作者
William D Rooney, Yosef A Berlow, William T Triplett, Sean C Forbes, Rebecca J Willcocks, Dah-Jyuu Wang, Ishu Arpan, Harneet Arora, Claudia Senesac, Donovan J Lott, Gihan Tennekoon, Richard Finkel, Barry S Russman, Erika L Finanger, Saptarshi Chakraborty, Elliott O'Brien, Brendan Moloney, Alison Barnard, H Lee Sweeney, Michael J Daniels, Glenn A Walter, Krista Vandenborne
发表日期
2020/4/14
期刊
Neurology
卷号
94
期号
15
页码范围
e1622-e1633
出版商
Lippincott Williams & Wilkins
简介
Objective
To quantify disease progression in individuals with Duchenne muscular dystrophy (DMD) using magnetic resonance biomarkers of leg muscles.
Methods
MRI and magnetic resonance spectroscopy (MRS) biomarkers were acquired from 104 participants with DMD and 51 healthy controls using a prospective observational study design with patients with DMD followed up yearly for up to 6 years. Fat fractions (FFs) in vastus lateralis and soleus muscles were determined with 1H MRS. MRI quantitative T2 (qT2) values were measured for 3 muscles of the upper leg and 5 muscles of the lower leg. Longitudinal changes in biomarkers were modeled with a cumulative distribution function using a nonlinear mixed-effects approach.
Results
MRS FF and MRI qT2 increased with DMD disease duration, with the progression time constants differing markedly between individuals and across muscles. The average age at …
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WD Rooney, YA Berlow, WT Triplett, SC Forbes… - Neurology, 2020