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Abstract Details

MG-ADL Subdomain Score Changes With Eculizumab or Ravulizumab: An Analysis of the Global MG SPOTLIGHT Registry
Neuromuscular and Clinical Neurophysiology (EMG)
P4 - Poster Session 4 (8:00 AM-9:00 AM)
9-008
To assess changes in Myasthenia Gravis Activities of Daily Living (MG-ADL) subdomain scores after complement component 5 (C5) inhibitor therapy (C5IT, eculizumab and ravulizumab) initiation among patients with generalized myasthenia gravis (gMG). 
The global MG SPOTLIGHT Registry (NCT04202341) collects data on real-world clinical safety and effectiveness of the C5ITs eculizumab and ravulizumab in adults with anti-acetylcholine antibody-positive (AChR-Ab+) gMG. 
MG-ADL subdomain scores were assessed in patients enrolled in the MG SPOTLIGHT Registry who received eculizumab (eculizumab subgroup), ravulizumab (ravulizumab subgroup), or transitioned from eculizumab to ravulizumab (eculizumab-to-ravulizumab subgroup) with data available prior to C5IT initiation and during treatment.

As of July 1, 2024, 178 Registry patients fulfilled inclusion criteria for analysis (male: 55.7%; mean±SD age at MG diagnosis: 54.7±19.0yrs) with 89, 49, and 40 patients in the eculizumab, ravulizumab, and eculizumab-to-ravulizumab subgroups, respectively. Statistically significant reductions (P<0.05) in mean scores were observed for all MG-ADL subdomains after C5IT initiation. Reductions from prior to C5IT to last assessment during C5IT treatment ranged from 41.0% to 53.0% (ocular), 49.9% to 73.1% (bulbar), 40.7% to 58.6% (respiratory), and 33.7% to 71.4% (limbs) across the 3 subgroups. The proportions of patients with complete or partial improvement in individual MG-ADL subdomains during C5IT treatment were similar between the eculizumab and ravulizumab subgroups: 60.7% and 63.3% (ocular), 58.4% and 59.2% (bulbar), 51.7% and 49.0% (limbs), and 33.7% and 26.5% (respiratory), respectively. Among the eculizumab-to-ravulizumab subgroup, the proportions of patients with complete or partial improvement in individual subdomains were 67.5% (ocular), 67.5% (bulbar), 65.0% (limbs), and 45.0% (respiratory) at last assessment during ravulizumab treatment. Results with extended follow-up will be presented.

These results from clinical practice show the broad benefit of complement C5 inhibition with eculizumab and ravulizumab in improving ocular, bulbar, respiratory, and limb function as indicated by MG-ADL subdomain score in patients with AChR-Ab+ gMG.

