Popeye's sign and transthyretin amyloidosis.

Abstract

A 76-year-old man with a history of cervical open-door laminoplasty for ossification of the posterior longitudinal ligament presented to our hospital with a symptom of exertional dyspnoea. A 12-lead electrocardiogram revealed I-degree atrioventricular block (PQ interval 262 ms), left axis deviation, and complete right bundle branch block (Panel A). A transthoracic echocardiogram indicated left ventricular hypertrophy with a maximum wall thickness of 17 mm and a left ventricular ejection fraction of 61% (Panel B1). Strain echocardiogram using speckled tracking showed marked diminution of global longitudinal strain with apical sparing (Panel B2). Physical examination found subconjunctival hemorrhage in right eye, several purpuric lesions in upper extremities, and a large bulge on his left upper arm when he flexed his arm, so-called Popeye’s sign, indicating a hallmark of biceps tendon rupture (see Supplementary material online, Video S1; Panel C; arrow). A monoclonal component was not detected by serum and urine immune-electrophoresis and free light chain assays. 99mTc-labelled bone scintigraphy showed grade 3 myocardial uptake (Panel D). Eventually, endomyocardial biopsy and genetic sequencing analysis of the transthyretin gene confirmed the patient as a wild-type transthyretin amyloidosis (Panel E). Popeye’s sign is uncommon, but pivotal to diagnose transthyretin amyloidosis.