Abstract
Rationale and ObjectivesT2-weighted magnetic resonance imaging (MRI) hyperintensity assessed visually in the corticospinal tract (CST) lacks sensitivity for a diagnosis of amyotrophic lateral sclerosis (ALS). We sought to explore a quantitative approach to fluid-attenuated inversion recovery (FLAIR) MRI intensity across a range of ALS phenotypes.Materials and MethodsThirty-three classical ALS patients, 10 with a flail arm presentation, and six with primary lateral sclerosis underwent MRI at 3 Tesla. Comparisons of quantitative FLAIR intensity in the CST and corpus callosum were made between 21 healthy controls and within patient phenotypic subgroups, some of whom were studied longitudinally.ResultsMean FLAIR intensity was greater in patient groups. The cerebral peduncle intensity provided the strongest subgroup classification. FLAIR intensity increased longitudinally. The rate of change of FLAIR within CST correlated with rate of decline in executive function and ALS functional rating score.ConclusionsFLAIR MRI encodes quantifiable information of potential diagnostic, stratification, and monitoring value.
Highlights
The neurodegenerative disorder amyotrophic lateral sclerosis (ALS) remains predominantly a clinical diagnosis with significant heterogeneity in rate of disability accumulation
This novel quantitative analysis demonstrated that fluidattenuated inversion recovery (FLAIR) intensity is increased in the corticospinal tract (CST) and the corpus callosum (CC) in ALS patients compared to healthy controls
The small subgroup of primary lateral sclerosis (PLS) patients demonstrated some of the highest FLAIR intensities, but there was no simple relationship with burden of upper motor neuron (UMN) signs
Summary
The neurodegenerative disorder amyotrophic lateral sclerosis (ALS) remains predominantly a clinical diagnosis with significant heterogeneity in rate of disability accumulation. In a very small proportion of cases, termed primary lateral sclerosis (PLS), there are only UMN signs clinically and consistently slower overall progression [8]. The “flail arm” variant of ALS is typically slower in rate of progression, but predominantly LMN clinically with the main burden of disability characteristically confined to the upper limbs and symmetrical for a period of years [9]. In this and all other subtypes of ALS, investigations are aimed at the exclusion of mimic disorders [10], typically involving MRI to consider structural lesion mimics [11]. An objective marker of UMN involvement that could be derived from a routine MRI sequence might have value diagnostically in stratification and in assessing the effects of candidate therapeutics
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