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Myasthenia gravis with antibodies to MuSK: an update

Abstract

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.

Authors:   Amelia Evoli, Paolo E. Alboini, Valentina Damato, Raffaele Iorio, Carlo Provenzano, Emanuela Bartoccioni, Mariapaola Marino
Journal:   Annals of the New York Academy of Sciences
Year:   2017
Pages:   n/a
DOI:   10.1111/nyas.13518
Publication date:   21-Dec-2017
Facts, background information, dossiers
  • rituximab
  • reductions
  • diagnosis
  • tyrosine
  • myasthenia gravis
  • antibodies
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