Myotonic dystrophy (DM1) and dysphagia: the need for dysphagia management guidelines and an assessment tool

Abstract

Myotonic dystrophy (DM1) is the most prevalent muscular dystrophy occurring in adulthood. DM1 is a multi-systemic disorder resulting in early-onset cataracts, cardiac rhythm problems, muscle weakness, ptosis, and cognitive and psychiatric manifestations. Dysphagia is one of the most problematic symptoms of DM1 because it may cause weight loss, aspiration pneumonias or sudden death. The purpose of this review is to describe the characteristics of DM1 that make dysphagia management problematic, and to address the need for disease-specific guidelines and a clinical tool to aid in diagnosing and managing dysphagia in this population.