From the Department of Neurology, Rutgers University - New Jersey Medical School, Newark, New Jersey (JK, HFA, MZ, JRB, AK, EF, NS); St. Joseph's Children's Hospital, Paterson, New Jersey (JK); and Department of Neurology - New York University School of Medicine, New York, New York (HWS).
Am J Phys Med Rehabil. 2020 Oct;99(10):895-901. doi: 10.1097/PHM.0000000000001438.
The aim of the study was to investigate differences between flail limb syndrome and amyotrophic lateral sclerosis.
A retrospective chart review identified 16 cases of amyotrophic lateral sclerosis and 16 of flail limb syndrome. Revised Amyotrophic Lateral Sclerosis Functional Rating Scale, compound muscle action potential amsplitudes, and rate of loss of vital capacity were compared.
Comparing amyotrophic lateral sclerosis and flail limb syndrome patients, rate of loss of vital capacity was 5.26% ± 0.33% versus 0.54% ± 0.06%, respectively (P < 0.05). No patient in the flail limb syndrome group had a rate of loss of vital capacity more than 0.65% per month. No patient in the amyotrophic lateral sclerosis group had a rate of loss of vital capacity less than 4.6% per month. The average ulnar nerve compound muscle action potential amplitudes were significantly lower in flail limb syndrome (P < 0.05). No significant difference was observed in the rate of Revised Amyotrophic Lateral Sclerosis Functional Rating Scale decline or average peroneal, tibial, and median nerve compound muscle action potential amplitudes.
In flail limb syndrome, an average monthly decrease in vital capacity exceeding 0.65% may suggest a spread of motor neuron loss to higher cervical anterior horn areas and raise the possibility of progression to amyotrophic lateral sclerosis. Larger prospective studies are needed to investigate the rate of VC decline in flail limb syndrome and limb-onset amyotrophic lateral sclerosis and to establish whether a cutoff score combining rate of loss of vital capacity and compound muscle action potential amplitude mainly of the ulnar nerve might predict progression of flail limb syndrome to amyotrophic lateral sclerosis, the knowledge of which can facilitate appropriate patient counseling.
本研究旨在探讨连枷臂综合征与肌萎缩侧索硬化症的差异。
回顾性图表分析共纳入 16 例肌萎缩侧索硬化症和 16 例连枷臂综合征患者。比较修订后的肌萎缩侧索硬化症功能评定量表、复合肌肉动作电位幅度和肺活量损失率。
与肌萎缩侧索硬化症患者相比，连枷臂综合征患者的肺活量损失率分别为 5.26%±0.33%和 0.54%±0.06%（P<0.05）。连枷臂综合征组无患者的肺活量损失率超过 0.65%/月。肌萎缩侧索硬化症组无患者的肺活量损失率低于 4.6%/月。连枷臂综合征组的尺神经复合肌肉动作电位幅度明显较低（P<0.05）。修订后的肌萎缩侧索硬化症功能评定量表下降率或平均腓总神经、胫神经和正中神经复合肌肉动作电位幅度无显著差异。
在连枷臂综合征中，平均每月肺活量损失超过 0.65%可能提示运动神经元损失向更高的颈前角区域扩散，并增加进展为肌萎缩侧索硬化症的可能性。需要更大的前瞻性研究来探讨连枷臂综合征和肢体起病型肌萎缩侧索硬化症的肺活量损失率，并确定是否可以结合肺活量损失率和主要为尺神经的复合肌肉动作电位幅度建立一个截值评分来预测连枷臂综合征向肌萎缩侧索硬化症的进展，了解这些可以促进对患者进行适当的咨询。
