Clinical findings in MuSK-antibody positive myasthenia gravis: A U.S. experience
| Autor: | Sharon P. Nations, Gil I. Wolfe, Mazen M. Dimachkie, Richard J. Barohn, Tulio E. Bertorini, Laura Herbelin, Steven Novella, Jaya Trivedi, Jonathan Goldstein, Luis A. Chui, Ronan J. Walsh, Angela Young, Shin Oh, April McVey, Lawrence H. Phillips, Ted M. Burns, Anthony A. Amato, Mamatha Pasnoor, John T. Kissel, Gwendolyn C. Claussen, Tahseen Mozaffar, Marcel Hungs |
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| Rok vydání: | 2010 |
| Předmět: |
Adult
Male medicine.medical_specialty Adolescent Physiology medicine.drug_class medicine.medical_treatment Severity of Illness Index Disease-Free Survival Medical Records Cellular and Molecular Neuroscience Atrophy Physiology (medical) Internal medicine Myasthenia Gravis Severity of illness medicine Humans Receptors Cholinergic Age of Onset Child Aged Retrospective Studies Plasma Exchange biology Electromyography business.industry Remission Induction Immunoglobulins Intravenous Receptor Protein-Tyrosine Kinases Retrospective cohort study Middle Aged Thymectomy medicine.disease United States Myasthenia gravis Surgery Treatment Outcome biology.protein Prednisone Corticosteroid Female Immunotherapy Neurology (clinical) Age of onset Antibody business |
| Zdroj: | Muscle & Nerve. 41:370-374 |
| ISSN: | 1097-4598 0148-639X |
| DOI: | 10.1002/mus.21533 |
| Popis: | 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 (> 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. |
| Databáze: | OpenAIRE |
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