[A case of transient left ventricular ballooning ("Takotsubo"-shaped cardiomyopathy) developed during plasmapheresis for treatment of myasthenic crisis]

Abstract

An 83-year-old woman was admitted to our hospital complaining of respiratory distress. She developed blepharoptosis and dysphagia two months previously. On admission, blood pressure was 150/84 mmHg. There were bilateral blepharoptosis and bulbar palsy. Tendon reflexes were brisk, but pathological reflexes were absent. Repetitive stimulation test demonstrated decrement. Tensilon test was positive. Thus, a diagnosis of myasthenic crisis was made. Vital capacity was 0.71, which necessitated intubation and artificial ventilation. During the first course of immunoadsorption plasmapheresis, blood pressure fell to 80/50 mmHg. ECG showed deeply inverted T waves in leads V2 through V6. Echocardiographic examination demonstrated extensive akinesis around the apex. CK was slightly increased. A diagnosis of "Takotsubo"-shaped cardiomyopathy was made. To maintain blood pressure, dopamine was continuously injected. Left ventricular function returned to normal within a week. No thymoma was seen on the chest CT scan. She was treated with pyridostigmine and tacrolimus. Ten weeks after admission, she was weaned off the ventilator and she had no dysphagia anymore. Anti-acetylcholine receptor antibody titers had decreased. In Japan, approximately 17% of patients with myasthenia gravis are in the seventh decade or older. Approximately 10% of the patients of this age group suffer from severe generalized form. Careful ECG monitoring is necessary while treating elderly patients with myasthenic crisis, because they are at potential risk of developing "Takotsubo"-shaped cardiomyopathy.