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

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Brought to you by the Residents & Fellows Section of Neurology®.

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]

References

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.

For more clinical pearls and other articles of interest to neurology trainees, visit Neurology. Visit the E-Pearl of the Week Archive. Listen to this week'sNeurology Podcast

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