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杜氏肌营养不良症男孩MRI-T2的纵向测量:年龄和疾病进展的影响

Longitudinal measurements of MRI-T2 in boys with Duchenne muscular dystrophy: effects of age and disease progression.

作者信息

Willcocks R J, Arpan I A, Forbes S C, Lott D J, Senesac C R, Senesac E, Deol J, Triplett W T, Baligand C, Daniels M J, Sweeney H L, Walter G A, Vandenborne K

机构信息

Department of Physical Therapy, University of Florida, USA.

Department of Physical Therapy, University of Florida, USA.

出版信息

Neuromuscul Disord. 2014 May;24(5):393-401. doi: 10.1016/j.nmd.2013.12.012. Epub 2014 Jan 11.

Abstract

Duchenne muscular dystrophy (DMD) is characterized by an increased muscle damage and progressive replacement of muscle by noncontractile tissue. Both of these pathological changes can lengthen the MRI transverse proton relaxation time (T2). The current study measured longitudinal changes in T2 and its distribution in the lower leg of 16 boys with DMD (5-13years, 15 ambulatory) and 15 healthy controls (5-13years). These muscles were chosen to allow extended longitudinal monitoring, due to their slow progression compared with proximal muscles in DMD. In the soleus muscle of boys with DMD, T2 and the percentage of pixels with an elevated T2 (⩾2SD above control mean T2) increased significantly over 1year and 2years, while the width of the T2 histogram increased over 2years. Changes in soleus T2 variables were significantly greater in 9-13years old compared with 5-8years old boys with DMD. Significant correlations between the change in all soleus T2 variables over 2years and the change in functional measures over 2years were found. MRI measurement of muscle T2 in boys with DMD is sensitive to disease progression and shows promise as a clinical outcome measure.

摘要

杜兴氏肌肉营养不良症(DMD)的特征是肌肉损伤增加以及肌肉被非收缩性组织逐渐替代。这两种病理变化都会延长MRI横向质子弛豫时间(T2)。本研究测量了16名DMD男孩(5 - 13岁,15名可独立行走)和15名健康对照者(5 - 13岁)小腿肌肉T2的纵向变化及其分布情况。选择这些肌肉是因为与DMD近端肌肉相比,它们进展缓慢,便于进行长期纵向监测。在患有DMD的男孩的比目鱼肌中,T2以及T2升高(比对照平均T2高⩾2标准差)的像素百分比在1年和2年期间显著增加,而T2直方图的宽度在2年期间增加。与5 - 8岁的DMD男孩相比,9 - 13岁男孩比目鱼肌T2变量的变化显著更大。发现比目鱼肌所有T2变量在2年期间的变化与2年期间功能指标的变化之间存在显著相关性。对患有DMD的男孩的肌肉T2进行MRI测量对疾病进展敏感,有望作为一种临床结局指标。

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