Clinical findings in MuSK‐antibody positive myasthenia gravis: A U.S. experience
2009; Wiley; Volume: 41; Issue: 3 Linguagem: Inglês
10.1002/mus.21533
ISSN1097-4598
AutoresMamatha Pasnoor, Gil I. Wolfe, Sharon Nations, Jaya Trivedi, Richard J. Barohn, Laura Herbelin, April McVey, Mazen M. Dimachkie, John T. Kissel, Ronan J. Walsh, Anthony A. Amato, Tahseen Mozaffar, Marcel Hungs, Luis A. Chui, Jonathan Goldstein, Steven Novella, Ted M. Burns, Lawrence H. Phillips, Gwendolyn Claussen, Angela M. Young, Tulio E. Bertorini, Shin J. Oh,
Tópico(s)Thyroid and Parathyroid Surgery
ResumoWe 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 (> or =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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