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Amyotrophic lateral sclerosis with upper motor neuron predominance: diagnostic accuracy of qualitative and quantitative susceptibility metrics in the precentral gyrus

  • Magnetic Resonance
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A Commentary to this article was published on 22 August 2023

Abstract

Objective

The study aims at comparing the diagnostic accuracy of qualitative and quantitative assessment of the susceptibility in the precentral gyrus in detecting amyotrophic lateral sclerosis (ALS) with predominance of upper motor neuron (UMN) impairment.

Methods

We retrospectively collected clinical and 3T MRI data of 47 ALS patients, of whom 12 with UMN predominance (UMN-ALS). We further enrolled 23 healthy controls (HC) and 15 ALS Mimics (ALS-Mim). The Motor Cortex Susceptibility (MCS) score was qualitatively assessed on the susceptibility-weighted images (SWI) and automatic metrics were extracted from the quantitative susceptibility mapping (QSM) in the precentral gyrus. MCS scores and QSM-based metrics were tested for correlation, and ROC analyses.

Results

The correlation of MCS score and susceptibility skewness was significant (Rho = 0.55, p < 0.001). The susceptibility SD showed an AUC of 0.809 with a specificity and positive predictive value of 100% in differentiating ALS and ALS Mim versus HC, significantly higher than MCS (Z = −3.384, p-value = 0.00071). The susceptibility skewness value of −0.017 showed specificity of 92.3% and predictive positive value of 91.7% in differentiating UMN-ALS versus ALS mimics, even if the performance was not significantly better than MCS (Z = 0.81, p = 0.21).

Conclusion

The MCS and susceptibility skewness of the precentral gyrus show high diagnostic accuracy in differentiating UMN-ALS from ALS-mimics subjects. The quantitative assessment might be preferred being an automatic measure unbiased by the reader.

Clinical relevance statement

The clinical diagnostic evaluation of ALS patients might benefit from the qualitative and/or quantitative assessment of the susceptibility in the precentral gyrus as imaging marker of upper motor neuron predominance.

Key Points

• Amyotrophic lateral sclerosis diagnostic work-up lacks biomarkers able to identify upper motor neuron involvement.

• Susceptibility-weighted imaging/quantitative susceptibility mapping–based measures showed good diagnostic accuracy in discriminating amyotrophic lateral sclerosis with predominant upper motor neuron impairment from patients with suspected motor neuron disorder.

• Susceptibility-weighted imaging/quantitative susceptibility mapping–based assessment of the magnetic susceptibility provides a diagnostic marker for amyotrophic lateral sclerosis with upper motor neuron predominance.

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Abbreviations

ALS:

Amyotrophic lateral sclerosis

ALSFRS-R:

ALS Functional Rating Scale-Revised

ALS-mim:

ALS-mimicking diseases

c-ALS:

ALS patients with no clinically defined predominance

HC:

Healthy controls

LMN:

Lower motor neurons

LMN-ALS:

LMN-predominant ALS

MCS:

Motor Cortex Susceptibility

QSM:

Quantitative susceptibility mapping

SMD:

Suspected motor neuron disease

SuscKurt:

Susceptibility kurtosis

SuscMean:

Susceptibility mean

SuscMedian:

Susceptibility median

SuscSD:

Susceptibility standard deviation

SuscSkew:

Susceptibility skewness

SWI:

Susceptibility-weighted images

UMN:

Upper motor neuron

UMN-ALS:

UMN-predominant ALS

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Acknowledgements

Authors would like to thank all the subjects that participated to the study.

Funding

No funding was received for this study.

Author information

Author notes

  1. Francesco Lo Russo and Valeria Elisa Contarino contributed equally to the study.

Authors and Affiliations

  1. Neuroradiology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via Francesco Sforza 35, Milan, Italy

    Francesco Lo Russo , Valeria Elisa Contarino, Giorgio Conte, Silvia Casale, Luca Caschera, Valentina Genovese & Fabio Maria Triulzi

  2. Department of Pathophysiology and Transplantation, Università Degli Studi Di Milano, Milan, Italy

    Giorgio Conte, Vincenzo Silani & Fabio Maria Triulzi

  3. Department of Neurology and Laboratory of Neuroscience, Istituto Auxologico Italiano, IRCCS, Milan, Italy

    Claudia Morelli, Francesca Trogu, Claudia Maria Cinnante & Vincenzo Silani

  4. Neuroradiology Unit, ASST Santi Paolo e Carlo, San Carlo Borromeo Hospital, Milan, Italy

    Sara Sbaraini

  5. Department of Electrical Engineering and Computer Sciences, University of California, Berkeley, CA, USA

    Chunlei Liu

Authors
  1. Francesco Lo Russo
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  2. Valeria Elisa Contarino
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Corresponding author

Correspondence to Giorgio Conte.

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Guarantor

The scientific guarantor of this publication is Claudia Morelli.

Conflict of interest

Fabio Maria Triulzi is a member of the European Radiology Scientific Editorial Board. The remaining authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article.

Statistics and biometry

The statistics were defined by Conte and Lo Russo

Informed consent

Written informed consent was obtained from all subjects (patients) in this study.

Ethical approval

Institutional Review Board approval was obtained.

Study subjects or cohorts overlap

There exists a partial overlap with:

• Contarino VE, Conte G, Morelli C, et al (2020): Development of the QSM method, no SWI assessments were performed

• Conte G, Contarino VE, Casale S, et al (2021): Evaluation of the QSM measures accross the ALS clinical phenotypes, no SWI assessments were performed

• Conte G, Sbaraini S, Morelli C, et al (2021): Development of the SWI method, no QSM assessments were

Methodology

• Retrospective

• observational

• performed at one institution

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Lo Russo , F., Contarino, V.E., Conte, G. et al. Amyotrophic lateral sclerosis with upper motor neuron predominance: diagnostic accuracy of qualitative and quantitative susceptibility metrics in the precentral gyrus. Eur Radiol 33, 7677–7685 (2023). https://doi.org/10.1007/s00330-023-10070-y

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  • DOI: https://doi.org/10.1007/s00330-023-10070-y

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