Abstract Details

Title Identification of an Atypical Variant of Logopenic Progressive Aphasia

Topic Behavioral Neurology

Presentation(s) P06 - (-)

Poster/Presentation
Number 074

Objective

Background BACKGROUND: lvPPA is a clinical syndrome characterized by fluent speech, phonological errors, and difficulty with repetition. It is unclear how well neuropsychological function beyond the verbal domain and neuroimaging abnormalities correlate with aphasia severity and whether they represent good biomarkers of disease progression in lvPPA. In addition, it is unclear whether aphasia severity correlates well to disease duration. Understanding the relationships between these features of lvPPA is important for determining patient prognosis.

Design/Methods DESIGN/METHODS: We prospectively recruited 21 subjects who met clinical criteria for lvPPA. All subjects completed comprehensive speech-language and neuropsychological evaluations and 3T MRI. Aphasia severity was measured using the Western Aphasia Battery Aphasia Quotient (WAB AQ). A neurocognitive global z-score was calculated based on averaging z-scores from each cognitive domain. Volume of the left temporoparietal cortex was measured using atlas-based parcellation. Pair-wise correlations were assessed among variables, and cluster analysis used to identify lvPPA subtypes based on the WAB AQ and disease duration.

Results RESULTS: There were significant correlations between aphasia severity and neurocognitive severity as well as temporoparietal atrophy, but not disease duration. Cluster analysis identified three lvPPA variants: (1) subjects with mild aphasia and short disease duration (mild typical lvPPA); (2) subjects with mild aphasia and long disease duration (mild atypical lvPPA); and, (3) subjects with severe aphasia and relatively long disease duration (severe typical lvPPA). All three variants showed temporoparietal atrophy, with the atypical group showing the least atrophy despite the longest disease duration. The atypical group also showed mild neuropsychological impairment.

Conclusions CONCLUSIONS: We identified a distinct atypical variant of lvPPA with mild aphasia and neuropsychological impairment despite long disease duration, which may represent a slowly progressive variant of lvPPA.

Authors/Disclosures
Mary M. Machulda, PhD (Mayo Clinic) PRESENTER	The institution of Dr. Machulda has received research support from NIH.
Jennifer Whitwell, PhD (Mayo Clinic)	The institution of Dr. Whitwell has received research support from NIH.
	No disclosure on file
	No disclosure on file
Pamela Dean (Mayo Clinic)	No disclosure on file
Matthew Senjem (Mayo Clinic)	Matthew Senjem has received stock or an ownership interest from Align Technology, Inc.. Matthew Senjem has received stock or an ownership interest from Inovio Biomedical Corp.. Matthew Senjem has received stock or an ownership interest from Johnson & Johnson. Matthew Senjem has received stock or an ownership interest from Mesa Laboratories, Inc.. Matthew Senjem has received stock or an ownership interest from Nvidia Inc.. Matthew Senjem has received stock or an ownership interest from LHC Group, Inc.. Matthew Senjem has received stock or an ownership interest from Natus Medical Incorporated. Matthew Senjem has received stock or an ownership interest from Varex Imaging Corporation. Matthew Senjem has received personal compensation in the range of $100,000-$499,999 for serving as a IT Technical Specialist II with Mayo Clinic.
Clifford R. Jack, Jr., MD (Mayo Clinic)	The institution of Dr. Jack has received research support from NIH. The institution of Dr. Jack has received research support from Alexander Family Alzheimer's Disease Research Professorship of the Mayo Clinic.
Keith A. Josephs, Jr., MD, FAAN (Mayo Clinic)	Dr. Josephs has nothing to disclose.
Theresa A. Zesiewicz, MD (University of South Florida)	Dr. Zesiewicz has received personal compensation for serving as an employee of Medscape. Dr. Zesiewicz has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Lexeo. Dr. Zesiewicz has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Steminent. Dr. Zesiewicz has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Larimar. Dr. Zesiewicz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biohaven. Dr. Zesiewicz 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. Zesiewicz has received intellectual property interests from a discovery or technology relating to health care. Dr. Zesiewicz has received intellectual property interests from a discovery or technology relating to health care. Dr. Zesiewicz has received intellectual property interests from a discovery or technology relating to health care.

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