock in Alector, Inc.. Dr. Chow has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant on grant U19AG079774 with NIA.
Facundo Manes	No disclosure on file
Jordan Grafman, PhD (Cognitive Neuroscience Section)	Dr. Grafman has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. Dr. Grafman has received research support from NIH. Dr. Grafman has received publishing royalties from a publication relating to health care.
Stefano F. Cappa, MD	Dr. Cappa has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elservier. The institution of Dr. Cappa has received research support from Italian Minsitry of Health.
Morris Freedman, MD, FAAN (Baycrest Health Sciences)	No disclosure on file
Bruce L. Miller, MD, FAAN (University of California, San Francisco)	Dr. Miller has nothing to disclose.
Murray Grossman, MD, FAAN (University of Pennsylvania)	Dr. Grossman has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology. The institution of Dr. Grossman has received research support from NIH.

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