Difference in cortical activity of lumbar- and bulbar-form of Amyotrophic lateral sclerosis: an fMRI study

Objective: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder of upper and lower motor neurons with a progressive limbic or bulbar muscular weakness and wasting. Survival of patients with bulbar onset is known to be shorter than of patients with lumbar onset. In this study we investigated the cortical activity during movements of tongue, eyes and extremities with fMRI. Methods: We studied 22 patients with ALS, 18 patients with lower motor neuron symptoms including five patients with Kennedy-syndrome and 22 healthy controls, using BOLD-fMRI, while they performed an acoustically paced motor task.…