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Updated: Mar 7, 2026
Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis ALS
Published on: March 17, 2012
Longitudinal evaluation of cerebral and spinal cord damage in Amyotrophic Lateral Sclerosis
Milena de Albuquerque1, Lucas Melo T Branco1, Thiago Junqueira R Rezende1
1Department of Neurology and Neuroimaging Laboratory, School of Medical Sciences, University of Campinas - UNICAMP Rua Tessália Vieira de Camargo, 126, Cidade Universitaria "Zeferino Vaz" Campinas, SP 13083-887, Brazil.
Objective:
To evaluate MRI-based parameters as biomarkers of Amyotrophic Lateral Sclerosis (ALS) progression.
Methods:
Twenty-seven patients and 27 controls performed two clinical and MRI acquisitions 8 months apart. ALSFRS-R scale was used to quantify disease severity at both time points. Multimodal analyses of MRI included cortical thickness measurements (FreeSurfer software), analysis of white matter integrity using diffusion-tensor imaging (tract-based spatial statistics-TBSS) and measurement of cervical spinal cord cross-sectional area (SpineSeg software). All analyses were corrected for multiple comparisons. The standardized response mean (SRM = mean score change / standard deviation of score change) was calculated for all methods herein employed and used for comparison purposes.
Results:
There were 18 men and mean age at first examination was 51.9 years. Mean ALSFRS-R scores at baseline and follow-up were 34.0 and 29.0, respectively. There was no region with progressive cortical thinning, but there was significant brainstem volumetric reduction (p = 0.001). TBSS analyses revealed progressive increase of AD (axial diffusivity) and MD (mean diffusivity) at the corpus callosum (p < 0.05), whereas SpineSeg showed progressive cord area reduction (p = 0.002). Cervical spinal cord cross-sectional area reduction was the only MRI parameter that correlated with ALSFRS-R change (r = 0.309, p = 0.038). SRM for ALSFRS-R was 0.95, for cord area 0.95, for corpus callosum AD 0.62 and MD 0.65, and for brainstem volume 0.002.
Conclusions:
Structural MRI is able to detect short term longitudinal changes in ALS. Cervical spinal cord morphometry is a promising neuroimaging marker to assess ALS course.
Insights
Cervical spinal cord cross-sectional area reduction detected by MRI is a promising biomarker for tracking Amyotrophic Lateral Sclerosis (ALS) progression over short intervals. This neuroimaging marker correlates with disease severity changes, offering a new way to assess ALS.
Area of Science:
- Neuroimaging
- Biomarkers
- Amyotrophic Lateral Sclerosis (ALS)
Background:
- Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease.
- Identifying reliable biomarkers for ALS progression is crucial for clinical trials and patient management.
Purpose of the Study:
- To evaluate magnetic resonance imaging (MRI)-based parameters as biomarkers for Amyotrophic Lateral Sclerosis (ALS) progression.
- To assess the ability of structural MRI to detect short-term longitudinal changes in ALS patients.
Main Methods:
- Twenty-seven ALS patients and 27 controls underwent two MRI scans 8 months apart.
- MRI techniques included cortical thickness, white matter integrity (diffusion-tensor imaging), and cervical spinal cord cross-sectional area.
- Disease severity was quantified using the ALSFRS-R scale, and standardized response means (SRMs) were calculated for comparison.
Main Results:
- Significant brainstem volumetric reduction was observed in ALS patients.
- Progressive increases in axial diffusivity (AD) and mean diffusivity (MD) were noted in the corpus callosum.
- Cervical spinal cord cross-sectional area reduction was the only MRI parameter that significantly correlated with changes in ALSFRS-R scores.
Conclusions:
- Structural MRI can detect short-term longitudinal changes in ALS.
- Cervical spinal cord morphometry shows promise as a neuroimaging marker for assessing ALS disease course.
