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Updated: Jan 31, 2026
Evaluation of Motor Impairment in C. elegans Models of Amyotrophic Lateral Sclerosis
Published on: September 2, 2021
Value of fluid-attenuated inversion recovery MRI data analyzed by the lesion segmentation toolbox in amyotrophic
Anna M Wirth1,2, Siw Johannesen1, Andrei Khomenko1
1Department of Neurology, University Hospital of Regensburg, Germany.
Background:
MRI fluid-attenuated inversion recovery (FLAIR) studies reported hyperintensity in the corticospinal tract and corpus callosum of patients with amyotrophic lateral sclerosis (ALS).
Purpose:
To evaluate the lesion segmentation toolbox (LST) for the objective quantification of FLAIR lesions in ALS patients.
Study Type:
Retrospective.
Population:
Twenty-eight ALS patients (eight females, mean age: 50 range: 24-73, mean ALSFRS-R sum score: 36) were compared with 31 age-matched healthy controls (12 females, mean age: 45, range: 25-67). ALS patients were treated with riluzole and additional G-CSF (granulocyte-colony stimulating factor) on a named patient basis.
Field Strength/Sequence:
1.5 T, FLAIR, T1 -weighted MRI.
Assessment:
The lesion prediction algorithm (LPA) of the LST enabled the extraction of individual binary lesion maps, total lesion volume (TLV), and number (TLN). Location and overlap of FLAIR lesions across patients were investigated by registration to FLAIR average space and an atlas. ALS-specific functional rating scale revised (ALSFRS-R), disease progression, and survival since diagnosis served as clinical correlates.
Statistical Tests:
Univariate analysis of variance (ANOVA), repeated-measures ANOVA, t-test, Bravais-Pearson correlation, Chi-square test of independence, Kaplan-Meier analysis, Cox-regression analysis.
Results:
Both ALS patients and healthy controls exhibited FLAIR alterations. TLN significantly depended on age (F(1,54) = 24.659, P < 0.001) and sex (F(1,54) = 5.720, P = 0.020). ALS patients showed higher TLN than healthy controls depending on sex (F(1, 54) = 5.076, P = 0.028). FLAIR lesions were small and most pronounced in male ALS patients. FLAIR alterations were predominantly detected in the superior and posterior corona radiata, anterior capsula interna, and posterior thalamic radiation. Patients with pyramidal tract (PT) lesions exhibited significantly inferior survival than patients without PT lesions (P = 0.013). Covariate age exhibited strong prognostic value for survival (P = 0.015).
Data Conclusion:
LST enables the objective quantification of FLAIR alterations and is a potential prognostic biomarker for ALS.
Level Of Evidence:
3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2019;50:552-559.
Insights
The Lesion Segmentation Toolbox (LST) objectively quantifies MRI FLAIR lesions in amyotrophic lateral sclerosis (ALS) patients. These FLAIR lesions, particularly in the pyramidal tract, are associated with poorer survival, indicating LST
Area of Science:
- Neuroimaging
- Biomarkers
- Neurology
Background:
- Amyotrophic lateral sclerosis (ALS) is associated with MRI-detected white matter hyperintensities.
- Fluid-attenuated inversion recovery (FLAIR) MRI shows abnormalities in the corticospinal tract and corpus callosum in ALS patients.
Purpose of the Study:
- To evaluate the efficacy of the Lesion Segmentation Toolbox (LST) for quantifying FLAIR lesions in ALS.
- To assess the relationship between quantified lesions and clinical outcomes in ALS.
Main Methods:
- Retrospective analysis of 28 ALS patients and 31 healthy controls using 1.5 T FLAIR and T1-weighted MRI.
- Lesion Segmentation Toolbox (LST) with lesion prediction algorithm (LPA) for extracting lesion maps, total lesion volume (TLV), and total lesion number (TLN).
- Statistical analysis including ANOVA, t-tests, correlation, and survival analyses (Kaplan-Meier, Cox-regression).
Main Results:
- Both ALS patients and controls showed FLAIR alterations; TLN was dependent on age and sex.
- ALS patients exhibited higher TLN than controls, particularly males.
- FLAIR lesions were most pronounced in the superior/posterior corona radiata, anterior capsula interna, and posterior thalamic radiation.
- Pyramidal tract lesions correlated with significantly inferior survival; age was a strong prognostic factor.
Conclusions:
- The Lesion Segmentation Toolbox (LST) provides objective quantification of FLAIR alterations in ALS.
- FLAIR lesion load and location, particularly in the pyramidal tract, may serve as a prognostic biomarker for ALS survival.
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