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Abstract Details

Accurate Test of Limb Isometric Strength (ATLIS) as an Outcome Measure in ALS and as a Marker of Upper Motor Neuron Involvement in Primary Lateral Sclerosis
Neuromuscular and Clinical Neurophysiology (EMG)
S11 - ALS and Motor Neuron Disorders (2:48 PM-3:00 PM)
010

ATLIS measures strength using a fixed load cell. It was shown to be a sensitive outcome measure in ALS.  However, its utility in ALS variants like Primary Lateral Sclerosis (PLS), is unknown.  Prior studies focused on peak force, but the raw data can be used to evaluate additional parameters besides force that could serve as UMN markers.

Quantify strength longitudinally in patients with ALS and variants using ATLIS and compare these changes to ALSFRS-R and forced vital capacity (FVC).  Identify putative markers of upper motor neuron (UMN) involvement.
126 participants with motor neuron disease have been enrolled.  ATLIS, ALSFRS-R and FVC are collected every 3 months. To compare the outcome measures, a linear regression analysis was performed for 36 participants followed for at least 12 months. To evaluate putative parameters of UMN involvement, a retrospective analysis of the 126 participants identified 7 with PLS, 7 UMN-predominant, and 11 primary muscular atrophy or LMN-predominant ALS. The following ATLIS parameters were compared between the variants: time to peak force, rate of ascent to peak force, time to maximum rate.

ATLIS upper and lower extremity mean-scores declined by 34.2% and 30.8% annually, respectively.  ALSFRS scores decreased by 17.9% per year, while FVC decreased by 14%.  Linear regression modelling demonstrated less variability with ATLIS than with FVC or ALSFRS.  A preliminary cross-sectional analysis demonstrated time to peak force and time to maximum rate are higher in PLS when compared with LMN variants.

Consistent with prior work, ATLIS is a sensitive outcome measure in ALS. More importantly, we identified novel parameters that could serve as potential markers of UMN involvement in ALS variants. To expand on these findings we are recruiting PLS participants to study these potential ATLIS parameters longitudinally and determine their utility as measures of disease progression in PLS and/or UMN ALS.
Authors/Disclosures
Samuel Frank (Cedars Sinai Medical Center)
PRESENTER
Dr. Frank has nothing to disclose.
Peggy Allred (Novartis Gene Therapies) Dr. Allred has received personal compensation for serving as an employee of Novartis. Dr. Allred has received stock or an ownership interest from Novartis.
No disclosure on file
No disclosure on file
Richard Lewis (Cedars-Sinai Medical Center) Dr. Lewis has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Argenx. Dr. Lewis has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for CSL Behring. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Grifols. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Takeda. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Nuvig. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Dianthus. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Janssen. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Medscape. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Boehringer Ingelheim. Dr. Lewis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alnylam. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Lewis has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Shernoff et al. Dr. Lewis has received publishing royalties from a publication relating to health care.
Robert Baloh (Novartis Institutes for BioMedical Research) Dr. Baloh has received personal compensation for serving as an employee of Roche. Dr. Baloh has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Acurastem. Dr. Baloh has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Irell. The institution of Dr. Baloh has received research support from NIH and CIRM.
Matthew Burford (Cedars-Sinai Medical Center) The institution of Dr. Burford has received research support from Brainstorm Cell Therapeutics. The institution of Dr. Burford has received research support from Pharnext Inc.
Frank Diaz (Cedars-Sinai Medical Center) Dr. Diaz has nothing to disclose.