Abstract Details

Title A Cross-Sectional Study of Inclusion Body Myositis: Novel Exam Findings for Early Screening and Detection, Correlation with Items in the Current Functional Rating Scale

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P3 - Poster Session 3 (5:30 PM-6:30 PM)

Poster/Presentation
Number 12-011

Objective

Identification of a method easily applied across all clinical settings to screen for potential IBM and how they correlate with IBM-FRS.

Background Inclusion body myositis (IBM) is the most common acquired myopathy after the age of 45 but challenging to diagnose in its early stages due to its uniquely indolent onset. Methods applicable for early screening is needed. There is also a lack in methods to accurately reflect disease progression. Inclusion Body Myositis Functional Rating Scale (IBM-FRS) has been used in clinical trials to demonstrate therapeutic efficacy, but we do not know how well it reflects finger flexor weakness, a finding more specific to IBM compared to other neuromuscular conditions. IBM-FRS has some validation data, but we are in need for improved methods to characterize this uniquely indolent but unrelentingly progressive disorder.

Design/Methods 191 subjects were recruited at the national Myositis Conference 2018. Patients self-identified their diagnosis as IBM, dermatomyositis/polymyositis, other disorders, and healthy individuals. Photograph of dorsal hand are graded as (1) loss of distal creases (2) potential loss (3) unlikely loss (4) no loss, to determine sensitivity/specificity of this finding for IBM. Additionally, grip strength of the dominant hand measured by dynamometer and finger flexor strength is obtained. We are looking at correlation between finger crease loss, finger flexor/grip weakness and IBM-FRS.

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

Conclusions This study points to an interesting observation of loss of distal dorsal finger creases in patients of IBM and aims to fill the knowledge gap regarding accurate ways to characterize physical and functional decline in IBM patients.

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
	No disclosure on file
Benjamin J. Koo, MD	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, FAAN (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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