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Abstract Details

Clinical, Neuroimaging, and Neuropathologic Features of Primary Progressive Aphasia Lacking Core Features of Nonfluent and Semantic Variants
Aging, Dementia, and Behavioral Neurology
P9 - Poster Session 9 (11:45 AM-12:45 PM)
3-012
To examine clinical, neuroimaging, and neuropathologic features of primary progressive aphasia (PPA) lacking features of nonfluent/semantic variants and to provide practical additions to the 2011 consensus criteria for PPA.

Evidence has accumulated that the 2011 consensus criteria do not fully capture features of logopenic variant PPA (lvPPA/LPA).

This was a retrospective examination of data from 2 observational cohort studies where patients with PPA were prospectively recruited. Based on performance on 2 cardinal features (repetition and comprehension), patients were classified as: pure-LPA (poor repetition, acceptable comprehension), Wernicke-like (poor in both), anomic-like (acceptable in both), and transcortical sensory aphasia–like (TCSA-like) (acceptable repetition, poor comprehension).
The entire cohort consisted of 102 patients with PPA lacking features of nonfluent/semantic variants. The proportion of repetition-preserved PPA (anomic-like and TCSA-like) was more than double that of repetition-impaired PPA (pure-LPA and Wernicke-like) (73% vs 27%). Regarding clinical course (n=31), the anomic-like subgroup was a prodromal state of the pure-LPA or TCSA-like subgroup, whereas the pure-LPA and TCSA-like subgroups were a prodromal state of the Wernicke-like subgroup. There was left temporoparietal atrophy on MRI and/or hypometabolism on 18F-fluorodeoxyglucose-PET in all groups. Regarding pathologic diagnoses (n=23), 70% had Alzheimer disease (AD). The pure-LPA, Wernicke-like, and TCSA-like subgroups all showed AD pathology. Only 53% of the anomic-like subgroup had AD. The remaining 47% showed Pick disease (7%), frontotemporal lobar degeneration with TDP-43–immunoreactive pathology (20%), and Lewy body disease (20%).

This observed clinical heterogeneity reflects different time points/severities of the same disease process and hence can be reconceptualized as an AD-related aphasia spectrum, incorporating lvPPA and the 4 subgroups (pure-LPA, Wernicke-like, anomic-like, and TCSA-like). Specifying moderate/severe repetition deficits as a core feature of lvPPA in the 2011 consensus criteria can enhance its pathologic correlations. Recognizing progressive anomic aphasia (anomic-like) as an additional PPA variant could lessen pathologic heterogeneity of lvPPA.
Authors/Disclosures
Hiroyuki Watanabe, PhD
PRESENTER 		Dr. Watanabe has nothing to disclose.
Joseph Duffy The institution of Joseph Duffy has received research support from NIH. Joseph Duffy has received publishing royalties from a publication relating to health care.
Heather Clark The institution of Heather Clark has received research support from NIH. Heather Clark has received publishing royalties from a publication relating to health care.
Mary M. Machulda, PhD (Mayo Clinic) The institution of Dr. Machulda has received research support from NIH.
Jonathan Graff-Radford, MD, FAAN Dr. Graff-Radford has received personal compensation for serving as an employee of Mayo Clinic. Dr. Graff-Radford has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for NINDS/NIH. Dr. Graff-Radford 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. Graff-Radford 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 Open evidence . The institution of Dr. Graff-Radford has received research support from NIH. The institution of Dr. Graff-Radford has received research support from Eisai. The institution of Dr. Graff-Radford has received research support from Cognition therapeutics. Dr. Graff-Radford has received personal compensation in the range of $5,000-$9,999 for serving as a Faculty Member with IMPACT AD .
Nha Trang Thu Pham (Mayo Clinic) Nha Trang Thu Pham has received personal compensation for serving as an employee of Mayo Clinic.
Dennis W. Dickson, MD (Mayo Clinic) Dr. Dickson has nothing to disclose.
Val J. Lowe, MD (Mayo Clinic) Dr. Lowe has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for AVID Radiopharmaceutical. Dr. Lowe has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eisai Inc. The institution of Dr. Lowe has received research support from AVID Radiopharmaceuticals.
Jennifer Whitwell, PhD (Mayo Clinic) The institution of Dr. Whitwell has received research support from NIH.
Keith A. Josephs, Jr., MD, FAAN (Mayo Clinic) Dr. Josephs has nothing to disclose.