Abstract Details

Title Non-Ataxic Symptoms Contribute to Gait Dysfunction in Spinocerebellar Ataxias

Topic Movement Disorders

Presentation(s) (-)

Poster/Presentation
Number 009

Objective

Background SCAs are complex and heterogeneous neurodegenerative diseases. While SCAs are characterized by ataxia, they also include non-ataxic symptoms. The presence and magnitude of these symptoms, and the degree to which non-ataxic symptoms contribute to gait dysfunction in SCAs, are not well known.

Design/Methods 26 patients with SCA were rated using the SARA (Scale for the Assessment and Rating of Ataxia), and received general neurological examinations from movement disorders and neuromuscular experts. Eighteen patients received nerve conduction studies (NCS). No patients were diabetic or abused alcohol, and there were no known patients with vitamin deficiencies or comorbid conditions that would cause gait dysfunction.

Results Diagnoses of participants were as follows: SCA1=1, SCA2=2, SCA3=5, SCA6=2, SCA7=1, SCA13=1, SCA14=1; sporadic SCA=13. Moderate impairment was noted with gait and balance (=9.90 (3% of maximum possible), speech (=1.46 (37% of maximum possible), limb coordination (=10.44 (39% of maximum possible) and spasticity (=1.92 (24% of maximum possible). Mild to moderate lower limb spasticity was noted in 81% of patients. Decreased lower limb vibratory sensation was noted in 58% (15 of 26) of patients. NCS was suggestive or showed evidence of peripheral neuropathy in the vast majority of these patients (83%). Thirty percent of patients had mild lower extremity weakness. Worsening symptoms of spasticity, weakness, vibratory and position sense, and pin sensibility were strongly correlated with higher 25' walk times (r=0.57) and the SARA total score (r=0.54).

Conclusions Non-ataxia symptoms, including spasticity, sensory,and motor dysfunction contribute to gait dysfunction in SCAs. Patients should be assessed in a systematic fashion to identify these symptoms. Further research is needed to determine the impact of treating non-ataxic symptoms on the overall function of patients with ataxia.

Authors/Disclosures
Tuan H. Vu, MD (University of South Florida) PRESENTER	Dr. Vu has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Dianthus. Dr. Vu has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ImmunAbs. Dr. Vu has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ARGENX. Dr. Vu has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Vu has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Vu has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen (Horizon). Dr. Vu has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Johnson & Johnson. Dr. Vu has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Vu has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for CSL Behring. Dr. Vu has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Argenx. The institution of Dr. Vu has received research support from CSL Behring. The institution of Dr. Vu has received research support from Alexion. The institution of Dr. Vu has received research support from RA Pharma/UCB. The institution of Dr. Vu has received research support from Mass Gen Hospital/Healy Platform Study. The institution of Dr. Vu has received research support from ARGENX. The institution of Dr. Vu has received research support from Horizon/Amgen. The institution of Dr. Vu has received research support from Woolsey Pharma. The institution of Dr. Vu has received research support from CSL Behring. The institution of Dr. Vu has received research support from Cartesian Therapeutics. The institution of Dr. Vu has received research support from Janssen/Johnson & Johnson. The institution of Dr. Vu has received research support from Immunovant. The institution of Dr. Vu has received research support from Regeneron. The institution of Dr. Vu has received research support from Dianthus. The institution of Dr. Vu has received research support from COUR.
Kelly Sullivan, PhD (Georgia Southern University)	Dr. Sullivan has received intellectual property interests from a discovery or technology relating to health care.
	No disclosure on file
Israt Jahan, MD (Parkinson's &Neurology Clinic, LLC)	No disclosure on file
	No disclosure on file
Joseph Staffetti, MD (University of South Florida)	Joseph Staffetti has nothing to disclose.
Clifton L. Gooch, MD, FAAN (University of South Florida)	Dr. Gooch has received publishing royalties from a publication relating to health care. Dr. Gooch has received publishing royalties from a publication relating to health care.
Patricia Greenstein, MD (Beth Israel Deaconess Medical Center)	Dr. Greenstein has nothing to disclose.
Theresa A. Zesiewicz, MD (University of South Florida)	Dr. Zesiewicz has received personal compensation for serving as an employee of Medscape. Dr. Zesiewicz has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Lexeo. Dr. Zesiewicz has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Steminent. Dr. Zesiewicz has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Larimar. Dr. Zesiewicz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biohaven. Dr. Zesiewicz 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. Zesiewicz has received intellectual property interests from a discovery or technology relating to health care. Dr. Zesiewicz has received intellectual property interests from a discovery or technology relating to health care. Dr. Zesiewicz has received intellectual property interests from a discovery or technology relating to health care.
Antonio M. Omuro, MD, FAAN (Stanford University)	Dr. Omuro has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ono Therapeutics. Dr. Omuro has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Telix. Dr. Omuro has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Curevac. The institution of Dr. Omuro has received research support from NIH. The institution of Dr. Omuro has received research support from Arcus Biosciences. The institution of Dr. Omuro has received research support from Denovo Biopharma. The institution of Dr. Omuro has received research support from Ono Pharmaceutical. The institution of Dr. Omuro has received research support from Servier. The institution of Dr. Omuro has received research support from Nanopharmaceuticals. The institution of Dr. Omuro has received research support from Denovo.

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