Abstract Details

Title Memory Performance in Asymptomatic and Prodromal Familial Frontotemporal Lobar Degeneration: Findings from the ALLFTD Consortium

Topic Aging, Dementia, and Behavioral Neurology

Presentation(s) P13 - Poster Session 13 (8:00 AM-9:00 AM)

Poster/Presentation
Number 7-001

Objective To assess performance on memory measures among asymptomatic and minimally symptomatic members of familial frontotemporal lobar degeneration (f-FTLD) kindreds.

Background Memory impairment is generally considered an exclusionary criterion for the diagnosis of overt behavioral variant frontotemporal dementia (bvFTD).

Design/Methods We analyzed performance across 7 measures in the ARTFL LEFFTDS Longitudinal Frontotemporal Lobar Degeneration (ALLFTD, ) Consortium protocol that assess memory functioning. The measures include the National Alzheimer’s Coordinating Center Uniform Data Set Version 3 – Craft Story Immediate Recall (CSImm) and Delayed Recall (CSDel), Benson Figure Delayed Recall (BenDel), and the California Verbal Learning Test – Learning (CVLearn), Immediate Recall (CVImm), Delayed Recall (CVDel), and Recognition (CVRecog). Testing was performed in asymptomatic [defined by global FTLD Clinical Dementia Rating (CDR) score of 0] non-mutation (AsymM-) and mutation (AsymM+) carriers and minimally symptomatic (defined by FTLD CDR=0.5) mutation carriers (SymM+) in kindreds with f-FTLD. Performance was considered abnormal (Abnl) for z-scores ≤-1.5.

Results Data from 255 AsymM- (mean age 47±13 years, 38% male), 181 AsymM+ (mean age 43±14 years, 45% male), and 36 SymM+ (mean age 56±12 years, 39% male) participants were analyzed. The frequencies of Abnl between AsymM- and AsymM+ for ≥2 tests (23.9% vs 26.4%), ≥4 tests (5.3% vs 7.5%) and ≥6 tests (0.4% vs 0.6%) were not different (p>0.05). The frequencies of Abnl between AsymM+ and SymM+ for ≥2 tests (26.4% vs 44.1%, p=0.038), ≥4 tests (7.5% vs 20.6%, p=0.018) and ≥6 tests (0.6% vs 8.8%, p=0.001) were significantly different. The frequencies of Abnl in the SymM+ group per memory measure were: CSImm 23.5%, CSDel 29.4%, BenDel 20%, CVLearn 22.9%, CVImm 26.5%, CVDel 29.4%, and CVRecog 29.4%.

Conclusions Among mutation carriers with prodromal bvFTD clinical features, a significant minority have impairment on memory measures. The presence of memory impairment should not preclude a diagnosis of prodromal bvFTD.

Authors/Disclosures
Bradley F. Boeve, MD, FAAN (Mayo Clinic) PRESENTER	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.
Julie A. Fields, PhD (Mayo Clinic)	The institution of Dr. Fields has received research support from National Institutes of Health. The institution of Dr. Fields has received research support from Patient-Centered Outcomes Research Institute.
Jeremy Syrjanen	Jeremy Syrjanen has nothing to disclose.
Walter Kremers	The institution of Walter Kremers has received research support from NIH. The institution of an immediate family member of Walter Kremers has received research support from NIH.
Adam Staffaroni	Adam Staffaroni has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alector. Adam Staffaroni has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Aviado Bio. Adam Staffaroni has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CervoMed. Adam Staffaroni has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Prevail Therepeutics/Eli Lilly. Adam Staffaroni has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Takeda. The institution of Adam Staffaroni has received research support from NIH. The institution of Adam Staffaroni has received research support from Bluefield Project To Cure FTD. The institution of Adam Staffaroni has received research support from AFTD/ALSA. Adam Staffaroni has received personal compensation in the range of $500-$4,999 for serving as a Scientific Advisory Board member with AADF.
Edward Huey	No disclosure on file
Katya Rascovsky (University of Pennsylvania Perelman School of Medicine)	No disclosure on file
	No disclosure on file
Leah K. Forsberg, PhD (Mayo Clinic)	Ms. Forsberg has nothing to disclose.
	No disclosure on file
Adam L. Boxer, MD, PhD (University of California, San Francisco)	An immediate family member of Dr. Boxer has received personal compensation for serving as an employee of Kaiser Permanente. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ono. Dr. Boxer has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Oscotec. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eli Lilly. Dr. Boxer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Arrowhead. Dr. Boxer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Neurocrine Biosciences. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Switch. Dr. Boxer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Arvinas. Dr. Boxer has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alector. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Boxer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Transposon. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen . Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Dr. Boxer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Boxer has stock in Alector. Dr. Boxer has stock in Arvinas. Dr. Boxer has stock in Neurovanda. The institution of Dr. Boxer has received research support from Biogen. The institution of Dr. Boxer has received research support from Eisai. The institution of Dr. Boxer has received research support from Regeneron. The institution of Dr. Boxer has received research support from NIH. The institution of Dr. Boxer has received research support from Bluefield Project-5. Dr. Boxer has received research support from Rainwater Charitable Foundation. Dr. Boxer has received research support from GHR Foundation. Dr. Boxer has received intellectual property interests from a discovery or technology relating to health care.
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.

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