肌萎缩侧索硬化束颤分布研究和卒中后下运动神经元病变的电生理、超声和病理研究

目的：肌肉束颤作为早期诊断肌萎缩侧索硬化（amyotrophic lateral sclerosis，ALS）的重要提示，一直以来受到广泛关注。超声具有无创性，且检测束颤的敏感性高于针极肌电图，目前已成为观察束颤的重要辅助手段。本研究旨在探讨超声下ALS不同部位束颤的差异及其与临床因素之间的关系，以探索ALS的束颤分布规律。 方法：我们纳入了113名ALS患者，收集其临床资料。在超声下检测患者球部（颏舌肌、咀嚼肌、额肌、胸锁乳突肌、斜方肌），颈段（上肢近端屈肌和伸肌、上肢远端屈肌和伸肌、小指展肌、拇短展肌、第一骨间肌、C5-C7脊旁肌），胸段（T2、T6和T10脊旁肌，T6和T10腹直肌），腰骶段（下肢近端屈肌和伸肌、下肢远端屈肌和伸肌、L1-L5脊旁肌、S1-S5脊旁肌）的束颤。根据束颤频率和涉及部位将其分为5级。 结果：颈段肌群的束颤级别最高（2 [0-3]），其次是腰骶段（1 [0-3]），胸段（0 [0-1]）和球部（0 [0-1]）最低（P<0.05）。腰骶段脊旁肌的束颤级别最高（0 [0-3]），其次是胸段（0 [0-1]），颈段脊旁肌的束颤级别最低（0 [0-0]）（P<0.05）。在颈段肌群中，上肢近端（2 [1-4]）和上肢远端（2 [0-4]）肌群的束颤级别最高，其次是手部肌群（1 [0-2]），颈段脊旁肌最低（P<0.05）；在腰骶段肌群中，下肢近端肌群的束颤级别最高（2 [1-3]），其次是下肢远端（1 [0-2]），腰骶段脊旁肌最低（P<0.05）。在肢体起病的肌群中，起病节段的束颤级别更高。除颈段脊旁肌外，起病节段肢体的肌群由近端至远端的束颤符合由多到少的趋势。束颤最常见于肌力为2-4级、出现轻-中度肌萎缩的肌群。 结论：（1）ALS患者的束颤最常位于颈段，其次是腰骶段，而胸段和球部的束颤相对较少；近端至远端的束颤分布有一定的规律，即肢体近端最多，其次是肢体远端，脊旁肌最少；在ALS患者脊旁肌中，束颤最常位于腰骶段，其次是胸段，而颈段相对较少。（2）肢体起病的ALS患者起病部位的束颤多于其他部位，近端至远端的束颤符合由多到少的分布规律，但是非起病部位无此规律。（3）ALS患者的束颤多少与肌力和萎缩程度存在一定的相关性，当肌力为2-4级、肌萎缩程度为轻-中度时束颤最多Objective: Muscle fasciculations, as an important indicator for the early diagnosis of amyotrophic lateral sclerosis (ALS), have long garnered widespread attention. Ultrasound (US) is non-invasive, and its sensitivity in detecting fasciculations is higher than that of needle electromyography, making it an important auxiliary tool for observing fasciculations. This study aimed to explore the differences in fasciculations at different sites of ALS under ultrasound and their relationship with clinical factors, so as to elucidate the distribution pattern of fasciculations in ALS. Methods: We enrolled 113 ALS patients and collected their clinical data. Fasciculations were detected via ultrasound in the following regions: bulbar region (genioglossus muscle, masticatory muscles, frontalis muscle, sternocleidomastoid muscle, trapezius muscle), cervical segment (proximal upper extremity flexors and extensors, distal upper extremity flexors and extensors, abductor digiti minimi muscle, abductor pollicis brevis muscle, first dorsal interosseous muscle, paraspinal muscles at C5-C7 level), thoracic segment (paraspinal muscles at T2, T6 and T10 levels, rectus abdominis muscles at T6 and T10 levels), and lumbosacral segment (proximal lower extremity flexors and extensors, distal lower extremity flexors and extensors, paraspinal muscles at L1-L5 level, paraspinal muscles at S1-S5 level). Fasciculations were classified into 5 grades based on their frequency and involved regions. Results: The fasciculation grade was highest in cervical region muscles (2 [0-3]), followed by the lumbosacral region (1 [0-3]), while the thoracic (0 [0-1]) and bulbar regions (0 [0-1]) had the lowest grades (P<0.05). The fasciculation grade was highest in lumbosacral paraspinal muscles (0 [0-3]), followed by thoracic paraspinal muscles (0 [0-1]), and the lowest in cervical paraspinal muscles (0 [0-0]) (P<0.05). Among cervical muscles, the proximal (2 [1-4]) and distal upper extremity muscles (2 [0-4]) had the highest fasciculation grades, followed by hand muscles (1 [0-2]), and cervical paraspinal muscles had the lowest grades (P<0.05). Among lumbosacral muscles, proximal lower extremity muscles had the highest fasciculation grade (2 [1-3]), followed by distal lower extremity muscles (1 [0-2]), and lumbosacral paraspinal muscles had the lowest grades (P<0.05). In limb-onset ALS patients, the fasciculation grade was higher in the affected segment muscles. Except for cervical paraspinal muscles, the fasciculation count from proximal to distal in the affected limb muscles showed a decreasing trend. Fasciculations were most commonly found in muscles with a muscle strength grade of 2-4 and mild to moderate muscle atrophy. Conclusions: (1) Fasciculations in ALS patients most frequently occur in the cervical region, followed by the lumbosacral region, while thoracic and bulbar regions have relatively fewer fasciculations. The distribution of fasciculations from proximal to distal limbs follows a certain pattern: proximal limbs have the most fasciculations, followed by distal limbs, and paraspinal muscles have the least. Among paraspinal muscles of ALS patients, fasciculations most frequently occur in the lumbosacral region, followed by the thoracic region, while the cervical region has relatively fewer fasciculations. (2) In limb-onset ALS patients, the affected segment has more fasciculations than other regions, and the fasciculation distribution from proximal to distal limbs shows a decreasing trend, while this pattern is not observed in non-affected segments. (3) The number of fasciculations in ALS patients is correlated with muscle strength and the degree of muscle atrophy; the most fasciculations are found when muscle strength is grade 2-4 and muscle atrophy is mild to moderate.