肌肉超声束颤定量分析有助于肌萎缩侧索硬化的鉴别诊断

doi:10.24920/004282

摘要/Abstract

摘要：

目的研究超声束颤对肌萎缩侧索硬化（amyotrophic lateral sclerosis，ALS）的鉴别诊断价值。

方法前瞻性收集2017年至2020年在北京协和医院就诊的ALS患者，同时招募神经根神经病患者及健康者分别作为病例对照组和健康对照组。采用医学研究会（Medical Research Council，MRC）量表评估肌力。采用肌肉超声评估ALS及病例对照组患者双侧上下肢16组肌群的最大束颤级别、束颤总分及束颤级别。采用受试者工作特征（receiver operating characteristic，ROC）分析评估最大束颤级别、束颤总分及束颤级别诊断ALS的特异度和敏感度。

结果束颤最大级别大于2级的ALS患者和神经根神经病患者的比例分别为84.9%的和9.8%，两者比较具有统计学意义（χ² = 101.3，P < 0.001）。ALS和神经根神经病患者的束颤总分分别为29（15，41）和3（0，8）[中位数（四分位数间距）]。ALS患者中，MRC评分为2和4分患者的肌肉束颤最明显，MRC评分为5分患者的肌肉束颤次之，MRC评分为0和1患者的肌肉束颤最不明显。束颤总分诊断ALS的敏感性和特异性分别为80.6%和93.4%（截断值为14）。对于ALS 患者，MRC评分为4和5的肌肉中，肌肉束颤级别≥3的比例分别为42.3%和24.1%；对于神经根神经病患者，MRC评分为4和5的肌肉中，肌肉束颤级别≥3的比例仅为1.7%和0。

结论肌肉束颤级别联合肌肉MRC评分有助于ALS的鉴别诊断。

关键词: 肌萎缩侧索硬化, 神经根神经病, 肌肉超声, 束颤, 肌力

Abstract:

Objective To determine the diagnostic accuracy of the intensity of fasciculation evaluated by muscle ultrasound in the differential diagnosis of amyotrophic lateral sclerosis (ALS).

Methods We prospectively recruited patients who had ALS and neuropathy-radiculopathy attending Peking Union Medical College Hospital from 2017 to 2020. Healthy adults from a community were recruited as healthy controls. Muscle strength was assessed using the Medical Research Council (MRC) scale. At the first visit to the hospital, patients were assessed for maximal grade of fasciculations, total fasciculation score, and fasciculation grade in 16 muscle groups of bilateral upper and lower limbs using ultrasonography. The sensitivity and specificity of maximal grade of fasciculations, total fasciculation score, and fasciculation grade for the diagnosis of ALS were assessed by receiver operating characteristic analyses.

Results The percentage of limb muscles with a maximal fasciculation grade higher than grade 2 in ALS patients and neuropathy-radiculopathy patients was 84.9% and 9.8%, respectively (χ² = 172.436, P < 0.01). Of the 16 limb muscles detected, the total fasciculation score [median (interquartile range)] was 29 (15, 41) in ALS patients and 3 (0, 8) in neuropathy-radiculopathy patients (Z = 9.642, P < 0.001). Remarkable fasciculations were seen in ALS patients whose muscles with a MRC score ranging from 2 to 4, followed by patients with MRC score 5, and then in those with MRC score 0 and 1. The sensitivity and specificity of total fasciculation score for diagnosis of ALS were 80.6% and 93.4%, respectively (cut-off value 14). In patients with ALS, for muscles with MRC score 4 and 5, the percentage of muscles with fasciculation grades ≥ 3 was 42.3% and 24.1% respectively, while in neuropathy-radiculopathy patients, the percentage for muscles with MRC score 4 and 5 was only 1.7% and 0, respectively.

Conclusion A combined analysis of fasciculation intensity and MRC score of the limb muscles may be helpful for differential diagnosis of ALS.

Key words: amyotrophic lateral sclerosis, neuropathy-radiculopathy, muscle ultrasonography, fasciculation, muscle strength

Jing Fan, Yi Li, Jing-Wen Niu, Nan Hu, Yu-Zhou Guan, Li-Ying Cui, Ming-Sheng Liu. Differentiation Between Amyotrophic Lateral Sclerosis and Mimics Using Quantitative Analysis of Fasciculation with Muscle Ultrasound[J].Chinese Medical Sciences Journal, 2023, 38(4): 265-272.

图/表 5

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Variables	ALS (n = 139)	NR (n = 61)	HC (n = 22)	t/χ²	P value
Age (yrs, mean ± SD)	53.1 ± 12.5	48.7 ± 17.7	53.6 ± 11.7	31.5	< 0.001
Sex, female [n (%)]	64 (46)	17 (27.9)	10 (45.5)	5.99	0.05
Height (cm, mean ± SD)	166.1 ± 0.7	167.7 ± 0.7	169.0 ± 1.3	5.22	0.073
Weight (kg, mean ± SD)	65.3 ± 1.2	73.4 ± 2.5	63.8 ± 2.7	13.7	0.001
BMI (kg/m², mean ± SD)	23.6 ± 0.4	28.6 ± 3.6	22.3 ± 0.8	1.4	0.003
Disease duration (months, mean ± SD)	15.1 ± 1.1	34.9 ± 5.9	19.1 ± 7.9	1.7	0.41
ALSFRSR (score, mean ± SD)	41.1 ± 5.1	-	-
MRC score (% of patients)
0	2.15	1.85	0
1	2.15	1.85	0
2	9.50	4.90	0
3	14.60	16.90	0
4	24.70	24.50	0
5	46.90	50.50	100

Groups	Grade 0	Grade 1	Grade 2	Grade 3	Grade 4	Total
ALS	626	383	502	200	513	2,224
NR	761	132	60	9	14	976
HC	328	19	5	0	0	352
Sum	1,715	534	567	209	527	3,552

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