Muscle acetylcholine receptor (AChR) autoantibodies are characteristic but not diagnostic of myasthenia gravis (MG). They are found in 13% of patients with Lambert-Eaton Syndrome (LES), which is rare in children. The patient's autoantibody profile is more informative than the result of any single test for supporting a diagnosis of MG.
Titers of AChR antibodies are generally higher in patients with severe weakness, but severity cannot be predicted by antibody titer. Seronegativity is more frequent in children with prepubertal onset of acquired MG (33%-50%) than in adults (<10%). Thymoma is rare under age 20, and striational antibodies (see STR/8746 Striational [Striated Muscle] Antibodies, Serum) also are rare, except in the context of MG related to neoplasia (usually thymoma or neuroblastoma), graft-versus-host disease, autoimmune liver disease, or D-penicillamine therapy. This laboratory has recently noted muscle-specific receptor tyrosine kinase antibody in children with "seronegative" acquired MG, but the frequency of this antibody in pediatric MG has not been determined.