Abstract Details

Title Poor response to Eculizumab in Caucasian Patients with Treatment Refractory Generalized Myasthenia Gravis

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P7 - Poster Session 7 (8:00 AM-9:00 AM)

Poster/Presentation
Number 11-006

Objective

To report three cases of poor response to eculizumab in young caucasian patients with treatment refractory gMG.

Background

Eculizumab, a C5 complement inhibitor, has been approved for management of patients with treatment refractory Acetylcholine receptor positive (AChR+) Generalized Myasthenia Gravis (gMG). Though the majority of patients receiving eculizumab experience clinical improvement, a small number of patients may have poor response.

Design/Methods Case series

Results All three patients were young, caucasian, thymectomized, females with MGFA class III, treatment refractory MG on multiple immunosuppressant medications. All three patients had initial worsening of MG- ADL score, 1 month post eculizumab, followed by an unchanged MG ADL and MGC score, 3 months after eculizumab therapy. No changes were noted in the number of acute exacerbations of MG, pre and post eculizumab therapy. All patients were eventually started on maintenance Plasma-exchange (PLEX) therapy, post eculizumab failure and had clinical improvement in MG-ADL and MGC scores and reduction in the number of acute exacerbations of disease.

Conclusions The exact mechanism contributing to poor clinical response to eculizumab in gMG patients remains unclear. Further studies are warranted to undermine the underlying pathogenesis.

Authors/Disclosures
Nakul Katyal, MD (University of Kentucky) PRESENTER	Dr. Katyal has nothing to disclose.
Raghav Govindarajan, MD, FAAN (HSHS St. Elizabeth Medical Group)	Dr. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for MT pharma. Dr. Govindarajan 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. Govindarajan 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. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Catalyst. Dr. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche . Dr. Govindarajan 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. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amicus. Dr. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Govindarajan has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for MT pharma . Dr. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Catalyst. Dr. Govindarajan has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Argenx. Dr. Govindarajan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biohaven. Dr. Govindarajan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. Dr. Govindarajan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Takeda. Dr. Govindarajan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Abbvie. The institution of Dr. Govindarajan has received research support from Band of Hope . The institution of Dr. Govindarajan has received research support from Alexion. Dr. Govindarajan has received publishing royalties from a publication relating to health care.

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