Paediatrics Publications
Title
Body composition in patients with congenital myotonic dystrophy.
Document Type
Article
Publication Date
8-2019
Journal
Muscle & Nerve
Volume
60
Issue
2
First Page
176
Last Page
179
URL with Digital Object Identifier
https://doi.org/10.1002/mus.26509
Abstract
INTRODUCTION:
Congenital myotonic dystrophy (CDM) is a rare neuromuscular disorder characterized by severe hypotonia and muscle weakness at birth that tends to improve with age. Understanding lean body and bone mass in this population has important research and clinical implications. The main objective of this study was to determine whether older children with CDM had muscle mass similar to healthy controls.
METHODS:
Thirty-five patients with CDM (3-13 years old) were enrolled. We analyzed lean body mass (LBM) and bone mineral content using the mechanostat framework, which allows calculation of z-scores for sex, age, and height.
RESULTS:
All patients had low LBM z-scores (muscle mass); however, higher LBM z-score was positively correlated with age (r = 0.45, P = 0.006), showing that it is closer to normal in older patients.
DISCUSSION:
Unlike other neuromuscular diseases, older children with CDM have a muscle mass closer to age-matched controls, consistent with the clinical profile of increasing strength in childhood. Muscle Nerve 60: 176-179, 2019.
