Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience

Mamatha Pasnoor; Gil I. Wolfe; Sharon Nations; Jaya Trivedi; Richard J. Barohn; Laura Herbelin; April McVey; Mazen Dimachkie; John Kissel; Ronan Walsh; Anthony Amato; Tahseen Mozaffar; Marcel Hungs; Luis Chui; Jonathan Goldstein; Steven Novella; Ted Burns; Lawrence Phillips; Gwendolyn Claussen; Angela Young; Tulio Bertorini; O. H. Shin

doi:10.1002/mus.21533

Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience

Mamatha Pasnoor, Gil I. Wolfe, Sharon Nations, Jaya Trivedi, Richard J. Barohn, Laura Herbelin, April McVey, Mazen Dimachkie, John Kissel, Ronan Walsh, Anthony Amato, Tahseen Mozaffar, Marcel Hungs, Luis Chui, Jonathan Goldstein, Steven Novella, Ted Burns, Lawrence Phillips, Gwendolyn Claussen, Angela YoungTulio Bertorini, O. H. Shin

Research output: Contribution to journal › Article › peer-review

152 Scopus citations

Abstract

We performed a retrospective chart review on 53 muscle-specific kinase antibody (MuSK-Ab)-positive myasthenia gravis (MG) patients at nine university-based centers in the U.S. Of these, 66% were Caucasian, 85% were women, and age of onset was 9-79 years. Twenty-seven patients were nonresponsive to anticholinesterase therapy. Myasthenia Gravis Foundation of America improvement status was achieved in 53% patients on corticosteroids, 51% with plasma exchange, and in 20% on intravenous immunoglobulin (IVIG). Thymectomy was beneficial in 7/18 patients at 3 years. Long-term (≥3 years) outcome was very favorable in 58% of patients who achieved remission and/or minimal manifestation status. Overall, 73% improved. There was one MG-related death. This survey reinforces several cardinal features of MuSK-Ab-positive MG, including prominent bulbar involvement and anticholinesterase nonresponsiveness. Facial or tongue atrophy was rare. Most patients respond favorably to immunotherapy. The best clinical response was to corticosteroids and plasma exchange, and the poorest response was to IVIG. Long-term outcome is favorable in about 60% of cases.

Original language	English (US)
Pages (from-to)	370-374
Number of pages	5
Journal	Muscle and Nerve
Volume	41
Issue number	3
DOIs	https://doi.org/10.1002/mus.21533
State	Published - Mar 2010

Keywords

Acetylcholine receptor antibody
MuSK
Myasthenia gravis

ASJC Scopus subject areas

Physiology
Clinical Neurology
Cellular and Molecular Neuroscience
Physiology (medical)

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Pasnoor, M., Wolfe, G. I., Nations, S., Trivedi, J., Barohn, R. J., Herbelin, L., McVey, A., Dimachkie, M., Kissel, J., Walsh, R., Amato, A., Mozaffar, T., Hungs, M., Chui, L., Goldstein, J., Novella, S., Burns, T., Phillips, L., Claussen, G., ... Shin, O. H. (2010). Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience. Muscle and Nerve, 41(3), 370-374. https://doi.org/10.1002/mus.21533

Pasnoor, M, Wolfe, GI, Nations, S, Trivedi, J, Barohn, RJ, Herbelin, L, McVey, A, Dimachkie, M, Kissel, J, Walsh, R, Amato, A, Mozaffar, T, Hungs, M, Chui, L, Goldstein, J, Novella, S, Burns, T, Phillips, L, Claussen, G, Young, A, Bertorini, T & Shin, OH 2010, 'Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience', Muscle and Nerve, vol. 41, no. 3, pp. 370-374. https://doi.org/10.1002/mus.21533

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abstract = "We performed a retrospective chart review on 53 muscle-specific kinase antibody (MuSK-Ab)-positive myasthenia gravis (MG) patients at nine university-based centers in the U.S. Of these, 66% were Caucasian, 85% were women, and age of onset was 9-79 years. Twenty-seven patients were nonresponsive to anticholinesterase therapy. Myasthenia Gravis Foundation of America improvement status was achieved in 53% patients on corticosteroids, 51% with plasma exchange, and in 20% on intravenous immunoglobulin (IVIG). Thymectomy was beneficial in 7/18 patients at 3 years. Long-term (≥3 years) outcome was very favorable in 58% of patients who achieved remission and/or minimal manifestation status. Overall, 73% improved. There was one MG-related death. This survey reinforces several cardinal features of MuSK-Ab-positive MG, including prominent bulbar involvement and anticholinesterase nonresponsiveness. Facial or tongue atrophy was rare. Most patients respond favorably to immunotherapy. The best clinical response was to corticosteroids and plasma exchange, and the poorest response was to IVIG. Long-term outcome is favorable in about 60% of cases.",

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AU - Pasnoor, Mamatha

AU - Wolfe, Gil I.

AU - Nations, Sharon

AU - Trivedi, Jaya

AU - Barohn, Richard J.

AU - Herbelin, Laura

AU - McVey, April

AU - Dimachkie, Mazen

AU - Kissel, John

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AU - Amato, Anthony

AU - Mozaffar, Tahseen

AU - Hungs, Marcel

AU - Chui, Luis

AU - Goldstein, Jonathan

AU - Novella, Steven

AU - Burns, Ted

AU - Phillips, Lawrence

AU - Claussen, Gwendolyn

AU - Young, Angela

AU - Bertorini, Tulio

AU - Shin, O. H.

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AB - We performed a retrospective chart review on 53 muscle-specific kinase antibody (MuSK-Ab)-positive myasthenia gravis (MG) patients at nine university-based centers in the U.S. Of these, 66% were Caucasian, 85% were women, and age of onset was 9-79 years. Twenty-seven patients were nonresponsive to anticholinesterase therapy. Myasthenia Gravis Foundation of America improvement status was achieved in 53% patients on corticosteroids, 51% with plasma exchange, and in 20% on intravenous immunoglobulin (IVIG). Thymectomy was beneficial in 7/18 patients at 3 years. Long-term (≥3 years) outcome was very favorable in 58% of patients who achieved remission and/or minimal manifestation status. Overall, 73% improved. There was one MG-related death. This survey reinforces several cardinal features of MuSK-Ab-positive MG, including prominent bulbar involvement and anticholinesterase nonresponsiveness. Facial or tongue atrophy was rare. Most patients respond favorably to immunotherapy. The best clinical response was to corticosteroids and plasma exchange, and the poorest response was to IVIG. Long-term outcome is favorable in about 60% of cases.

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Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience

Abstract

Keywords

ASJC Scopus subject areas

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