• Peter Bede, Arun Bokde, Marwa Elamin, Susan Byrne, Russell L McLaughlin, Norah Jordan, Harald Hampel, Laura Gallagher, Catherine Lynch, Andrew J Fagan, Niall Pender, and Orla Hardiman.
• Trinity College Institute of Neuroscience (TCIN), Lloyd Building, Trinity College Dublin, Dublin, Ireland. bedepeter@hotmail.com
• J. Neurol. Neurosurg. Psychiatr.. 2013 Jul 1;84(7):766-73.

BackgroundBody region of onset and functional disability are key components of disease heterogeneity in amyotrophic lateral sclerosis (ALS).ObjectivesTo evaluate patterns of grey matter pathology in the motor cortex and correlate focal structural changes with functional disability.MethodsWe conducted a single-centre neuroimaging study of a cohort of 33 cognitively normal patients with amyotrophic lateral sclerosis (ALS) and 44 healthy controls. A voxel-wise generalised linear model was used to investigate the distribution of disease burden within the motor cortex in relation to clinical disability.ResultsPatients with bulbar onset have bilateral focal atrophy in the bulbar segment of the motor homunculus compared with patients with limb onset who have focal cortical changes in the limb segment of their motor strip. Furthermore, the extent to which different body regions are affected in ALS corresponds to the extent of focal grey matter loss in the primary motor cortex. Cortical ALS pathology also extends beyond the motor cortex affecting frontal, occipital and temporal regions.ConclusionsFocal grey matter atrophy within the motor homunculus corresponds with functional disability in ALS. The findings support the existing concepts of cortical focality and motor phenotype heterogeneity in ALS.

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