Cortex; a journal devoted to the study of the nervous system and behavior
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Cortical neuron degenerative process underlying upper motor neuron involvement in amyotrophic lateral sclerosis (ALS) spreads to extra-motor regions as disease progresses. This is associated with cognitive and behavioural worsening in more severe disease stages. However, the clinical variability of ALS patients might reflect different cortical involvement in extra-motor areas. ⋯ Looking at ALS patients irrespective of their cognitive phenotype, motor and extra-motor cortical involvement is consistent with neuropathological studies of disease dissemination. Segregating patients according to their cognitive status, a distinctive trajectory of cortical thinning emerged for ALSimp patients, suggesting a specific course distinct to that of the classic ALS phenotype.