Quantitative vs qualitative muscle MRI: Imaging biomarker in patients with Oculopharyngeal Muscular Dystrophy (OPMD)

被引:6
作者
Melkus, Gerd [1 ,2 ,3 ,8 ]
Sampaio, Marcos L. [1 ,2 ,4 ,8 ]
Smith, Ian C. [2 ]
Rakhra, Kawan S. [1 ,2 ,4 ]
Bourque, Pierre R. [2 ,4 ,5 ]
Breiner, Ari [2 ,4 ,5 ]
Zwicker, Jocelyn [4 ,5 ]
Lochmueller, Hanns [2 ,4 ,5 ,6 ]
Brais, Bernard [7 ]
Warman-Chardon, Jodi [2 ,4 ,5 ,6 ,8 ]
机构
[1] Univ Ottawa, Dept Radiol Radiat Oncol & Med Phys, Ottawa, ON, Canada
[2] Ottawa Hosp Res Inst, Ottawa, ON, Canada
[3] Carleton Univ, Dept Phys, Ottawa, ON, Canada
[4] Univ Ottawa, Fac Med, Er Poulin Ctr Neuromuscular Dis, Ottawa, ON, Canada
[5] Univ Ottawa, Ottawa Hosp, Dept Med Neurol, Ottawa, ON, Canada
[6] Childrens Hosp Eastern Ontario, Genet, Ottawa, ON, Canada
[7] McGill Univ, Montreal Neurol Inst & Hosp, Genet, Montreal, PQ, Canada
[8] ES08-1053 Carling Ave, Ottawa, ON K1Y 4E9, Canada
基金
加拿大健康研究院; 加拿大创新基金会;
关键词
Intramuscular fat; Semi-quantitative; Magnetic resonance imaging; Neuromuscular disorders; Disease progression;
D O I
10.1016/j.nmd.2022.09.010
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Oculopharyngeal muscular dystrophy (OPMD) is a genetic muscle disease causing ptosis, severe swallowing difficulties and progressive limb weakness, although atypical presentations may be difficult to diagnose. Sensitive biomarkers of disease progression in OPMD are needed to enable more effective clinical trials. This study was designed to test the feasibility of using MRI to aid OPMD diagnosis and monitor OPMD progression. Twenty-five subjects with Dixon whole-body muscle MRI were enrolled: 10 patients with genetically confirmed OPMD, 10 patients with non-OPMD muscular dystrophies, and 5 controls. Using the MRI Dixon technique, muscle fat replacement was evaluated in the tongue, serratus anterior, lumbar paraspinal, adductor magnus, and soleus muscles using quantitative and semi-quantitative rating methods. Changes were compared with muscle strength testing, dysphagia severity, use of gait aids, and presence of dysarthria. Quantitative MRI scores of muscle fat replacement in the tongue could differentiate OPMD from other muscular dystrophies and from controls. Moreover, fat fraction in the tongue correlated with clinical severity of dysphagia. This study provides preliminary support for the use of Dixon-based quantitative MRI images as outcome measures for monitoring disease progression in clinical trials and provides rationale for future prospective studies aimed at methodological refinement and covariate identification.(c) 2022 Published by Elsevier B.V.
引用
收藏
页码:24 / 31
页数:8
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