Abstract
Amyotrophic lateral sclerosis (ALS) is characterized by progressive loss of upper and lower motor neurons. Advanced MRI techniques such as diffusion tensor imaging have shown great potential in capturing a common white matter pathology. However the sensitivity is variable and diffusion tensor imaging is not yet applicable to the routine clinical environment. Voxel-based morphometry (VBM) has revealed grey matter changes in ALS, but the bias-reducing algorithms inherent to traditional VBM are not optimized for the assessment of the white matter changes. We have developed a novel approach to white matter analysis, namely voxel-based intensitometry (VBI). High resolution T1-weighted MRI was acquired at 1.5 Tesla in 30 ALS patients and 37 age-matched healthy controls. VBI analysis at the group level revealed widespread white matter intensity increases in the corticospinal tracts, corpus callosum, sub-central, frontal and occipital white matter tracts and cerebellum. VBI results correlated with disease severity (ALSFRS-R) and patterns of cerebral involvement differed between bulbar- and limb-onset. VBI would be easily translatable to the routine clinical environment, and once optimized for individual analysis offers significant biomarker potential in ALS.
Highlights
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive loss of upper motor neurons (UMN) and lower motor neurons (LMN)
The mean extent of significant intensity increase ranges from 4.1% in the left subcentral region to 8.7% in the posterior limb of the internal capsule (PLIC)
This study employed a novel and sensitive method for detecting white matter pathology in ALS, based on T1 images that could be acquired from a routine clinical scanner, in theory as part of the patient’s diagnostic work-up
Summary
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive loss of upper motor neurons (UMN) and lower motor neurons (LMN). It is regarded as a multi-system disease, showing consistent extra-motor involvement and has genetic, clinical and pathological overlap with frontotemporal dementia (FTD) [1,2]. Routine clinical MRI sequences may reveal corticospinal tract (CST) hyperintensity, but this is not sensitive or specific for ALS [5]. Diffusion tensor imaging (DTI) has defined a core white matter pathology, involving the CST and corpus callosum in ALS [6,7], it is not yet a routine clinical sequence and will be challenging to harmonize across international centres
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