Motor Neuron Diseases: Amyotrophic Lateral Sclerosis

Motor neuron diseases (MNDs) are motor syndromes (sensory sparing) that begin insidiously, are chronically progressive, can be distal, proximal, or mixed, and can have different combinations of upper and lower motor neuron findings. These are usually sporadic while some forms can be familial. Amyotrophic lateral sclerosis (ALS), perhaps the most common of MNDs, is a progressive disorder of motor neurons in the brain and spinal cord. ALS is fundamentally a clinical diagnosis, supported by neurophysiological testing. There are no cures for ALS, but there are evidence-based guidelines for standards of care.