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Fasciculation score: a sensitive biomarker in amyotrophic lateral sclerosis

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Abstract

Objective

The aim of our study was to elucidate the characteristic of fasciculation distributions in amyotrophic lateral sclerosis (ALS) using a fasciculation score (FS) of muscle ultrasound (MUS) and to compare the diagnostic values of three MUS fasciculation parameters in patients.

Methods

Thirty ALS patients, 16 ALS mimics, and 10 healthy subjects were involved. MUS of unilateral 10 muscles in each patient and needle electromyography (EMG) of total 204 muscles were performed to detect fasciculations and spontaneous activity respectively in ALS. Control groups underwent only MUS. Fasciculation was graded semiquantitatively with FS.

Results

Three hundred fifty muscles in ALS and 260 in controls were examined. The fasciculation detection rates, total FS, the number of muscles with fasciculation, and the total number of fasciculations in ALS were all significantly higher than those of controls (P < 0.001). ALS patients exhibited a multifocal continuous pattern of fasciculation in limbs, whereas there were few fasciculations in controls. Compared with other parameters, total FS had the largest area under the curve (AUC) (AUC = 0.899, P < 0.001) in ALS diagnosis. The detection rates of lower motor neuron (LMN) acute lesions by MUS (70.6%) and EMG (72.1%) were nearly the same, and a positive correlation between the FS and spontaneous activity grades (P < 0.001, r = 0.359) was proved.

Conclusions

ALS patients exhibited the multifocal continuous pattern of fasciculation in limbs. FS showed high sensitivity and specificity in differentiating ALS from non-ALS patients, and the optimal cut-off value was determined as 4. The combination of MUS and EMG can provide additional information about specific muscles.

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Funding

Shanxi Science and Technology Department, China (grant ID: 201704D13111584).

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Author notes

  1. Jing Ma and Qi Wen contributed equally to this work.

Authors and Affiliations

  1. Department of Neurology, First Hospital, Shanxi Medical University, No.85, Jiefang South Street, Taiyuan, 030000, People’s Republic of China

    Jing Ma, Qi Wen, Xiaomin Pang, Shan Huang, Jing Zhang, Juan Wang, Xueli Chang, Junhong Guo & Wei Zhang

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  1. Jing Ma
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  2. Qi Wen
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  3. Xiaomin Pang
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  4. Shan Huang
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Correspondence to Wei Zhang.

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The authors declare no competing interests.

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All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Informed consent was preliminarily obtained from all patients and controls before their inclusion in the study.

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Ma, J., Wen, Q., Pang, X. et al. Fasciculation score: a sensitive biomarker in amyotrophic lateral sclerosis. Neurol Sci 42, 4657–4666 (2021). https://doi.org/10.1007/s10072-021-05166-6

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  • DOI: https://doi.org/10.1007/s10072-021-05166-6

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