Brogna Claudia, Cristiano Lara, Verdolotti Tommaso, Norcia Giulia, Ficociello Luana, Ruiz Roberta, Coratti Giorgia, Fanelli Lavinia, Forcina Nicola, Petracca Giorgia, Chieppa Fabrizia, Tartaglione Tommaso, Colosimo Cesare, Pane Marika, Mercuri Eugenio
Pediatric Neurology Unit, Fondazione Policlinico Universitario "A. Gemelli", IRCCS, 00168 Rome, Italy.
Nemo Clinical Centre, Fondazione Policlinico Universitario "A. Gemelli", IRCCS, 00168 Rome, Italy.
Medicina (Kaunas). 2021 Nov 18;57(11):1267. doi: 10.3390/medicina57111267.
: The aim of this study was to evaluate longitudinal changes using both upper limb muscle Magnetic Resonance Imaging (MRI) at shoulder, arm and forearm levels and Performance of upper limb (PUL) in ambulant and non-ambulant Duchenne Muscular Dystrophy (DMD) patients. We also wished to define whether baseline muscle MRI could help to predict functional changes after one year. : Twenty-seven patients had both baseline and 12month muscle MRI and PUL assessments one year later. : Ten were ambulant (age range 5-16 years), and 17 non ambulant (age range 10-30 years). Increased abnormalities equal or more than 1.5 point on muscle MRI at follow up were found on all domains: at shoulder level 12/27 patients (44%), at arm level 4/27 (15%) and at forearm level 6/27 (22%). Lower follow up PUL score were found in 8/27 patients (30%) at shoulder level, in 9/27 patients (33%) at mid-level whereas no functional changes were found at distal level. There was no constant association between baseline MRI scores and follow up PUL scores at arm and forearm levels but at shoulder level patients with moderate impairment on the baseline MRI scores between 16 and 34 had the highest risk of decreased function on PUL over a year. : Our results confirmed that the integrated use of functional scales and imaging can help to monitor functional and MRI changes over time.
本研究的目的是利用肩部、手臂和前臂水平的上肢肌肉磁共振成像（MRI）以及行走和非行走型杜氏肌营养不良（DMD）患者的上肢功能（PUL）来评估纵向变化。我们还希望确定基线肌肉MRI是否有助于预测一年后的功能变化。27名患者在基线时以及一年后进行了12个月的肌肉MRI和PUL评估。其中10名患者可独立行走（年龄范围为5 - 16岁），17名患者不能独立行走（年龄范围为10 - 30岁）。随访时，在所有部位的肌肉MRI上均发现异常增加等于或超过1.5分：在肩部水平，12/27名患者（44%）；在手臂水平，4/27名患者（15%）；在前臂水平，6/27名患者（22%）。在肩部水平，8/27名患者（30%）的随访PUL评分较低；在中间水平，9/27名患者（33%）的随访PUL评分较低；而在远端水平未发现功能变化。在手臂和前臂水平，基线MRI评分与随访PUL评分之间没有恒定的关联，但在肩部水平，基线MRI评分在16至34之间且有中度损伤的患者在一年中PUL功能下降的风险最高。我们的结果证实，功能量表和成像的综合使用有助于监测随时间推移的功能和MRI变化。
