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- Richard S Dowd, T Jayde Nail, Knarik Arkun, James Kryzanski, Oscar Soto, Teresa Fogaren, Kelly Harrington, Ayan Patel, Raymond Comenzo, and Ron I Riesenburger.
- Department of Neurological Surgery, Tufts Medical Center at Boston, Boston, Massachusetts, USA.
- World Neurosurg. 2019 Nov 1; 131: 104-107.
BackgroundTransthyretin wild-type (ATTRwt) amyloidosis is a systemic process resulting in deposition of misfolded transthyretin protein in several different tissues throughout the body. It is known to be a cause of progressive, life-threatening cardiomyopathy and lumbar spinal stenosis and carpal tunnel syndrome.Case DescriptionHere we present the case of a 71-year-old man who has clinical manifestations of all 3 entities over several years, providing a picture of the natural history of ATTRwt amyloidosis. This patient eventually underwent a heart transplant because of progressive cardiac amyloidosis (CA) resulting in end-stage heart failure. However, symptoms in carpal tunnel and lumbar spine manifested years before the symptoms of heart failure. ATTRwt amyloidosis may present as a clinical triad of lumbar stenosis, carpal tunnel syndrome, and heart failure. Recently developed medications have shown efficacy in slowing the progress of systemic and cardiac amyloidosis.ConclusionsKnowing that extracardiac symptoms may occur first, we propose that sending ligamentum flavum and flexor tenosynovium for pathologic evaluation may be a unique opportunity to screen and treat a population of patients at risk for developing CA and heart failure.Copyright © 2019 Elsevier Inc. All rights reserved.
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