Authors/Disclosures
Joshua P. Alpers, MD (UT Erlanger Neurology)
PRESENTER
Dr. Alpers has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenx. Dr. Alpers has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. Dr. Alpers has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Alpers has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Alpers has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Argenx. Dr. Alpers has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Alpers has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for UCB. Dr. Alpers has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Janssen / Johnson&Johnson.
Vern C. Juel, MD, FAAN (Duke University Medical Center) Dr. Juel 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. Juel has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Immunovant. The institution of Dr. Juel has received research support from argenx. The institution of Dr. Juel has received research support from Alexion. The institution of Dr. Juel has received research support from Janssen. The institution of Dr. Juel has received research support from NIH Rare Diseases Network.
Samir P. Macwan, MD Dr. Macwan has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alexion,Argenx,Grifols,Kabafusion, UCB. Dr. Macwan has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Abbvie,Alexion,Argenx,Grifols and UCB. The institution of Dr. Macwan has received research support from Alexion, Argenx,Immunovant, UCB.
Francesco Sacca, MD, FAAN (University Federico II) Dr. Sacca has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alexion. Dr. Sacca has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Argenx. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lexeo. Dr. Sacca has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genpharm. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Medpharma. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Madison Pharma. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Zai Lab. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Dianthus. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Reata. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sandoz. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neopharm Israel. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Johnson&Johnson. Dr. Sacca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Sacca has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Astrazeneca. The institution of Dr. Sacca has received research support from AIFA. The institution of Dr. Sacca has received research support from FARA.
Andrew J. Gordon, MD (Northwest Neurology) Dr. Gordon has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Catalyst. Dr. Gordon has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Gordon has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Avion . Dr. Gordon has received personal compensation in the range of $0-$499 for serving as a Consultant for Vertex. Dr. Gordon has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Gordon has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Lundbeck. Dr. Gordon has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Abbvie. Dr. Gordon has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Cassiday.
Lida Zeinali, MD (Alexion Pharmaceuticals, Inc.) Dr. Zeinali has received personal compensation for serving as an employee of Alexion Pharmaceuticals, AstraZeneca Rare Disease. Dr. Zeinali has stock in AstraZeneca.
James M. Winkley, MD Dr. Winkley has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alexion. Dr. Winkley has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Sanofi. Dr. Winkley has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Teva. Dr. Winkley has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Roche. Dr. Winkley has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Argenx. The institution of Dr. Winkley has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for J&J. Dr. Winkley has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for amgen . Dr. Winkley has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for TG therapeutics. An immediate family member of Dr. Winkley has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for UCB. Dr. Winkley has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for BMS. Dr. Winkley has received personal compensation in the range of $100,000-$499,999 for serving on a Speakers Bureau for Teva. Dr. Winkley has received personal compensation in the range of $100,000-$499,999 for serving on a Speakers Bureau for Alexion. Dr. Winkley has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for BMS. Dr. Winkley has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for amgen . Dr. Winkley has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for UCB . Dr. Winkley has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for J&J. Dr. Winkley has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for TG therapeutics. The institution of Dr. Winkley has received research support from Noveratis. The institution of Dr. Winkley has received research support from Merck. The institution of Dr. Winkley has received research support from Sanofi. The institution of Dr. Winkley has received research support from Biogen. The institution of Dr. Winkley has received research support from Alexion.
Chuang Liu, PhD Dr. Liu has received personal compensation for serving as an employee of Alexion.
Gary R. Cutter, PhD (University of Alabama At Birmingham) Dr. Cutter has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for onsulting or Advisory Boards: Alexion, Antisense Therapeutics/Percheron, Avotres, Biogen, Clene Nanomedicine, Clinical Trial Solutions LLC, Endra Life Sciences, Cognito Therapeutics, Genzyme, Genentech, Immunic, Klein-Buendel Incorporated, Kyverna Therapeutics, Inc. , Linical, Merck/Serono, Noema, Neurogenesis, Perception Neurosciences, Protalix Biotherapeutics, Regeneron, Revelstone Consulting, Roche, SAB Biotherapeutics, Sapience Therapeutics, Scott&Scott LLP, Tenmile.. Dr. Cutter has received personal compensation in the range of $50,000-$99,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Data and Safety Monitoring Boards: Applied Therapeutics, AI therapeutics, AMO Pharma, Argenx, Astra-Zeneca, Avexis Pharmaceuticals, Bristol Meyers Squibb, CSL Behring, Cynata Therapeutics, DiamedicaTherapeutics, Horizon Pharmaceuticals, Immunic, Inhibrix, Karuna Therapeutics, Kezar Life Sciences, Medtronic, Merck, Meiji Seika Pharma, Mitsubishi Tanabe Pharma Holdings, Prothena Biosciences, Novartis, Pipeline Therapeutics (Contineum), Regeneron, Sanofi-Aventis, Teva Pharmaceuticals, United BioSource LLC, University of Texas Southwestern.. Dr. Cutter has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for JASN.
Dubravka Dodig, MD (University Health Network) The institution of Dr. Dodig has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for argenx . The institution of Dr. Dodig has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion AZ. The institution of Dr. Dodig has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UBC. The institution of Dr. Dodig has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amicus Therapeutics. The institution of Dr. Dodig has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for argenx. An immediate family member of Dr. Dodig has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion AZ. The institution of Dr. Dodig has received research support from Alexion AZ.
Rup Tandan, MD, FRCP, FAAN (University of Vermont (UVM)/UVM Medical Center) Dr. Tandan has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Tandan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Apellis. Dr. Tandan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amylyx. The institution of Dr. Tandan has received research support from Alexion. The institution of Dr. Tandan has received research support from Apellis. The institution of Dr. Tandan has received research support from Cytokinetics.