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From the Division of Neurology (Drs. Sanders and Massey), Duke University Medical Center, Durham, NC; Department of Neurology (Dr. El-Salem), Jordan University of Science and Technology, Irbid, Jordan; Department of Neurology, Royal Victoria Hospital, Belfast (Dr. McConville); and the Neurosciences Group (Dr. Vincent), Weatherall Institute of Molecular Medicine, Oxford, UK.
Address correspondence and reprint requests to Dr. Donald B. Sanders, Box 3403, Duke University Medical Center, Durham, NC 27710; e-mail: Donald.Sanders{at}Duke.edu
Serum antibodies to muscle-specific receptor tyrosine kinase were detected in 12 of 32 patients with generalized seronegative MG. All were women, with onset between ages 21 and 59 years. Seven had prominent neck, shoulder, or respiratory muscle weakness and little or delayed ocular muscle involvement. The response to cholinesterase inhibitors was variable, and electromyographic findings suggested myopathy in several. None improved after thymectomy. All patients improved after plasma exchange, and most had a good response to selected immunotherapy. MuSK antibody status should help diagnose MG with atypical presentations and ensure appropriate patient treatment.
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