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Abstract Details

Myasthenia Gravis, Myocarditis, and Pericarditis in a Patient with Thymoma
Neuromuscular and Clinical Neurophysiology (EMG)
P3 - Poster Session 3 (12:00 PM-1:00 PM)
1-006

To describe a case of myo/pericarditis in a patient with Myasthenia Gravis and thymoma.

Cardiac involvement has been reported in patients with myasthenia gravis. Manifestations include pericarditis, giant-cell-myocarditis, Takotsubo cardiomyopathy, and arrhythmias. Myocarditis and pericarditis are associated with thymomas. Though the exact pathogenesis is unclear, cardiac involvement has been associated with the production of antibodies against striated muscle, including anti-titin and anti-ryanodine, anti-mitochondrial-7, anti-smooth-muscle-alpha and anti-citric-acid-extract. Prognosis is varied; however, myocarditis associated with myasthenia gravis can be rapidly fatal.

Case Report

A 27-year-old male with newly diagnosed generalized acetylcholine-receptor-antibody-positive myasthenia gravis with underlying thymoma without prior cardiac history was admitted to the ICU for acute type-2 respiratory failure secondary to myasthenic crisis. He was recently treated with a complete course of IVIG.  Rapid taper of oral prednisone, and concurrent infection possibly resulted in the current crisis.

The patient had a screening Echocardiogram and was found to have reduced cardiac ejection-fraction of 40%. His anti-striational antibodies were negative. He did not have co-existing polymyositis and his CK was normal. Cardiac MRI however, (performed after Plasma Exchange) revealed pericarditis.

He was extubated and discharged on Prednisone 60 mg/day. Repeat Echocardiogram (after Plasma Exchange) showed return of myocardial function to normal (EF 68%). He underwent successful video-assisted-thoracoscopic-thymectomy and is currently on a slow taper of steroids.

Anti-striational antibodies are positive in 97% of patients with Thymoma. Our patient was treated with immunosuppressives at presentation and hence likely tested negative. Constrictive pericarditis with invasion of the thymoma into the pericardium and pericarditis from post-radiation therapy of thymoma has been described. Based on the improvement post Plasma Exchange in our patient, we have reason to believe that his myo/pericarditis was an immunological process. There are few cases described in the literature with myo/pericarditis with thymoma/myasthenia gravis, challenging the guidance of treatment and prognostication.
Authors/Disclosures

PRESENTER
No disclosure on file
No disclosure on file
William Jimenez, MD No disclosure on file
Rabia Choudry, MD (St Lukes Neurology Associates) Dr. Choudry has nothing to disclose.
No disclosure on file
Aparna M. Prabhu, MD Dr. Prabhu has nothing to disclose.