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肌肉MRI在患有耳肌肉发育不良的患者:一个纵向研究

Rosemarie H M J M Kroon¹, Johanna G Kalf¹, Bert J M de Swart¹

¹From the Departments of Rehabilitation (R.H.M.J.M.K., J.G.K., B.J.M.d.S.) and Neurology (B.G.M.v.E., C.G.C.H.), Radboud University Medical Center, Donders Institute for Brain, Cognition and Behaviour, Nijmegen; Department of Radiology (L.H.), University Medical Centre Utrecht; Department of Imaging (J.W.J.d.R.), Radboud University Medical Center, Nijmegen; and Department of Neurology (C.G.C.H.), Medical University of Innsbruck, Austria.

|January 2, 2024

概括

定量肌肉MRI揭示了眼肌肉发育不良 (OPMD) 患者显著的脂肪透,特别是在舌头, adductor magnus和soleus肌肉中. 这种成像技术在20个月内显示出进展,支持其作为OPMD生物标志物的使用.

科学领域:

神经学神经学
肌肉骨系统疾病肌肉骨系统疾病
医疗成像医学成像

背景情况:

眼肌肉发育不良 (OPMD) 是一种罕见的,进展性神经肌肉疾病.
肌肉MRI是评估神经肌肉疾病中的肌肉变化的一个关键技术.

研究的目的:

用定量核磁共振 (MRI) 来表征OPMD患者口腔面部和腿部肌肉中的脂肪透模式.
评估MRI作为OPMD进展的成像生物标志物的潜力.

主要方法:

在基线和20个月后,对43名OPMD患者 (≥18岁) 进行了定量MRI.
在口腔面部和腿部肌肉中评估了脂肪透,并与临床措施进行了比较.

主要成果:

舌头, adductor magnus和soleus肌肉显示最严重的脂肪透 (平均脂肪分数为37.0%,31.9%,27.9%).
在20个月的时间里,26个肌肉中有17个肌肉的脂肪分数显著增加,其中唯一肌肉的脂肪分数增长最大 (3.8%).
在MRI脂肪分数和临床评估之间,以及疾病持续时间和脂肪透之间观察到显著的相关性.

结论:

定量肌肉MRI有效地识别了OPMD中显著的脂肪透,特别是在特定的肌肉中.
随着时间的推移,观察到脂肪透的增加支持MRI的响应能力作为OPMD进展的生物标志物.
这种技术对于监测疾病恶化和在未来OPMD临床试验中使用有价值.

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