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  • Title: Different characteristic phenotypes according to antibody in myasthenia gravis.
    Author: Oh SJ, Morgan MB, Lu L, Hatanaka Y, Hemmi S, Young A, Claussen GC.
    Journal: J Clin Neuromuscul Dis; 2012 Dec; 14(2):57-65. PubMed ID: 23172384.
    Abstract:
    OBJECTIVES: To find the characteristic phenotypes of 3 different types of myasthenia gravis (MG). METHODS: The clinical and electrophysiological features among 15 cases of muscle-specific kinase antibody positive (MuSK Ab+) MG, 59 cases of double seronegative (DSN) MG, and 161 cases of acetylcholine receptor antibody (AChR Ab)+ MG in the University of Alabama at Birmingham were compared. RESULTS: AChR Ab was positive in 69% of cases and MuSK Ab in 6% of cases. MuSK Ab+ MG was more common (14%) in African Americans compared with whites (4%). AChR Ab+ MG is characterized by male predominance, later onset, a fewer cases of ocular MG, and a higher association with thymoma. DSN-MG is characterized by a greater prevalence of ocular MG, milder forms of MG with less number of crisis, and fewer abnormalities in the repetitive nerve stimulation test. MuSK Ab+ MG is characterized by younger age at onset, severe and bulbar forms of MG, predominant faciobulbar neck weakness, and a poor response to edrophonium, anticholinesterase, and intravenous immunoglobulin. Long-term outcome showed no difference among 3 types of MG. CONCLUSIONS: AChR Ab+ MG and DSN-MG are similar, with the exception of less severity in the latter. MuSK Ab+ MG has distinct clinical and electrophysiological features.
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