Kim Sunyoung, Lim Yong-Hyun, Kang Kyunghun, Park Donghwi, Lee Ho-Won, Park Jin-Sung
Department of Neurology, University of Ulsan College of Medicine, Ulsan University Hospital, Ulsan, South Korea.
Center of Self-Organizing Software-Platform, Kyungpook National University, Daegu, South Korea.
Front Neurol. 2020 May 19;11:392. doi: 10.3389/fneur.2020.00392. eCollection 2020.
Recent studies on Myotonic dystrophy type 1 (DM1) have shown profound impairments in gait, leading to falls. We analyzed functional ambulation profile (FAP) score that reflects the temporal and spatial gait characteristics and investigated correlations with the lower limb muscle magnetic resonance imaging (MRI) and 6 min walk test (6MWT). Twenty patients with DM1 and 20 controls participated in this study. The 6MWT and gait analysis including FAP scores via GAITRite were performed in all patients and controls. DM1 patients displayed slower gait, shorter stride length, shorter stance length, and lower FAP score. Among lower extremity muscles, the gastrocnemius, soleus and tibialis anterior showed the most severe fat infiltration and these crural muscles significantly correlated with FAP and 6MWT. Among crural muscles, tibialis anterior was the most important muscle affecting gait speed, whereas the gastrocnemius contributed substantially to gait instability. FAP score correlated with the muscle imaging and 6MWT in DM1. Therefore, FAP score maybe used as an non-invasive marker that reflects deterioration of gait and a possible surrogate biomarker in DM1.
近期关于1型强直性肌营养不良（DM1）的研究表明，患者步态存在严重障碍，易导致跌倒。我们分析了反映步态时间和空间特征的功能性步行概况（FAP）评分，并研究了其与下肢肌肉磁共振成像（MRI）及6分钟步行试验（6MWT）的相关性。20例DM1患者和20名对照者参与了本研究。所有患者和对照者均进行了6MWT及通过GAITRite进行的包括FAP评分的步态分析。DM1患者步态较慢，步幅较短，站立时间较短，FAP评分较低。在下肢肌肉中，腓肠肌、比目鱼肌和胫骨前肌脂肪浸润最为严重，这些小腿肌肉与FAP和6MWT显著相关。在小腿肌肉中，胫骨前肌是影响步态速度的最重要肌肉，而腓肠肌对步态不稳起重要作用。FAP评分与DM1患者的肌肉成像及6MWT相关。因此，FAP评分可作为反映DM1患者步态恶化的非侵入性标志物及可能的替代生物标志物。
