Where Neurosurgery Meets Heart Failure: A Case Report of a Patient with Amyloid Transthyretin Wild Type in the Ligamentum Flavum and Cardiac Tissue with Bilateral Carpal Tunnel Syndrome

• Published in: World neurosurgery Vol. 131; pp. 104 - 107
• Main Authors: Dowd, Richard S, Nail, T Jayde, Arkun, Knarik, Kryzanski, James, Soto, Oscar, Fogaren, Teresa, Harrington, Kelly, Patel, Ayan, Comenzo, Raymond, Riesenburger, Ron I
• Format: Journal Article
• Language: English
• Published: United States 01-11-2019
• Subjects: Aged; Amyloid Neuropathies, Familial - complications; Amyloid Neuropathies, Familial - pathology; Cardiomyopathies - etiology; Cardiomyopathies - surgery; Carpal Tunnel Syndrome - etiology; Carpal Tunnel Syndrome - surgery; Heart Failure - etiology; Heart Failure - surgery; Heart Transplantation; Humans; Ligamentum Flavum - diagnostic imaging; Ligamentum Flavum - pathology; Ligamentum Flavum - surgery; Lumbar Vertebrae; Male; Spinal Stenosis - diagnostic imaging; Spinal Stenosis - etiology; Spinal Stenosis - surgery; Heart failure; Carpal tunnel syndrome; Lumbar stenosis; Amyloidosis; Cardiac amyloidosis; Ligamentum flavum
• ISSN: 1878-8750; 1878-8769
• DOI: 10.1016/j.wneu.2019.07.172

Transthyretin 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.
Here 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.
Knowing 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.