E-Pearl of the Week: Anti-MuSK Antibody Myasthenia Gravis

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July 29, 2013

Anti-MuSK (muscle-specific tyrosine kinase) myasthenia gravis (MG) was first described in 2001, with the associated antibody isolated a couple of years later.  MuSK-Ab MG is more commonly seen in woman, with a diagnosis frequently made in the fourth decade, as compared to the second or third decade in typical AChR+ Ab MG.  [1] It has a preference to affect bulbar and respiratory muscles.  Although it can present with a generalized picture, the presence of bulbar symptoms at onset suggests a worse prognosis.  A rapid progression to respiratory crisis is not uncommon after symptom onset.  In comparison to AChR+ Ab MG, response to acetyl-cholinesterase inhibitors (AChE-I) is not as common in patients with MuSK-Ab MG. [2]


1.  Guptill JT, Sanders DB and Evoli A.  Anti-MuSK antibody myasthenia gravis: Clinical findings and response to treatment in two large cohorts.  Muscle and Nerve 2011; 44: 36-40.

2. Zebardast N, Patwa HS, Novella SP, Goldstein JM.  Rituximab in the management of refractory myasthenia gravis.  Muscle and Nerve 2010; 41: 375-380.

Submitted by James Addington, MD Resident Physician, University of Virginia

Dr.  Addington reports no disclosures.

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