Lu sur :https://www.ncbi.nlm.nih.gov/pubmed/29461627
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Myasthenia Gravis with Muscle-Specific Tyrosine Kinase (MuSK) Antibodies- A Narrative Review.
Morren J1, Li Y1.
Muscle Nerve. 2018 Feb 20. doi: 10.1002/mus.26107.
Growing evidence provides new insights about myasthenia gravis with antibodies against muscle-specific tyrosine kinase (MuSK-MG) including its pathogenesis, clinical and electrophysiological manifestations, and treatment.
Data now support the presence of both presynaptic and postsynaptic dysfunction in MuSK-MG.
This is one of many key differences between MuSK-MG and acetylcholine receptor antibody-MG (AChR-MG), especially as it pertains to potential therapeutic implications.
In comparison to AChR-MG, MuSK-MG is generally more refractory to treatment.
However, with MuSK-MG being better understood and more readily recognized today, there are more reports of a relatively benign course.
The most effective immunotherapies for MuSK-MG are corticosteroids, plasmapheresis and rituximab.
With appropriate therapy, most MuSK-MG patients achieve minimal manifestation status or better on the post-intervention status outlined by Myasthenia Gravis Foundation of America.
A minority of patients remain refractory to treatment, and optimal management for this group remains a considerable challenge.