Abstract Details

Title Unraveling globular glial tauopathy: a clinical and neuroimaging study

Topic Aging, Dementia, and Behavioral Neurology

Presentation(s) P7 - Poster Session 7 (8:00 AM-9:00 AM)

Poster/Presentation
Number 3-004

Objective To describe the clinical and neuroimaging characteristics of patients with autopsy-confirmed globular glial tauopathy (GGT) and compare brain atrophy patterns in GGT to atrophy patterns in patients with progressive supranuclear palsy-Richardson’s syndrome (PSP-RS) with autopsy-confirmed PSP pathology.

Background Clinical presentations in patients with GGT, a 4R tauopathy in the frontotemporal lobar degeneration spectrum, often overlap with syndromes linked to other 4R tauopathies, including PSP. Neuroimaging data are scarce and non-specific in GGT.

Design/Methods Brain bank at Mayo Clinic (MN, FL) was queried for GGT cases with ante mortem imaging available with the following imaging modalities included: MR (n=10), [18f]-FDG-PET (n=4), and tau-PET (n=1). Voxel-based analysis was performed comparing gray and white matter atrophy in patients with GGT to 10 age- and sex-matched PSP-RS patients with PSP. This study is funded by NIH grants: P30-AG62677, P50-AG016574, U19-AG63911, R35-AG11378, R01-AG041851.

Results Eleven GGT cases were identified: 9 (82%) female, median onset 65 years, median age at death 76 years. Six patients (55%) had positive family history. Most patients (55%) had speech and eye movement abnormalities, signs of pyramidal tract involvement, and parkinsonism. Primary progressive apraxia of speech was the most frequent initial diagnosis (36%), five patients (45%) evolved into Parkinson-plus syndrome, three patients met criteria for behavioral variant frontotemporal dementia (bvFTD). Asymmetric frontotemporal pattern of hypometabolism/atrophy was characteristic for most patients. Flortaucipir uptake was observed in areas of atrophy/hypometabolism. Compared to PSP, GGT patients had more gray and white matter atrophy in temporal lobes, particularly poles.

Conclusions Patients with GGT are likely to present with speech problems, have positive family history and evolve into a Parkinson-plus syndrome or bvFTD. Asymmetric frontotemporal atrophy/hypometabolism is the most frequent neuroimaging finding in GGT patients with greater temporal lobe atrophy compared to PSP. Tau-PET uptake may be a marker of underlying tau burden but equally likely reflective of regional neurodegeneration.

Authors/Disclosures
Marina Buciuc, MD PRESENTER	Dr. Buciuc has nothing to disclose.
Nha Trang Thu Pham (Mayo Clinic)	Nha Trang Thu Pham has received personal compensation for serving as an employee of Mayo Clinic.
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.
Farwa Ali, MD (Mayo Clinic)	Dr. Ali has nothing to disclose.
David S. Knopman, MD, FAAN (Mayo Clinic)	Dr. Knopman has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for DIAN TU study. The institution of Dr. Knopman has received research support from NIH.
Bradley F. Boeve, MD, FAAN (Mayo Clinic)	Dr. Boeve has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Rainwater Charitable Foundation. The institution of Dr. Boeve has received research support from Alector. The institution of Dr. Boeve has received research support from EIP Pharma. The institution of Dr. Boeve has received research support from Transposon. The institution of Dr. Boeve has received research support from Cognition Therapeutics. Dr. Boeve has received publishing royalties from a publication relating to health care.
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 .
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.
Dennis W. Dickson, MD (Mayo Clinic)	Dr. Dickson has nothing to disclose.
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.

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