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Abstract Details

Anti-MuSK Antibody Positive Myasthenia Gravis Patient Demographics, Symptomatology, and Treatment Responsiveness in the University of Southern California Neuromuscular Clinic: A Retrospective Analysis
Neuromuscular and Clinical Neurophysiology (EMG)
P4 - Poster Session 4 (5:30 PM-6:30 PM)
1-005
To contribute to the limited existing literature regarding Anti-MuSK Antibody Positive Myasthenia Gravis (MuSK-MG) by describing our MuSK-MG patients’ demographics, symptomatology, and treatment responsiveness.
MuSK-MG is rarer than and differs from seropositive Myasthenia Gravis (MG) in regards to prevalence, demographics, symptomatology, and treatment responsiveness. Existing literature regarding MuSK-MG is limited when compared to that of seropositive MG.
Retrospective analysis of our MuSK-MG patients' demographics and disease course characteristics.

Of 93 MG patients, 6 had MuSK-MG. 83% were female. 50% were Caucasian, 33.33% were African American, and 16.67% were Filipino. 66.67% had symptom onset in their 20s, 16.67% in their 30s, and 16.67% in their 40s. 

50% had initial and later respiratory dysfunction. 16.67% developed exclusively later respiratory dysfunction. 66.67% had initial and later dysarthria. None developed exclusively later dysarthria. 33.33% had initial and later dysphagia. 50% developed exclusively later dysphagia. 83.33% developed later muscle of mastication weakness, while none had this initially. 33.33% had initial and later diplopia and ptosis. 33.33% had exclusively later diplopia and ptosis. 33.33% had exclusively later neck weakness, while none had this initially. 16.67% had initial and later extremity weakness. 33.33% had exclusively later extremity weakness.

33.33% had initial MuSK titer > 1:1000 and 33.33% had Musk titer > 1:2000.

33.33% had initial bulbar crisis. 33.33% had bulbar crisis on chronic immunosuppressive therapy. 100% had non-severe bulbar dysfunction worsening while on chronic immunosuppressive therapy. 16.67% treated acutely improved with IVIG alone, 33.33% with IVIG and PLEX, and 16.67% with IVIG, PLEX, and rituximab.

None improved with pyridostigmine alone. 33.33% improved on steroids alone. 33.33% required one steroid sparing immunosuppressive drug (SSID) and 33.33% required two or more SSIDs. None were able to discontinue steroids with or without SSIDs.

Our MuSK-MG patients’ demographics, symptomatology, and treatment responsiveness were in concordance with phenotypes described in existing literature.
Authors/Disclosures
Victoria A. Cannon, MD
PRESENTER
No disclosure on file
Said R. Beydoun, MD, FÂé¶¹´«Ã½Ó³»­ (University of Southern California Healthcare Consultation Center 2) Dr. Beydoun has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. Dr. Beydoun has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. Dr. Beydoun has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Janssen. Dr. Beydoun has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for CSL. Dr. Beydoun has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for AstraZeneca. Dr. Beydoun has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenx. Dr. Beydoun has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB. Dr. Beydoun has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pfizer. Dr. Beydoun has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Takeda. Dr. Beydoun has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Alnylam. Dr. Beydoun has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Beydoun has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for CSL. Dr. Beydoun has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for UCB. Dr. Beydoun has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Takeda. Dr. Beydoun has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for AstraZeneca. Dr. Beydoun has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Argenx. Dr. Beydoun has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Janssen. The institution of Dr. Beydoun has received research support from Abcuro. The institution of Dr. Beydoun has received research support from Sean Healy & AMG Center for ALS. The institution of Dr. Beydoun has received research support from Regeneron. The institution of Dr. Beydoun has received research support from RemeGen. The institution of Dr. Beydoun has received research support from Sanofi. The institution of Dr. Beydoun has received research support from Novartis.
Leila Darki, MD, FÂé¶¹´«Ã½Ó³»­ (USC NeuroSciences) Dr. Darki has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Trinity Partner. Dr. Darki has received personal compensation in the range of $0-$499 for serving as a Consultant for Guide point Global. Dr. Darki has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Global Access Meetings. Dr. Darki has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amylyx . Dr. Darki has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amylyx.