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Abstract Details

Increased Prevalence of Developmental Stuttering Across lvPPA, nfvPPA, and CBS Spectrum Degenerative Disorders
Aging, Dementia, and Behavioral Neurology
Aging and Dementia Posters (7:00 AM-5:00 PM)
021
To investigate early-life developmental differences within nonfluent variant primary progressive aphasia (nfvPPA) and related disorders.
Increased rates of language-based developmental disorders have been observed within primary progressive aphasia (PPA), notably in logopenic variant PPA (lvPPA). A higher rate of non-right-handedness has been described in semantic variant PPA (svPPA). Specific developmental associations with nfvPPA have eluded us to date.
Clinical histories across nfvPPA (n=155) and related disorders, corticobasal syndrome (CBS) (n=100) and progressive supranuclear palsy (PSP) (n=138), were compared to svPPA (n=190), lvPPA (n=216), posterior cortical atrophy (PCA) (n=113), early-onset Alzheimer’s disease (EOAD) (n=555), and late-onset Alzheimer’s disease (LOAD) (n=661) for the presence of speech and language-based learning differences.
19.4% lvPPA possessed a history of either a speech or language-based developmental disorder. Splitting these up into language-based and motor-speech developmental disorders, revealed statistical elevations in both compared to general population estimates (15.3% vs. 10% and 4.2% vs. 0.72%, for developmental dyslexia and developmental stuttering respectively). PCA, EOAD, LOAD, and svPPA lacked elevations in either of these developmental disabilities. 11.0% nfvPPA possessed a history of either a speech or language-based developmental disorder, 6.5% of which were dyslexia, and 4.5% were stuttering, reflecting a select increase prevalence of stuttering. Only 1.4% PSP reported histories of speech or language-based developmental disorders compared to 20% CBS, half of which were dyslexia and half stuttering. The amount of stuttering within CBS reflects twice that observed in lvPPA or nfvPPA and is 14-fold higher than expected.
Across Alzheimer’s and frontotemporal lobar degenerative spectrum disorders we confirmed the specific association between developmental dyslexia and lvPPA and captured novel increases in motor-speech developmental disorders across lvPPA, nfvPPA, and CBS. These findings add substantial support to the hypothesis that domain-specific developmental differences associate with domain-specific degenerative disorders, with implications for neurodegenerative disease prediction and prevention.
Authors/Disclosures
Zachary Miller, MD (UCSF Memory and Aging Center)
PRESENTER 		Dr. Miller has nothing to disclose.
Rian L. Bogley (UCSF) Mr. Bogley has nothing to disclose.
No disclosure on file
No disclosure on file
Michael G. Erkkinen, MD (Brigham and Women's Hospital) Dr. Erkkinen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen.
David Perry, MD The institution of Dr. Perry has received research support from NIH/NIA.
Salvatore Spina, MD (UCSF Memory and Aging Center) Dr. Spina has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Techspert.io. Dr. Spina has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lumanity. Dr. Spina has received research support from Tau Consortium. Dr. Spina has received research support from Chan Zuckerberg Initiative, LLC. Dr. Spina has received research support from Bluefield Project to Cure FTD. Dr. Spina has received research support from NIH. Dr. Spina has received research support from Larry L. Hillblom Foundation. Dr. Spina has received personal compensation in the range of $500-$4,999 for serving as a Invited Speaker with Peking Union Medical Center Hospital.
Lea Grinberg Lea Grinberg has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Curasen Inc. The institution of Lea Grinberg has received research support from NIH. The institution of Lea Grinberg has received research support from Eli Lilly. The institution of Lea Grinberg has received research support from BrightFouus. The institution of Lea Grinberg has received research support from Rainwater Charity Foundation.
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 Â鶹´«Ã½Ó³»­ interests or activities. Dr. Rosen has a non-compensated relationship as a consultant with Alchemab that is relevant to Â鶹´«Ã½Ó³»­ interests or activities.
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.
No disclosure on file
Gil D. Rabinovici, MD, FÂ鶹´«Ã½Ó³»­ (UCSF Memory & Aging Center) Dr. Rabinovici has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Eli Lilly. Dr. Rabinovici has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alector. Dr. Rabinovici has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Merck. Dr. Rabinovici has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Rabinovici has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. Dr. Rabinovici has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novo Norodisk. Dr. Rabinovici has received personal compensation in the range of $500-$4,999 for serving as a Consultant for C2N. Dr. Rabinovici has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Johnson & Joihnson. Dr. Rabinovici has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Peerview. Dr. Rabinovici has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Medscape. Dr. Rabinovici 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 JAMA Neurology. Dr. Rabinovici 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 JAMA. The institution of Dr. Rabinovici has received research support from NIH. The institution of Dr. Rabinovici has received research support from American College of Radiology. The institution of Dr. Rabinovici has received research support from Alzheimer's Association. The institution of Dr. Rabinovici has received research support from Rainwater Charitable Foundation. Dr. Rabinovici has received personal compensation in the range of $500-$4,999 for serving as a Topic Chair, Course Director and teacher with Â鶹´«Ã½Ó³»­. Dr. Rabinovici has received personal compensation in the range of $500-$4,999 for serving as a Grant reviewer with NIH. Dr. Rabinovici has received personal compensation in the range of $500-$4,999 for serving as a Invited speaker with ANA.
Bruce L. Miller, MD, FÂ鶹´«Ã½Ó³»­ (University of California, San Francisco) Dr. Miller has nothing to disclose.
Maria Luisa Gorno Tempini, MD, PhD (UCSF Memory and Aging Center) The institution of Dr. Gorno Tempini has received research support from the NIH.