Abstract Details

Title Hippocampal and Medial Temporal Sclerosis in Behavioral Variant Frontotemporal Dementia

Topic Aging and Dementia

Presentation(s) S44 - (-)

Poster/Presentation
Number 007

Objective

Background Both HS and MTS are characterized by selective neuronal loss and astrocytic gliosis of the hippocampus and/or neighboring cortex and are associated with memory impairment. HS/MTS frequently coexist with dementia, and previous single-center neuropathological studies have demonstrated an association between HS/MTS and bvFTD, particularly in tau-negative cases with TDP-43 proteinopathies.

Design/Methods We identified patients with a clinical diagnosis of bvFTD (n=107) who were included in both the Neuropathology and Uniform Datasets maintained by the National Alzheimer's Coordinating Committee (NACC). We excluded 12 patients with ambiguous pathologies and 21 patients with predominant Alzheimer's disease pathology and focused our analyses on the remaining 74 patients with predominant frontotemporal lobar degeneration (FTLD) pathology. This cohort was subdivided by neuropathological findings into tau-negative (n=51) and tau-positive (n=23) groups. We compared the prevalence of HS, MTS, and memory impairment between groups.

Results The tau-positive group, comprised of cases with Pick's disease, progressive supranuclear palsy, FTLD and Parkinsonism with tau-positive or argyrophilic inclusions, and other tauopathies, was characterized by a higher prevalence of both HS (25%) and MTS (30%) than the tau-negative group (HS: 5%; MTS: 10%; both p's=0.02). However, the two groups had similar proportions of patients presenting with memory impairment on initial neuropsychological examination.

Conclusions Our results indicate that HS/MTS is more common in tau-positive than tau-negative bvFTD cases, and run counter to the hypothesis that HS/MTS is primarily associated with TDP-43 proteinopathies. These findings suggest that HS/MTS is an independent marker of neurodegeneration, associated with tau in some cases and TDP-43 in others, and most reflective of neuronal/synaptic loss and reactive gliosis.

Authors/Disclosures
Aditi A. Joshi, MD PRESENTER	No disclosure on file
Edmond Teng, MD, PhD (Genentech)	Dr. Teng has received personal compensation for serving as an employee of Genentech. An immediate family member of Dr. Teng has received personal compensation for serving as an employee of Evidation Health. Dr. Teng has stock in F. Hoffman-La Roche. An immediate family member of Dr. Teng has stock in Evidation Health. Dr. Teng has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Teng has received intellectual property interests from a discovery or technology relating to health care.
	No disclosure on file
	No disclosure on file
Mario F. Mendez, MD, PhD, FAAN (VA Greater Los Angeles Healthcare System and UCLA)	Dr. Mendez has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Medical ��ɫ�� Speakers' Bureau. Dr. Mendez has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for UpToDate. The institution of Dr. Mendez has received research support from NIH. Dr. Mendez has received publishing royalties from a publication relating to health care.
	No disclosure on file
Bernard M. Uitdehaag, MD, PhD, FAAN (Amsterdam University Medical Centers)	Dr. Uitdehaag has nothing to disclose.

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