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Abstract Details

Intermuscular Coherence as a Biomarker for Primary Lateral Sclerosis
Neuromuscular and Clinical Neurophysiology (EMG)
P10 - Poster Session 10 (8:00 AM-9:00 AM)
9-019

We investigated whether intermuscular coherence in the beta-gamma (20-40 Hz) frequency range (IMCβγ) can serve as a biomarker for PLS.
Primary lateral sclerosis (PLS) is a sporadic neurodegenerative disorder characterized by progressive degeneration of upper motor neurons (UMNs). It represents one extreme of the motor neuron disease spectrum, with amyotrophic lateral sclerosis (ALS) being the most prevalent condition within this category. In its early stages, PLS must be distinguished from an UMN predominant variant of ALS. A diagnosis of PLS has important prognostic implications, as PLS has slower disease progression and significantly longer survival than ALS. Currently, there is a need for a widely accessible neurophysiological biomarker that can detect and monitor UMN dysfunction. 
IMCβγ was evaluated in upper and lower limb muscle pairs in 21 individuals with PLS along with 42 age- and sex-matched neurotypical control subjects. 
IMCβγ was significantly lower in PLS patients across all muscle pairs. Area under the receiver-operating characteristic curve ranged between 0.71 and 0.77 for differentiating PLS from neurotypical control subjects based on measurements in a single limb. Using an abnormal coherence measurement in any two limbs as a diagnostic test for PLS, test sensitivity was 0.76, specificity was 0.86, and accuracy was 0.83.
IMCβγ measured in the arms legs helps distinguish PLS patients from neurotypical control subjects, supporting its potential as an accessible biomarker of UMN dysfunction.
Authors/Disclosures
Naoum P. Issa, MD, PhD (University of Chicago)
PRESENTER 		The institution of Dr. Issa has received research support from NIH. Dr. Issa has received intellectual property interests from a discovery or technology relating to health care. Dr. Issa has received personal compensation in the range of $0-$499 for serving as a Study Section Member with NIH. Dr. Issa has a non-compensated relationship as a Scientific advisory board member with Theta Neurotech that is relevant to AAN interests or activities.
Elena Badillo-Goicoechea, MS Ms. Badillo-Goicoechea has nothing to disclose.
Serdar AYDIN, MD No disclosure on file
Sean E. Smith, MD, MPHS Dr. Smith has received personal compensation in the range of $500-$4,999 for serving as a Consultant for inThought.
Nathan Carberry, MD (Univsersity of Miami) Dr. Carberry has nothing to disclose.
Doreen Ho, MD (MGH) Dr. Ho has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals.
Mark Garret, MD Dr. Garret has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Advarra. The institution of Dr. Garret has received research support from Biogen. The institution of Dr. Garret has received research support from Target ALS. The institution of Dr. Garret has received research support from uniQure. The institution of Dr. Garret has received research support from Mass General Brigham. The institution of Dr. Garret has received research support from Patient Centered Outcomes Research Institute. The institution of Dr. Garret has received research support from NIH/NINDS. Dr. Garret has received research support from FDA. Dr. Garret has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant with Massachusetts Department of Health.
Avinash Moses Mr. Moses has nothing to disclose.
Betty C. Soliven, MD (University of Chicago) Dr. Soliven has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CVS Pharmacy. The institution of Dr. Soliven has received research support from NIH. The institution of Dr. Soliven has received research support from NIH. The institution of Dr. Soliven has received research support from Alexion. The institution of Dr. Soliven has received research support from Roche/Genentech.
Kourosh Rezania, MD, FAAN (University of Chicago) Dr. Rezania has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Akcea. Dr. Rezania has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alnylam.