Abstract Details

Title A New Assessment Tool for Ataxia Syndromes: The "STAND" (Scale To Assess Ataxia and Neurologic Dysfunction)

Topic Movement Disorders

Presentation(s) (-)

Poster/Presentation
Number 001

Objective

Background Neurodegenerative ataxias, such as spinocerebellar ataxia (SCA) may include non-ataxic symptoms. There is a need for an assessment tool to better evaluate non-ataxic symptoms in patients suffering from neurodegenertive ataxias. The Scale for the Assessment and Rating of Ataxia (SARA) is a valid scale, but does not assess these symptoms. The "INAS" (Inventory of Non-Ataxia Symptoms) "counts" nonataxia symptoms, but does not rate them. We conducted a pilot evaluation of a new assessment tool called the "STAND" to better evaluate non-ataxic and ataxic symptoms.

Design/Methods The STAND is a semi-quantitative 109 point scale that tests gait, balance, speech, coordination, tone, peripheral motor, and sensory function. Two movement disorders neurologists administered the SARA and the STAND during one visit and a neuromuscular expert performed nerve conduction studies (NCS) in SCA and Friedreich's ataxia patients. Interrater reliability was examined using intraclass correlation coefficients (ICC). Correlation between STAND and SARA totals and sub-scores were tested using Spearman's correlation test. Internal consistency was assessed by Cronbach's ?.

Results Thirty-seven ataxic patients participated in the study to evaluate the scale, and 25 patients received NCS. The scale showed good internal consistency (Cronbach's ?=88). Ataxia components of the STAND were strongly correlated with the SARA (r for total ataxia score = 0.88). Interrater reliability for the overall scale was excellent (ICC=0.97) and was similar for sub-scales (gait and balance ICC=0.99; speech ICC=0.84; limb coordination ICC=0.91; tone ICC=0.76; peripheral motor system ICC=0.78; peripheral sensory ICC=0.87). Peripheral sensory scores correlated with the presence and degree of abnormalities noted by NCS (r=0.75).

Conclusions The STAND's quantification of non-ataxic neurologic symptoms should provide greater diagnostic and prognostic utility than scales that assess ataxic symptoms alone.

Authors/Disclosures
Theresa A. Zesiewicz, MD (University of South Florida) PRESENTER	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.
	No disclosure on file
Israt Jahan, MD (Parkinson's &Neurology Clinic, LLC)	No disclosure on file
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
Stephen J. Marks, MD	No disclosure on file
Joseph Staffetti, MD (University of South Florida)	Joseph Staffetti has nothing to disclose.
Patricia Greenstein, MD (Beth Israel Deaconess Medical Center)	Dr. Greenstein 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.
Tuan H. Vu, MD (University of South Florida)	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.

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