Journal of clinical neuromuscular disease · 2012
Different characteristic phenotypes according to antibody in myasthenia gravis.
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Oh Shin J, Morgan Marla B, Lu Liang, Hatanaka Yuki, Hemmi Shoji, Young Angela, Claussen Gwendolyn C
Аннотация
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.
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.
Предметные рубрики MeSH
AdolescentAdultAge of OnsetAgedAutoantibodiesChi-Square DistributionCholinesterase InhibitorsElectric StimulationElectromyographyEvoked Potentials, MotorFemaleHumansImmunoglobulins, IntravenousImmunologic FactorsLongitudinal StudiesMaleMiddle AgedMyasthenia GravisNeural ConductionPhenotypePyridostigmine BromideReceptor Protein-Tyrosine KinasesReceptors, CholinergicRetrospective StudiesYoung Adult