Lu sur :https://www.ncbi.nlm.nih.gov/pubmed/29266255
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Evoli A1, Alboini PE1, Damato V1, Iorio R1, Provenzano C2, Bartoccioni E2, Marino M2.
Ann N Y Acad Sci. 2017 Dec 21. doi: 10.1111/nyas.13518.
Myasthenia gravis with antibodies to the muscle-specific tyrosine kinase (MuSK+ MG) is a rare disease with distinctive pathogenic mechanisms and clinical features.
An acute onset and predominant bulbar muscle weakness are very common and highly suggestive of the disease.
On the other hand, a more indolent course, atypical ocular presentation, and signs of cholinergic hyperactivity may complicate the diagnosis.
Though MuSK+ MG is still a severe disease, over the years we have observed a steady reduction in the rate of respiratory crisis and a significant improvement in the clinical outcome, both likely related to earlier diagnosis and timely treatment.
Despite the improved management, MuSK+ MG patients tend to remain dependent on long-term immunosuppressive treatment and may develop permanent disabling weakness.
In uncontrolled studies, B cell depletion with rituximab proved effective in most patients with refractory disease, inducing prolonged clinical responses associated with a sustained reduction of serum antibody levels.
Promising results from experimental studies and case reports suggest that both 3,4-diaminopyridine and albuterol may be effective as symptomatic agents.