Abstract Details

Title Hypergraphia in Semantic Variant PPA

Topic Behavioral Neurology

Presentation(s) P05 - (-)

Poster/Presentation
Number 109

Objective

Background BACKGROUND: The semantic variant of primary progressive aphasia (svPPA) manifests with distinct features of semantic language impairment and behavioral abnormalities arising from anterior temporal and ventromedial frontal damage. Hypergraphia, extensive and often compulsive writing behavior, has been previously observed in patients with temporal lobe epilepsy and right hemisphere stroke, but has never been described in patients with temporal lobe degeneration.

Design/Methods DESIGN/METHODS: Symptoms were carefully described in three svPPA patients with hypergraphia, including neuropsychology, neuropsychiatry, neurologic exam, and 3T structural MRI scans. Voxel based morphometry (VBM) was performed to quantify brain atrophy patterns in the three patients against age-matched healthy controls.

Results RESULTS: All three patients displayed qualitatively similar new-onset compulsive writing behavior . Despite progressive word-finding and naming difficulties, these patients consistently produced a large volume of writing, typically with impoverished content. Though at different levels of disease severity, all three svPPA patients showed remarkable sparing in the left lateral temporal lobe (superior and middle gyrii ), while the medial temporal cortex (amygdala, limbic cortex) was uniformly atrophic.

Conclusions CONCLUSIONS: These findings suggest that in patients with temporal lobe degeneration, sparing of left lateral temporal language-associated regions, in combination with atrophy of medial temporal limbic structures involved in emotion regulation and reward, may lead to release of excessive writing behavior. While only a subset of patients with svPPA develop hypergraphic tendencies during disease, further analysis of specific brain-behavior correlates in larger groups is warranted to elucidate this phenomenon.

Authors/Disclosures
PRESENTER	No disclosure on file
Katherine P. Rankin, PhD (University of California San Francisco)	The institution of Dr. Rankin has received research support from Marcus Foundation.
Baber Khan, MD (Riverhills Neuroscience)	Dr. Khan has nothing to disclose.
Raman Sankar, MD, PhD, FAAN (University of California Los Angeles)	Dr. Sankar has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB. Dr. Sankar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Takeda. Dr. Sankar has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Jazz Pharmaceuticals. Dr. Sankar has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for UCB. Dr. Sankar has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Neurelis. Dr. Sankar has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for SK Life Sciences. Dr. Sankar has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for LivaNova. Dr. Sankar has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Eisai. Dr. Sankar has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for BioMarin. Dr. Sankar has received publishing royalties from a publication relating to health care. Dr. Sankar has received personal compensation in the range of $500-$4,999 for serving as a Grant Reviewer with NINDS, NIH.
	No disclosure on file
Zachary Miller, MD (UCSF Memory and Aging Center)	Dr. Miller has nothing to disclose.
Kavita Nair, PhD, FAAN (University of Colorado)	Dr. Nair has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bristol Meyers Squibb. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Nair has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for PhRMA Foundation. Dr. Nair has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genentech. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. Dr. Nair has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for MJH Lifesciences. Dr. Nair has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Anylam . Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Applied Patient Experience. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Esai. Dr. Nair has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for MHJ Life Sciences. Dr. Nair has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Sanofi-Genzyme. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as an officer or member of the Board of Directors for ICER. The institution of Dr. Nair has received research support from Genentech. The institution of Dr. Nair has received research support from Novartis. The institution of Dr. Nair has received research support from Genentech. The institution of Dr. Nair has received research support from Phrma Foundation. The institution of Dr. Nair has received research support from Bristol Meyers Squibb. The institution of Dr. Nair has received research support from Novartis. The institution of Dr. Nair has received research support from NINDS. The institution of Dr. Nair has received research support from Sanofi Genzyme. The institution of Dr. Nair has received research support from Kyverna Therapeutcis. Dr. Nair has a non-compensated relationship as a Board Member with Center for Improving Value in Healthcare that is relevant to AAN interests or activities.
Bruce L. Miller, MD, FAAN (University of California, San Francisco)	Dr. Miller has nothing to disclose.

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