| Summary: | <strong>Rationale and Objectives</strong> T2-weighted 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 FLAIR MRI intensity across a range of ALS phenotypes. <strong>Materials & Methods</strong> Thirty three classical ALS patients, 10 with a flail arm presentation, and 6 with primary lateral sclerosis underwent MRI at 3 Tesla. Comparisons of quantitative FLAIR intensity in the corticospinal tract (CST) and corpus callosum were made between 21 healthy controls and within patient phenotypic sub-groups, some of who were studied longitudinally. <strong>Results</strong> Mean FLAIR intensity was greater in patient groups. The cerebral peduncle intensity provided the strongest sub-group 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. <strong>Conclusion</strong> FLAIR MRI encodes quantifiable information of potential diagnostic, stratification and monitoring value.
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