ATTR Amyloidosis Concomitant with Parkinsonism and Cardiac Sympathetic Neuropathy.

Abstract

W ild-type transthyretin amyloidosis (ATTRwt) is an infiltrative disease in which amyloid fibrils derived from transthyretin are deposited in various tissues without mutations in TTR gene. Symptoms mainly manifest in joints, ligaments, and the heart, leading to carpal tunnel syndrome, spinal canal stenosis, cardiac hypertrophy, arrhythmia, and heart failure (1). 123 I-metaiodobenzylguanidine (MIBG) scintigraphy is widely used to evaluate cardiac sympathetic innervation in patients with chronic heart failure. In addition, it is applied to the differential diagnosis of Parkinson’s disease from other neurodegenerative parkinsonism, characterized by reduced accumulation of 123 I-MIBG in the heart (2). Here we report the case of ATTRwt presenting parkinsonism concomitant with reduced accumulation of 123 I-MIBG scintigraphy in the heart.