Abstract Details

Title Olfactory Dysfunction as an Indicator of Cognitive and Motor Impairment Severity in Huntington’s Disease

Topic Movement Disorders

Presentation(s) P6 - Poster Session 6 (8:00 AM-9:00 AM)

Poster/Presentation
Number 3-006

Objective To explore the association between olfactory dysfunction and Huntington’s disease (HD) symptoms.

Background

Although olfactory dysfunction in HD gene expansion carriers (HDGECs) has been previously reported, its association with the severity of HD clinical symptomatology is yet to be investigated.

Design/Methods

Cross-sectional study with 41 HDGECs and 43 age- and gender-matched controls. The olfactory function was assessed using the 12-item Brief Smell Identification Test (BSIT). Participants completed the Unified Huntington's Disease Rating Scale (UHDRS) total motor score (TMS), UHDRS Total Functional Capacity (TFC), and Problem Behaviours Assessment (PBA-s). The cognitive tests included the Montreal Cognitive Assessment (MoCA), Symbol Digit Modalities Test (SDMT), Stroop Color and Word Test (SCWT), and Social Perception-Affect Naming.

Results HDGECs presented lower BSIT scores than controls, indicating a poorer ability to discriminate familiar odors (10.17±0.99 versus 7.12±2.19, p<0.0001). HDGECs also performed significantly worse than controls in all cognitive tests. Among HDGECs, higher BSIT scores were significantly associated with better scores in the motor, cognitive, and functional capacity scales: UHDRS-TMS (r_s= -0.574; p<0.001), MoCA (r_s= 0.538; p=0.0003), SDMT (r_s= 0.610; p<0.0001), SCWT interference (r_s= 0.502; p=0.0008), social perception (r_s= 0.413; p = 0.0073), and TFC (r_s= 0.569; p=0.0001). The same associations were not found among controls. We did not find any correlation between olfactory function and PBA-s scores.

Conclusions Olfactory dysfunction was associated with worse motor and cognitive symptoms in HDGECs. Our findings suggest that olfactory changes can be used as an indicator of the severity of HD. In the next step, we will investigate the association between olfactory dysfunction and HD progression in a longitudinal study.

Authors/Disclosures
Shayan A. Zadegan, MD PRESENTER	Dr. Zadegan has nothing to disclose.
Madilyn L. Feik	Miss Feik has nothing to disclose.
Hannah Coco	No disclosure on file
Andi Liang, MD (Washington University in St Louis, Department of Neurology)	Miss Liang has nothing to disclose.
Frank Ramirez	Mr. Ramirez has nothing to disclose.
Jorge E. Patino Murillas, MD (University of Cincinnati)	Dr. Patino Murillas has nothing to disclose.
Natalia Pessoa Rocha	Natalia Pessoa Rocha has nothing to disclose.
Erin Furr-Stimming, MD, FAAN (University of Texas Health Science Center-Houston)	Dr. Furr-Stimming has received personal compensation for serving as an employee of Help4HD International. Dr. Furr-Stimming has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Medscape. Dr. Furr-Stimming has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MedPage. Dr. Furr-Stimming has received personal compensation in the range of $500-$4,999 for serving as a Consultant for PTC Therapeutics. Dr. Furr-Stimming has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Wave Life Sciences. Dr. Furr-Stimming has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Latus Bio. Dr. Furr-Stimming has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Atalanta Therapeutics. Dr. Furr-Stimming has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Furr-Stimming has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for SkyHawk Therapeutics. Dr. Furr-Stimming has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis . Dr. Furr-Stimming has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Atalanta Therapeutics. The institution of Dr. Furr-Stimming has received research support from Roche/Genetech. The institution of Dr. Furr-Stimming has received research support from Uniqure. The institution of Dr. Furr-Stimming has received research support from CHDI. The institution of Dr. Furr-Stimming has received research support from Huntington Study Group/Neurocrine Bioscienes. The institution of Dr. Furr-Stimming has received research support from NIH/University of Iowa. The institution of Dr. Furr-Stimming has received research support from Sage Therapeutics. The institution of Dr. Furr-Stimming has received research support from HDSA. The institution of Dr. Furr-Stimming has received research support from Prilennia. Dr. Furr-Stimming has received publishing royalties from a publication relating to health care. Dr. Furr-Stimming has received publishing royalties from a publication relating to health care. Dr. Furr-Stimming has a non-compensated relationship as a Committee member with AAN UES Committee that is relevant to AAN interests or activities.

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