Abstract Details

Title Cortical Atrophy in FTD/ALS Compared to FTD

Topic Behavioral Neurology

Presentation(s) P06 - (-)

Poster/Presentation
Number 057

Objective

Background BACKGROUND: FTD-ALS is a rare disease that embodies the spectrum of clinical manifestations and pathologic findings in FTD and ALS, involving symptoms in language, social/behavioral, executive, and motor domains. FTD-ALS is associated with TAR DNA-binding protein (TDP-43) cytoplasmatic inclusions and a recently discovered genetic mutation (hexanucleotide repeat expansion in C9ORF72). Diagnostic criteria, natural history and imaging characteristics of FTD-ALS are only beginning to be described, and only one prior paper compares FTD-ALS with FTD.

Design/Methods DESIGN/METHODS: 3.0 Tesla MRI scans were acquired from 5 FTD-ALS patients and 29 patients with FTD (including both behavioral variant FTD and Primary Progressive Aphasia), along with 34 controls. Quantitative cortical thickness analysis was performed.

Results RESULTS: Compared with controls, FTD-ALS patients demonstrated atrophy in all portions of the prefrontal cortex, precentral gyrus, anterior cingulate, and anterior temporal cortex. Compared with controls, FTD patients demonstrated atrophy in all portions of the prefrontal cortex, anterior cingulate, anterior insula, and anterior temporal cortex. Comparisons between the two disease groups are ongoing.

Conclusions CONCLUSIONS: In FTD-ALS, atrophy is prominent and frontal and anterior temporal cortical regions, in a pattern similar to that seen in FTD, and extends caudally to include the precentral gyrus. Further work will investigate whether cortical thickness in a subset of these regions in patients with FTD or ALS alone can predict the development of FTD-ALS.

Authors/Disclosures
Elena Ratti, MD (Takeda) PRESENTER	Dr. Ratti has received personal compensation for serving as an employee of Takeda. Dr. Ratti has received personal compensation for serving as an employee of Biogen. Dr. Ratti has stock in Takeda. Dr. Ratti has stock in Biogen.
Kimiko Domoto-Reilly, MD	Dr. Domoto-Reilly has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for MedBridge. The institution of Dr. Domoto-Reilly has received research support from Lawson Health Research Institute. The institution of Dr. Domoto-Reilly has received research support from NIH. The institution of Dr. Domoto-Reilly has received research support from Biogen. The institution of Dr. Domoto-Reilly has received research support from Lewy Body Dementia Association. The institution of Dr. Domoto-Reilly has received research support from Garvey Institute for Brain Health Solutions. The institution of Dr. Domoto-Reilly has received research support from Cognition Therapeutics. The institution of Dr. Domoto-Reilly has received research support from Takayama Family.
	No disclosure on file
Bradford Dickerson, MD, FAAN	Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Acadia. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arkuda. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eisai. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lantheus. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lilly. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novo Nordisk. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Dr. Dickerson has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arkuda. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Acadia. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Lilly. Dr. Dickerson has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. Dr. Dickerson has received publishing royalties from a publication relating to health care. Dr. Dickerson has received publishing royalties from a publication relating to health care.
Albert Saiz (Hospital Clinico De Barcelona)	Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janseen. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for novartis. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen.

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