Abstract Details

Title Mesial Temporal Sclerosis in Frontotemporal Lobar Degeneration: An Association with Age, Cerebrovascular Disease and TDP

Topic Aging and Dementia

Presentation(s) P05 - (-)

Poster/Presentation
Number 100

Objective

Background BACKGROUND: MTS, traditionally viewed as an epileptogenic phenomenon, has been of recent interest as a sequelae of neurodegeneration and a distinct dementing condition in the elderly. Its relationship to pathogenesis, evolution and clinical manifestation of FTLD is unclear.

Design/Methods DESIGN/METHODS: The pathology information system at SHSC was searched for autopsy cases from 2000-2012 with a diagnosis of FTLD who were assessed at our Cognitive Neurology Clinic (N = 52). H&E/LFB stained slides of bilateral hippocampi were examined for MTS. Immunohistochemistry was performed on the appropriate anatomical regions to document and stage other co-existing neurodegenerative phenomena including limbic TDP43 pathology, Alzheimer's pathology (beta-amyloid and AT8 tau), Lewy bodies (alpha-synuclein), argyrophilic grains (AT8 tau), and the presence of cerebrovascular disease was noted.

Results RESULTS: MTS was the most frequent co-existing neurodegenerative phenomena in this cohort of FTLD patients being present in 21/52 (42%) cases, and was associated with older age, cerebrovascular and TDP43 pathology. 15/21 (71%) cases with MTS had TDP43 immunopositivity; either a primary FTLD-TDP diagnosis (12/21) or co-existing mesiotemporal TDP43 inclusions (3/21). Of the 16 FTLD-TDP cases, 12 had MTS and the 4 without MTS were younger than 61 years of age. All of the FTLD-tau cases with limbic TDP43 pathology had MTS. Less than a third of cases with MTS had significant Alzheimer's phenomena. Whether the presence of MTS altered the 'accuracy' of clinical and radiological FTD classification will be explored.

Conclusions CONCLUSIONS: This study adds to the scant literature detailing MTS in FTLD; further study into the impact of co-existing neurodegenerative phenomena such as MTS on clinical phenotype of FTD is needed.

Authors/Disclosures
PRESENTER	No disclosure on file
Sandra E. Black, MD, FAAN (Sunnybrook Health Science Center)	Dr. Black has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Hoffmann-La Roche. Dr. Black has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Black has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Hoffmann-La Roche. Dr. Black 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. Black has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Eisai Limited . Dr. Black has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Eli Lilly. Dr. Black has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen. The institution of Dr. Black has received research support from Hoffmann-La Roche. The institution of Dr. Black has received research support from Biogen. The institution of Dr. Black has received research support from GE Healthcare. The institution of Dr. Black has received research support from Eli Lilly. The institution of Dr. Black has received research support from Genentech. The institution of Dr. Black has received research support from NovoNordisk. The institution of Dr. Black has received research support from UCB Biopharma. The institution of Dr. Black has received research support from Alkahest Inc. The institution of Dr. Black has received research support from University of Southern California - AHEAD 3-45 Study.
	No disclosure on file
Mario Masellis, MD (Sunnybrook Health Sciences Centre)	Dr. Masellis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arkuda Therapeutics. Dr. Masellis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ionis. Dr. Masellis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alector. Dr. Masellis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Wave Life Sciences. The institution of Dr. Masellis has received research support from Canadian Institutes of Health Research. The institution of Dr. Masellis has received research support from Ontario Brain Institute. The institution of Dr. Masellis has received research support from Weston Brain Institute. The institution of Dr. Masellis has received research support from Washington University. The institution of Dr. Masellis has received research support from Alector. Dr. Masellis has received publishing royalties from a publication relating to health care.
Olaf Stuve, MD, PhD, FAAN (UT Southwestern Medical Center)	Dr. Stuve has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Stuve has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Stuve 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 Therapeutic Advances in Neurological Diseases. Dr. Stuve has received research support from US Department of Veterans Affairs. Dr. Stuve has received research support from National Multiple Sclerosis Society (US). Dr. Stuve has received research support from Merck KGaA.
Patricia K. Coyle, MD, FAAN (SUNY At Stony Brook)	Dr. Coyle has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Accordant. Dr. Coyle has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. Dr. Coyle has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Sanofi Genzyme. Dr. Coyle has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. Dr. Coyle has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for GlaxoSmithKline. Dr. Coyle has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Horizon Therapeutics. The institution of Dr. Coyle has received research support from CorEvitas LLC. The institution of Dr. Coyle has received research support from Genentech/Roche. The institution of Dr. Coyle has received research support from NINDS. The institution of Dr. Coyle has received research support from Sanofi Genzyme. The institution of Dr. Coyle has received research support from Cleveland Clinic.

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