Hand Preference and Learning Disability Differentially Distribute across PPA Variants (P03.093)

OBJECTIVE: To describe developmental characteristics of handedness and language learning disability in Primary Progressive Aphasia. BACKGROUND: Primary Progressive Aphasia consists of 3 variants - logopenic, nonfluent, and semantic. Each subtype correlates with distinct anatomical involvement and typically with discrete underlying pathology. Prior literature on the development of PPA suggests a correlation with neurodevelopment as evidenced by increased rate of learning disability among patients with PPA and 2 cases of left hemicranial hypoplasia in semantic variant PPA. DESIGN/METHODS: We screened the UCSF Memory and Aging Center9s PPA cohort for hand preference and history of LD. The study included logopenic variant PPA (lvPPA), n=47; nonfluent variant PPA (nfvPPA), n=54; semantic variant (svPPA), n=96. An independent sample of svPPA, n=36, provided by Mayo Clinic Jacksonville and of PSP, n=107, obtained from UCSF were screened for solely for handedness. RESULTS: There was nearly twice the prevalence of nRH in the svPPA cohort, half the prevalence of nRH in nfvPPA, and nRH consistent with handedness in the general population in lvPPA. These handedness findings were corroborated in our independent groups. In the lvPPA group there was a much greater likelihood of LD in comparison to the svPPA and nfvPPA groups. Within the lvPPA group patients with LD were younger and had increased global functioning at presentation than lvPPA without LD. CONCLUSIONS: A unique pattern of hand preference and history of language learning disability appears to correlate strongly with each subtype of PPA. This further strengthens previous findings in PPA and may provide new insights into how neurodevelopment affects neurodegenerative disease susceptibility. Supported by: National Institutes of Health (grants P01 AG19724, P50 AG023501, P50 AG1657303, P50AG16574, R01-AG032306, and R01 NS050915-05A1) and its contents are solely the responsibility of the authors and do not necessarily represent the official views of the National Institute on Aging or NIH. Additional funds include the Consortium for Frontotemporal Dementia Research, the NSF Graduate Research Fellowship, and the Tau Research Consortium. Disclosure: Dr. Miller has nothing to disclose. Dr. Rankin has nothing to disclose. Dr. Mandelli has nothing to disclose. Dr. Henry has nothing to disclose. Dr. Wilson has nothing to disclose. Dr. Babiak has nothing to disclose. Dr. Frazier has nothing to disclose. Dr. Bettcher has nothing to disclose. Dr. Wu has nothing to disclose. Dr. Block has nothing to disclose. Dr. Ketelle has nothing to disclose. Dr. Edwards has nothing to disclose. Dr. Heggeli has nothing to disclose. Dr. Rosen has nothing to disclose. Dr. Kramer has nothing to disclose. Dr. Rabinovici has received personal compensation for activities with Eli Lilly & Company......Dr. Rabinovici has received research support from Avid Radiopharmaceuticals and Eli Lilly & Company. Dr. Graff-Radford has received personal compensation for activities with Codman. Dr. Graff-Radford has received personal compensation in an editorial capacity for the Neurologist. Dr. Graff-Radford has received research support from Janssen, Pfizer Pharmaceuticals, Medivation, Forrest, and Allon. Dr. Seeley has received personal compensation for activities with Korea Novartis, Summer Street Research Partners and Bristol-Myers Squibb for speaker, consulting and scientific advisory boards. Dr. Miller has received personal compensation for activities with Allon Therapeuctics, Inc. and TauRx Therapeutics, Ltd. Dr. Miller has received research support from Novartis. Dr. Gorno Tempini has nothing to disclose.