Abstract Details

Title Striving to improve outcome measures for inclusion body myositis through novel dynamometer and modifications of the current functional rating scale

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P16 - Poster Session 16 (5:30 PM-6:30 PM)

Poster/Presentation
Number 1-001

Objective (1) Determining efficacy of novel dynamometer for finger flexor strength measurement (2) Does addition of NIH-published patient reported outcomes to current IBM-FRS improve ability to detect change

Background Inclusion body myositis (IBM) is a indolent but relentlessly progression myopathy that remains refractory to current modalities of treatment. Limitation of clinical trials for novel therapeutics include insufficient outcome measures to faithfully reflect changes in deep finger flexor strength and upper extremity function. IBM-FRS has some validation data, but our previous work showed that items designed to reflect hand function did so poorly. Stemming from this work we aim to (1) evaluate new patient reported outcome published by the NIH and how well they augment appreciation of deep finger flexor weakness (2) determining the reliability and feasibility of a new pinch dynamometer.

Design/Methods 123 subjects recruited at the national Myositis Conference 2019, including 84 IBM patients and 39 healthy controls. Motor function is measured by grip dynamometer (Jamar), manual distal finger flexor testing, commercial pinch dynamometer (Citec) previously used in assessment of IBM, and novel pinch dynamometer (Piezo). The Piezo dynamometer consist of a light weight, thin, membranous force-sensing resister capable of detecting force applied between 2 surfaces (pinch) up to 100lbs. Our IBM cohort filled out a modified IBM-FRS which included items from patient reported outcomes published by NIH and additional section to free text questions that they feel would be more appropriate when assessing their hand function.

Results Data analysis is ongoing and will be published at the time of the poster

Conclusions This study aims to improve outcome measures by (1) field test NIH patient reported outcomes in a large IBM cohort, (2) comprehensive comparison of 3 different methods of pinch strength evaluation, that is specific to IBM but can be challenging to assess.

Authors/Disclosures
Ava Y. Lin, MD (University of Michigan-Division of Pediatric Neurology) PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
Conrad Weihl, MD, PhD (Washington University in St. Louis)	Dr. Weihl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sarepta. Dr. Weihl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Casma therapeutics. Dr. Weihl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Abata. Dr. Weihl has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sarepta. Dr. Weihl has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sarepta. Dr. Weihl has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Acceleron. Dr. Weihl has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Wilson Elser Moskowitz Edelman & Dicker LLP.
Leo H. Wang, MD, PhD, F�鶹��ýӳ�� (University of Washington)	Dr. Wang has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for AskBio. Dr. Wang has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Fulcrum Therapeutics. Dr. Wang has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Wang has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi Tanabe. Dr. Wang has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenx. Dr. Wang has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Avidity. Dr. Wang has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB Pharma. Dr. Wang has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lupin. Dr. Wang has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sarepta. Dr. Wang has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Scholar Rock. The institution of Dr. Wang has received research support from National Institute of Health. Dr. Wang has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant with US Government.

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