Clinical findings in MuSK‐antibody positive myasthenia gravis: A U.S. experience |
| |
| Authors: | Mamatha Pasnoor MD Gil I. Wolfe MD Sharon Nations MD Jaya Trivedi MD Richard J. Barohn MD Laura Herbelin BS April McVey MD Mazen Dimachkie MD John Kissel MD Ronan Walsh MD Anthony Amato MD Tahseen Mozaffar MD Marcel Hungs MD Luis Chui MD Jonathan Goldstein MD Steven Novella MD Ted Burns MD Lawrence Phillips MD Gwendolyn Claussen MD Angela Young MD Tulio Bertorini MD Shin Oh MD |
| |
| Affiliation: | 1. University of Kansas Medical Center, 3901 Rainbow Boulevard, Mail Stop 2012, Kansas City, Kansas 66160, USA;2. University of Texas Southwestern Medical Center, Dallas, Texas, USA;3. Ohio State University, Columbus, Ohio, USA;4. Brigham and Women's Hospital, Boston, Massachusetts, USA;5. University of California, Irvine, Orange, California, USA;6. Yale University, New Haven, Connecticut, USA;7. University of Virginia, Charlottesville, Virginia, USA;8. University of Alabama at Birmingham, Birmingham, Alabama, USA;9. University of Tennessee, Memphis, Tennessee, USA |
| |
| 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. Muscle Nerve, 2009 |
| |
| Keywords: | myasthenia gravis MuSK acetylcholine receptor antibody |
|
|
