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Abstract Details

Diagnosis of Lambert-Eaton Myasthenic Syndrome (LEMS) May Be Missed Among Patients with Myasthenia Gravis and Autonomic Dysfunction: Results from a US Database Analysis
Neuromuscular and Clinical Neurophysiology (EMG)
P4 - Poster Session 4 (11:45 AM-12:45 PM)
11-018

To evaluate the potential for missed diagnoses of Lambert-Eaton myasthenic syndrome (LEMS) and autonomic dysfunction among patients with myasthenia gravis (MG) in the United States (US). 

MG and LEMS are both characterized by muscle weakness, but autonomic dysfunction is more common with LEMS. LEMS patients may initially be diagnosed as MG. Autonomic dysfunction in patients diagnosed with MG may indicate a missed LEMS diagnosis. Delayed diagnosis of LEMS, an underrecognized syndrome, can worsen morbidity, including delayed recognition of associated small cell lung cancer (SCLC).

Healthcare claims from a large US de-identified dataset (Symphony Health's PatientSource®, 3/1/2014-6/30/2023) were used. Eligible patients had ≥2 claims ≥30 days apart for MG and/or LEMS. In patients with MG, subsequent diagnoses of LEMS, lung cancer, and the receipt of SCLC therapies (etoposide + platinum-based chemotherapy) were ascertained according to the presence of autonomic disorder claims.

Among 130,762 eligible patients with MG, 400 (0.3%) also had LEMS diagnoses. Claims related to autonomic dysfunction were observed in 5.0% (n=6,562) of patients with MG. LEMS diagnoses were more frequent in patients with MG and autonomic dysfunction (0.8%) than in patients with MG without autonomic dysfunction (0.3%). Among 6,508 patients with MG and autonomic dysfunction, without a LEMS diagnosis, 138 patients were diagnosed with non-thymoma lung cancer. Of those who received SCLC therapies (n=4/138), none had LEMS codes in their claims history. In a subset of patients with MG diagnoses 1/1/2022 or later, VGCC antibody testing claims were infrequent but more common when autonomic dysfunction was present (2.3% vs 1.5%).

LEMS should be suspected in patients diagnosed as MG with autonomic dysfunction, especially in those with SCLC. Based on our analyses, at least 2% of this MG population may actually have the diagnosis of LEMS and be appropriate for alternative treatments.

Authors/Disclosures
Steven Vernino, MD, PhD, FAAN (UT Southwestern Medical Center)
PRESENTER
Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving as a Consultant for antag. Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CSL Behring. Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for argenx. Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Autonomic Neuroscience (Elsevier). The institution of Dr. Vernino has received research support from Takeda. The institution of Dr. Vernino has received research support from NIH/NHLBI. The institution of Dr. Vernino has received research support from Lundbeck. The institution of Dr. Vernino has received research support from Regeneron.
David Morrell (Catalyst Pharmaceuticals) No disclosure on file
Guy Shechter (MedTech Analytics LLC) No disclosure on file
Regina Grebla No disclosure on file
Jeffrey Rosenfeld, MD, PhD, FAAN (Loma Linda University School Medicine - NEUROLOGY) Dr. Rosenfeld has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for ML Biosolutions. Dr. Rosenfeld has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for MT Pharma America. Dr. Rosenfeld has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CSL Behring. Dr. Rosenfeld has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Rosenfeld has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. Dr. Rosenfeld has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Argenx. Dr. Rosenfeld has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for NeuroSense. Dr. Rosenfeld has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Catalyst. Dr. Rosenfeld has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Annexion. Dr. Rosenfeld has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB Pharma. Dr. Rosenfeld has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neurizon. Dr. Rosenfeld has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Serano/EMD. Dr. Rosenfeld has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Anelixis. Dr. Rosenfeld has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AcuraStem. Dr. Rosenfeld 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. Rosenfeld has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for NeuroSense. Dr. Rosenfeld has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for MT Pharma America. Dr. Rosenfeld has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Argenx. Dr. Rosenfeld has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Strongbridge Pharma. Dr. Rosenfeld has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amylyx. The institution of Dr. Rosenfeld has received research support from MT Pharma. The institution of Dr. Rosenfeld has received research support from Alexion. The institution of Dr. Rosenfeld has received research support from Healey ALS Trial Center Mass General.