Nara Takeru, Shibuya Kazumoto, Ikeda Shinobu, Kuroiwa Ryota, Otani Ryo, Ogushi Moeko, Suichi Tomoki, Shiko Yuki, Takahashi Kohei, Misawa Sonoko, Murata Astushi, Kuwabara Satoshi
Department of Rehabilitation Medicine, Chiba University Hospital, Chiba, Japan.
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
BMJ Neurol Open. 2025 Apr 24;7(1):e001065. doi: 10.1136/bmjno-2025-001065. eCollection 2025.
The usefulness of muscle ultrasonography for detection of fasciculations has been increasingly recognised, particularly in amyotrophic lateral sclerosis (ALS). This study aimed to elucidate distributions and characteristics of fasciculations in spinal and bulbar muscular atrophy (SBMA) and to compare the results of those in ALS.
In 24 SBMA and 16 ALS patients, muscle ultrasonography was systematically performed in the tongue, upper limb muscles (biceps brachii, triceps brachii, first dorsal interosseous (FDI), abductor pollicis brevis and abductor digiti minimi), trunk muscles (Th10 paraspinals and rectus abdominis) and lower limb muscles (vastus lateralis, biceps femoris, tibialis anterior and gastrocnemius). We assessed the presence of fasciculations and the fasciculation intensity (scored from 0 to 3) for each muscle.
All SBMA and ALS patients showed fasciculations at least in two muscles. In SBMA patients, fasciculations were most frequently found in the tongue (100%), FDI (93%) and tibialis anterior (80%), whereas less frequently present in the proximal limb and trunk muscles, irrespective of age, disease duration and CAG repeat numbers. By contrast, in ALS patients, fasciculations were more diffusely distributed including the proximal limb and trunk muscles. When fasciculations were present, the intensity was higher in ALS patients, except for the tongue.
Whereas both diseases exhibit extensive fasciculations, the distribution and intensity are different. SBMA is characterised by prominent involvement in the tongue and distal limb muscles, suggesting different pathophysiology of motor neuronal death in SBMA and ALS.
肌肉超声检查在检测肌束震颤方面的作用日益受到认可，尤其是在肌萎缩侧索硬化症（ALS）中。本研究旨在阐明脊髓性延髓肌萎缩症（SBMA）中肌束震颤的分布及特征，并与ALS的结果进行比较。
对24例SBMA患者和16例ALS患者，系统地对舌肌、上肢肌肉（肱二头肌、肱三头肌、第一骨间背侧肌、拇短展肌和小指展肌）、躯干肌肉（第10胸椎旁肌和腹直肌）及下肢肌肉（股外侧肌、股二头肌、胫骨前肌和腓肠肌）进行肌肉超声检查。我们评估了每块肌肉中肌束震颤的存在情况及肌束震颤强度（评分从0到3）。
所有SBMA和ALS患者至少在两块肌肉中出现肌束震颤。在SBMA患者中，肌束震颤最常见于舌肌（100%）、第一骨间背侧肌（93%）和胫骨前肌（80%），而在近端肢体和躯干肌肉中较少出现，与年龄、病程和CAG重复次数无关。相比之下，在ALS患者中，肌束震颤分布更广泛，包括近端肢体和躯干肌肉。当出现肌束震颤时，除舌肌外，ALS患者的震颤强度更高。
尽管两种疾病都表现出广泛的肌束震颤，但分布和强度有所不同。SBMA的特征是舌肌和远端肢体肌肉受累明显，提示SBMA和ALS运动神经元死亡的病理生理学不同。
