Clinical aspects of MuSK antibody positive seronegative MG

DB Sanders, K El-Salem, JM Massey, J McConville… - Neurology, 2003 - AAN Enterprises
DB Sanders, K El-Salem, JM Massey, J McConville, A Vincent
Neurology, 2003AAN Enterprises
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 …
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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