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• W3138796553 abstract "Objective: 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. Background: 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. Design/Methods: Retrospective analysis of our MuSK-MG patients’ demographics and disease course characteristics. Results: 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. Conclusions: Our MuSK-MG patients’ demographics, symptomatology, and treatment responsiveness were in concordance with phenotypes described in existing literature. Disclosure: Dr. Cannon has nothing to disclose. Dr. Beydoun has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Grifols,Shire, CSL, Alexion, Mitsubishi Tanabe, Akcea, Alnylam. Dr. Beydoun has received research support from Argenx, Pfizer, Mallinckrodt, UCB, Catalyst. Dr. Darki has nothing to disclose." @default.
• W3138796553 created "2021-03-29" @default.
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• W3138796553 date "2020-04-14" @default.
• W3138796553 modified "2023-09-23" @default.
• W3138796553 title "Anti-MuSK Antibody Positive Myasthenia Gravis Patient Demographics, Symptomatology, and Treatment Responsiveness in the University of Southern California Neuromuscular Clinic: A Retrospective Analysis (2921)" @default.
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